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Theutlulive dinributo. in the Americal and Canada ;1 Thieme New York 333 Seventb Avtnue New York. NY 1000l United States or America (BOO) 782-30188
The "clUllV' d,.tribul.or QIIt.11ida tha Americas 18 Thieme inl<emlOtionai Rudigentraase I~ Stuttgart. Germany +49 (0) 711-8931-126 Wbrary orCongress C"t.logins-;n-PubliClltion Data fa ayaiiable from the publi.h@r HANDBOOK OF NEUROSURGERY MatI< S. Greeobere: ISBN 1-58890-.57_1 (ThIeme Ne ... Yorl.) ISBN 3-13-U0886-X (Geare: Thiem. VerlaS Stutts.rLJ Capyrillht C 2006 Marl< S. Greenbe'll. All nghl.l
re~e",e-d.
Third ed.itiun, 199eci1Y the Ilrength ofth. dlta. For th_. the natu .. of the d:lu ",ill 1M gi-..en. A CKNOWLEDGMENTS
I would like 10 ockno .... ledge.U the 1OIlt'CU .. Hod ror tile malQrialln thit book.. 'J'hia lnelud "~cy:sl.~ ...t ..... ~ ..
A cerebrnJ v8Godi istor , ;nCfIIO"ei CBF and Jep. Decrl!&llet> CMR01 which tends to tluBe II competlli8 toty Wlsoetlnst riction.
GENERAL CARE
1. 1. Neuroaneslhetia
~
sevoftu rane (Ultaoe®l
\
I
DRIIGIUFO
\
,
Mildly incre.uu CBP nnd [CP, find reduoe5CMRD,. MUd negat;ve inotrope. Cll.rdiac w;t.h isoflura.n e or deIlflun.ne .
putpll l not fill ....·1'11 ,mllou.inP.Ol . fill
INTRAVENOUS AGEtffS
BARBITURATES IN AtjESTHESIA
Produce flignifiClln~ redu~tion il\ CMROa and scavenge free radical l llJUongl.>ther e ffed.• (mo pC.8' son Produce dose-depr:nd<mt EEG BUpp'Usion which U n be u.ken all the way to illOC!le.:tric. MiuimaUy affect EP, MOlit a re anticonvulsant, bul methohellita l (B . .. vitol®) ~an IOWf' the ""ZllTIl th .... shold (u( pase ,36), MyocaniialluPPreniofl and p"ripher/l. l vllfiOdillltation from harbi turatea may cauu hypoten sion and compro mio;e CPP , e$pe.:ially in hypovolem ic plltienta .
NARconcs IN ANESTHESIA
increa..e CSF nbso'l'tion ""d minimoU)' reduce ce rebral metabolism . TIley Blow r.he EEG but wilillCll. produ~ ao Iaoelect rlc tracing. II All nlln:otks «IU6E' dOOle-dependent respiratory depression which tan re/iu lt in hypercarbra and concomitan t increased IC? in non -ventilated pali~nt.ll . MOl"p h.lne: d~lnot sign irlcantly CI'(IIIS the BSB. • Oisadvantagt'll in neuro pali\mI8' I. ca uRS hi stam ine release which A . "'",y pro Z4 hre, at II. .aloe ~ 10 ~mtn, 01' in renal failure: DIC ifth.iOl'Ylinat.e La".I, ,. 10 nlll"l>1, tuhyqrdia, Ulldu'PhJtluil, hypo\.en· .ion which ~an extend II" Ml "coronary l \.eal", "'""id in p~ancy. & rv drip O.:u.·8 l.I&fl /
,..---/
labet9.lol
\
/
OFIUGorfll
\
(Nurmooyne®, Trandllte®)
8101'1(11 III seleelivi. B norHlelective (potency < propranolol). ICP nduc:u Or DO chance'. Pol.. rite: deeresses Or no chang • . Cardiac output does notchao~. Does not nM~erbDte 0;:0.0011")' ilO;.lu!mia. May be uM(! in eonLroUed CHP. but not in oYen CHF. C!>nl ra.indl~ted;n ,.. tbml. Renal failore: urne do... Sm~ ~rn:cra: raligue. din.iIl Inlravenou. (IV) Ol"ll •
1.2. Critielll care
GENERAL CARE
DIC Bronchospllsm 1.,. likely th"n othllr beta bloc:klll"ll . Avoid in CHF. Rx 500 llgi'kg loading dOH over I min. folio,", .... itb. mill infUllion atamng .... ilh 60 ~glttnn. Repeal loading dPS'! and increment infullion rllte by 60 ).Ig/kglmin 115 mins. Rarely", 100 1Ig/k&lmin requJr.. d. l)o;g~s ",:200 IIsfkg1min add li llll•.
fenoldopam (Corlopam®)
\ I
\
VII"oollftlOr, Onse: "rllction < 5 minutes. duroLion 30 mil\$. Rx tv infullion (no bolus dOMJ1: IIUln .... ith 0.1·0.8 mcglkpmin. tilAla by 0.1 mcglkefm.in q 15 min up to II maximum of 1.6 mrg/'k&lmin .
.---I
prop ranolol (Inderal®)
\
I
00I.0C31N1'O
\
\
Main use tv is tQ Q)un terflcl LIIchycardia with yasodilatoTII (Ullually d~en'l lower BP aIIICK pat;etll$. See,"r.loprilr.1 a bove ror IV use. Rx Initial dose 2 .5·5 mg in onl! doee: maintenance 5 ·40 mil in lor 2 desr.s.
1.2.2.
Hypotension (shock)
Classification: L hypOVolemic: fir~1 .ign usually tachycardio. :> 2IJ..40% 0(111000 vol luna 10&11 mUllt OCCUr berore perfUlio n orvital orgon5 is impairl!d . Includu: A. hemorrhage (exte rnol or internal ) B. bowel Dbstructic.n (w; th third .pacing) 2. septic: lAostonen due to gram negative sepsia ;I. COrdJogeOlC! ,ncJlldes ,'1l, Clrd'omyopllthy, dytirhythm las ('ndndlr'lg .... fib) 4. neurtlgunic: e.g. pual:; s" due to spinal cord inju.ry. Blood pools in venolla capacItance vessels 5. miscellaneoll. A. anaphylBll.ia B. Insulin reaction C ARD IOV ASC ULAR AGENTS FOR SHOCK
Pla.rna expanders. Indudo;s: l . ",rysl.alloids: normal saline hos less tendency to promote cerebral edama than others (&ee tv fluids , page 657 unducontrol of elevated !CP) 2. a:>l1njds; e.g. het.astarch lHespan~) . • CA1J'l'ION: repeated administl"lltlon(lYt!Ta pariQd orda)'. mtlY prolong PTIP'rT and dotting UrofS and may ine:.reoee \.be ri~k of rebleeding in oneurysmal SAH" ($« pG//! 7871 3. blood pradu~t.I; upensive . Risk nrtra.ns m;ssible (liseBllU \lr tnIlLBFu~iw:> reaction
PReSSORS phenylep hrin e
(Neo--Syneph ri ne@)
\ !
\
Pure alpha sym pathomimetic. Useful il"l hypotension ilSl.o ci8ted with tachycardia (a triol tachyarrhythmiaa). EIe"Oh!8 BP b)' we .... uing SVR via VASOCOnStriction, cause, rt'1I"" in H)% mllY ... acerbate myocardial ischemia. more cornmon at dC~1 > 20 "glkg/min. Optima! ~ requires hemodynantic monitoring, PIlssibl" pLal.elet f'~nctiOfl inhibition. Rx usua l range 2.5 - 10 I'gr\g/min ; rilrelydOfie5 up to 40 ",sed (t.o prepere: put SO mg in 250 IIlL DtiW to yield.200 I'g/ml).
/
amrinone (Inocor®)
,-----/Nonadrene.rgic cmiotoni.:.
\
/
DfIUO",O
\
':c"L,------=-----:-:----'~,
Pho~phQdie8t1!ra!1e inhibitor. eITecl.ll l ilDilftr to dobIIt amine (i ncluding exacerbation or mYDC8n!ial ischemia). 2'10. incldeuce ofthromboW dO'le!l . lncruJlea puJmonlu)I voscula. ,..,.i~ laMe.
,-----/
nore pin e ph rine
\
epinephri ne
\
~
I
~IIfFO
\
,
& 0.5-1.0 rug of I: 10,000 ~lutfon [VP: may repeat q 5 minu tes(may boJua p.!' ET lube). Drip: llartat 1.0 J.lglmin, titl'\lle up to 8 J.lglmin (1.0 prep:ire: put I 109 in tOO 101 NS or05W).
DirectB l timulatlon (jIQ.'!it;ive iuolNpic.and ohrDnot",pi~ ) Rx If tart drip at IH2 J.lglmio ; maintenance 2· 4 I'gfmin (0.1>-1.0 mVminl (to prepare: 2 lOgin SOD rnl NS ~r D5W to yield 4 Ilgfcc).
1.2.3.
Neurogenic pulmonary edema
A rRra ""mi ition
GENERAL CARE
a~"""i"t.!echd (prior to th ~ AM hy· dromrtj,uoedose), a.nd the hydrorortisone is tapered by2,5 mg W"'lkJy un!.ii 10 mgtd is n!ached (Ia wer lirnit.s of physiologic) C. then, every 2-4 WHM, theAM cord. ol level i. dre .. n (prior to AM dose) until the 8 AIlI cortisol is > 10 1'81100 ml, indica t ing retum ofbll5elineadrenal I'uncLion O. when this return of baseline adreu.1 function DOCU" : 1. daily steroid..!l a~ stopped, but strMS dosu must stili be given whn needed (u. below) 2, monthly cosyntro]lin Klimulatioo leaL!! (_ pogt 444) Nre JH'rfnrmoo ....ntll oonnll1. The need for stress doaes ofsteroid~ ceases when a PQ5' itive lut is obt ained. The riak for adrenal illl!Ufficie.ncy perl;latii-..2.. )'jlJI.[5, sf\erce$atlon of chronic neroids (upecially thlO first year)
GENERAL CARE
1.3. Endocrinology
,
STFIESS DOSES Duri.rl, ph)'lliologic ".l.rHl" ill , nonnal .dr, nal ",nd proc!uott. .. 250-300 ml bydroooortilOnoid l Y. With cilronie,llucorortiroid therapy leither It pr~lnt, or in lut ].2 yB), fLlppr ulion oftha tIOrmal ".t,ess·r&,pon..- ntoeHeitat. lIupplenHlnt.1 d OIft.
In
Table 1·5 s.erokl alt, u dol· u 10' "IItcUve . urgery
pIIt~" ....!tll
.. l uppreued IIPA axi.: for mild mn.-.l • •, . un, co mmon cold), linal. dental eJrtracuon: double t he dally do .e (if off I l.;lroidl, stvII 40 IlII1 hydroo:o/'1ilOn e OlD)
fa, modera\e . tl1!ll' (e.,. nU l, minor I W"jIwry lUId e . iIlalllneathe.11I (endO&tOpy, multiple den. tal utraction. ...): giVII 50 InlI hydrocortison •
• '0
for m-,j or Illne.1 lpnllumon;a, l )'tle." ic ln fectione, high fever), I16Yi!~ trauma. or llmergenC)'
I Ulpry under il8oe rlllllOe.lith l'llll : giVI tOO mg hydnl }-' o r "'miner alocorticoid e merge ll oy" U&u.Uy not naCBfUliI.-. F .... tu,..,. comUlOn to patients who develop CPM a""" deloy in the diagnoeisofhyponatremla with .... sultant respiratory BrTi'8t or MlI~ure with probsble hypoxemic "enl rapid ron-eetiOlI to normo· or hYP"I'-nlltremia (> l S5 mEqIt. ) .. llhln 48 houtll of initiation ofthenpy increase oflJ(lt\lm fOdlum by , 25 IUEqIL within 48 hours or;nitiation of therapy O'Ier-ronecting5l1rum ~UID in patil!l1ta with hepatic encephalopathy .NS, many patienta de,".,IDping CPM wen! victims of chn"'icdebilitat.lng disellse.
"
VI.. Fluid$ and Ele-etrolyt ...
GENRRAL CARE
malnourishment, or alcoholism and neve r had hyponatr-tmi • • Ma ny had an episode of hypox,ialanoxia'" p1"QO!IlCII ofhyponatremi. > 2~ hlll prior to treatment" TR£ATMENTOF HYPON",TREMIA
PUientl with hypon. tremi. of unknown durMKln probably h.ve chronic hyponatre. mia ifminimally . ympto .... tie. and ehould be trealed _lowly, preferably with flu id re· striction. Thoee .. lUI acute Iymptomatic hypon.tremia (convull ionl, Itupor or coma) . hould be treated promptly _inee the! pr_n«o of eNS . ymptoros h.. been _hown to be auocilted with brain edema lrad)ographica Uy and I t necrop.y) and m.y henold impendh" hern.illiOtl and cardio...... prralOry Symptomatic r.:lientl with hyponatremia ofunknown duration InI t heOtle, a t ril k of neurologic HqUl .e.lnd one . boukl,lartoft" .. ith. I"col"l"fttion, followed by. more cradulltrutment u outlined below". TIM followina method for con-ec:ting hyponatremia UNa') < 12S mEqfL) iSll&Ocilled with low riB of deveKlping CPM (although it may not be poMible to define. note of co~tion th.t I, conai. tently free of m k): 1. • CAUTION: avoid oormo- 01" hyper.n.tremia during con-en1 thoot ..... undeaT. edema doe. nOt ooc:ur. can~r.
E ti o logies o f SlAD H The hyponatremia ofSlADH must be differentiated from that due to «Tebral A lt wast ing (CSW) (u, ~Iow). SLADH may be KIn in the fDllowin6 ... ttin", (KI refe",noel' for more utensive list): L malignant tumD": especially bronchogenic 2. numerous intnerani. 1 prooeISN including: A. men.ingiti.: ... pecially in pediatric patientl•• tao .. ith TB mf'~ti. B. tr.uma: seen in 4.6'1r of head trauma patientl C. increaaed lep D. tumDn E. post cnoniotomy F. SAM (NB: ..... Ie-out CSW, whidl ~ui r.. dilfenl!nt tre.tment. _lH!low) 3. numerous pulmonary dillarden A. malignancy B. pulmonary TB 4. 5. 6. 7.
C. aspergillOiIl m.y lO.IIleti mH occur . ecooda.ry to enemia with !tren, ..vere pain. nau ... Dr hypotension (all can st imulate ADH rele...) ooc:uion' Uy _ ... with acute i nterrnjt ~nt porphyrilo (AlP ) d..... gl: A. ehlorpropramide(Oi.binese¢l): mayeauSflI "n1.lIve" SLADIi byincr...ing
GENERAL CARE
1.4. Fluid. and Elactrolyt.ea
"
the renal ... n,it;";ty to endogenOUi ADH 8 . OJIytoci ..... hal""'6 ·croo activity" with Al) ~I , and UlII.)' al., bI contarninllt. eel with ADH C. thiuide diurr.tiet: h.Ydt«hlorothluide . 00 po~ 18) D. arbamaupine ' 1'egretola)
uri"", and DO ."idenc- of ren, 1 or Idrlllnlli dY'f\metion. 10'1 more detai l: 1. Iow ... rum lOdium lhyponatreOl.iI): u.ulllly < 1$4 m£qll.. 2 . low I61,Im o.mollliity. < 2110 mOnnIL 3. hl,h Urilllll')' lIOdiumA :... It wutin,,): 01 le ... l ,. 18 mEqIL. often S()'160 -4 . high rltio o(Urlnl:M n;lm Q8tnOUolil),: onen 1.5-2.5:1, but may be 1:1 $ . norttlllll'f:na\lUndion (ebec:1r. BUN &. ~.Ihlille): BUN eommon ly < 10 6 . normal.d .. n.aol function (no hypoiAnaioD, flO hype rlu lemia) 7 no hypothyroidi.m 8. no .1.". ofdoth)'dl'lltion or _ r hydra t lon (in many pat>enUi with lieull! b rain d,.ea... there i. signLiiaont hypO"Olemia often due to CSW (ou /H!/Qw ) Ind II thi '!& I atimulu. for AOH lIr."ellC)n , the ADH relulM IM.lI be "appmpriQIa"") \(further \.eStin, ito required, thl wate ... load Iell Wcmlidered to bl the defini t ive. Ielt-. The pati.nt Ia ulud to con.ume a wal.er load 0(20 m.tIq up to 1:100 mi. In the lib· tenet or,.drenal or ren&! inlu n-'dwty, the fQi\ure to I!;(~rete 65'110 of the ",. lH load in 4 mor ~ in.5 lin indkall's StAnH. . CAUTION : lhito te!lt i. danll~roWl if tile at.arting IM!rllm !NII'I iI ~ 12-6 mEqIL o~ iftbe patient h .. I)'lIIpUlIIlII ofhypormtremil, Ah.emll~ively, OM! may n:e .... u"' IM!ruJD _mEN>"
BellUre that hyponatremia IS notdu .. to CSW 1_
Mloui ) bef CENTRAL DI ~of ADH ","cretorycapacily mUllt be lost bef...... din~al 01 ens ...... Cha racteri$lic high urine O\Ilput (polyuri.) with low urine ""molality, and (in the conscious patient) cr.ving ror w.ter {polydipsi.). especi.lIy ife-water. Different ial diagnosil of 01: 1. (Detlf"O«!ftic) di.betea iosipidus 2. nephrogenic diabetes insipid ", 3. psychogenic A. idiopathic: from resetting of the osmOila1 B. psychoglenic: polydipsil 4. osmotic diures"' : e.g. following mannitol. or ",ith renal glu- 600 lished, and further leating NORMAL is not needed (exrept to differential c.m
CRyOPRECIPITATE lrIInsfu!liGn mteri e; I heonophiJia A
Rfoeom~nded
2.
vOn Willebrand d isea ....
3. documented fibrinog~nfr.dor VIII deficienc,y 4 docwnen~ di55eminated inl.rll~allCUla r t'OIIgulation tDI C~ alonl with oLber m.odeoI oftberapy
1.5.2.
Coagulation
1.5.2.1.
Anticoagulation
AN~GLt.ANT CON51OERATIONS IN NEUROSURGERY
Contralndication l to beparin tberapy Many U'adilional contraJndkation.aalll bein, r~nlIidered and challenced. M"';Vf
PE producinghemodynun itO)mprom iH should bI! treated witlli nticoogulalion in mOll~ dupite in traut.nial rUb. ContrRindiCl tillnt to h,~rin include; .-..cent !leVeR hud injury te«nt uarllolomy: ..e IMI"", palj..,ta wi th roIIplopa thia hemorrhagic infarct ion bleed.inlulcer or otbu ,nllcc85.bl ... blH.hng si te 'Hitolllrollto bl" hypertan5jon Vvelll hepat ic 0. renal dr_v immedia tely before inv..ive procedu .. (au ~ for ar.riocnphy 0. my~IOI"T" phy) b... in tu mor;,"~
cue.I
10
pati~lltllwltb
unM.lphu'-ed (l ocide otal) ce rebra l ane u rylIm ,
Nllioo.l\IlMion 111&, no!. ilKrN'" the rillil of he,""rrba~(i .e. r upturtl. hO'Wevt'r, . hould nrplu .... occur, 8111ticotJUJItion would most likely inl!l'fue volumeorhlmotTh.~e.
'lid
o,u. ;nCff'a. morb'di\,)' and mortallt,.
In plltieou wic.b braJn tumllr Sonw au thora are .... Iuclanl \0 adminil ter heparin l(I any p!!Iti~nt with a brain lum~. , Itl\ouih a numbr!:ror.tudJ. found no hi~u ri.k in th .... patientawhm treal' f:tiwi\h her-rin til' DrIII.nt~ lat;"" •..·(PT.hould be follO'Wellrin activates ."tl·lhrombin lJIand can CUU$e plateletaggrega· tion ) which caQ rEDult in 10111. CVAs, OVl'$. PEa. etc. Thrombocytopenia: tranSient mild thromboo;ytop.tnill i& (airly common in the 61'11t few days "fter initiating heparin therapy. however se>'1'rE thrombocytopenia IK:CUr8 in L-~ of Piltients l'eCf!iYlng hepQrin " 4 dil)'ll (usually has a delayed Dn5l!tof6·12 dilY~. ilDd is due 10 CDlUlumpt;on in heparin·induced thrombas" or toantibodill!l rottlloo. ~g3il1fit~ hep$rin·plllteletpTOu,in ooltlplllX I. Conijider LIM-of l~pjrudin (",~ tHlow) iD thrombocyr.openic patients. Chrollk th4!rIlPY may cRUle OIl· teopOrlI6~ .
II
1.5 Hematology
GENERAL CARE
Low mo lee ular w e ight be pa ri n IJ"" Low tnQlec:ular wei,hl hepl.rinJI (LMWH) (.v~llIge mol~ lar weight .. 3QOO.OOOO daltorwlare derived rrom Wlrractionllted hepllr in (IVlrIlPMW", 12,OOO· 15,OOOr:\altoM). LMWH. dllr•• from UnrtllCI;onnted Ileparin becaute th ey h.v • • higher ... 1;0 of IInli·flllt.tua lly hmu: colII!ulable ( _ d i l l ' to rl!e Type liD wheT" faclOT$ may be ab· normlll 0. miMing). R.o; 0.3 OJg/kg (u8fl 50 ml nfdiluentfor dovl ~ 3 OJg. usot 10 Ill] for :loses> 3 11K) given over 15·30 minute.o 30 minu\.(,s prior 1.0 a s urSica l procedlJ,te, ELEVATED PRE-oP PIT In a palient with no h'i tory ofOOllgulopathy. a $,gnlfiCB otly eJevatc(\ pre-op P'IT,5 eommon]y d ..... to I!ither a foetor deficieney o r to lupus ant,eollgulan~ Worku p: I. milting study 2. lupus coagulont
If the mixins s tudy cor,..., .... !he el"v8U!d P'l'T, th"'n there- ia probably B factor defi. ciency. CoNU! t II hemalologi8l. Lupus anticoagulant: I flh~ test for lupUJ pn t ieuagula n t ;5 ~ilive.lhen tM major risk to tM pa tient wi th surgery i. o.ol blllEdin" rath~r it i. thromboombolism . ManD!:ement reNlmm~ndBtions:
"
1.5. Hllmlltology
GENERAL CARE
as soon as feasible post-op.SLart patient On heparin (Met page 22 ) or LMW heparin (_ page 23), e.g. Love nox 2. at the ume time SLart warfarin, and maiotain therapeutic antiooagulation for S· 4 weeks (the risk of DVTIPE is attuaUy highest in the fint few weeks !X'et-op) 3. mobilize ft8 soon as p08.!lible post-op 4. ronalder vena-tava intelTUption filter in patients for whom anticoagulation is contraindicated 1.
THROMBOEMBOLISM IN NEU ROSURGERV
Deep-veiD Ih ro m ~i 6 (DVT) is of ooncem primarily because of the potential for material (dot. platelet dumps ... ) to dislodge aod form emboli (i ncluding pulmonary ern· boli. (PE» which may cause pulmonary infarction, sudden death (from cardinc arrest), orce rebral infarction(from a 8()o.called paradoxical embolus. which mayoa;ur in tbe pres. ence ofa pateot forameo ovale. see Cal"diOllfllic brai" ~mbolirm. page 773). The reported mortality from OVT in the LEs ranges from 9-50%"". OVT limited to the calf has a low threat « 1%) of embolization. however. these dots later extend into the proximal deep veins in 3Q..5O% ofca5es", from where emboliutioo may OCCur lin 40·50%). or they may produce postphlebitic syndrome. Neurosurgical patients are pa rticularly prone to developiog DVTa (estimated risk: 19-50%) due at least in pa rt to the ..... lative frequency of the following: 1. long operating tillles of some procedures 2. prolonged bed rest 3. paralyzed limbs (e.g. in spinal oord injuries or stroke pa!ienLl) 4. alterations in COlIgUlation status A. in patients ..... ith brain tumors (_ below) or head injury'" I . n)'lated to the eondition itself 2. due t!! reduced by pre·op use of aspi rin" 3, suba rachnoid hemorrhage 4. head trauma 5. stroke: incidence of PE = 1-19.8%. with mortality of25·1{)()% 6. patients undergoing neurosurgical operation PROPHYLAXIS AGAINST DVT
OptiON includa: I. general measures A. passive range of motion B. ","~u1at.o ~"ptup riat~ pati~" ts as ~'... Iy .... pob.ibl~ 2. mechanica l te Reeo mm enda tion. RecomD>eod«l
prophyluia ".rie, with the risk ofdevel· oping DVT. a • •Uu.-
Table 1. 12 Rl,k & p. ophylaxl a of OVT In neu rosurgical pa118nl'"
trated in Tobk J·12 -. AJIJoO I « pogr 105 fot det.aig orprophylaxil in cervical . pI. nal cord injuria. DIAGNOSIS OF OVT
The clinical di agt>O$i. of OV'l' ill very unreliable. A patient with tha"d,uie liJ'lI" of. hot. awol· leo. nnd \.ende. enlf, ora poII itlyt Homaou' . lgn (calfpll;n on dor • • inexlon ofthe a nkJe) will hpva. OVT only 2().50% of til." tim ..... 00·60% o'f patienLl with OVT will not hav" thesa f,ndiop.
5. wear anti·embolic stocking on affected LE indefinitely (limb ill always at risk of T" EMH
The exuberant tissu e is very radiU6ensitive, however, the patient may be somewhat dependent on the hematopoietic capacity of the tiuue. Treatment Sw-gical excisiun followed by radiation therapy has been tlle recommended treatmenL Repeated blood transfusions may help udure EMH and may be """ful po$t-op in· stead ofRTX except for reft-actory cases" . Su rgery on these patients is difficult because 0(: I. lowplateletc:ount 2. poor condition of bone 3. cardiomyopathy: increased anesthetic risk 4. anemia, coupled with the fact that mOllt uftbese plI.tien:" are "iron·toxic" from multiple previou t transfusions 5. total removal of the maBS is not always possible
1.6.
Pharmacology
1.6.1.
Analgesics
For a discussion of types of pain and pain procedures, H' pagt 376. GENERAL PRINCIPLES
The key to good pain con!.rol is the early use of adequate levels of effective /Ulalge· sics. For cancer pain, &eheduled dosing is superior to PRN dosing, aod "rescue" medication ~hould be available". Nonopioid analgesics should be tootinued 85 more pm..nl medications an d iavasi"e techniques are utili2ed. ANALGESICS FOR SCM: SPECIFIC TYPES OF PAIN
Visceral or deafferentation pain May sometime. be effectively treated with tricyclic antidepress/Ults (ou pag~ 33). Tryptophan may be effective (8# pagt 33). Carbamuepioe (Tegretol®) may be useful for paroxysmal, lancinating pain. n:u~ta.8tatic bone d i sease Steroids, aspirin, or NSAIDa...,.., especiaUy helpful , probably by reduciog prostag· landin mediated sen9i ti!.lltion of A·delta alld C ftbeno. and therefore may be preferred to APAP.
Tho! anU.i nfl&m",atot)' propertiell orNSAID. i. pri"" .,.;!), d"e to ; nhlMt'on orth~ ~n· ...,. ..... eycl_y,ftlHe (COX) .. hlch part,cipalell;" the !lyntheslll of pl'OIIl.I,!.nduul ond thromoolu. nu'" . Chal1lc~ri.tic:5
of nonwlective ""'ISUlroid al anti·;nnalJto'latory drug>::
.n a~ """n 0 . .11)' eao;ep4. Mtorol • ., t rornethDmine ('I'oradolol!l . (Ut below ) no dependence develofUI addiliveeffKt imprOY'fllhe ptlin reHfJwith opioid anal,esies NSAlo.land APAP) deruonnf1lte a "",mD, ened : a maximulIl dOlt! aboy" whlt h no fU rthe r llIIaJaaiio ilobta.lned. Foraspirin and APAP, Ihi, lor " l ua lly bf:l;'ween 650-J300 me, and ia ofLen higher ror oUt... NS Al08 whkh m.~ alllO IUIYe a longer d"f1Ition 0( ""lion ri>; k or Gl "PMI;& ..,mlllOlI . 1I>Ore .."rioua rho of hel"'totox.idty"", or GI ulcer· al iOll. hemotTbaee , 0 ' ;:>erlo .... tion Irt! I/!!!$ """mmon lIking medication with mu la Or I nl.lcicb hal not been pI'1l'«'A effective in reduo-IniGi sideeffKW. ~ti~p fO$toI ICytotd). a pl'OIItagiandin, maybe "ff'« tiye in mitipling NSAlD·U\d.lUd pamc el'Ollioo or peptic ulm I'. Colltl'l'nrlical.ed in p. ......cy. Rx 200 Ili PO QID willi food as lone ... patient Is on NSAlDlI. Ifnol tolenled. w;e 100 1-'( • • CAUTION; an .bwtjrjlCl~nL Should not be ,""en tCl preg. nant womt n or women Clf clli ldbearing polen till most l'ev",..ibly inhibit pI.telet runction and Pro1onl bl\'lO'd;nll tim .. (nonaceil}'llted .alkylal.ea hIve Ies& antiplat.elet action, e.l . • al ..late, trililli~l.te , n.b"lIIet.onle) ...... pirin. unlike all nthll' NSAlDS, irrflvtClibly bincb to c~IOOXYleniH and thUll inhim .. platelel ruocUon for the &·10 day lire oflhe platelel an c...... MkliuOl and .... leI' ~ntion and ca ny ~ rloI k ofNSAl D.lnd u«yp.'1IC1iYIIv (IrcIucIIng "",,",Ulll _ _ U'K), ....... '...., ".."iJeOi _given nl~ \.6. I;>hannacolcgy
.."""
Tabl,1-18 Equ!,n. lgu!c dOlle. lor S EVERE pain , AGONIST/ANTAGONIST opIoldl (relerenced 10 10 mg 1M morphine) Drug 01l1li: genetic
...
1101,1'1
(!Iroprlel.r)8j
~(Supronexe)
'M Sl
MIX 'M
"'......
...
(Nubm"f)
"""",
(T'twIr0f;)
demcina(~)
--
" "POt 'M, ~
,. '.3 ,
....
",., ""
(."
,
O.S. I
,,.>,.•
..., 10
I~O~
1&0 ($1M 0 SO) 1.S.2 10
.
"'~
..,••. ••
.,."",.,.
!)Ir1iI!J9OAI"
I
1'10 $Igm1l1C1j1101
.....~t
,...,
.. ..... lItfICIdIIte _8w.1'~" pI'lente pIIySIC8lIy rill"",,,..,, on ~ 1I'0OI ~ "'''''. ~ oIg land thercby may redu{'O!
the reqllitOO dC>H) 1'ric),elle a nl ldepre ..... n~:.~ ~ 376.
'rryptop b"n: an emillOlrid e nd • pl'l'CUrIIOI" ofwrotonm. wly work by illCTUti", .crolonin !eye I,. ~u'"" high d(l<ellllnd luu bypnolH: ~ffectll , w....,fo.... 1.t..2 gro giv"'n usually q hi , MIIII gi"c daily MVI U" chron ic trypcophln thel"llP1 depleta: viWoin 8..
ADtlhlelaro!netl: ~,II\.Aminel play I role in oociception . .... ntim-tamine.. wkith ant ,lao I nJUo!ytk 'ntillmBtic, and mildl y hypnotk Int@fTecll"eB. Bnll!ge8i.-or uadj,,· "lints. HydroxYline (A(oIra41, VisLaril®): fU . tart with rx> mf PO q AM .nd 100 mg PO q hi. May incre."" up III _ 200 mg dllily. AnliconvulPnll; tlIroa .... zepine, c!onazepem, plMnyt.c>ill or flbilpttnlin mly he effKti"e in ncuro{)ltthlc pa in from dinbel;t neuropttthy, lrigemin&l ".U".lgil, pafl. ""' .... petie nlluNllgil, gl_pharyngeal ne urll!gill , lind neuraJgi". due t.o ne,,'e Injury or intil· lration with C8nurOO Phe'lOtblui o u : Inme cause mild reduction in nodoc:a pt;on. Mo .. are t".nqu,lldng Ind IOliemetie. Best Imo.... n for tbis U~ ia fluphlJluinl! \ Prolwn®). usually given with • lricyeli~ I ntidepresunt for n~uroPl'thic JNlin, .e.lJiI:JM/;t tl.f ~ropee ."alp.ie ,ir.." of APAP, ASAoribuprofen in HlA., Ofllliutpry pIIill.nd post.-partWll pIIm.
u..
1.6.2.
po-.._.
Antispasmodics/muscle relaxants
0 ...1tf'nlI"llUy·aclinll mUlti/! n!laxan18 have. Md/lting ,-Ifed. On the c:ent.raJ net'V\lU5 a),litem, a nd lben> I. little e>'ideuce of any othu beneficial f'fTO':(t. Elfa:acy ofu5e in~ tienta WIth Mut@lowblockproblema r. dubioUl'" (uo: /XlIl!l291). Only d 65 yrs old uperience noc:tUn;laI crtlmpl at -orne time IUluaU,. In 1M l~, _I~;n the han(II,. No weU-cnnlrolltd lrial' todoelI"",nl efl"ertivlllllM_Mda -aMl,ysl. luggesltd Ihlt lhe frequency ofCfDmp5 C~II be ~ duUld by ~ 2MI. _ r 2 weeks of trealnltlnl, and by InOT1! ",,"r' w ...1u. bu~ Ih"re WM 110 challie in ....enty o:r dunoo ...... Avoid in pr~n~ (aborti facien t). CaUlion: even low dose can cau.. 'I'TP In stn,itive palienta_ re~nted doae&call cau5ecinchoism (watch for tinnilus. HfA. NN, hearillj' Iou)-_Rulf:-out uremk neuropnth,. bero~ trl!(ltinl't-po,gt'
"",.
R..r Ad"l1 200 o:r:roo ..... PO 'I lis f'RN (better ..tficac;r seen wit .. n!t(Uhlr lI"";nl).
1.6. PhannacolocY
GENERAL CARE
1.6.3.
Benzodiazepines
Alto!1!e &dilfiliU & paralyticl, page 36. All are elre-c:tive for \.reating aru ~derly
pa-
tients. May be ",vel1!led with nUlYIIUeniJ (Ii'!! IH>/ow l. SmK~' v.. ntilatory dep..-ioo anti hypowo,s.ion u"cer1l.t&d by opioids, w,,'ee in palien"" with COPD. AlI contraindicetad in fin! trirn""ter orpre~lInon il ... tran-.ated , intra_ .rterial ln~tioo ~ n~tOti., . git.ation itin,ieeted . Iowl)" lin IIntj.n. !gnic mJOC'/lrd ial deprUlll nl., hypotelWon in hypovolemic patlen".. & Adult.: ini l ie! eonfflltra\.ion ,hollid oot ucae \
pI!~u"neou,
,
rhllO-
GENERAL CARE
where pellen! need. t.o be .edlted IUId I w&.ll.ent ~
haloperidol (Haldol®)
\
I
0l'II.I0'*'. .... chloral hydrnteat
50 rog!kg C.- Niow). pentobarbital (Nembutlll®~ 2 mg/'ka' IV II ~ hnlPRN
OR OR
fentanyl (Sublimawe): 1 NIka: IVP di,upam (V.liuum). 0.1-0.50 mglltg« not to ..xefti 10 mg) lit miduolam (V.r-.tIIl
AKA -lytic r;Odt.lail" Corabine in 1 . ynnp.nd rive at deolp]M ;11-
.iac:tion: • ...
IIW!peridine (Denu!rote): 2 mlVkf (mu: don 60 mg) pl'OlIlethnina (PhenerganCl): 1 mgllo;g (a COnl rll;lId;clltad;n patient.! < 2 yn age) chlOll'I'OIDI,iI\e rJ'hGradnt«l): J mN
GENEItU CARE
1.6. PhnnnlleolollY
"
1.6.4.3.
Paralytics (neuromuscular blocking agents)
CAU'J'ION: "'quirt's venc.ilatiDn (intubation or AmbtJ-bAglmnskl. Reminder: pH ... Iywd potienl.ll may still be~DnsciQIIII.lUld thereforellb!~ to !eel pll.in, th ,imultllneQua use. of8edaliou i, !huJI required fD' cou$ciaus pMienu. Early routine uae in head·injured patienulowens ICP (e.l . f.am lI.uctioning' O') and mon...lity. but doe!> not improve ovenilU outcome'" . Neuromuscular blQcking alente (NMBAa) Ille ch:ll!lpa.m "",,1m! during IIttempted intubatiDn uRing othe~ IIgents. S id e effeds • CAUTIONS: usually increases serum K' by 0.5 mE:Qfl. (on rBre Decaf;on CBUses "",veee bype rkalemia (!K·I up 10 12 m&yl.) in patient8 with nel,U'l)nal D~ mu..:ular pathol. ogy. uu~ing tllTdiac compHt~UDru; which cannot 1Jo:o. blocked ). Lherefcre contraindiuted in IlCUt.e phS$!! of iDjury fDllDwing major bUm6, multiple trauma Dr nt.e1Uive denervatioo of.keletlll mUl! INTERMEOIATE ACTING PARAL'r'TlCS
~
vccuronium {No rc u ron®)
\!
0RUCl1NFO
\
,
Nondepolariting (competit ive) NMRA, Adequatto pnra!Ylis for intubation within 2.5-.3 minut"" of pdmini~Lratlou, About onR third mol'll rtent th an plUlcuroruum •• hotter dllI'Dti"" o{netion (huts _ 30 rnmutu "fl..>. tailisl dQlJOl . Unlike pe.n""...,,,i,,,,,, vcry I,ul o vegal (i .~. ClIrdiOY ..scular) err-acta. No CNS .. dive mel.8bolite.s. DOoe'!I not alretl. lCP Or CPP. Hepatically l1lel.8bol i~ed . Due to active metabolite, . partlly,Ca hn been reported to taM 6 hrs to 7 days to recede following diswnt;nuotiOll of the druB Dfler it 2 dll.)'8 uae in pIItient.s with I'f!nal f~Hllr.-.I". MU8L be mixed to use.
Doaing BWP .... !(;g: .10 rng freeu-dried enkc. requiring rec,;mst;lution . Uao wit hin 24 h..... Rx Adul~ahd ch ildren .> 10 yeors age: 0.1 mglkg (fOT llIoe~adults use 8-10 mg as ini_ tial dose). May repeo t q 1 hr PRN . lnfu gion : 1-2I'gfkglmin. Hz Pedlatril:;!:hiI.dD:.Il ()- IOyrs) requil'll sligh tly higher dose and morefrequenLdOl!lIng than adult. lofllol.l (7 weeki - 1 yr); slightly more ~ensitive Iln a mglkg basi. thaD adullil. tak"" ~ 1.5 ){ longer to recover. Use in n"'lnet.el and ~ont.inuou~ infu9ion in ch il _ drr.ll i~ in sufficiently studied.
GF.:NF.:RAL CARE
1.6. Phannacology
"
:-./
cisatncunum (NimbeX®)
\
/
DfI\IIlIHO'O
\
,
Nondepolarizing (rompetiti"e) blocker. Thi s illOmu of Btrll{:urium does no~ relelllle unlike it& plU"eP l C(lmpOuud (lee ~Iow). Providllll about I bour ofparaly~i • • Also undergoes HofmBM degradatifln. with laudanosine III one nfl1.3 metaboli1.(s. Rx Adult and children> 12 years age: 0.15 or 0.2 mglkg 113 part of propOfoVnitroui hi&tamin~
oxideJWi:ygen ioduction-intubdion ~ ique produ~ mU$ele paralyeil adequllte for intubation within 2 or l.~ min n.es, respedively. Infm Do!:ling SlIPPUUI: S " 10 ml ampule. c.f 10 mg/IDI concentration. Rx Adult & ~hlldren > 2 yr$ age : 0.4·0.l~ mglkg lVP. ReduCOl ~b~quentdDfieli to 0.02 mg/kg Rx ~(I month· 2yrs), 0.3·0.4 mglkg.
LONG ACTI NG PAR ALYTICS
,----/
pancunm;um (Pavu\on ®)
\
I
DAl./GItjR)
\
,
"Prototype" nondepolari-QIIij (CQm petitivtl) para lytil'- Puk; 3·5miDII, duration up to 60 mins . Revel'9ible with Bnticholineat.erage$lucil as neos tigmine. Renal elimination. SlDI:D"1'ECTa1 usefulnel!s a limited because the drug is ~ and IIUl illliiNctlym· pathOlnimBtic whid> incretlliell cardiac OUtput. pulse rate. and rcp ConsIder "l'curonium II1S an alt.em ative.($n' aoo ... ). DOlling ~
Rx :t:i£::Imawl: espe-cialJy Hnaitive. t 1.6.5. St~68
Acid inhIbitors ulce ra; in oeuro!fllrge .y"
The m~ of st,...ss uicer' (SU);$ high in c,; tically HI patienUi w;~h CNS puthology. 17':lofSU. produ~ dio icallyaignificant hemorrhage. eNS risk r""tan. indude inlmen· nial patholOgy: brain injury (upeda Uy Gl asgow Coma scale IfCOr e < 9), brain t\lOlO"" in· tracerebral hemorrhage. SIADH, CNS infection, l~cbemic CVA, 118 ~11 as spinal cord Injury. The odds a re jncreallf! 3 wlI'!lui), bums> 25% of body turfl1 0.1110 inactiyales pl'psin. Other therapies that don't in~olve alterationa of pH thllt may be elf~tive indllde ello.ralfa le and enteral notri tion (controver lial),". Titrated antscidl or s lIcralrale IIPpear La be superior to'}l, antaconiu8 in reducing the inddence ofSL·•• RolltJ.ne prophylllJtia .... hen .leroma are used fs not .... PlTant.ed unle" one of the rol· lowing r is!< fac:tors I ' e p~nt: prior PUD, tonCUrTen~ use or NSAlOs. lIepot'c or renal failure, malno urish ment. or prolonged steroid therapy >:1 weeu. In
KIwrwI>I. M .... C . _ M I. M.rOO F... aI" Th< """~""., or II"""""",. ,n the ""~ipid· .. ,it>ody.ynd"""" . N r..... J ~ ))20 99)·1. I99S. Ill''bll< hoo"b "" i>ur cereb ri .••) 3 4
irrita t ion!W Inflammlltion ofmeningel! m@ningiti. tumor: with Or without ele~aU!d ICP (no. JXlIt 4!l.S) C. lou! plIlhoiolD' oft~ eye, nuopha ryr\ll:, OT utracn.ni"J tiRuea (;"duding ~U"lt
<ell artenti.,.- pagt. 58)
o following head t ' II"llIa (~tcor>c:uU;Y" ayndrorne}: ~~ PfJ~ 682 E. f.lllowtllg ainlolllmy t".ynd rome 01 th. trephIned"): 1ft pagt 6J2 A ,,,!Yere new HlA. (It. chaos , in the plltI\!m oflllo"8.tarulinl,: Of' rKUrTenl HI" ( in~
eluding developing IInoclllt.l(l NN, Or ~n "lmormal neurologic enm) w.rran~ (.. r1MoT in· Yt$tiption with CTorMR I' ,
2.2.1.
Migraine
Migraine attacks ua uallY'ICCLlr in indiyicillaLt pr.dilpeud IA'l ,h"condition, lind mly
be activated by factorl alleh a.\ urill,lli,ht, Itrfla. diilt ch, npll. trauma, adminiNrillioI'I ofrttdiologic contrast
m~i.
(eJpedaUy 8JlgiOgtIlPhy) and vesodilalol'l ,
CLASSIFICATION See aJfrO indu under HtodQl;/u" e.lI. [or; crash pogr 782, pOIH,.. myeIOjfraUl headache ptJ~ 46".
migr~ine
(thundndap headachel
COMMON MIGRAINE
Ep;Booie HI" with:;.lN and photopl:lobil, w;thoutllu", or fleurologiedelkit, CLASSIC MIGRAIIII£ migrai~ ~Ive comp1t~ly
... aur. , May have HlA with occuional foelll neuro\orlC dt:firiuI ) in .. 24 hra . Over halfofthe Ir""delll neurologie dilturblln~ ~f'It vilUll\' and uluan,. _no!" poIIitiyt phenomena (lpArk photoplil, ,Ian., IXlmplu 1000metrie pIIuema, fortifiCAtion apectra) which mllY leaye negative phenOOltl\a (goloma. hemianopia , monocular or bin. orular yjauplloQ , .. ) in their wake. The HlCond IIIlXt malloon symptOma a,.. .amatoRn.. frOry involving the hand aod lower rac~ ..... fn!quently, defldu may (:QIUI\1t orapha&ia, .... mipa ....,is. or unil,,~ral elu"",;n ...... A slaw mardl·like progrnllion ofdeficit i. Cha rllllVlril'itk. Tho! risk of ~Iroke is probably inatlaMd ;" palientll wilh mign,,""'. Com mon
Ihlll
COMPLIC.'o TEO MIGAAIN£ Occuional IIll.8eU ofdaJ.4jcmigte;ne with minimal or no auociated HlA, and comple~ re&Olution ofneuroJorie deficit ..... 30 dap
M/OAAINE £OU/VAL£f'/T Nelll'Olork 'ymplomf INN. visualaLlrJ.. etc. ) without HlA (_phaJg.e migraine), Seen mc:.11y in childnn UsuIII,. dev&lopll into typical migraiae with I~ Au.. lIIay be ehorVlned by opening and _an_inl contenb of a 10 IIll!I ni(ed,pine capw]e'". HEMIPLEGIC MIGIM/Nf
lilA typiC*lly pr-'eII hemiplegia whkh may penlSt eve"
Il~r
HlA t«olves.
CWSTER HEADACHE
AKA h;'l.8min~ mllTlllIl.. Actually a neUl'OY8ICUIllt ""tnt. di$tincl from true mi. IUrne. RtcutTtnl unila~tll altacks ofseYeN! pa;". Ulually oculoCrontal or IICulolempGfli wilh occuional radiation Intothejaw, ulu_Uy r«Ilnin,on theslme , id"of the head. lpe:ila~ralautonomic: UVl~
(UW)juntliyal injectIon, nau.l
conlle~tion ,
th ino rrhea ,
lacfllUlltion, fad.1 nUllhin,) .... comtll(ln , PBrtial Hamer'aayndrome (ptoai. and ml .... \s) _ellme. o«u,.. Male;f~ale ratio U. ~ 5;1, 25~ of pat ienlll hay" a petllOllal or fami ly hi.tory of m igTIJM. H"adache. ch'''doI'riu~Uy h.... no prodrome. I.t SO-SO minu\.O$, and ~r one or mote limes daily u...al\y (or 4- 12 wHIu, often lit a .imila t time day. following which ther. is typieally, remillion fat an average of 12 month.",
or
NEUROLOGY
2.'2.. Headache
BASILAR ARTERY MIGRAINE
Essentially restr icted to adnlellc~nce. RecurT~nt episod811 lastln!: minutes to hours oftral"llnent neurologic ddidu. in distribution ofverwbrobMilar system , Defioil& iru:.iuu; v~rt.igo \ moat common ), ga;~ ataxia, ..,pual disturbance(acotD,Data, "'Ialoeml blindn_), dysarthria, fo1!owad by ... veu HlA and DC:C8IIjonaLly nausea a nd vomiting". Fami ly his_ tory of mrrrBln~ is present in 86%.
2.2.2.
Post LP (myelogram) HlA
AKA "poatapinai headach e" or "spinal helldath~·. MaY·IIIIG folio .... proc:edure. other than l P/myelogmm, such 811 dunil opt!ninc (..." pa/le 308). Can alro o.....,ur with 5pontane· O1.Ir intratrauial hypotension i!eep"'¥t 178).
CllDi ca1 rea tures Important distinct ive cho.ract.eris t lc: HII'. (Ittt.ITS .. hen patient is e~, and ia corn plelely or partially (but eignifitantly) reliev..:! .. hen recumbent. May be lll!wcisted .... ith nsusea. vomi ting. diuiness, or v;"'uaJ dioturbances. Tim e CO~: MO$t pD6t_LP headachea IPLPllA) hav"-,, delayed OM~1 2" ....8 hl"l after the LP, and although they may octU . ....eek~ poet-LP. mll&tal~o develop within 3 day". The duraliao ofPLPHA varies, with a mean of 4 dBY"''', IUId r eports of dumtion of mon t hs" IlIId even> 1 yeB. '". Pat b o pbY!lio logy Thought to be due \.0 continued CS F leakage IhrQugb the hole in the duro " . which redUCN the CS F ' cuahion" of the brain, 10 the uprigh t po.9i ~iM\. the pull of gravity on II ... bmi n produces t m clicmon lh~ blood veMelaQ nd any .trucluu s tetherinR the brlli n to t he pain-sensitive dura. CSf' may sonleti mu be.delllon~trabl~ in the tl1id.w:a.I.pacc.
Ep id e mi o lo gy ro llowiog LP Reported lnddenct: " ng" is2-40%(typically ~ 2~). bigheraftudiagnoatic LP than for epidural anuthuia". For variobles in LP tl1at impa~t upon the ri$k ofPLPHA, Sef' paMe.617
T REATMENT FOR tvA FOLLOWING LP
Ini(ial 1, 2. 3. 4_ 5. 6.
'cOIlservative" me:saurea include!'.at in bOO for at leat1l24 hI"!! hydm tion (PO or [V) analgeuCii for lilA ~il!:ht abdominal binder de,oxyrortiaone acetate 5 mg 1M q 8 brs" colTeine lodiwn benzoate 500 mg in 2 ~c IV Q B hr. up to:l d mllll (70% of potienlS had relief with 1 or 2 iqj.,dlo~" 7, high-doae .teroi,a, report ofsuccK8ln 8 case orinll1lUao.ial hypoWlls;on auoeis led witl1 "ponllln&OU8 Blit ventricles tapering down from a MArting d05l! "rd",,amethasone 2{j mgldayll 8. blood patch if refractory 1_ below)
E PIDURAL BLOOD PATCH
For refractory PQSt-lumb.o.r punctu r~ or poll-myelogram HlA. W-oru in one a pplica_ tion in over ~ of caul. mil)' ~ repea\.ed ;fine lTective" . Theoret.ital ri8q: inflKlioo, caude equi na com prcasion, failure \.0 relieve HII'.. Tec hn iq u e ACCl!uing epidurall1pace (one ofuvera! techniquu): prDC:etId as rouline LP. When ligame4Ls a re Itavened. and lleedJe tip iB neating apiDel cane!. style!; is renwved. Then. either plllC.l drop of st.erile u line in hub (hanging drop tEf acetylch ollne.n Ihl! b. , a! ganIl:1i,.
I DIOPATHIC PAAALVSIS A01TANS
CI .. ¥icn l Patkin!.On'. rlilellae. AKA ! lIaking pa l.,..
Table 2-1 Cla sele triad 01
C lin ica l
Parktnlon'l dlHalll
Aff«tA: -l'*'ofA,mt n canl>apliO)'f'I". M.le:remaJ.
I':
- :"= , ~1'**ludobulbar palsy (mask·like facies with marked dysarthria and dysphagia, hy· peractive jaw jerk, emotional incontinence usually mild) 3. axial dystonia (especially of neck and upper trunk) N!sociated finding" sutCQrtical dementia (inconstant), motor findings of pyramidal, extrapyram id al and cerebellar systems. Average age of onset: 60 yrt!. Males comprise 60'1>. Response to antl.parkinson drugs i, u8ually very short lived. Averagesu ..... ,val after diagnosis: 6:1 yrs . Differelltiating from Parkinson·s disease (IPA): Patienu with PSNP h8ve a pseudo-parkinsonism. They have mask facies, but do not walk bent forward (they walk ered), and they do not have a tremor. They tend to rail backwards. Course I. early: A. mDny fall., due to dysequilibrium + downgaze palsy (can't !lei! noor) B. eye findings may be normal initially, subsequently may develop difficulty looking down (t"Specially to command, l~ to following), calories have nor· mal tonic component but al»ent nystagmus (cortical component) C. alurred spe~h D. personality ch!lllges E. difficulty eating: due to pseudobulbar palsy + ;nabili~ to look down at food on plate
..
2.3. ParkinsoniBUI
NEUROLOGY
2.
I .~
It. eye. fIXed ~utTaUy too response to oxulocepbal ic. or otulovullbular1); OIl1.11 •• ImmotiU ty ill due Lo f. (Hltlll lobe laio n.
8
"""k atiffe ... in "",ten,lon l. etnK'OIllI)
SURG ICAL TREATMENT FOR P AAKINSON'S DISEASE BerGn Ih .. introdudion or t..dGp. in til- I. te 19&0'., .te' f'Otactic tblilemo!.Omy ... n widely used fGr Park ....... diteaae. Tb .. loaItlon ullimll.ely targelN for lel io",;ng"'u the .... nll"Ol.III,...1 o"deu •. Th .. p.-lu...... Q1'ked better for Nlifl.vinr the t~mor tbflll for the bradyllinUla. however It "'1\1 the lillie. lympLom tbllt we.. mOll;t.diubliog. Thi s ~ tedu ", eanoot be done bihllllrfl Uy ... ilhoul .igni licunl rislJ to 'PH\:h function The p.ocedun. f.1I oUt GfflYOr wh.n more effective drop ~lIme IIvIIU.b!e" See SW-I,",lll'WOlm,M 0( f>Qr1ti1lWll', di __ 0"' pap 365 !'or rurther lofonnation.
2.4.
Multiple sclerosis
A derllYlLlin,ti.Q( dlll,",_ (Iffecti", "nly while malto.r) IIfth. al1!brom . optIC neTVl!II, . nd s ploaJ mrd (espec:i.lI". oorti«llp!nal lUlU .nd the pOIIl@rior 'JOJumru). Ptod"Cftmultiple pl.que. of VII no ... ..,;n dilfu ... loxe~1\II in tl!ll CNS. eapec:illily in th" poi"riv~ntri.,. UID r white maUer. LHiolalniti,lIy eYOQ a", innIR'ma~ response with monoqteo and ]ympl»cyllc pl:nvucul., ""mnfi;. bllt with 'g~ fedle down to el ial SClil ri.
EPIDEMIOLOGY Utili] IIp.of onMt ; 10·59 yell,..., " 'i lh lb. gTellli'!lt peAk bet.... een agell20- ~O y,",,~. Ma le 10 femal~ I'Itio: 1.J§,1. Prevalence v.nOM __.th latitude, e.nd is < 1 peer 100,000 rtf:1I r iheequator, lind if - 3Q80 per 100,000 la Ihe north*m U.s. and Canada.
CUNICAL Cauj;eS exac:erb.a tioRa.n d ~rnis T.ble 2-2 Cl1nk:aL MS l ions in variou$locationJ In the eNS (diuemioati~ aDd liIDfl ). Com· mon Iyrnploms! visual utu rbantu (diplopill, bl"rring, fielcl :U1ll1I• .,.,1.0. mil. ,pastic par.pa res;lI. and bJ,ddp... distllrbllntK. N01'llf:11t1l1turfl for tbe. tima 1>'1,,'$11 o( MS if. . h ...... n i", 1'bbh: 1'. 2"1. Re1~P8iDg-rem'lting MS i, the IOOiit com· mon pattem at OMet,.nd hal 1M beat I'f!Sponlie to therapy, b,,~> 5~ of cues ev"ntually become semn~al')' pt'Ction ofthe CNS. Condl· tiou. that mllY t losal)l aunic MS di nio:slly and Gn d iagnoltic to.lt;1\I include.; I !\C1,ta d.i~rnin .t.ed ensing loiS I"I'IU$I ","I crilowing o/IgocIc>nalt>an and can demOlI9tl'at& dissemination of Ie· sions in time and space. Recommended" brain MRI criteria for diagu08ing MS are . hown ill Table 2·4"'n . Le5ions are normally" 3 rum diameter". M~R[ shows multiple white matter abnormaliti8$ in 80% of patients with MS (compared to 2:9% for CT)'"· ... LesionJI are high signal 011 T2WI, and acute lesions tend to enhance with gadolil!.ium mOre than old Iesion a do. Periventricular lesions may blend in with the tignal from CSF in the ventricles on T2WI. these leaioll8 are shown to better advantege On proton density images as higher intensity than CSF. Spinal ~ming hOI cri1ical. buI 3 mont/I$ b cord lesions normally show little or no .welling, should be:it 3 mm but < 2: vertebral SfI. The etiology of ALS illtill not known with certainty. CLINICAL
Involvement il ofvoluotary mu..:I.., eparing tile volunury ~ 1Il,,"lee and urinary ephincter. Classically, presents initially with weaueIII and auoplly of the hands (lower motor ncuron) witll l PIIllicity and lIyperunexia of 1M lower extremiti"" (upper motor neuron). However, LEI may be hypon!fleltic if the Io"'e r motor neuron delicita predominate. Dyu.rthria and dysphagia a ... cau..sed by a combination of upp"r and lower motor neuron pathology. Too,gue atroplly and fasciculations may occur. Although eornitive deficits an! generally considered to be abosent m AlS. in aetual· ity 1·2% ofca_ a ... auoc:ialed with dementia. and cognitive changes may occasioo.ally predllte tha ul ual fea t ura of ALS" . DIffERENTIAL DIAGNOSIS
It is important for the neurosu'¥eOOkott Of" rnay be non-eJ.ittent by - 18 month," ". At tile tima of thi. writin" the d"., it aVlilabLe onLy for prema:rkl!tln. trials. and cannot be procured comTOCTcially. Much of care i. di~ted tow.rd, minimiain, diubility: I. ..pintion may be treat$;\ witll 2.5. Amyotrophic iat.e!'al lCleroai,
NEUROLOGY
A. Iroch"""IllIllY B. gtostroslllllll' tube III allow cootinued fceding C. voce] eord ill,jedion wit h "(!f1on 2, a paslidty thaL occun wh(!11 upper Illntor neuron defrcit.s predomin~~ may ~ trea~d (usually With ! hort.· UO'ed response ) with: A. bsdafen.: alM! may re~eve t.becommonly oc:currlng cramp. (,el': fX18t 368)
B
dia~epam
PROGNOSIS ~IOlIt patients die within 6 years oranset (median Bu,.".i"al, 3-4 yrsl. "h ... e wIth prominent or(lpharynlfulsymptol1t$ may haO'e 8 . harter lif....span usually due ta compJi. ClI.tio"" q{ IIspinl.lion .
2.6.
Guillain-Barre syndrome
t Key features •
acule onselofpenphet a! neuropathy with pf'Oll"l!Sl ive .... uscle weakne.u (more BeVe~ Drol{imal!~ with arefleria, Teaches mllll'lmum o"e r 3 dllYI t.o 3 waeu cranIal neuropal : 8 180 conunotl, may include fadal diplegia, ophthalrnopltlf;ia little ar nose.uory invo!veml'n t (paru thesiu lire not uncommon) onset often 3 dayo.·6 weeki fo UowI,IlB" "ira! URI. Immunillltion, ar s urgery p&thol~: foeal Rgnlental dem!eli nlltion with endoneuria1 monocyl.ic infll lrat.l! elevated CSF protein wil.baul p eocytosia (albuminocytalogie d,s6Ociation)
AKA BCUU! id iopal.blc pol)'TlIdiculoneuritiB. "he IIlQIlt common acquired demyeli nat.ing neull>\>athy. lncidenoe is _ VIOO,OOO."he lifetime risk forar.y one indlvidulIl gettin!! Guillain·Barri &yndro"" (GBS) is _ VI,GOO. Mild cases of CBS m8y pre&ent only with alllJ, by S wI\! in 80%, and by 4 wu in ,,9Q"!ssioll without T«Overy 8. 3phin~r dys function (usually spared!: a,e. bladder paralyalo!
>"
NEURO/..OGY
2.6. Guillain.Bam; syndfOlD8
"
f.
eNS involvl!ment (controversial): e., . ataxia. dy!l8.lhria, Babin .k.i aigna B. CSF !indings: a lb" m ,nocytolog"i 2 mOl. CIDP produces progrellllive, symroetric:al, proximal &. diua l weaknes.s, depre.. ion of muscle atretch noflex .... and ,·.riable soellSOry laq. Cranial ne""es are usually spared (faci al muades may be involved). Balance diffi· cullin are common. NHd for respiraWry IUpport i. rare. I'l!ak incidence: age 40-60 )'TI. ElectrodiagnOltjCl lllId nerve biopsy findings an!' indicative of dem~lination. CS t' findings .r. aimilar w CBS (_ obow). Most respond to immun""uppnuive therapy (npeciaUy predniliOlone &: plasmaphereaia) but relapaea arecommDn. Refractorycuea may be Ina~ with IV gamma. globulin , cyd"" porin ." ", tolal body lymphoid irradiation or inu.rferon-." The MiIle, · t ·ilher v.nant of CBS indoo... ataxia. an!nexia and ophthalmoplegia. TREATM£ftIT' LoununoclobuliNi m.ybe helpfUl. lnKIWc.....,ea rlyplaamaph ..... is haa\.eM!.he recovery and reduaoa the ....id""l defici L Its role in mild c:aaea Is l,In«rtain. S\.erOids . re not. helpful". Mecba\1.ical venlilationlJ.>d meuu,"" to preven t aa piration , ... used .. a ppropriate , l n e. 1IQ of raci. 1 dipleril , the eyn muSl be protected from eXl""'ure keratitis.
CNTCOME Recovery may not be complete !'or H veMl I montlu:. 35'" of untre,led patienla have .... id""l wealtn .... and atrophy. Reairreneeor CBS .lUr achievina: maximal recovery DCronl in . 2...
2.6. Cuillain·a. ... . yndrome
NEUROLOGY
2.7.
Myelitis
AKA acute transverse myelitis (ATM). The terminology is confusing: myelitis over· laps with "myelopathy". Both are pathologic conditions of the spinal eord. Myelitis indicot 1.
accepted etiologies include: infectious and post-infectious A , primary infectious myelitis I. viral: poliomyelitis, myelitis with viral encephalomyelitis, herpes ~OIIter • • abie! properly ro th. ,
34 patients with ATM ": age of On· set ra nged 15-55 yrs, with 66'l1>occurTing in 3rd 8nd 4th d"",ade. 12 patienUl (35%) had s
Nt'UROLOGY
2.7. Myelitis
"
"iral ·like prodrome. Pruent;ng symptoms are shown in Tabk 2·1, with otherpresenting . ymptoma of unspecified frequency including"': fev .. r and rash . Pr elHlDling leve l The level . at pretentation in 62 pationu with ATM aro shown in Table 2·8". The thoradc le"el is the molt common ten · sory le~ l . ATM i. rarely the pre.enting.ymptom of MS I_ 306'11 of patiMtI with AT M de"elop MS).
Table 2-8 Level of
MnSOl'Y deficit
PROGRESSION Pro8l'usion i. usually rapid, with 66% reaching maximal deficit by 24 hn, however the interval between fint . ymptom lind mlllUmal deficit "aries from 2 hn·14 days-. Finding. It th e time of maximal deficit a re .hown in rabk 2·9.
EVALUATION
MY.florram CT &..M.SI: nO characteri.· tic finding. One pape r reparts 2 patients with fusiform cord enlargement". High ...... olution MRI with thin CUtl may be able to demonstrate al'8S ofinvol~ment within the cord . Patient.hould hIve imagi ng to IUO compressive lesion. C5.E: nonna l d ur ing acute phase in 38'11 ofLPs. &roaioder 162%) had elevated protein (usually:> 40 mg%) or ploocytOS i$ (Iym. phocyte$. PMNs, or both ) or both.
Tabl.2·9 Symptom' " time 01 maJlk mald.lell (62 patieotl..-i l h ATM"')
,
EVALUATION SCHEME
10 a patient developing acute myelopa· thy/paraplegia. espc(:ia lly when ATM is considered likely. the fint uslof choice is an emergency MRL Iffl()t readily available, a royelogTam (with CT to foI!ow ) direc:ted at the region of illesensory If!>'e l is perfonned ICSF may be sent in this ci rcumstan« once bloek is ruled Dull.
T REATME NT
Suggested efficacy ofhigh..:lose s teroid treatmen t in 1 patient .... ith ATMG (methyl· prednisolone 250 mg IV q 6 h,.. X 24 h ,.. . 125 Ing IV q 6 h,.. .. 24 h,.., 125 mg rv q 12 h1'$ I. 48 hrs. then 30 mg PO q 6 hno. Ulperilld gradually. Rc>&imen sllould probably be individ· ua liud baaed On ..... ponsel.
P ROGNOSIS
In a $eries of34 ATM palien U willi" 5 yrs follow·up (FlU)"": 9 pal iellUl 126%' had good rltCOYery (amb",1ale well mild urinary symp&o ....., minimal $eraory and UMN l ign l ): 9 (26'110) lIad fai r recov ....y (functional g. il with $Orne degM!! of $p8sticily. urioary u.rgency , obvioua .. ntory '[1"5, par' pereaill; 11 (aN) poor (paraplecic. a btent sphiDcter fIOIItro\); 5 (15%) died wilhi n 4 ..- or illn ...... 18 palientl (62':1. of survivorsl becama am· bulatory (in th_ cues,.1l c:ould walk with support by 3-6 ..-). In a Hri " 01'59 pllienU-{FIU period unl pl!('ified 1: 22 (37'11) ....d ,ood recovery; 14 (24") poor; 3 died in K U\.a stage (ru pinotoTy inl ufficiency in 2, Hpli5 in U. R«overy oc· curred between 4 weeki and 3 _ after on$et IfI() improvement oc:curred .fter 3 rnos).
2.8.
Neurosarcoidosis
SI.rcoidosi, i • • gran",lon,atou. di _ that il ulu,Uy I y, terni.:, and may indude the CNS (_ lied neuroaan;:oidotl ia). Only 39& or cue. have CNS fiodinp without IY.' temic: manifut.alion. ... The ('8uHorthe diM_ i, ",nknow n. An infeo:tiou l Igeol il pot. NEUROLOGY
CNS lall:oid().9ia primanly InV()lvl!Ii the lep~omeningea, however parenchyma l invpaion often occu.rtI. Adhesive a rachnoiditia wilb nooule form.t.iOD may al-o oco.:u r llloduJ~ have" predil~iOll rOT the pc., nerior £(658). Oiffuse. meningitis ~r roellmgoeneephalitis may occur, and may be 1II011! pronounced al the balll.! oft bl! brain {bR8IIl meningitia l Bnd in the aubependymal region of the third ventricle (including the hypotbalamwo ). Cons tan t min"Oll~pic featW"1!5 of neurO$&TCoidNi. in clude n~a;,eati ng JlTllllulomilS with Iymph lion in l,rt"Oid08i.t! il nat limited to Wle region imroed.i·
sis 01neurosareoldosla
au.ly ~uN"Ound;n G bl~ V_18 "" It;8 in CA, .... he ro ""len6;v8 disruption Qrrne vueel wall mayo"'_~......,-...ybt~
2.9.1 .
Giant cell arteritis (GCA)
f
TKey fuw ....
formetll D~" ~fured ta al l.emporal arteritis d ' romc vallCUht it!oflac]{e and ~um celibtr v_la, primlri l, LDvolvlnSeTl' nill b",ncl;>ea of Ole Imnft trisioS from the .0000e...::h age,. 50 yean: atrKU WOme 80 IIIm1hr with .bcJo,·e CtUII Polymyalgia rheumatlca
2.9.2.
PolymyaJgia Theumat;c:a (J' MR) and giant ",,\I arteritio (GeN i_fXJR£1i8) may be diffe rUlj pointl! on /I condnuum orche ume disease.
Epidemiolo gy)' Both GCA & PMR occur in peopl .. ~ 50 years old . The incidence increaaes with age and peMa betw~n 70-80 yeat8 and is higher a~ higher latilud"".
Polymyalgia rb e umati cs (PMR) " an inflammatory cond ition afunknow n etiology clinical rna",clenst\a A. aching and morning stiff/ieM in the c"rvical f' IlDOnth. The pain llstUllly increases with movement I . sh oulder pIl;n: present in 7 Na .... obttruetion and e,ulting ... the ulual initial findill8l1. Arthralgia (nOt true ill present in > 50'1&. Neurologic involvement .... uaUy eon$isU of cl"lUlial oerve d,..runetion ( UIIUIUy II, 111, IV, &. VI; 1_ often V, VlI, &. VI II ; and leal t commonly IX. X, XI, &. Xli) and peripheral neuropathiU, wi t h diabetn illlipi du'(OOX.lionally pt«edingothet8)'ll1ptom. by up to 9 montha). Foealle.ionl of th e Drain lind spinal «Iro occur In. frequently.
.rthrit~)
Differenti.l diagnosis indudu! "Ia tbal m idline I/Tanu lo ma" (may be similar or identical to Jlulosis) may evolve into lymphoma. ~boy cauu r... lminant loca l dutrud;on ofth. nasal tiu ue. Dift'erenlilltion il crucial II thill cODdilion ;1 treated by radiation; one l hould avoid immune.uppruaion (a.,. eyciophOllph,mide). Problblydoet: not involve troe granulom... Reoal and tTacb •• 1 involvement do nOl. occur fungal dille..e: SpOrothriz fChfncitii &. Cocci dioidet may ca\IM identicallyn. drome other vasculitidu: up«i~lIy Ch ul'1-Stnu.. I yndrome (uthma and peripheral eosinophi lia usua lly _ n), and PAN (,,"anulomas ulvally IACki.ng)
LYMPHOMATOID GRA NULOt.'-"TOSIS
Rare; alfecu mainly the IUlISI, akin (erythemalOUs maeulu Or .. ndurat.ed plaequet in 40%) and nervOuS Iy,tem (CNS in 20 .... p"'ripberal neuropathiell in 15'1» , Si nuee" lymph nodn, Ind spleen are usually lpa red.
B EH(:ET'S SVNOROME
Relap!;ingoculaf lesion. and recu!l'entoraJ I nd genital ulcer., with occ.a.ional . k.in les;ons, thrombophlebitis, and arthriti' .., H/A ooeur in,. 50". Neurologic involvement includes peeudoturnor, cerebellar ataxia, paraplegia, aeilurea, and dural linus thrombo.i•. Only 5% have neurologic aymptoma as the presenting c:omplainL 86% bave CSP pleocyto&s and protein elevation . Cerebral Ingio(raphy is ul uaUy nonnal. CT may show focal areal of enhancing low deMity. Steroi: III bioJMY materi.l.hould be cultured. Brain parenchyma biapey ;nf~uently , how. vl.ICuliti .. LeptOmt ninpal bioJMY invariably involvement.
.mow.
"
2.9. Vllculiti. and vllculopathy
NEUROLOGY
H YPERSENSITIVITY VASCULITIS Neurol~c involv.ment if not I prominent fe~tl.Ire ofthis gl'j)up of"8MUlitidl!$, whIch include: drug induU:d enel'l1c V.~uli li. cul.neo .... V8KUlill. HrulTl siwOII': rna)' - ancep/llllopolh)" Mbu ...., coma, p!!nphe'!ll l>~urol"'Lhy .nd br8th.ial pluopa tb, H~noch-&:h6nlein purpura
D Rua INDUCED VI, SCULms A numlHlrord",i' !l.""".lIOCieied with th"d~elopment of~".ebTl.I vlUlCUlit ia. ,odude metfulfllphellolfline. (".peed"), tOeIIine.{fl'llok vI\SCuliti. ClCCU ..... but is nlJ1!). he ..
n,ne
oln and ephedrine.
2.9.4.
Fibromuscular dysplasia
._i.ted
A vnculopa thy (oogiop.thylatrectilll pnm anly brllndMs o(!.he eoTta, wltll reo.1 nrtery involvement ill S5'i11 of caH. (thl. motl IlOmll"llln .Itel.nd eIImmonly wIth hypertM5101>_no" di_1e hI' an i.otldenee of ~ 1'11> •• nd .esulls in mui t ifoaolll ...... rial cO"'l"lrietions ,"d i,rne"""nirlc regiol'l5 of.oeu~,n.l dilatallon, The leical illt.emal carotid (priml rily nenrCI_2), with fibromu!iC1.ll&.r dyspluill iFMD) 'Ppe.rinlon l \t.ofearotid llIriograml, making FMD the &eCOnd molt ~mmon causeofexLr&crani.1 clrol.id 'UI~. Bllaleral cervicnl lCA involvemrnl O«UI'II il> _ ~ or~. SKof pal"nla wi lh Uon)ljd Ji'MD haye renal FMO, Patienls wilb FMO ""ye an Inc:rnted rUk d ontu"ranill I"'U.,.."" lind neophu;ms, ami ~ probably II higher ri&k of tllrotill di56edion
ETIOlOG Y The ItCtual etlolo1O' remains unknown, alt.hout:h«lncenital df~ of the media (muscula r layer) and ir.lem&i ~a.tk layer oftbe sr\eria bar bee.;> identified which mIoy prediSpoII8 the arteries to injwy from otlll!T"oCe • anticoaglliantl controversl.l. ,tneBUy di$CO\l.~ged An a,,",nym enr ClTebrlll .... ulO$Omyl Dominant Artuiapll1hy with Subeorticallnf· a r~.nd Lomkoe,""'phalnpath,.. . .... f.mililll d;~ with onnt in ea r;y ad ... 1thood (meDn .ge lit omet: 45 '" II )'nil, mapped to chroflllMOfDt 19. Clinical and neuroradiglogic fca IU ' " IN!limilarlOlhoIe aeen wilh mullipielubcorticul mro~U from HTN, ucepl t here II no eviden~ofH1'N. The vlISCUloplltlly i5 disti nct "om thot Ieiln in lipO/lyal'nasis. ar1.eri000erosi••nd amyloid lI.IIgiopal hy. and cause.s thiek. ning of the ~ia of lepl.ol!leniniNl ""d penOI1lI,,,,, anerie, m-.uring 11)0.400 "," ," dia.nw!Li!r. Clinical inYillvell>ent: rerurnontsubmnkat io rlln:~ 184~), prawns;v"' .... u"pW'5e dtmentia (3 1"), l1IigTaine "'ito'! li' UI"lI (2'~), a nd depress.on {\tOOIi-). AlIl}'mptomaU( Dnd IS'l\ofUympt.omalie patlentil had prommenuu bcort.ieal whit..·matterand basal ,anlll ill hypennten$it;u on 1'2",1 M Rl. Trutment: Cownadinf>is ua.ed byaome.
2.10.
Vascular dysauloregulalory encephalopalhy
This Hnil pl, ""'tl p!I"'" ..... """'''''' A ""ubi< "...!y. Htad.oo:hO 19: 6)11-1. 1m. 1..>II«JW.B,.ndlC8,,... lumb" PO"""" . (.0",," 301): ",. '!9.\ lles ~ • R. i".oc...;'1 bypOltn>_ ...... ,Ii, "< m. ",,,,,,,, .. oIhrlil,,*,', di111,1. E.I...wIOA"' : """i • Fi ... 'o( ,..., pano. N Ell" J Mtod JJ9: 11)004.'1. l'11ll. K" ...... o M ():~ ..... _katp.ol.,·IO ).. ... Iote,. Ae .. Nmtol S i< ~ 1""'1'" of i.. ""my.II ... '"I kleoll) 1... 10dMI1 .... "",,"1 W .C ......."'" O. IJ"m.1 W•• ,,,/, T.......... '" <Jo .... k ,.n ... __ .,.por ... A, J "'.uo .. 1'1 ..... ....,.. "'~1.1'l'6O' It..I.;, 199Ii Gotwo K C. Ropp« A H, CI'''' e O• .,.I.: T... ,_ mo.' or c~,.",;. ;,,11 . ...... ''''' .
" "
lIIHp!i..:l IrtII "'
iIo!,...-., .....Il}''''''''''''''''-,.
Gu,tloi... 6ar.t.,ncIrDm&. IAJouI II I. lU.-?O.
I'I'1J .
" ". ~
~
" M,
..
".
k'",,'" I c. OJ'''' M L! M1IogJ. 6>1:" ..01. 8 _ I~", ~J. (ed,.). Hupo, IaoJ"'kl&II " " " " n
Sr. area 17: primary vi .ual Clp oj venll.c:le5lo $lWll lanclfTWksAI:It>«Iv~liont : jF E ~ OOlall»'n. B E t)IIU1ion (Red.awn lrom -Inlroduelion 10 BaoiC Neurology'". by Ha.ry O. PallOn. JoM W. Sun NEUROSURGERY
3. NeutOOnat()my and physiology
"gu,e 3-8 Sdlomillc ($11\Qf1l/T1 01 ~I COld a_1aI su~ty (Modi!ief ayl\.e1llS have been deacribed to addresses thli inlure • M3·4 : distal braoch .... • 11015: t..nn;nal branch post..rior cerebral (PC,I t ) (several nomenclature schem .... ""i8:"''' ~ • PI : PCA from thllQrigin l.O post..rior CQmmuni~ating artery (AKA melM!n · cephalic, precommlUlicating. circular, peduncular, ba,,;lar ... ). 1'be long lind .. hon circumflex and tbalamoperforating atteri .... arise. frem PI • P2; PCA from origin of p-comm to the origin of inferior toomporal arterieol (AKA ambient, potitoommWlieating, perime!lencephaUcJ, P2 traverses the nmbient ciBt..rn. Hippocampal, antoorior t..mporal, peduJlcular perforating and medial po.tenor choroidal arterie!'rise from P2 • P3; PCA frem the origin of the inferior temporal branches to the origin of the t..nninal brlU'che" (AKA quadrigeminal ~gmentl. P3 travenlu the quadrigeminal d stem • P4: ~egment BitU the origin oflhe parieto-oecipital and ca lcarine Qrteries. indude~ the cortical branch"" of the PCA
•
•
oplicn. (er N. II)
. I
FIgure '·10 C"tlf-ol WiUis viewed Irom In lronl of nne below 11>8 briM Key po in t: the anterioroe~ral anenes PI'~9 over the supe.rior ," urface of the optic chi asm.
"
3. NeuroanatQmy "lid
phy~jololO'
NEUROSURGERY
ANTERIOR CIRCULATION
INTERNAL CAR OTID (ICA)
Acutely occluding one carotid arr.ecry will eause a stroke in 15·'20'ltofpeople.
Segm e n ts of th e lCA and its branc hes "Caroti d sipho n": begiO!l at the posterior oond of the CaVemoUli ICA. and enda at the ICA bifurcation (thus incorporating the cavernous. ophthalmic and communicating segmentsl" . C I (ce rvic aJ): begins Dt carotid bifurcation. Travels in oarotid sheath with IN and vagus nerve. encircled with postganglionicaympathetic nerves (PGSN). Lies posterior & medial to the external carotid. Ends wheTe it enters carotid canal of ~trou8 bone. No branch~s C2 (petroua): still surrounded by PGSNa. Ends at the posterior edge of the fon· men laoerum (f.Lac) (inferomedial to lheedgeofthe Oso.serian ganglion io Meck. el's cave ). Three legments: A. verticalaegment: ICA ascends then bends as the .. B, posterio;>r loop: anterior tocochlea, bends antem.medially becoming th e ... C. horizontal segment: deep and medial \0 greater and lesser superficial ~tros.ol nerv'ea, anterior \0 tympanic membr/lne (TM ) ClI (Iace rum) : the ICA passes over (but not t hrough) the f·Lac fonning the lateralloop. Ascends in the canalicular portion of the f·Lac tojuxtaBeliar position, piercing the dW'8 as it pasae8 the petrolingualligament \0 become the eavemo\UI segment. Branc h.... (usually not visible angiogrnphicallyl: A carotiwtyropanic (inconsistentl - tympanic cavit y B. pterygoid (vidian) branch: passes through foramen laceTUm, pruent in 30%, may wotinue a~ artery of pte rygoid canal C4 (cavernou s); covaNd by vascular memb.-ane linin, sinus, s!illsurrounded by PGSNs . Passu anteriorly th~n supe~mediaJly, bellOI!leriorly (mediall{){)p ofiCAJ. travelshori:tOlltally, and bends antenorly(part of an len o r I{){)p oflCA) to anterior clinoid process. Ends at the pro~mol dural ring (incomplete encircles ICA). Many branchea, main ones indude: A, meningohypophyseal trunk (1argeat & most proximal) 1. A. "ftentorium (AKA arte ry of Bernasconi & Cas6lnari) 2. dOnll!.l meningeal a. 3. inferior hypophyseal s. (- poIItenor lobe of pituitary): occlusion causes pit uitary infarcts in post-partum Sheehan's necrosis, however , OJ is rare because the stalk is spared . B. anterior meningeal a. C. a, to inferior portion of cavernous sinus (present in ~) D. cap$ular aa . of'McConnell (in 30%): supply the capsule of the pituitary" C6 (clinoid ): eods at the distal dural ring (wmpletel)' encirdes ICA) where the ICA becomes intradural C6 (ophtha lmic): begins at distal dural ring, endsjuat proximal to p.comm A. ophthalmic a.: the origin from the ICA is distal to me cavernous sinus in 89%(intracavemous in 8%, absent in 3%"). Pssses through the optic canal into the orbiL Has s characteristic bayonet-like ~nk· 00 lateral angi~ iTam. B. superior hypophyseal a. branches _ anlerior lobe of pi t uitary & stalk (1st branoh ofsupraoiiooid ICA) C. posterior commWlicating a. (p-comm ) I. few anterior thaJaOloperlorat(! .... (- opt ic tfllet, chiasm & po!Iwrior bypothalamus): see Plnttrior t ireulation below D. a.n te rlor c ho roldal a r tery": takeoff2-4 mm distal top-wmm - (variable) portion of optic t r&ct, medial globus pallidus, g~nu of internal capsule a C) (in 50%1, inferior half ofJ>Ol!lerior limb of IC, uncus, retrolenticular fibers (optic rad iation), lateral geniculate i»dy (seepage 778 for occlusion syndromes) I , plexal segment: enters supracomual reteU oflemporal horn, - only this portion of choroid plexus 2, cisternal segment: passe! through CTUtal ciltern C 7 (eommun;ea t ing): begi... just prox.iJnal to p-tomm or igin, tTavels bet"""",n Cr. N, II & Ill. wnninstesjust below anterior perforated l ubstance where it bi. furcates into the ACA & MCA
NEUROSURGERY
3. Neuroonatomy and physiology
"
ANTER IOR CEREBRAL (ACA) r"un between Cr. N II . nd ante rior perfol'llurd 5ubostMce. Su 1'16",..5·2. pap 132 for angiornm lind bran~bBlo. MIDDLE CEREBRAL (MC A)
$eU"lIurt 6 ·3, pag~ 132 fllr a"llogram and branch"'. PO STER IOR CIRCULATION
VeRTEBRAL ARTERY (VAl 'The VA i8 t he first and usually the l.rGut brllncil ortlle.ubel.'·;"n a rt.l!1")'. V.rianl: the len VA Bri., oJT the B()I'tje arch in . ... Oi.mft-olr. 3 101)'1. M"e blood flow _ ISO mllm,n. Thlliell VA i, dominant in 6nti.te, Ht JXl6t 136); I. "ytotOld ,,: BBB is closed. the ... f,,", no protein utrav ..... tilln. then!fure nG en·
cr
hanoement on or MRI . Cell ••well then .brink. Seer. e .•. in h u d i.,jury 2. v" lIOg .. ni,,: BBB disrupted . Prol.ein (serum) INk. out of v..cular IYttern. end the ... fon may .. nhllJ\Ceoo imagin,. Extra"",llular'paee fECS) upand •• Ce ll •• re stable. Responds to cortioosl.e...,id. (..... dex.met ........ nel. Seen ' " .lUrTOund inll metastatic brain tUmGr 3. i,..,bemic : . combination of the.~ BBB c\oted initia lly, but th.n may open. ECS shrinkll then upandl. Fluid .x tra vallal.ellate. May aUN d.. l.yed del.e'ioration following inlra"", ... bral hernorrh"'. (_ ~ 8.S$)
3.7.2.
Regional brain syndromes
This section IoervU to brieRy describe typiaol.ynd .... me .nociated with Ieoion. in variou8 areu of the braIn. Unlu. otherwiMl not.ed, Inionl conlidered Ire dtJ!oli:m:... I, r.on t allobo!! A, unilaterlll il\iury: l , may produ"", few clinic.1 findi .... ,x"'pt with very I.rge Inioou 2. bilaur.1 or I..ge unilal.e.,1 lesionl: apathy, abulia 3. the frontal.ye field (fGr contral8l.er.1 gazel dlocated in the poIIl.erio. frontal lobe (8 • • a. ea 8, , hown I I th u triped area in Fitu", 3·J. page 68). Deetructive le,ions impair gaze to thecont •• lal.eral aide (patient lookl .llU!Llllh the .lde of the lu iGn), whereas irritative lesions (i.e. lei · lurn) caule the center to activate, producing contralateral gau (pa. tient looltll ~ )'!'lll. from the side Gfthe luion) . Also IIU pagt 584 B. bi lateral injury: may produce apat hy, abul ia C. Glfactory fioova rtgiGn : may produce Foster· Kennedy syndrome (see bf,low) 0 , ~fronta lobet control "exec utive runction": planning, prioriti:;ng. orga· niling tholl4lhta, suppressing impul8el, understanding the OOflsequencet of dedsionl 2, parietal lobe: major featuretl Cst. pog# 87 for deta il9} A , either ,Ide : conical aenlo,y ' yndrome, sensory extinction, contralate.ral homonymous hemianopia . contralateral neglect B. donei,,"n! parietal lobe luion (len in rnSt): language disorders (aphasilll), eerttmann', sy ndrome (_ M t 87). bilateral astereognosis C. """.domin,"" parietal lobe lei''''''' topographie raemory Iosa, anosogTlOliia and dressing apra~i. 3. occipiullobc: homonymGul hemianopsia 4, ""'rebellum A. leaiGni of the cerebellar Mmi.pM", cause atuie In the jpsilateuJ l'robs B. letion. o{ the cerebellar vennis cau"" truncal aluia 5. brainstem: usually produces a mixture o{cranial nerve deficilll and long tract findings (1ft Mu.w fo.- some specific brainsl.em syndromes) 8. pineal region A. p.rinaud'. ,yndl"OV'le; 8ft pap 86 FOSTER-KENNEOY SYtJQROME Ul ually from olfactory groove CIt media l third aphenGid wif\i: tumo , (ulua)ly menin_ gioma). Now nlre due to earlier detection by CT KaD. ClaAic triad : 1. ipsil.teral anosmia 2. ~ centra l scotoma {wi th optic I.1aUili:t: due pressure on optic nerve} 3. contralatergl papilledema (from elevated lCP)
O«lIIiooally ipsilateru l proptosis will also OCCur due too.bi",l invuion of tumor.
3.7.2.1 .
Brain stem and related syndromes
W EBER'S SYNDROME Cr. N. III pally with contra later.l Mmipa reti. (a lso 1M LDcu ..o. "fOott" PIIp 716). Third nerve palsin frGm parenchymal leaionl may be ..l.tively pupil .pa rin • .
NEUROSURGERY
"
BENEDIKT'S SYNDROME
Similar to WebB'a, plus red nucleullellioll. Cr. N. 111 pals)'wilb rontrnl8~TlII hemi· Uceplllml which hn hYP" rk.ine8ia , atu.io, and a CO'ndbrlin Io!ymentum i"Y01 ~; nl! ~ !lucie"., brllthium col'\lu llttl.V1,m , /llIIf fllaeitlel oflU. panstS
M II.I..ARD-GU8LER SYNDROME Facial (VI I) " abducenl ]VJ) pal.y . contralat.er~ IM!miplegia ((:(Jr tlcoapinsl lTatt) from LeaiouSi.) E. bilateral ideomotor .p.....,u. [ioabllity to;.:any out vuba l tonunandl for lie· dvi lles th a~ c:an oth_1M! be ~rformed apontanl!9U1ly wIth NN) S. addiliOflaJ effecu of """,-d.omillOlll parietal lobe te.iollll C.... uaU,. nihtl: A. topOgr:llphil: memory 10lIl B. anOlOgnOllla and drealil\r.pn>t..
'p_
NEUROSURGeRY
87
CORTIC AL SENSORV SYNDROME
Letlion ",fpostamtrnl gyT11S, £specially 81"118 that mUJlII 10 hand. llensory defitit ~ A. loss of position ,""nN and of pa!l8lVe mOVemCnl sanae B. inability 10 Io ca.
3.7.6.
Bladder neurophysiology
CENTRAL PATHWAYS
The primary
coordjn~ting
eenl.O't ror bladder fundion re-
sides within the nudeu8locu~ coerule u8 of the pons. Thi BOOnIA.lr 8ynchroniu!'I bladde r eontraction with relaxation orthe
urethral sph,lIcle, during voidin, .. VOIW\t8ry cortieal rontrol primarily "wolvei inhibition of the ponline ren~, "lid Otigin81.O'8 in the anternmedial por. FlgrJr. 3-15 LocaliOfl 01 lion "rthe frontal lobes .lId in the genu of the rorpu~ callospinal cord bladoer eN.. • 8Um. In an uninhibitt!d bladder (e.g. infanQyl the pontine voidingcelll.O'T rUllclions wilhuut ""rtiCIII il,lhibi tion !lIld lite detrosor IIl"aderontr.ctswhen lite bladder ru.ch ua critia,,] capaci ty. Voluntary .uppre"i~n from theeol'tel< via the pyl1llOidal tract may r:ontratL tha e~ternal aptlinclerllnd t:lay alIIo inhibit detruwr rontraction . (."orticalle.e.iofUI in this 10eaU"n - urgency ineontinence willt inability to luppr,," th~ micturition renex"".'''IJ. Elfen!llt.i; to Lhe bladd .... trllyel in IhedoNl~1 portian ofth .. !al 3. !'leuroanlllolny gnd phYlliology
"
nal cord (ahaded areas in Figan 3·/5).
MOTOF! There are two aphinctera that pre,·ent the flow of urine from the bladder: internal (a utonomic, involuntllry control), end external (striatOO mu""le, v"luntllry control). Paruymp athe Hca (PS N): the detrusor mo""leofthe b!addercontracts and the in· ternal sphincter relaxes under PSN stimolation. PSN preganglionic cell bodies reside in the intennediolater al grey orspinal cord segmenta ~_ Fibe,... exit e8 ventral nel"l/e root.s and travel via pelvic splanchnic nerves (n ervi e ri&" UAJNAAY 8LADOEA DY SFUNCTION
The tenn ne uroge nic bladder describes blAdde r d}'$Function due to leBion~ within thecentrai or peripheral nervous systems. Some use the tenn synonymously with detru· sor arene1u n~ w rflrotl e:lectrodes. VoIu,,uny .phincter CUnlra~I>Ofl ...... IlIl.aM:tnea ol l uprlUlpinal innervllti(ln. When CClmhin!l; due III high int,..,yesiculu preslUres) and opti millition or utinllry rontinem:e. Palient$ "',minad~uate emptying or incre.sed bladder preuure I,.., often II\3nlged by intermittent c.th.~riutiGIII I nd I1Iticholinergica. Anlicholinerti""'lInd beiulvio ... 1 the..py an used fur patients with mIIintained voluntary blldder emp~y'ng w,th Utlnl!"), frequef1cy or urgency incontinen~.
3.B.
, ,
References
_1li_.'''I. ~ 1I'
_
1»M. I"'-1.
I [ C,-·..u.. af~ "' " ". N.
'_flf'l II
NEUROSURGERY
_""'I.
J N........"" 7~: 1IJ7.
y .... "') 1t.(N 1 "' ......... &111.
l>ioI:""'''''''' ,.....
.... 1119' .
~ , .
Gil>o H ,t..."~yC.~"L.. Mo~ P C.~,riIr II B. "'_""" t o . .,oII.• fOll'.I. WO ~.I'b;"",,,lpioio. 1O/1«1 .• IWIi. Y'" !. Cbopl 2.
cysts with tnore:complex lining which may slaoOllntain neuroglia, ependyma, and O lh~r
l i,o;su.,
type~
PRESENTATION
MoslAe" thaLbecome symptomatic do so in enrly childhood'. The presentation varies with location of th e ~Ylt. and of'l.entiruu appear mild con sideri ng the large 'Ii ze; of some. Typical prllsent.a-
tions are sMwn in Tllblf "_1" and i ndud ..,
Tabla 4-1 Typical presenlaUona 0 1 araehnold cysta
- --Mldd ll los·
.. ,yt l.
SuprastUar cy5l1 wllh
hycl,ocep!lalul
-, ~ant.l1
headache
h)1le1lftnSior1
hemiparesis developmental CIeIir~ 'iWa11oss
D,l1uaa I Up"'" or Inlra!flllOf1. I I cyJts with hyc!racephalus inUlICIa"lia! hype; are notoriou . for hemorrbagedu!! III t.ear1ng of bridging vejlUl . Solue sparta organj~tion~ do notaUow participation in OIIntact$port5 for thue pat ienUr
B, dlletoruptureoflhecyat at a focal protr"u~;o n aflh., s kull wilh focal BignliaymptOn-.. ofa space occupying le~ion incidental finding di scovered during evaluation for unrelated condition 7, su prasellar cysts mlly additionally prellent with': A. Jl,)'drocephaJIUI (probably dne to compfl'alIion oflhl' third ventricle) B. endocrine sympwma; QCCUN in up 1Il6O%.lncludea precociou./l puberty C. head bobbing (th e t a.ca lled "bobble-bud doH ayndrolue"): con. idered lug. gt!'St.iVl'-o{ , uprll'l4!!i IlT cYlib!. bu t O«UTS ,n aa few at 10'J>
4. o. 6.
4. DevelopmenltlJ anom~Jres
NEUnOSUnGERY
D.
vi.ual lmp&ir!llen~
OISTRIElUTION Almon all OC:CU r in ,ela lion to a n arachnoid cisU!rn {ncept;\ln: iotrasellu, the cmly one that;, (!>i tradural, • • TobI~ 4.2). RetrOCt CYST SHUNTING
Probe.bly Ihe ben O~1'1I1I treatment. For . hunting into peritoneum, """' a ~ I(u......, II ronm....,..t ~.nlrkulomeIt8Ly. on. may 8imult&n~u!ly place" ventocu.l •• Ihuntle.lI'_ I.htoUJb a -r conneccorl. Ultratound. ventriculllion (difficult Lo apprec'iate on M RI J'~_ Oi rrerentisl diagrOllia on CT' primarily between dermo~ cyst, teratoma" and ge rminoma " . MR I: characteristic finding l& a midline lesion with signal ch... raCterlslU:~ orfot (higb int.enaily on Tl.Wl. low inten5ity OIl '1'2\\'1/.
P r p.s e n ta tio n Often discovered incidentally , u lr'ge Lipomas may be assoc:ial.ed with sei~un!8, hy. pothalamic dyarunction, or hydrocephDlu8 (pos6lbly from oomp r1'Ssion of the eq ue 1'reatm ent Direct eorgical approach is Hldom nE'Cll&Sll1')' for il)tlllcr~niallipom.81'·. Shuuting OlDy be required ror caSO!'! where hydrocephalu8 n!sulta fron, obstruction ofCSP circu lation"
4.3.
Hypothalamic hamartomas
Hypothalamic hamartomas 8m rnrll n(Hl-neop!a.tic oong.mitAl DUllfonnDliona lCOpicapproach: difficull because vel1tritl" aM ra rely dilated
NEUROSURGER Y
4. [)"v.. lllpml!flUlI
anlllllDHe~
4.4.
Neurenteric cysts
No uniformly ao;cepl.ed nomendature. Working detinitOo n: . CNSc)'It lined byendo. thelium primarily rtllembl..ina thatoftheGi tract, or le$&ol\en, the rapil1ltory tl'toct. Not true neop{umll. The mostcolDnlon .ltenatt term ill e n ter oge no ul c y a t , with 1,.. oom·
cyst, and endodenllal c)'It. Uwally affect UM upper thoracic and cervical ,pine, a nd other associated developmental vertebral.nomalin .r. commoo", &'-0 oc tit."
r elluj"" theoomblned effort. of. neU(OiIurgtoOn and eraD.iof.cillllurgeon. and /NO)' nead In till ~t8Hed in lOrD" ca_. Ri.u of I Uritry ,"elud.: blood 10M, te\1,,-,,",. I tnlU.
DIAG NOSIS Many caJeiI of -'yoOllw.ia" .... ",Ilty dua to p(NI;tionlll noUe nil\f (e. r . "I.Ey l.tmb· doid". au IH:lowJ. U thit i. '1Upeete\t. inslJ'uet parenU to kttlp need olfo(n"IWied are. and tllcbl!Ck palie al in 6·8 wttlu: ifilwu poiIit.ional, it lhould tA impl1M!d. if II was CSO then il ul uully dk l" ..... ILllelr. The di .......it a f CSO may be . i~ed by . 1. PIIlJaotin of .. bony pl'OminellC1e DYer the IUspected 5ynoatotie autura\UOIption. lambdoidall)'1l(lltDllil,.t b./ow) 2. I':~ntlfl finn pr~u~ ... IUl tha thumb. Wl.t to I'\!1JU;Y8 moye~nt of the bones on either lide of the luture 3 plaia sku\! x·ray.: A. laek ofnBI'"/I'UIJ Juoen l;)' In centet"ohut"l"e. Someee_ with oonna l x· ....y"". pearance of the ""tu", (even 00 CTt "'B)' be due \(J fOUl ! bon)' .picule ronn .. tion"" B. boos ten coppt!r calvaria 1_ ~~ 101), sutural d'liSllIlht snd .."",iOll ofth", Hils maybe seen in cases ofinc",8IIed ICE-t CT_n: 1 eso
- pla gioceph81y with forehead on affeeted .ide nattened Or concave above ~e(narmal side fa lsely appesMl to bulge abnormally), luprao(l.bital margin higher than norma l .ide ((III skull x-ray - h8rl equin eye l ilt'l). The orbit rotates out On the abnormal l ide, ond can produce omblyopia. Wi thou t treatmeot, flattened cheeks develop and \.he no. devi_ ates to the normal side ( mot of nOM tend . to rotate t.oward s deformity). Bilateral coroaal eso (usually io craniofacial d)'lmorphi l m with mul t iple lut ure CSO, e.g. Apert's) - brachycephal y with broad , nattened forehe"d (acrocepbaly). When combiooo wilh p~malUre d08L1A offrootosphenoidlland front.oethmoidal l utLlrel, resu lts in foreshortened anterior f0618 with maxillary hypoplatill , . hallow orbits, progN$Si,-e proptosis. Surgiealtreatment Simple strip craniect.oml oflhe involved sutUA has been used, often with excellent cosmetic """It. HoweYer, some argument that this may nol be adequlte hili been presented. Therefore, a moreculTf!nt recommendation is todofrontal -Ste¥!MOn, Muenke, Jaclu!on·Wei!lS ayndromell}. All exhibit autoBOm31 do.uinlllll inheritance.
Ave, age agio: at prellenlatiM ie "1 yell"l I ..... oge: 12.1a yn). Slight female preponde .... anctl (female:mllie a 1.3:l t Average duration of.ympt.ows clearly rell'ltcd 1(1 Chlan malformalin" is :t t)'l"$ Irange: t mMth_20 )ITs); if non~pec:i fic eompJair,t/i, e.g. lilA , a re induded, this bec:nmes 7.3 ye/ml". Thi. lateCley is probably low~r i~ the MRI ef$..
CLINICAL Par.ient.6 with Chia ri type t malfonnatic)Il /lUI}· present due tu;my o r aU " f the fol-
lowing: I. oompreasion " f brain stem "I the level of the ftooraroen mllgnum 2, hydrocephalus A. True hydnom.y. l", probabl,y a_n'l O,mperature eentlltion with preserved touch &'JPS), 0 I.....'"
1:"
ClIiIIrI m.lronzaalicm IIQd
.
•
In itially." Ii DUll wIIs ddined 8lI dearly pathologic:"'lwith 3-6 mm heincbordt'rlina). Bark.ovieh" found t.n$illar positions 88 Bho .... n inTabl~ 4·8. and Tabl~ 4 ·9 ehow. th e ef. feet of utililing 2 Vt. 3 mm (Ii the lowe$t lIo rmal position . Table 4-9 Cri lerla lor
Chlarll ~
Crileri.lor Iownt Sensitivity Speclllcity e~t 01 tanslls at> lor Cll\lrli IorC\'ltlrl I cepled II norlll!ll 21T)1'T1 below FM t OO'l\o ~.5" ~ 0 Surgical techniq u ell The mllSL frequently pe:rformed operation is poaterior fOSlln decomp",,,,,ion (su bocd pitlll er..rueo position being atCt ( 6~) . Fibrous adhes ions between dura, arachnoid s nd tonails with occlusion of forami _ na ofLUlIChka snd Magendie in 41%. The toMils sepa rated easily in 40%.
Table 4-11
r
In Chlarl I
I I below loramen magnum
"
"
SURGICAL COMPLICATIONS
CO
After suboccipital craniectomy plus Cl·Slaminectomy in 71 patients, with dursl patch grat\iog in 69, One death due to s leep apnea oceurTed 36 hra post-op. Respiratory depression was the most COmmon post..,p eomplicetion (in 10 pa· tients ), ..... u.al1y withi n 5 days. mostly at night. Clou respiratory monitoring is therefore rerommended". Other r isks of the procedure indude: CSF leak. hern iation of eerebellar hemisphe res. vascular illiuries (to PICA ... ).
unspeciIied level
~"
inverted foramen m8ll"1lm keel rJ bone
Cl aret! alresia
.tIn"'....
vncular abnOrmal ..... P ICA d~ateeaking of tectum 2. absence of the septum peJlucidum with enlarged interthalamic adhesion' absen~ of the septum pellucidum it thought to be due to necro.is with resorption secondary to hydrocephalus, and not a congenital absence'''''' ,,', 3. poorly myelinated '·II: I . swallowing difficulties (neu rogenic dysphagia) (69%)". Manifests as poor feeding. cyan0!5is during feeding, nasal regurgitation, prolonged feeding time, or pooling of oral secretions. Oag rene~ often decreased . More severe in neonat.e~ 2. apneic spells (58%): due to impaired ventilatory drive. More common;n neonates 3. stridor (56%): more common in neonates. usually WOn;\! on inspiration (abductor and occasionally adductor ,'O NEUROSURGERY
E. cerebe llomedullBI)' empr"''''on Lary ll gOSCOpy Performed in pa t i.nQ with lirido r to rule out croup or other upper ~"piratory tracl i"fection. TREATMENT
inw,t CSF . hunt for hydro«pholua (or check funct.ion 0( ex ialing ahunt ) if neurogenic dy.phagia, Htndor . or apneic apell. occur, upeditious poiI~rior r.... l a d«omp~ .. lon i. recommended (_ Nlo",) (r.quired in 18.7 Cerebell a, hypopluia without cerebellar herniat ion .
NEUROSURGERY
... Developmental a roomal ,es
,.,
4.7.
Dandy-Walker malformation
At.",;a offo' 8111'n. of Magtndie and Lu.c:h k,". Thl, l"uulU in &&frlesi, of tile ~r I I I~ posurior roan cyatcommunicating with lin enla rged 4th ventricle (.orne retrotl'l'i'beUar .nchnoid cyaU mi mic Oandy. Walker, but theAl! do not heve vennia" agene.is and the c)'IIt does I1l!& open into the 4th ventricle)
lObelia. venn's with
Hyd.""",phaJuaQ«un in 9O'Jl, ofcl.U$, and Dandy- Walker malformui(ln is present
of.n caHI 01 hyd.rocepha!IlI, Associated ab normalit ie. in
2-4 ~
eNS .bDOnJUllitia include .gents;, oft'" o;(Irp'" caIto-u rn in 17,.... a nd occipital encephaloc.le in 7<JIo., Olhu lindi"p indude heterotopi.., apina bifid" syringomyelia, microoephaly, den::ooid cyau. poreneephaly. and Klippel-Feil def for 1-5 yun: lbe longe.8~ W>i!;' '' 0 yrs. Although moat folio ..... !.hi! longstanding benign cou ~ there Ol'll rt!poru of "Ievue See Table 4·13 . Headachf 1'181 the QlOllI Cllmmon symptom. lind ~ ad charae .... rialiC4 of HlA 1I000000;au,d with e!e~ated ICP. Viauru ~h.ngu were next , lind liBuaJiy etlM'.lfod of blurring or 1051 of acuity End""rine cb lngu induded menstruol irre(Ularities, hypOthyroidism, aod h irsuti6m ,
Signs Papllledema was the most COmmOn lind· ini: (53%). Vi~ual fLel,u were normal in 78~, lM remainder having l'1:!duced puiphe ral vi, .ion, increased blind spota, quadranlic or hemianopic f'eld euta, or OU:Oto1ll8ta. lnwlle Migration abnorm a lities A I lightly different clauification scheme defines the following u abnormal ities of neuronal migration (!fOme are considered posl neurulation defect • . .."" "bow ): 1. l; _nce phaly: The most severe neuronal migration abnor mality. Maldevelopment of cerebral convolu t ions (probably an arrest of cortical development at an early fetal age). Infants are severely retarded and usually don't survive:> 2)'T11 A. agyri a : completely smooth s urface B. pac h ygyria: few broad &0 flat gyri with shallnw l ulci C. polymicrogyTh. , small gyri with shallow sulci. May be difficult to diagnose by CT/MRI , and may be confused with pachygyria 2. b et eroto pi a: abno rmal fod of gray matter which may be located anywhere from the auboortical white matter to the aubependymallining of the ventricles 3. ""hh.en ce pb .. ly: A. den that com municates with the ventricle (as lTU'Iy be 4. Developmental anomalies
NEUROSURGERY
MICROCEPHALY
Definition: head circumference mare than 2 standard devia,jol\ll belnw the mean fnr sex and geSl.8tional age. Terms thalare sometimes used synonymously: mionx:rania, mi_ crocephalus, NCIt a Bi ngle entity, many afthe «mditions in TabU: 4-14 may be a!lSO AKA macrencephsly, AKA megalencephaly (not to be confused with mru:rouphaly, which ;8 enla rgement of l he s kull (",e page 919)), Not a tingle pathologic entity, ~ enlarged brain wltich may be due to: hypertrophy of gray matteralone, gray and white mat.ter, presence of additional structu res (glial overgrowth, diffuse gliomu, heterotopias, metabolic storage diseases, .. ). May be soon in neurocutaneous ~yndromes (especially neurofibromatosi s). Brains may weigh upto 1600-2650 grams.IQ may be nnrmal, but developmental de· lay, retardation, spasticity and hypotonia may oc As&oe ia ted neuropa tho logic fi ndin g." pore_ph_I), microgyria interhemi.phe.ric 1iJl2mu and li poma. or lbe COrpUI callosu m (Ht page 96)
art.ineo",*phaly
optic atrophy ~obom..
hypopl ••if, oftha limbic ' ,Item bundl" ofProbtt: aborted btcion inp of corpu. .,.Ikwum, b'Jlge into late",1 ven_ lrid. 10.. ofhorizonta] orien lation of d ngu late gyro, tnQHr\ll~1y 01 ~Ml"'IisIue . C/rIe t.iftl1la'III _ rIIIIIOIogoc deId. myelomeni'lgocelt ~I 6e1ecl in v&!Iebr&Iardles wilt! eys!ic diltUlliorl of mri'oge$ and IlTul;luIil 01 f\n;IiOrIallbnormal'fy 01 spiIIl eord 01 caUIIIlQUN [see~. SPINA BIFIDA OCCULTA
OcCUI"l in ~ 20·SO'h of North Amt rk l.nl. Often.n inciden tal finding. u ..... lly ofllO clinical im por\llnce wh en it o«un alona. However. it II)II YOCClllion.lly beassoci.ated with diutematornyeli a, \.ethe...o cord. ]i porno, or de rmoid tumor. Wh eo IYlllplomatit from one of the IlUOc:lll\ed c:onditionl, the ~senl.&tioo i" tha t or
'"
4.
~veloproenUl I
anomalies
NEUROSURGERY
tettlered tord (gai t dinurbance, leg weakness and atroptly, urinary diaturba nce, foot d~ (oralities ... , see Tf/heret! t»rd .yndromf, page 120). The derl!(:t trUly be palJ.>llble. a nd there may be ove rlyiog C'J lsmloua mani festation. (.ee culo:rnrour " igrruolo: 0( dylnlphi,m in Tablt 4· 17, ""ge 121). M YELOMENINGOCELE
EPIDEMIOLOOY/OENETICS
In cidence of.pina bifida with meningo«le or myelomeningocele (MM) 1.1 1-211000 live birtlll (O.I .O.21to). Rislr inc rea_ to 2-31to ifttler. i. one previou. birttl with MM .•nd 6-81to after t ..·o "fTec\ed ctilldren. The nlk i•• Iso IncruHd in f. milies where cloH relllUvel (e.g.• iblinp) hav, given birth to MM clUldren, esp. ultanlOus . hunt if overt hydl"ll«phalul (HCP) at birth): see 1'I'm i"4 of MM dNU,.. below 2. neurological . . . ssmenl and manaK"mlnt: A. it.o.>ml related to . pinal lesion I . watch for l pontaneoUS roovemelltofU>e LEs(good spontaneous melVe-ment correlates with better later functional outeam"') 2 . alMss Iownt level ofneurologic function (_ TCJblf 4·1$ ) byd'e Since sympl.Oln$ are due to \ 1) tethering orthe spinal cord. especially during growth spurta, and ( 2) compression due to progussive deposition offat, especially duri ng periods ofrapid w.. ight gain; the goals ofsu rgery are to release the tethering and redu"" the bulk of fatty tumor. Simple cos",etic treatment of the subcutaneous fat pad does not prevent neurologic defIcit, and lJIay !Oake later definitive repair mOre difficult Or impossible. Surgical treatment is indicated when the patient reaches 2 months or age, or at the time of diagn osis if the patient preaents later in life. Adjuncts to su rgica l treatment inel""' .. evoked potential monitoring and laser. Overall, with ,urgel}', 19'1> will improve, 75% will be ullchanged , lind 6% will worsen. Foot deformitiee olten progress regardless. D ER MAL SINU S A tract begilUling at the sk.in surfllce, lined with epithelium. UsuaUy Ioc8ted at ei· ther end of neural tube: ceph alic Or caudal; most common location i, lumho$ll.cml. Prob· ahly results from failure oftbecutaneous ectoderm to separate from the neuro~toderm at the time afelosure of the neural groov .....
SPINAL DERMAL SINUS
May appear as a dimple or as a sinus, with Or without hai rs, usually very close to midline. witb an opening of only 1-2 nun. SUrTl)unding sk.in may be uormal. pigmented (·port wine~ diseoloration), or disl.Orted by an underlying mass. The si nus may terminate s uperficially, may connect wil-h the coccyx, Or may travenle hetween normal vertebrae or through bifid spinee to the dural tube. It may wid_ ell at any point along its path to form a cyst; called an e p id e rmoid cyst iflined with stratified $quamous epithelium and containIng only keratin from desquamated epitheJium,orcalled a dermo id cy3t ifalso lined with dermis(conUiningskm appendages. such as hili< follicles and seba~u . glandsl and also containing sebum snd hair. Although inDOCuous in e ppearance. they are a potentIal PIIthway for intradural in· fection wlUch mlly result in meningitis (sometimes recu,nmt) and/or intra thecal abscess. Le"" serious, a local infection may occur. The lining dermis contains normal skin appendages which may result in hair , sebum, desquamated epithelium and choleeterol, withln th" t,·"d. lui. Ii re~ult, u,~ ..... "~" .... "f1.4,, .i"u~ trae1. ,.. ~ inil ..!i", ,ULJ ~ ...., eli""" ... ...,nl" (chemical) meningitis with possible de)IIyed uechnojdjtjs ;tit enters the dural sPlIce. InCidence ora prf's"med sacr al sinus (a dimple whO$ Radiologic e va luation
When lean at blrlh,llikJI.I~.lI.U'1 i. the belt meanl to evaluate for 'vim. bifida and a poIIsible nlll.. in. ide the o;8nlll. Ifleen initially followinll biM, lin MRI .hould be oOLained. Sagittal ima&d may demon$t.r~te the ttllct .nd ia pOint of.tWo.hmenl. MRlal*O Optimally demoNtc.t.eI m..uea (lipomas. epidermoids ... ) within Ihe CllnaJ. Pillin ,,·tII),1 lind CT Ira unllble to do:tmOr\&lrate Ihe fine ttllct which 01/lJ' .. ist between th skin lI11ld Ih .bnn~orthe patteno, t iementa with an enlarged foramen m'fIlum and fiud hyperemmion postu re I.ItaUed In. ieocepbal)' and il ,arl. lneio;lenceofKlippo:l·r",il il unknown dIN! 100 iLl rarity alld tbe fact that it " frequrnlly uymptomatk Clauic clinkal triad (an 3 at'" pHUnt in < 6011>): low poIoterlor hairline, ,oofU""d neck (brevictlJli.), aod limitalion of neeIt motinn (may nDt. be e~ident if < 3 venebne ar. fuMd, iffusion illimitl!d only to the 1 _ et1'VieaIl_lJIO. or ifhYJll'rmobllily of non· ruNd """iI!n~ compensala). Limit.tiDtI ofmovlrnent I, more wmmon In rotation than flexion.f:lltanl1Dn 0' lat>eral bending, May a«u r in COI\iunction with othu fonpnital o:em",llpine al'lOmaliH t>a.eilar iDlprfiSion and atlanto-ottipitalhDioo. Other dini"",l.ssociftt;onl include fICOl;· 01;' io 6O'lto. f.eial .. sym meuy. tortinoUMi , _tlbinl or the ofCk (~aUed pttlryVwa colli wbeo II!Yrre). Spre~I'a d efnrmity in 25-3S'it {raiHd IleIPull due 100 fal lure o1the ",pula to properly d l'AOC'nd from ita recton 01 rarmetion !\ilh in the ned< to itt normal position lbout!,he Um' time elllle KlIppe.L·Feillnion oce ..... l. IY"k lnwl ]mirror mo·
.,,m nel"V~ roots by presenoe oftbllucteiistic lIquiggly vtssel on Buriace of rtlum . Also, under tbe micros~ope.. the filum 11M Ddistinctively wh i{er app"'aranll a Umula Uon wnd "",urlling of.n .. l . phlno"", EMG .... e
more definitive. OUTCOME
In MM , it if; us ually ImpmlllibJe 1. NE:UROSUROE:RY
References
4.14.
v... {)O:, Me1"";"'III!11.
f""""",
_la"
PlllllaurQl" 1r20-r1cil Nd lifllbC
R..,"" O.,.",1IIIl E, Ciooai'i G... (1{~ "'",,",M' r.:.-
.......11>.'''... II> "'pcn'• • )'noilSrus
Iki"_ ..
Mit ......1.... of "" .... IDJIII"" "" ~ . Rodl·
I_
" " "
bobb_ ....l l.)_lrl"~ .....ln
"'"P'' ' ' ........u.u.
_OflIO>I""" ...
,~
I\~OC1OuIctEM . I """'''C A.S.U.... L N, ".>I. Op.no:" ."'0>tl_ A i .....,...io ' _ ......... ,01 ...."""""' •• .-.1 "",.....1Od _ ...pII;., ..... lo ollil4l .... ,111....., ..., _•• J~; fj91-9. '11'16, Cbodd",, ~ W M.C\IotW"". J 8 , 600p F .... Th< .ut.18""""'1"""0.", No ...... .... .or) lO.II6I·7 !, '9n, G.... G F.Duo< E I( 0....:,.,.0(.... .,.,.,..,.."'.. II)' " " " ' ' ' ' ' _ R... lokro 1I.5; 6U.7 1. 1915.
".
M~
Spdl .... I O. Po'o!! C. Jonco ... M: o.-.cloptncnoll -..0.1"... 10 "'" """",of ,Iw. I",""",n ...."""' •
_-o.t...
8.... 80, lI ·st 19»-
1'001 K S, LI" R fl .St/Iwo, F"',n oj . ... ",.11......." .., Ro ..... aJ7 1,-"J N.......v ,~ !3' !D·7.19IIl O. _G . ~C.llbmi ...... F. «61. a.;... l .,oI/onno, 1ott Pan I. Con ' .... p 16
r.m. I·7,:/004.
No,,,........
$I11d S,So.,P II! • "'on. J'I ....
4. Of!veIQPn:tenU,t! anomB lie$
c...
". ". " ".
.
". ". n.
". ". ". ". n.
"""'~ J2: J06.9 . 1\19)
1..\liobon ond I. pOl"''''' ...;'" Ch,an mallormat_ "'I" ... 'i""" . _ h wi," MR imo,i.J. J Com"". "..", To""'V9! IOll·6.I'ISS. 8..-1:". ... " J. Wippold FJ .Shmno"J L. ,, J(".,......_ idri .
... ... ". ".
V_ _ I . H1_R,"""'·' ....... oI_6o ••J, or .."",I 'obo ""1« ... JA M ~ 27': 1089·91 . ,~, S~wG M. Velie E M.Su1 D. "'~hI" L love r8(li (Optiray®) U~
• NOl ror int rathecal use (ret Clbot.!). and eoncentration$ include:
NEUROSURGERY
s . Neuroradiology
arteriography: Optirtly 300 (lov".80164%) or Optiray 320 (iovenwl68'l&). Total procedural dose should oot usually exceed 200 ml IV contrast enhanced CT I01 receive IV iodine e"~en with thi~ prep, unless absolutely lI~aaary. Caution: th e patient may still have serious reaction (modified'). utilize 'IOn-ionic oontrast medium (e,g. iohuo]) whenever poII.'Iible steroid ($I" pogt 8 for further details ofsterDid doting) • prednisone 50 mg PO: 20-24 h rs, 8-12 hi'll & 2 hra before study • equivalent dOlle lumedrol® (tnethylprednisolone) f~r TV use would be ~25 mg diphenhyd ramine (Beoadry~) 50 mg, EITHER 1M 1 hr before, OR TV 5 min before st udy optional: H1 antagonis:, e.g. cimetidine 300 mg PO Or TV I hr before Sludy have emergency equipment a vailab)e durin8" study
5.1 .2.
Reactions to intravascular contrast media
BETA BLOCKERS Beta blockersea.n increall'l the risk of contrast media ,..,action8. a nd may mask Some manifestations of an anaphylactoid reaction. They al$o make use of epinephrine inadvisable since the alpha effects of epinephrine will predomioat.! (bronchospasm, vasoconstriction, increased vagal tone). If treatment is required for hypot.!nsion. OlS)· try g lucagon 2-3 rog TV bolus, followo:d by 5 mg TV drip OVer 1 hour (glucagn has positive inotropic and chronotropicefTect that i$ oot mediated through adrenergic pathways).
I DIOSVNCRATIC REACTIONS AND TREATMENT
For treatment ofinadvertent intrathecal injection ofionie contran agents,:lee fIOIl~ 126. HYPOTENSION WI1l-i TACHYCARDIA (ANAPHYlACTOID REACTION) 1. mild: 1Tendelenburg position . IV fluids 2. ifno response but remeins mild: e pin ep hrin e (use with caution in patienta with COl"Qllory artery disease. limited cardiac reserve, hypertension, Or unclipped cerebrsllUleurysm) A. 0.J.O.5 ",I "f 1:\000 SQ (0.3·0.5 !ilK) q 1:;...20 UJim. (...,w.: 0.01 "'~ ) B. OR, ASEP recommendations (especially for elderlyor patienLa in ahock): 10 ml of 1:100,000 TV over 5 to 10 min (put 0.1 ml of 1:1000 in 10 rnl ofNS, or dilute 1 amp of 1:10,000 to 10 ml with NS) 3. moderate to $f!ver~ or ....Qrsenin8" (anaphylaxis): add: A. rv colloidal nuids. e.g. hetasl.arch (Hespan®)6%(colloids a re required si nce there is extravascular shift.ofnuids due to seepage, then agents also caIT)' a Bmall risk of aOerg'ic reaction) B. epinephrine (..... ",00",,). May repeat It I C . 0 1 2-6 Urnin per NC. Intubate ifnl!0
~IPOru~,
add,
A. atropine 0.15
mr tv. may rep~at up to 2·3 mg over 15 mille PRN. VII'! with
u ... t ion in patients with ... nderlying hean dillea.. B. t:KG andlor emiae monitor: eapedaJly if atropine OT dopamine are uBed 3. ifl>O rupon"": add dopam ine •• tart at5 I'r/kglmin (.n 1X'Il. 7) URTICARIA
L. 2.
ro.ild : . elf limited. No t reatment ne«Mllry moderate: A. diphe nbydramlne(Benadryl 2. if respiratory di.tr...: 0 1 2·6 Um;n . Intubate if nece. S&ry 3. diphenhyd ramine: I « ooot.oe 4 . eimetidine:.u o~ 5. if angioedemail accessible. add iee pack 6. maintain IV line B RONCHOSPASM I. mild to moderate:
A. epinephrine: I « obotot. May repeat up to I ml B. if respi ratory distreu: 0,2-6 Umin. lntubete ifne«sury C. maintain tv line D. inhalatioll3L therapy with a IJ-adrenergic agon;lt. e.g. albutenll (Pl"'OYen· til®) if respiratory therapy is availablll, otherwise, me\.ered dOH inhaler e., . pirbuterol (Maxai.rGl) OT metaprotertnol (Meta pre Ie). 2 purrs 2. MVere: l~at as above for moderate reaction. and add: A. aminophylline 250-500 mg in to-20a: NS.low tv over I~O miru. Monitor for hypoteruion and arrhythmias B. intubate 3. prolonged: add the following (will not have immediat.e e!Tect): A. hydromrtisone 250 mg tv B. diphenhydramine: 1ft obcM: C. cimetidine: I « oboue PUlMONARY EOEMA
L 0, 2·6 Umin per NC. Intubate if necessary 2. raise bead and body 3. futoMmide (l.asixe) 40 I'DI tv 4. EKG 5. if hypo>tia deYeln.. (fDlly manifest aa agitation Or oombativene.u), add: A. morphine 8- 15 ml tv. May ~au" rupi ratorydepreuion, III! prepand to in· tubate B. epilllpbrlne: _ oOOw . • CAIJI'ION: UN only if MI can be RIO all cause of the pulmanllT)l edema. Patienlll with acute ;n \.raeran'al pathology may be at riak ofneul"OKf!lI;c pulmonary ede.ma (I« pose 7) SEIZURES
IflH'ilUIl! is notlH'lflimited . • tart with [oraaepam (Ativ~) 2.... mg tv for a n adul t. Take precautio... for .t.lul epi lept;"" (IJft pof. 265) and p~ to othe r d ....p .. in· dkated (I« pose 266).
NEUROSURGERY
'"
5.2.
CAT scan
Attenuation of the x- ray beam on Ii CT Scan i.o defined in HGunsfield units.
Table 5>2 Hounslield unll,
'f'Iwse unit.9 are not abll(llute. and vary
'canner
'01' II I8mple CT
between CT scanner models, ..... ith II SlImple beiog shown in T"bI~ 5·2.
If there are no calibration marks on BClln, ooe Cll n estimate average adult globe (eyeball) is 25 m m diameter (through. its equator).
Hcl '" 23% wIH cause an oeule SOH to be isod_ with brain
5.3.
Angiography (cerebral)
Risks Risk varies with the natw"(! afthe patholOgy being investigated and w;th the e~pe rienee of the angiogTophy team. Overall risk of II complication resulting in II pennanent neurologic deficit" '; 0.1%. [n ACAS, there was II 1.2% oom plication rate ($u P"8~ 873).
General information' In gen of patieoq supply their poste rior cerebralllrtery on one or both sidCli from the carotid (via p-oomm) inl tead of via the vertebrobaeilar syetem. 1'0 help find the middle meningeal artery on lateral ECA angio, follow the ante rior _O' 6. sh ra pnel; BB', (tome bullets are OK) 1. reJlIliv~ oontrawdicatioua:
A. da"atrophllbic pBtieDI.S: may b., abl~ to sedat.> adequately to perfoftU nud)' B. rntically ill patH'!nt.s: ability to monir.o. And BOO!!811 to pllit.nl are impaired. SpeciaUy designed non.magnetit ventilator may be reqIlLN!d. Cannot Ill;(! mlOn MRSoI {A) nonnaI brain, ""II (6) high Ii''''''' glioma
MULTI-VOKEL MRS
Colorroded KIn with &eleded overlay for NAA, eholine .. [email protected]\>« risk ofumpling error.
5.5.
Plain films
5.5.1.
Skull films
Tabla s.6 Norrtull d imension. 01 . he aellll iurcica ( _ Pl9ure 5-8) ~
S ElLA TURCICA
o {depIhj {mm)
NORMAL AOULT DIMENSIONS ON SKULL X-RAY Teehniqua: t",. late.. L, 91 em u.r~1.O lilm
Max
Min
" •5 "
An
10.6
d.istan«, centnl AY 2.5 COl anterior IDd 1.9 tD1 lu penor 10 EAr.!. See FiI"'" 6 ·8 for mUSlTation oflbe diroentiont. and Tob/.~ 6·6 for nOr· ma l vIlu ... Deptb (0 ): defined .. Ihe gn!atel~ mua urement from Iloor IG diaphraama seliH. Lenrth (L) : defined a. !.he gn!att.t AP diameter. ABNoRIML FINDINGS
Pituitary adenoma. tend 1.0 enl'rfl:e lb. sella, in «Intnost 1.0 ~ ..niopha.ryngiomu whkh erode th. posterior clinoidt.. Empty ..lIa .yndrome tendll to bIolioon the sella . ymmetrically. and "so ~ noterode the clinoid..
1)8
5. Neuronod)oloty
NEUROSURGERY
al50
",f' ahspell seUa 8U.tiM'- opti~ nuve glioma. It o;an tongenitally In H urler·.$aynlirom oco;,W"
polysaccharidosis).
MISCELLANEOUS Water's view: x-l1I.y tube ungled up.~S· lperpendiculli r to divusl . AKA submental verteK view. 1'91"01 '5 'tiel" ; K· ray tube angled down 45' . 1.1) vie .....
occipo,a. BASILAR IMPRESSION ~ure 5-8 M.nu,emaOl5 01 Several conditiol15 whose nomu are often (eNtlnem. sella IU'dca (lalora! view) OUllty) uged Interchangeab!y(eKact defirutionsara nOI en· tirelyagreed upon for all oCthe!;l!); 1. plnlyb8.llla, abcol"Ulal bll6ilar angle. OCHllle mOldkal i.:llporta,,~ (usO!"d in an· thropological dalal. May Cr may nOI btl s!i/!oci Med with basilar !mp~8!l ion 2. baallar ;mp", " ioZl (B1): UP"'lI.rd di&placeme nt offo ra men magnum IlIl1rglrur (i nc ludiog ocdpit(ll bon. l and urviclil ,pine lineluding :Jdo[ll.Qjd process) inlo Pfossa. Some use thi s term for upward di Splacement of dens only. May be selm in: A. tonpn"ital condilions (BI is !.he mon tommon congenilal anomaly of lhe crani E.
hype.tpan~idlsm
Same IO"asu remenl.& of ...... (rerer to FiG" '"" 5.10, page 141, and Figu,",,5·9 below/: I. Mc Rae's line ("MeR" in FiellrY. 5· /0): drown acTOlls rammeD magnum ttip of clivus (buion) loopist!Uon )'". Should 00 :> 19 mill ( avera~ : 35). No part of odontoid should be SOOv t !.hisl'ne (th e /DOIIt sccumta fo~ 2. C hnmber laLn·.lin e ("CL" in Figurt ';· 10)" : posterior hard pailite \.(I p05terior margill offonmeo lIIagown lop;&thlon,. l.cno Ilio n 3 mm or half ofdena should be above thill line, ..ith 6 mm being definitelY p,uhologit. Seldom UKild be.cau8e opi sthion i' ofu!n hard to we on plain film IIna mayaJso be invaginAted ) 3. McCre£or'. bilKli.,,, Flgu", 5-8 AP Yiew ih,ough ",al'k>cervk:al lul"lCtion' (~MtG·ln FisurI5·/0"f"': POi' FOOL ", FISd"IgOId'. divUlric line. F~L ~ FiScilgOld " tenor margin of hard palate _.~ line, " ~ , .)CIaI 0Wft>a/!g '" C1 CIt> C2 (SI!' to most CAudal poinl ofoo_oi~PIIIl·7Zl) ciput. No more thaI> ~ . 5 m.m ofdeDll shOuld be above thia 4 . Wac k e.,belm'8 e livu8- tl>o. ... an!" ronl.Our lin ... (AKA arcu.!.e liOQ). Non..aU), f8C"h should ronn II smooth, pMle "" .....e UN FiIlUrl5·10); I . po6terior mIlrIPna.llin. (PML) ; _10113 poIIUrio. Cl)n~ lunace. or" ....I~b ....1 \.Jod. jllll (VB). Marks the uteriOf' llIargin or'pinal eanal 2. a"!.eriOl" margiruolline (.4.' 1L): alonr anterior cortical surfac:ee of VB, 3. "Pi nQI. ",;n ll . lin ", (SU,: along base ohpinou s proalS ...L Tb. pIIII!.erior ",argin of the . pinal eMal 4. pclllLerior spinal.ll line {PSW: akmr lips or s pinaul pro bral fractW"e, d islocation, or ligamentou l
,,
dis ruption". NonuaJ values are shown in Tobie 5-8. NB : the IU!Ilsitivity ofth.esJe measurement'! ie only ~ 60% a~ C8 and 5% aLC6" . Increased PVST i . more like· ly with anterior than posterior injwie5" . FaJae pD.itivllS may (Je(ur with bessl skulVfaci al fr8~tUrl!'B, e~peciaJly with
NEUROSURGERY
.5. Naurorad iology
'"
rTliclure of the pterygOid
pI9te~ ,
INTERSPINOUS DISTANCES C·~pine AP: a fractl,lUldi,tacation or [jgarnent d1i!ruption ","y be diagnDSe Poet myelQgra p hic. CT u,errallY Iellsi tiY ily and l peiI\lpc.j!>'l"
"
..
,
~
111-l>.lqsS.
ScI... od< k H tI, S" ... W H. (0.1>.). Op. ... ~ ........
II.ondo I, V............. I. Mo,; .. II... al ~ _T ...
6 . Ele(:uodiagnostics
NEUROSURGERY
o:>Verallaplnal tord funeliOn and ar~ a lTetted by manipulation or the .pinal cord and l~ chemie ev~nt.l, they mal' r~in un~hanged with aome injuri" to the anterior cord, ~anscran;al m otor e voked potentillia (TCMEPa) : tran&a"anial electric .. l or magnetic stimulat ion of O'\otor cortex and delcending motor 0::i~ to pennit a. 2 out of4 twitchel. De-cending evoked potentieJ. (D£P): (form erly refelTed to by the misleotding tern:> al oe uropnic molor eyok~ poten t ial,). Ros~al5ti!l\ula tion of w apina l corti wit.h n dey
I-Y pe.D: !alelq'
4.01 mS
H.pe3~ 1a1eflC,
l.\51!1S
4.63m 2.&61115
V nbsolote I~~ IH-V peaIIli!1erqo
S.1mS
6.27 m5
tfs·N'1 pealIlalMCY
9.38 m5
PuP. pe~ 1i1!!ocy
IS.62mS 2O.82mS
jlIOklrqaIion $U~ lesion belween pon$ inO coIicuII.G. oIkt" K_11e neu,oma proklngallOn ~t:; Ie:siotI be~ IoMr pons"& midbrain, may be seen irllo1.5
field Slimulauon. Monocular delect so.ggesli ~ de!edif! Ul.loloplic oerve anlMor to CIli~5m (e 0. M.S" g1i..eoma, ~pre$IOOI'Il8lifW cegeneralion). Bilale!i! de!ect does nc)llocali.l:e. ••
IIOIm" ... Iues on _ _ ... cnliCl l "alUM
6.3.
u~
as eutaW I... aDnDImli _
Eleclromyography (EMG)
There lire two po.lrtionB 1.0 thl! EMG exam: 1. conduction lI1eI,,;Urements 2. o .. edle exam A, ioserti01l1d actiVIty B. activity at refit: IhouJd be I lle nl when need le Is activity iuo3l1uieteoi
~talionary
a nd in :\Crtional
DEFINITIONS
Fibrillati on potenti"" a: following den~rvetion ofa
lOOIetime. up to 3·4 week.. after denervation. If tM nerve re<Xlye ..... it llIIIy reinnerva~ tM mu..:le. but with larger motor units N!. uiting in longer duration a nd de .
...-, .,. '.' "
". ..,
_1IIIOlI:RI:!an:
~
-
ALCOHOL WITHDRAWAL S'I' NDROME
Compen18lion for the CNS tllpraua nl elrecUI or EtOH aco::~ in mrooic alcoholi. m. Coneequently, rebound CNS hyperactiv ity m.y rellull from f.mng EtOH level •• Clinical !IIena of EtOH withdraw.I,,1I duaified .. mador o r minor Ithe devae 6r.tltonomic hyperKllvily lind the pr~noe(.bHnceorD"l'll difl'erenlilltel lh_ l, as well .. ,.,1)'(24_48
NEUROSURGERY
7 Neuro!OX.oology
'"
hrs) or late (> 48 hl'1l). SignslsymptQIilas syndrome include dehydration, fluid and electrolyte dislurbanc· es, infection, pancreatiti$, and alcoholic ketoacid"'is. and sbouJd be treated accordingly . Other medications lLSed for EtOH withdrawal it5elfioclude: 1. drugs useful for controlling HTN (ca ution : these agents should not be "sed alone beceUlle they do not prevent pTsta: do nOl use together with lI·blockers. Clonidine (SH JJOIe 5) has been exteru.oively studied, and can be given in pat.ch fOrnl (takes _ 2 days) 2. p~enobarbital : an alwmative to BDZ.. Long acting, and help$ prophyl8.llagailut se l ~ures
3.
badofen: a Smallsludy' fouod 10 mg PO q d X 30daya resulted in rapid reductioo of sympt "ertj~ cal), lateral rectu s palsy, cun,jugat.!·gllZe palsies. Gail aLaXia i.IIseen In 87%, 8fld results frolll a oombination of pOlyneuropathy. Cl're· t>ellard.Ysfunctiun. 8fld veatibular imwirroenL Sy.t.erruc 8ymptomo may indu de: vomiting. fever . M1U : May , h(IW high s iJ,'llal in 1'2WI and FLAJR images in the pa'raveDlricular (madlal] thalamus,.the floor ofth1l4th ventricle. and pe ri aqueductal ~9y of the midbrain . These changes may resulve with t realmeDI'. Atrophy of the mammill ary budj"," may alao be seen. Normal MRI does not RIO the dlagnO$u .
Treatm ent
Wernicke'. entephalopothy (WE) III a medical emergency. When WE i. $ullpect.ed, 100 mg thiarnine$hould begiven 1M Dr IV (oral route ia unre/iable,sa llbow)daily for 5 daY8 . • IV glucose CIIn pr..clPltat.e acute WE in thiamine derkle"t patients, :. give thia·
mine fint.
Thiamine adJUini~trat.ion improves eye findings within hl>Urs to day,,; ataxia and confusion improve in day~ to weeb. Many palienlll that Bumve are left. wic.h horizontal nystagmus, ataxia, and 3Ih\ have Kurnkoff'...yndrome {AI8p. uc adn!nergie n,,"'e term inal,. It ;. avaUabl" in 2 formt: roeIIine hydrochloride (h Ac ute pharmacologi c e ffe NOD· pba nn lco)ogic effects related to the ne rvous system I . pit .. it.ry deteneration: from chronic intra.naaal use 2. Cflebral vuculiti., leu common than with amphet.ami ..... 3. Hi zum' pot,ibly rel,ted to the Jocalanesthetk Pl'OP'!rtieI of cocai ne 4. cenbrov_ular a~ident (CVA. ttrok,),' A. intra~erebral hemorrhase: _ 1"'f"Gce..,bral Mmorrlw&e. E;1,'olOtl~1 on page
'50
B. lubarachnoid iM!morrhage'" OJ: pouibly .,a mult of HTN in thII, however. sometiroH no lHion it demoMtrated on angiOflTBphy". " Iay pouibly be due to ",rebral vaKuliti. C. Itchemic strolee'': may mult from vasoconl t n ction D. thrombotic s troke "
E. TIA" 6. .nterior Ipinalartery syodrome" 6. P.ft"_ of maternal rouille ..... on the fettli ne .... ou. lyatero include": microceph· aly. di.eorde.. of neuronal migration. neu ron.l differentiation and myel ination . ... rebra! i.. {amioo. s" ba..chnoid and intraee rebral h"mol"tll . . . and l uddeo in·
'"
7. Neurotox.iooloey
NEURm,'URCERY
fant death syndrome (S IDS) in the postnatal period TREATMENT OF TOXICITY Most cocaine toricity i8 too short-live-(\ to be treated. Aru fco-._ If"'.
M P.R~ . M 5 ......... I»4i.
wi ....... ".. ' A' .... JAMA 2n'
'S
""""...,dOubk·OIilOl .... roIl«I
"9·13. '994.
~"" "oC.~ ."""
F.Col'fillO E.
.,Q/, R",i 'OPII""""" of .'CII' o f o _ w~t.lrow" . M..r Le'Inically, tnIs io • SCI'" 01 _ e •• "O(> ma'lmpI'" ""''''PQnI~ range at I<Jt.II pants: 3 """"01) 10 ' S (nmnaI) _ _ i$$1Ihg eye oper.1l'e >S,
The follow;ni covers non t raumatic oomo(see Hood Irauma, paie 632 for thllt topic). Initilll evalulltion: il>(:ludea ITH!O.!IUre:!I to protect brain (by providini CB r , 0.. lind iluoose), assesses upper brllinatem (Cr . N. VII I), and rapidly identifies8urgica.l emergencies. Keep "peeudoooma" os II pol-lJible etiology in b8ck ofmilld. ApPROACH TO COMATOSE" PATIE"I>IT, OUTliNE" 1. cardiova~ulllr stabilizlltio n; establish airway, ched< cil"(:ullltion (heartbea t, BP.
cllrotid pulse), CPR ifnece56sry 2. obtain blood for tests A. STAT: eJectrolyte& (espend ouI"J B. pcnible Homar'. 'rndrome: conlIider ta.ro!.id _It>.ionldiasut lol> 3. bilater.1 pupillbnormalruu Ii pinpoill~ with m;nl.lta. .eec:tilll1 lbucan be det.ec:uod with l>illi'lifyl ng ¥I ...: ponline letion (.ymPl'tMtic inpu~ it 11If1; paru)'UIpllt.het.ics emerp at &d. i"le.·Watphlll nudeu. aDd I~ "nl,lppClSed) 8 . bilateral n~ed and dilated (1 · 10 trim): . ubtoul dllm.~ to medulla or lmmeI.
,1_
NEUROSURGERY
8. Com8
'57
diate post,.anoxi~ Or hypothe rmia (core temperature < SO' F (32.2' C)) midposition (4-6 mm) and fixed: more ex.tens;ve midb".ln lesion , prosumably due to interruption ofsympatheties and pare.sympathetics C. extraocular muscle functi on L deviations of ocular a>:es at re!lt A. bilateral conjugate deviation: 1. fron tal lobe lesion (frontal .::.enter fQrcontralateral gaze): looks toward aide of dea: n.octive lesion (away from hemiparesis). Looks away from side of /leiZllre focus (looks at jerk.ing side), may be statu s epilept icu &. Renex eye movements (ru below ) are norm al 2. p..",tioe leaioo: eyes look ~ from lesion and tooYards he miparesi s; caloriC!! impai red on side of lesion 3. "wrong way gaze": medial thaJamiehemorrhage. Eyes look aw .... y from lesion and towards hemiparosi . (an exceptioo to the axiom that the eyes look ~a destructive $upratentoriallesioo )' 4 . downward deviation: may be associated with unreactive PUpil8 (Parina ud's 8yndrome , oei1 pag~ 86). Etiologies: thalamic Qr mid brain pretecta l lesion s, metabolic coma (esp~iaJly ber bitW"ates), may follow a llelZUro B. unilateral outwa rd deviation On side of Iaeger pupil ([II palsy): uncal hemi· ation C. un ilateral inwa rd deviation: VI (abducens) nerve C.
O.
!;k~1l!i.2n
1. III Or IV nerve/nucleus lesion infratentorial lesion (frequenUy dorul midbrain) 2. spontaneous eye movements A. 'windshield wiper eYell": random roving conjugate eye movements . Non·l .... calizing. Indicates an intact 1lI nucleu s and medial longitudinal fasciculua B. periodic alternating gaze, AKA · pin&"·pong gaze": eyes deviste side to aide with frequencyd - 3·5 pe r seoond (pa using 2-3 &eC$ in uc h direction). Usu· ally indicates bilateral cerebral dysfunction C. QCulp r bobbing: repetitive rapid vertical deviation downward wiU, slow re~urn to neutral position. Pontine lesion (Ht paIJ' 588) 3. intern uclea r ophthalmoplegia UNO) : due to Ie. ion in medial longitudinal fasciculus (MLF) (fibers Cr0531ng to contraiaterallil nuc\eua are interrupted ). Eye ipsilateral to MLF lesion does not adduct on spontaneous eye movemen~ or in response to reflex maoeuvers (e.g. calories) ( sa P<JCI' 585) 4 . rolle .. e)"l mo\fflments (maneuvers to test brainstem) A. c>cw Qvest ibuln r eneltna 4.
1IJI$l.IIrmul without IOnic deviation (i ....Y" f8ma ;n In primllry lion) virtually di/lll(n08t ic of l'IIychopnlc wIDe c:ontrelatualaye r.u. t.o adduct: [NO (MLF lealonl
6.
~i.
B. optoki ne tic ny ltermu, ~oc. atronal, IU!;!;"ta paychOgo nic eon,' D. UlOI.Or: mu aele tone a nd l'1!f1exea, t8lljlOilBe W pAW. Bobinski (no'" a.ymmetries) I. ap propriate: impJi" cortiw.pi nlll trleta lind eort.x ",tact 2.. 8.ymmuric; lupl'lItentoria l lylon (t.one Ulually IRcrenO'd). unlike ly In meUiboJic S. incon, i.tentJv.ri.blt, HilU ~, POIytroBtnc 4. eymmatric: metaholic (""1I811J' decteJIl!f:d). Al tarixil , l!"tmor, n'yoclonu, rnft, tw present in metabolic: coma
..
5.
I!yporene;tlow ) ~
".roII
COMA FROM 5 UPRATEHTOfilAL MASS"
CantrllJ and unul hemlll t'or. eoeh ca U_ " differtil t fOND ofroatraJ-eauda l ae' . ";orlltiol1. Cfll tra l htm,at>on , H uh. In Nqlle"ti.1 ra;tu", 01' dian«phalon . midbra in. pon •• medulla (arf fHl6' 160). For ul\C8l herniation , 11ft' pap 161 . ' CllIu ic" l ien' ofin· o;rellSed ICP (HTN, bradyurdia , .1te~ ,,"piralor), patUom ) lUIUlllly MIn with p-fOM. Inionl may blabHn~ 111 alowly d~'optl\( au pflteatoria.! mlti",:tion betWl!tn cl!tl lTal and until hl!rnill ion i. ditTi~ull .. hen dysl\.lncti!>n ruche. Ihf- midhrai n 1,..1 gr below. Predielin. the roeatlGn a rthale4k)1I bAM m._.
hemiat.lon ayndrome ill unreha ble.
Clinical c horacteriat iu d ifferentiati"'i u ",c.a l from ctmtrlLl h e rniation dt\:reaH Differential di agnosis of s up-catentori a l etiologies L 2. 3. 4.
Mu Ltiyariate analy"s yieLds (XIt· come prognosticator'll shown in Tobie 8·:; &06 Table 8·6. NB: this lI.Oaly. l, ~pp1ies on.1:t to hypo,ao;..ischemie coma'· More r~nl6tudinwnfinn the poor prognosis of ..mrellct;ye pupils and !ack-ormotor ..,sponse loG pain"; if /lither orthese findi np 8M $/len within a rew houllI lI.l'ler cs,rdillO!aTTffl tbfte ;11. 8n 8O'lI ris k ofduth Or permanent vegetative I Ulte, 8nd if present at:1 day. these th'. r8le t(l9f 1(1100%. Clurocortiooids lau-roids) hllY~ ~n ahown tohll.Y81\O bt:neficill ~trKt on ~ urviy.! TaU- or neurolog'iC. V',_ A B. H"'Y" R Il!C""'V.o;)" ............... .,,'''',,, I/w 1onJd.J";""" p ,. • ,
, ,
II",," V l.aw..,C.YonIo0 BRAiN DEATH CRITERIA
lWcommendatlOD sL. ' : 1. !lbsenc&0I DrI_irISIem 'elIeUS A. absen'-"!of brai"8Iem refl ca·e.: A. liud lll4"~ t. lXular eumination: 6 BbsenI comeal relle.(eS A. rlX~d pupila: no ....~Pllnse. to bright light (tau: ion alter reaus, C. absfnI OC\IIOYestIWar ~tle~ citation: H e bflow). usulIlly midD. absenI ~hallc po!Iitiou (4·6 mID) bll ~ may VB"" E. BCseoI gag &. w.; gil IeIlel: 1.0 dilated rlngeB (9 mm) In size 1. B. abJ;ent corneal Taflel"'I!lC 3. no r~ to d\'ep~ pain C. abHnt lXuloc:..phalie (doll's 4, 'oi\I1~ eyes) reflu (coll lrain dicated If C·spine not elesr edj, _ {XlB" A. tori! temp > 32.~' C (90' F) /58 B. SBP '" 90 rrrn t'Ig D. ,b....moc ulovQ tibuJllrren .. '" iccld wewr ca Jo rl ell): it1lltill6(l.. 100 ml iea water into one eaddo not do ifTM perforal.ed ) wiLh HOB at 30' . Brsin death is e~duded ifan), sya moveroent (... ~ IJ06('. r58l. Wailalleut 1 minute for responBII, and::t a min before testing the op~it' side 2. absent oropharynC .. a! ....1'1,:< (gag) to stimulation of pDJIt.erior pharynx S. no rough TeSpOnsa 1.0 bronchial auctloning B. a pnea leat AKA apnea c:w.Uenge: nospontan~ou~ respirations D lifter diSCOM&L:!tion &om ventHaloT (aases_ function or ln.duJla). Sioce elevating f'aC01lncrease.ll tCP whl~h oould prec:lpi tate h IQ..,
EEG
ell! ~ do,", III bedlitl... R.eoqui... . .,;.nced int.lrpretar Don not del.a(:t brain. • t.lm artivity. and elec\rocerebr.l.il.nce (ECS) d ... not oelude the pouiblUly 01 ..... venibl. coma. ThuI, I I leul a:..bmu:a ~rvillon if, n!()Ommendad in cortiUDc;tlon with ECS, U.iDg RCS . . . cliniu] Ql)nUru'lltory t.I, t . lIould be doni only in pui. nu without drug intoxication, hypoth~ru'li. , nr . hoek. Definition of . leetl'O«lnbral all.nee 011 EEG: no elec:triwltCfiYl t)",. 211V with th e following rtquirelDl!nt.l! reeord inll'r absence of buLbar mU8Cui.aUlte movement: including oropharyngeal lind facial rnu~cll'll; absence of corneal, gag, \lOugh , $uck, lind r!lOting reflex 4. llhoien"" of ro~pi ... tor:v movem0at (usually tl!;lted alter other erilens met) 5. flacci d tone aod ahoieoceof5pont.aneoua or induced movemenUi (spinal myoclonus and spinal \lOrd I.llOvemenu, e.g. renex withdrawlare not included) 6. examination r""ulb should remll;n clllU"leZl~with brain death t.hrllughout observation periOd observation period~ according to age, A. in nawborns born at or art.er term (:> 38 wks): 7 days B , age 7 days · 2 m(llS: 2 l!Jl3mination.s and 2 EEGtl48 hn apll.r! {repeat ualn unnOOl!5Sary ifcerehrnl rlldionuclide IIngiDgl"Bm (C RAG) (ai .. to y;suali ZOl cerebralllI'tl!riesJ C. ai" 2·12 nlll~: 2 ua..rninatiofUI and 2 EEGs 24 hn apart lr"peatexam unlJ 100 with normal CVP) 4 . dise_ of the organs coosidered for dOJ)ation 5. anencephalic newbom~ : recent consensus is that the functioning brainstem in these inf8J1tJ; (e.g. spontaneous respirations) di9qualilie~ them froro the diagnosis orbrain death (furthennora, few such organs would likely oonefit others)" G uidelines for inclus ion (some recommendations from refe rence" included) These guidelines are constantly being revised . in part due to '".proved results with the use of cyclosporin in recipients. In general. consultation with. a transplant coordina· wr ia recommended to determine appropriateness of donation . 1. brain death in a prev ,ously healthy individu~1 2. organs: A. kidneys, age> 6 roO! (because oftize). Normal blood pressure, BllN.serum creatinine & UlA. No S I..E (because ofposllible lupus nephritis) 8. heart and beartlluolr. ageideaily" 40 years for males and "45 for females (above these ages. a cardiac catb i8 usually performed) but up to 60 yrs may be used depending on condition of heart and potential recipienta). Euro by cardiologist ind ,eating 00 heart disease (cardiomyopathy, valve defect. reo duced ejection ('-""tion. severe ASHD. SIP CASG). No roDM C. liver: age > I ".os. Nonnal hepatic fuTlCtion (nonnal Or acceptable AST, ALT.I..DH, bilirubin (direct , indirect & total) 8J1d nonnal dotting studiea) wit~ no history of liver disease D. pan cre as: age 15 - 40 yrs . No history of disbetes. Nonnal serum glucose and oroylase 3. tissues : A . J:lI.m9!: age" 1 yr. Neither cancer oor sepsis di5Qu"lifiea (rabiea and CreutzfeJdWakob disease are contraindications) B. Din: "ge 15-65 yrs. E~c1uded if cancer C. il®l::; age 15·65 yrs . E~duded if cllncer D. bone marr!lw: age ~ 50 yrs
'"
9. Braindeath
NEUROSURGERY
E. hgart valves: age" 55 YI"II
9.3.2.
Organ donation in patients with brain lumors
Asnong patients with a hrai,n tumor: 1. those that al1llll!t tandidnIQ fOT O'l[1Ul donotlon : A, meta"talk tumol'll to the b ra in B. brain tumcr~ rnat hllve been manipulated (biop.sied Or I!:lcised ) C, p/ltienl..$ with br/lin tumor" who hlV(' been 6h..."ted 2. tholle thAt m;ghtbe candida~, but eonlide.eII higl,..",k donora'" indude p,;p"IDtitd: A. glioblaslOtnIl rtlultifonne B. a naplastic astrocytoma C meduUnblBllOmp 3, unmlln;pulpted tumors that might not be considered high nsk A , henlsngiobiastnma B, meningioma
u.nma:.
Optimally, ifno mela!l!.ll.seSOI1l seen on CT,chest, abdomM lind pelvie) And no meu are found /It tune oforlClin procurem~nt, a brllin bioP"Y would beperfo,m"" II..fl&t Lhe 01" gan , aTil procured li t the same anesthetic bnd the organs would not be ~ele3Sed' until the biopsy prove... which of the 1I1x we o:IIU!gonl!.!l applle.s ,
9.3.3.
Management after brain death for organ donation
Note: OnCe b""i n dea th 0«Un. cardiovBlIC ular instability aventll.lly ensu e., genef'/Illy within 3·5 dayo, and management with preSSOI"ll; 8 u"ually requinod. Fl uid and elKtrolyte imballlnces from 10M of hYJIothalamk regulation mun be non:r"dl ~ed.ln eQme instanrtlS a beating·beart cadaYH can be maintbined for months". 1. ~osent: mustbeobUlined from donor'. legal guardian. NB: mUlltala.nance B. un c>olloid IFrP, a lbumin .,,) if unabla to rnalnlem BP by replacement C. UIIe V"Opruso ", if 8tiU hypotensive. Start with low dl'lSll dopmrn;ne, h~ c.... a~e u p to - 10 l'gikg/min, add dobutamine ,(still hypot.enS;VD at this d!l!le O. If UO;s still,. 300 mUhr afte r above lIleaSUri!$, U!iC ADH anlliog ' aqUfOUli v8llo prea:l i~ (Pitretl>in®J i. prefem!d over DOA VP to a void ren8J. hutdovm ) thyroslobulin given TV convertl50me cent from anaerobic to ,,~robic rneta\lolillm which rnay h elp .. tav~ oll'CIlrdiDY3i1Ctlla. collapse
LABORATORY EVALUATION U
Gel)er a l iDitiallabll I. 2,
3.
serology: VORL ~r RPR, H8&\(, HIV, CMV,ABO blood group, HLA tiS8ue Lype dtellli,t.ry. eleclrolytes. gJUCOOle, BUN , ( reatilline, cal(iulII, phOllphate, liv~r f..."I:' t ion t.ests, U/A ( unn~ D.nD!y~i$) hematology: c ac , PTIP1"r
to"
A, h,fb ·rlok ."...ns ".1 b< OOlI.id .. __ pou,ru, . s. ... oIoc- H' 108\1·91. 1984. J.. ' ........ I M S. Pow"" D. S..,.-d" I." ~t" Sponu· """". olr.,. ""..
GI-oaIJ 101«1 HO: 119.
"
Goc>dm>." J M.1l«1; Ll. M<m B o ,conr""",.,. 011n;,.0..1I1 wIlh 1IOf" '" _ .., """,,",' " ..... wof2G4_"',"" ....... N.. """..." 16: '91·7,19U 0"",,"" J .l'nud I. Comparison .1 .... 0l."Il111 CCIII'II during 80me Qr th~e procedLlres . An actu'" reliction t of all p8tienl.! with bead ;'I.1UI")'. 6O'lb ccur within days oftrallmli. 95% within 3 n,onthBU, 70% (of CIl$e;I oI'CSF rhjnOlTheo It.op withIn I wk, and uSLlally within 6 mOl in the N5t . Non·traLlmatic fQeS cease apontlllleoualy L'I only 33%. Adut~child tati" il 10;1. 1':Ilrf! beroN ago, 2 yn. AnO!lmJ.a is.eommon in trallmatic leaks
'"
10. Carebrospinal Ouid
NEUROSURGERY
(78~),
TIIN!;n sponLln.., ... " . Moet (8Q.85%) CS F otorrhu ceases in 5-10 days_ CSP liltull oecurred in 8.9% of 101 easel of pe netrating trauma, and intreases the infection rat. over those penetrating injuries without fistu la (50% vs. 4 .6~)". It i$ reported to occur flO't·op in up to 30'l0 of eases of .kull·ban surge.,,".
SPONTANEOUS CSF FISTULA
Nontraumatic leaks primarily occur in adul", > 30 y .... Often inald iou5. May be mil_ ""ken for allergic rhinitia. Unlike traumatic leaks. these tend to be intermittent. the ...... of 1mell i. ulu.lly prtHrved, an d pneumocephalus ia Wlcommon". Sometimu lMOCilted wit h the following" 1. Igenesi. of the noor of the anterior faua (eribrifonn plate) or middle f _ 2. empty leila . yndrome: prima." or poSt tranAphenoidal ,urp." (He poet 454) S. increased ICP andlor hydrocepbalus 4. infection of the p8ranU81.intUles 5 . tumor: including pi t ui""ry adenomas (He p4fe 438), meningioma. 6. I pe ... il tent rtmna nt of th crlniopharyngeal ClInal" 7. AVM" 8 . dehiscenteofthe footplaLl of the ."'pes(a congenital Ibnonnality) which can produce CS F rhinorrh.. via the 11lItachian tube"
P08terior fossa 1.
2.
pediatric: usually preaents with either meningitl. or hearinc lou A. preserved labyrinthine function (hearing and b.lanee): these ulually prtsenl with meningitil. 3 ulull rolIteaofr.. tula: 1. facial ~llJlal: oan fistulize into middle ear 2. petromastoid canru: along path of arteriallupply to 111lI 1.3 ,ulI"ta leak . If no le~k , the no. can be repacked and the study repea:.ed the following mom ,na· Leak. into fron tal sinul will ampt.y into nllOpharyu anterior I.laIOlde (260 mg PO QrD) t.o /"l!dUCf! CSF pN>dudioll D. modest nu;d A$tridion (eaution po OR
3.
B. continuous JumblU" drn..in.age (C LOt via percutaneous catheter. Ke-ep HOB el""al.dache. Atypical patients bave been described withcu.t lilA , or HlA tbat i$ !)On_pOiIitional, without pa.;hymeninll:e.. l enhancement on MRl", with dinicalaigns of encephalopathy, cervical myelopathy, Or parkinsonism". Sinti! some plltients roay have normal intracranial preuuTe. the term ·CSF hYJlD"olemia" has beeo suggested'". MRI evidence of brain df!S NEUROSURGERY
Ei Many mean8 to di li tingui.'lh hydrnnencephaly lind HCPhave been descnbo>d , and j"dude: 1. E£G , $ho w.! no ~'1I1iC1llactivity ,n hydraneneephaly lmuima] HCP typically produces lin abnormal EEG, but background activity wUl bE presenl throughout the. brain'l) and i. one of the be!;t way . to diffe .... ntiate the lwo 2 (;T). ~, MRJ Or ull1"UOund: mojo";ty of mtrlloranial space i8 occupied by CSF. U5ually do not see front al lobE ... o. rmn~l horns of lllteral vl'ntricleB (there may be remn.nts oftempcTII I. DCc ipiUlI Or lubfmnw corte..l. A , (nJcl ... re ton,istl ng of
'"
II Hyd""'ephalu,
NEUROSURGERY
bra iD$tem aodule (r(lundd thalamic roas$es. hypothalamu s) and medial ocelpitallobes sittingc)O the tentorium oeeupie. & midline position . urrounded byCSF. Posterior f\lS5a structures are grossly intact. The falx is usually intact (unlike alobar hoioprosencephaly), and is not thickened, but may he. displa~ laterslly. In Hep, some cort~aJ mantle i, usually identifiable 3. trilDsillumln a li o D of the skull: Ii hydroc T rea.tment Motl.urteONI advOCllte shunting the vent ricle ei lher with a separate VP shunt, or li n king into an existing .hunl. PotenUal complia t ionl ;nc:lude delayed injury UI the brainltem by thl catheter Up " t he bra'NI!em moves in Ul i~ norma l position with.drainage ofl ha 4th ventridl. Thilln8y be avoided by bringing lhe CIIItheter inlo the 4th ven· tricle 81 a .Iight aogle through t he cerebella r hemisphere. A Tork.ildsell ahunt (ve ntricu lociste roal th unt ) i. an option for obstructive hyd ..... cephall.. if,t i. certain that the ar achnoid granulationa are functional (\dua lly not the CMe with hydrocephal .... of infantile onMI). All LP . hunt may be considered WhM the 4th ventride outleta an patent.
CTfM RI CRrrERtA OF HYOROCEPH ALUS N uroerolll methodl h,,,. been deviMd UI attempt UI q uantitatively defilM hydrocephalus (HCP) (mo.! date back to the N rly CT experieoce). Some ... prt!!ented here for COOlplel HydrostatiC b ydrocep hal us Hydrottatic HCP i •• ugguted when eilher": A. the I oU of both tempOral born. (TIt) i.", 2 mm in width tift Fi/llOre 11· 1) (i n t he abMn", of HC P. the tempo ral homl .hould be berely vi,ible). and the Iy lvian'\ interhemi. pherie fi"url' Ind cef"flbral uk; are 001 vilible
OR
'"
11. Hydrocephal us
NEUROSURGERY
~ 2 mm , IruI the ratio ~~ > 0.:1 (",hel'll PH i. the lalllest ",idth of the
6 . botll TH ar.
frontal horlU, and ID ~ tll. l1Ilemal diameter fr1>m l.n ner· Ubl. to j"neNlble at thi. level ) (_ r-r /1 . 1) Other foatu,," IUlge,t,ve ofb.xd..Gt~ hydroceph·
F" .
1111&: I. t..lloonilLS of fronu l homs orrete •• 1 ventri· de. (" Mickay Mouse" Hntric]es]lrld 3n1 V~n· triela :l. pfriventricuLar J.o.N deMit,)' on CT, or periyentrkuLar high lnt@n~Lt,..ign .. l em T2Wl Oil MRI eUi!ftUIIlS trIInlepeodym,J IIb.... rpLioo Or milP'lIt;OIl orcs!" 3. II&ed along, tht ratio
'H! :10"10
$~Uesl.l
ET10LOGIES OF HYDR OC EPHALU S
HCP i. either due to luboormal CSF "'lb· sorption. Or rarely to CSf' overproduction ia.o Tabl.11-1 HCP In 170 ..vith wme choroid pie."" papilionlU; .... en here, reablorption i. probabl)' defec:tive in 80me &5 IlOrnUll individuals ~ould probably 1001"II\e thuliglrtly el eYlted CSF production 1'lI\e oftheMl tUIllOI'5). The etiolOjliN in onesenel of pediatric patienls lI5hltWn in Ttlbk 11. / congenital !t, Chlan Type 2 malformltioo and/or myelOmeningocele ( MM ) {ulually "'cur together) B. C hiari Type 1 malformation: HCP mIIY occur witb 4th ¥i!ntricle out.letobI truction C. pnma.,. aqueduclal . teno."" tUlullily p ........ t.o io infancy. taJ'l!ly;n adult-
hood ) D. uc<md ...,. Iqueduclalpiosia: due t tl ,ntrlulotrirw infec:tioo or ge,,,utU\! m~· Mmorrhage" E. DaDdy· Wall..,r mal formation: alrellia orfonlmina or Lughll .. &. Mar~nd. it 1_ PflIl //01. The incidence orth~ in patient! with HCP 112.4% P. ,..'" X·linked inhentH disorder Kqui red A. intKtiouI(the .....' common (3~ OrConHDlllliutinr HCP) I . pCIoIt-lI'IfninsitiA (upec:iaUy pllt\l~nl and basal, Inc)udingTB)
tn.,.
2.
t)'AWmI6LI
8 . JIO'" .... mcnharic (2nd IDlIISt common CIIUIW! ofcorn munic:aLinG HCP) I pINIt·SAJ-l lMc~783) :l. poIt-in~.v.ntticular hemBnhage ( IVB ], man)' ",ill develop laI.Il4iln\ JlCI' 2().SO" olJ'l'tienlir with la'lle IVlf d""elop perma nent Hel' C. HCOndary to II"IIMH I . non neilplastic: e.l, v.nuJa. malfbnnation 2. lleilpJllltic; mosl prodUCI oMlructive HCP by blockinr CS ~' palh",1IY1 E. r>eu rosar3olll';OIl. H_ev~r. M be" Ih i. .hould only "" colllid"red u lin ~djund to definitive trealment or a.s 8 temporizing I'IlflaluN. Seli5fact.wy con trol or Aep '"'11$ reported in _ ~ ofpatienu ofare (su ~Iow ).
Complication s: 1.
hypothalamic uuury
2. t ransient 3rd and 6th nerve palsies 3.
uooootrollable bleeding cardiac aTTest" 5. traumatic ha,ilar artery aneurysm": pO$.9ibly related to thennal injury from U911 of lase. in performing TV Succe$8 r ate: OveralllucceJ;;S rat(> is _ 56%(rangeof6l).94% for nontumoralaqueductal stenO$is" (AqS)). Higheat maintained patency rate i5 with pnviously untreated Il(:qu i~ AqS. SUCnllllic h,.. drothorax nece8IIitallnlf relocating d istal end . Recom~ only fM peuent.s" 7 yn.1t'! a, gall bladder C. ureter orbladder: CIIU",", electrolyte imbala_due!.O 10..... throuch urin .. 5. Lumboperitonea' (LP) ,hunt; A. only ror communie.rtinll HCP: pnmariiy pr;eudolulDOl' teTl!bri or r1.&lu)s". lhIeru l in lIi tullionl with IImall " .. ntricles B. o"er ale 2)""S. perculaJl~ul iDliertiot\ with Tvoby ne.IJe ia pre~~ 6 tyIIlor llubdursl,hunt: from 1IJ1Ielwoid CYlt Or 50bdural hygram. ClIvi!)" ususlly \0 ptriwne ul1l
csr
Djl!adyantages/con;r pi icationa of varioul ahunts I
thOMlthat m.y occu r with lillY 1hunt: A. ot..truction: the UlOIIt common cau'" or,hunt malrunction • proximal: v~ntrieullr CIItheter flhe mott tommon "i~ 1 • valve ml!'Cllanilm • distal : reported incidfflClof 12-3-4","". ~ in petito"'!a] cathel.er in VP Ihunt 411ft below). ln atrilll CIItheter in VA . hu."1t B. diKOnnection lit ajunr:cion, 01" break at IIny poIn t C. u.feclion O. hardw ..... ef"OlJlon thl'OUlh sk in, usually only in debilitated plltienlt (espedally preemies with Inlllrpd herodJ; and thin SCII lp rro m chronic " CP, who lay on OfIe aide of head due to elonll"lt.ed cr.nium). Mil,)' a lso indicate .ilicolHI allergy (_ belOUl) E. KWi1U (venlricula • • hunl.lonly}: (hue il - 5.Kriskol",ri~ul"f.in the first year aft.er pl_1Mn1 01. ,hunl which drops to - 1.1" ancr Ihe 3rd y ...... INa; this d_ nO{ IIINn th at the I hUll! 101" the caulII! or.1I of th_llri,urM) Sei~ure ritll ItqustlolUlbly hia:herwith ,","1.11 CIIth.,tertthan with parieto-lI«i piW F. Itt nl conduit fo.ntnlniuraJ mfllMlUftofCflrUln tu monr (e.1o medullo-
bI •• toml ) Thi, i. probably I relativlly low n&k rr G. "licone .Ue..,... rsrelifitOtt\lrt.1 1m. May 1'fIM00bIe .h uM InfKtion WIt h ,ki n breakdown and fllnptill(lI'.nulomsl. CSF i. in,tially Iterile bu~lster infection, mil,)' oc~u r. May requi rl f.tobrication of a ClJllOm $iJicone·free d .. viCII.(e.lf. poI)'urethane) 1. VP lhuDt; A. ,7'" ino;:idO!I>re inguinll hernf. {",Iny IIhuntl are il1Mrtad while pnlCIIIL~UII "I" nali, is patenU"" B. need t.>lengthtn artMI.fI" WIth ,",wth: I1Ily bt obviltAro:! by usin, 10111 peri.
08'
NBUROSURGBRY
too"ol catheter (IOU page 621) obstnJctio;>n of peritoneal catheter: • may be more likely with distal ~lit openings ("alit valves") due to occlusion by omentum or by trapping debris (rom the ahuntsystem" • by peritonelill cyst (or p8IIudoc)'!lt:r"': ""usHy lL$$OCiated with infection, may alllo be due to reaction to talc from aurgical g loves. It may rarely be necessary to differentiate a CSF collection from B urine collection in patlen!..! with overdistended blpo:idel"$ that have I"\Jptl1J"ed (e.g. seeondary to neurogenic bladder). Fluid Can be aspirated percutaneously and analyud for BUN and creatillille {which should beabsent in CS FI • severe peritoneal adhesions: reduce surface area for CSF resorptiOIl • malpo.'lition of catheter tip: at time of surgery: e.g. ;n preperitoneal fat tubillg may pull out of peritoneal cavity with growlh D. peritoniti s from shunt iofe ..
,
_ !low
!low (lode Yiew)
FIg"" I 1-$
PS Medio;aI-.cIatd -....:I "I~ NEUROSURGERY
nnw into the \il'ntridl! du ring the .)\,,,t step). The n whlle main:ainin& t his Pre5!lutll. (II!'ptell5 the ruervoirdome with 118/!(X)nd fin,ar. Ro!lel\se both finGers, and repea t .1'1lE! One-
way vllive reguJate8l'ihunt pfeMu re and p.... vl'nlll reflux of CSF during normal during the rel w;e
and
X'rpy c ba r acter iiticii The lhree ava il able valve pressuret an indicllt.ed by rndio-opaque dou. on th~ .,,,,lve (allow5 x-ray identification Qr"alve preuure), one dot :. low prell!lur~, two dots z medium. thfSle (lotll ..
high
r;.~:r~~~~::~~M~EP~,~:t::~,..
_.. ..... STB> 1
FIgura 1HI
Pu"lf'lng!~
cl!tprllSS poolp ctIamber
PS MedIcal vaIYe
Strata® program m able valve The Medtronic
Strata valve is aD utemll.llya!(jUlltable "alve thllt i. pro8Tllmmed (Ulling II magnUjtooneoffi"e perfof[oaoCill evel I"P/L- ) setting!! (Filllire J J-7).
Bi!cause tile
YDlve mil)' be inad_
venently reprogrammed by external magnet.a, the patient must be in formed to have the \/lI]ve setti llg thecked aFter an MRI performed rOT IIny reaSOn .
SOPHYSA USA Sophvsa USA, Inc;,
760 WEtSI 161h 51.61dg. N. Costa Mesa , CA 92627 USA (949) 546·6484
www.sophysa.cgm
P o l" n .. p ....gy...... m .. bl .. vw" .. The Polaris"V ... I,,", ial'" H lernally programma· ble valve thai \ISe!I two attracting Sarnor iUlDCobal ll1lagne.t8 to loel< the pressureaet\ingllod to ,.,.ilJl. inadvertent reprogramming by e.",i. ronDlent.ally encountered magnetssl.lch at MR! 5el!onen>, oelt phon"_ headphon es ..
Available in 4 rnooels (dim-rent preasure ranges. ea~h identified by II unique nllrn· ber ofradio-opaque dotal, each wit h 5 extemally adjustable posit ionL The ~-ray appeara nce and correBponding pr\l.'!SU'1!S lire .hown in Figu.re 11..8.
NEUROSURGERY
II. Hydroo:ephalU5
'"
NEUROCARE Oisllt (not a true shunt): A. periodic withdra ... al of accumulated fluid B. for injection of radioactive liquid (usually phospilorous) for ablation TECHNIQUE
(For LP shunt. see LP shun' ~!JfJ1ualion. page 622). There is a risk ofintrodudng infection with every entry into the .hunt system. With care, this may be kept to II minimum. I. ~h a ve area 2. povidone iodine solution prep _ 5 minutes 3. use 25 gauge butterily r>eedle or smaller (ideally a !>Oneoring neentll specifically designed to be tapped
To measure pressures Steps are outlined in TaMe 11·2. Table 11·2
a s hunt
neous IIow into btmertly tubing;
S-i.J.e in manometer
IN STRUCTIONS TO PATtENTS
All patients and families ofpatienta with hydrocephalus should be instTUoted reo garding the followjng: 1. signs and symptoms of ahunt malfunction Or infection 2. not to pump the shunt unleu instructed to do SO for a specific purpo:se 3. prophylactic antibiotiC!!: for the following si t uations (mandatory in vascular shunl.!i. somet imes recommended in other shu.ota) A . denUlt procedures B. instrumentation of the bladder: cy~l.O!!copy, CMG. etc.
'"
II . Hydrocephal\l.!l
NEUROSURGERY
4. in 0 growing child: th~ need for periGdicevaluation, including asses&mentofdi.r;tal shunt length
UNDERSHUNTlNG The sbunt malfunction rate is _ 17% during the finlt yea r of placement in the pediatric population. May be due til one or a numbe r of the following: 1. blockage (occlusion ) A. possible causes of occlusion: 1. obstruction by choroid plexus 2. buildup of prot.e.inaceQus accretions 3. blood 4. cells (inflammotllry Or tumor) 5. secondary to infeclian B. site of blockage I. bloek.sgeofven tricularend (lTI(Istcommon ): UBuall,y by cbaroid ple"u", may also be due to glialadhesioru;. intraventricular blood 2. blockage ofint.ennediate harowan! (valves. connectora, etc., tumor fil. ters lIlay become obstru~ by tumor cells, antis ipoon devi~ may dose due to variable overlying subcutaneoU8 tissue pressun!~) 3. blocked diltal end (U': after tapping the shunt, drain 2·3 roJ ofCSF and seml l rnl erCSF fo r C&S. l'1,iect radio-;sotoJle (e.g. for VP shunt in an adult, use I roC; of-1'c (techneatit B. these patienta m.y occ.as.ionaUy present with l)'Dl ptoJUlllnrelated to the . hllnt, e.g. troe migraine 2, 51it ventriclel)'lldrome (SVS): _n in < l~ ohll shunted patil!ntl. Subtypea: A. intennittl!nt shunt occl usion: ovel"$hllQtinglead. to ventricular c:ollap$f (Ilit ventricles) which caulles the ependymal tioing to occlu de the inJet porta of the vlntriclll.. catheter (by l:OIIpt.ation) prodllCing s hunt obIItrllCtion . With ti...." many oftheH patientl develop low ventricular oompliance"'. where t'Veo minimal dilatation results in high p","ure which prodUCl!ll Iympt.oml. Expanaion then f'lentu.lly reopell8 the inlet porta.Uowing resumption of drainage (Mil« the intermittent Iymptoms), Symptom. may rnembleahun t malfunction: intermittent .... ad.d..!s I,lfIrelated to pMtu.e, onen withNN, drowsiness. irritability .nd impaired menuUon. Signa may include6tb cranill nerve palsy. Incidence in Ihunud patientl! 2·~""""_ CT or MRI .can. may aJao .how t'Vidl!nce oft ...... epeodym.t .bilorption o(CSF B. totll,hWlt maUimttion (AKA nonnal volume hyd.oc:eph.lu"'): lilly occur Ind yet ventricles remain Ili t-like if the ventricl .... c.nnot expand betau. oI" l ubependymllgliol;'l, 0. due to the law o(Leplaee {whidlltatH th.t the praIIU", required llIeapand .I.a.,.e container .. 1(....... thIn the pre"u'l! reo quired to expand. Im.U container) C. VInoU' hypertension with norm.llhWlt funttloo: may result from paTti,1 veoo ... ocdlliioo that 0«11" io 110m. conditionl (e.J..t the lev.1 of th,jug_ IIlar foramen in CrouUln', Iyndrome)_ U'lIllIy sub.idea by adllithood 3. ....... patients with IdioPfotbic intracrani.1 hyputention (ptI!udOtllrno' ce.ebri. _ PfJIll 493) ha", .1it·Jika ventridu with COf\Iillantly ,l,vated ICP 4. intraCTInil1 bypotl!n.lon: .ymptom. onen relieved by recumbency (_ llbotoor)
EVAlUAnoN OF SLIT VENTRIClES Th e ,hllnt v.lve fill ' .Iowly ifpUJllped whl!n thl! ventrides Ire ooll_pHd. Monitoring CSF preesllre: eitheT via illmbsr dr.in . or with. hllttl!rOy in ... rted into the ,hllnt re ... rvoir(wltil thil method , preU llrecan be followed during poItll.al chan ges
NEUROSURGERY
",
to look for MKali~ prn, untt when upright; possibly higher risk of infection with thi~) . The.. patientl Ire . 110 monJtored fo r pr.U unlt spi kes, especiany dunng ,leep. Alternatively, th eM patiwu may be evaluated by "shunt-o ·gtam" (Ht aboutl. TREATMENT
In Iru tinr II pa tient with . Iit ventridell in imaging studies . iti, Impo rtant to aacer· tain int.o which of the " e&tegories (8ft .. bow) th e patient fal1a. [fthe pati ent can be catego:lrited, then the '!!al pres.ure h)"d rocepb.l ul 'ldeTl U n reliabl y open the device in an emergency B. in non·ahu nt dependent c. _ 1. .ny of the methods outlined sbove for sh unt dependent cases, or 2. temporari ly tying 01T the.hunt" C. inse rtion or an antj-aiphon device"' 2. draio.gl or the subdur.l 6pe<e to A. the ci!~",a magn.'" 8. to the peritoneum with a low presllure valve (or novalve"). Some authon have the Ure-gLver rrequently pump the su bdur ..: valve The goal iA to acb~ve a deli ... te balll...,., bet~n undershunti"ll" (producing symptoms of active hydrocep/lalua) .nd overshunting (promoting the nl.um of the SOH>. Fol_ Lowing lurw"ry the patient should be lI)()bili:u!d slowLy to p~ent recurrence of the SO H.
CRA NIOSYNOSTOSIS, MICFlOCEPHA LY
& SKULL DEFORMITlES
Also see Cronwl)'tWntotir. page 99. A number of skull chan," hav, bHo deacribed in infants after shun ting. indudin,",,: thickening and inw ard growth of tile bone oftbe skull base snd crani.l .... ult. decrease;n li:u! or the sell. turcica. reduction in lile of the cranial foramina . and cnmio. ynostosis. The moat common. . kull del"o.lII ity wal dolicl!ooeph.ly from llllgittalsyno.to&is" . Microcephaly lICOOunted for ~ 6""of skul1 derormi· ties after shunt,nc {.bou t haJfofthese had IRlgi Llal 1)'I"I05\ol1i.). Som, ofthoete ch. nget werl reversibl e (eue pt .... hen complete I)'1IOIIlO5il WII present) ifiO\.raCl"ln iai lIypert..n · lion recurred.
11 .3.
Normal pressure hydrocephalus
. I
1 Key feature s c1.ssic triad : dementia. Ca it disturbllnce. uriMry ineontinence • eommu nicating hydroceph alul on CT or MRI • DOrmat prell$ ure on r~ndom LP • symptoms remed i.bl, with CSF . hunting Flnt d~bed io 1966". nonnll l prenure hydrocephilu l (NPI{), AKA H.kim·Adsm. syndrome. ~ originally deacnbed. the hydrocephalul ofNFH Wit considered to be jdiAAllthk.
NEUROSURGERY
11. Hydroeephlllu.
".
However. in some ca~ an etiology can be identified: 1. poat.-SAH 2. pOIt-traumatic 3. post-meningitic •. following pntIterior fos!a .urgery ~. tumors. including carcinomatous meningiti s 6. also seen in ~ l~$ ofpatienUl with Alzheimer's d isease (AD) 7. denciency of the arachnoid granulations 8. aqueductal stenosis may be an overlooked cause
C LI NI CAL
Age usually> 60 yrs . Slig-ht male preponderance.
C linical tri ad" T'liad is not pathognomonic, aod may al so be seen in vascular dementia" , I . gait disturbance: usually precedes other symptom$. Wide based with short, shufmng step!! and ullSteadiuess on turning. Patien~ of\en fool like they are "glued to the noor- (so-caUed "magnetic gait-) and may have difficulty initiatingsteP8 or turns. Absence of appendicular ataxia 2 . dementis: primarily memory impairment with brndyphrenia ':slowness of thought) and bradykinesia 3. urinary incontinence: usually un witting (NB: a patient demell led for any rell80n Or with mnbili ty impa.i~ment may have in.:.;,ntinence)
DIAGNOSTIC PROCEDURE S
There is ill:I test nor radiographic imaging thst is pathognomonic (or NPH. Numerous diagnOllticmteria have been proposed for NPII to determille which pat ients are like· ly to respond to a shunt procedu.re in order to avoid poteotial complications (1ft below) and Wlnecesaary surgery without denyinl surgery to those who might possibly benefit. None has proven to be of adequate reHabi ity. Some are presellted here for complete"':''''. LuMBAR PUNCTURE (LP)
Normal LP opening prt'u ure (OP) should be < 180 mm H,O. The response to a single LI* (withdrawing 1~-ll0 ml CSF. or taking the OP down by ~ OM third) OT to serial LPs may be of90me pred.ictivto value. Consider ambulatory lumbar d,.s.inage b~ below) fOT patients who fail to improveaft.er a s ingle LP. CSF should besent for rnutine labs(_ page 616).
Patients with an initiol OP > 100 mm H 20 have a higher
respon~e
rate to shunting.
CONTINUOUS CSF PRESSURE MONITORING
Some patient,., with a normal OPon LPdemon$trnte pressure pealul > 270 mOl H20 Or recurrent B-waves". These patient/l also tend to have a higher response rate to$bunting. AMBULATORY LUMBAR ORAI/JA GE"
Lumbar sub8 radmoid drain is pLaced with Tuohy needle. connected lhrough a drip chambe r to a dosed drainage ayst.em. The drip chamber is placed at the level "fthe pa_ ti ent', ear when recumben t , or at the level of the shoulder when sitting or ambulating. A properly functioning drain should pul out _ 300 mI ofCSF per day. If symptoms of nerve root irritation develop during the drainage, the ca theter should be withdrawn several millimeters. Daily s urveillance CSF cell COWlta: aDd cultures fihould be performed (NB: a pleocytosis of ~ 100 cel1111mm 3 is expected just with the presence of the drain ). A ~ day trial is recommended (mean time to improvemen t: 11 daya). CT ANDMRI
FeatUCil8 Qn CT"" and MRI" I . prerequisite: communicating hydrocephalus 2. features that correlate with favorable rt'sponse to shunt (thes e featUrES sugge..c. that the hydrocephalu, ia1lll.i due to atrophy al"ne"): 11. Hydrocephalus
NEUROSURGERY
peri~ntri~ula r low denaity on CT or I'Iigtl inteluity on '{'2WI MRI: may rep!"eRnt tran.ependymal ahllorption orCSf'. May re!lolve wittl . tlunting B. oompresaiCtn of0fl.l!Yl. intracranial radioactivity within 24 tin , re OOfUIidertd to .... ve an adequateove,.lI abo\orptlon rate, and are unlikely to improve wittlltlunting. However others have bund no conelation of clearan~ to shunt rt!Spon5e S . one l tudy found thn If the ratio ofventrieuta r to t.otal intracranial actiyity (Vfr) at 2~ tlOUrll il > 32%, there would be a responae lO ahuoting. wherea, Vfr < 32% did DOt exclude th .. possibi lity of impmvement" M ISCELlANEOUS
Cerebra l blood now lC BF ) measuremen t.a: Although some Iludin indiea~ other· wile. CSF measu remenlS show no lpeel"c "ndings in NPH, and are not helpful io predicting who will respolld to shunting. However . inlnNed C 8F a~r ,tlunting ~Iates wit h cl inieal improvement". EEG; No specific findir.g. O PoTENTIAL COMPLICA f /ONS OF SHUNnNG
Com plitoti"n ra~~ may ~ III high a~ ~ 35% {due to the froWlity Ilrthe elderly brain~' 'M. Potentl .. 1 M1npiicotions indudalO': L subdu ral hem~tomlls Ill" hygroma (also _ ~e 198): higher rif,k with 1_ press ure va l ~ and old.e . patieots wbll to'nd to have ",,",bra l Dtropl'ly. Usually acco01panied by h..OOo[h ... m.... t re.solv",pontanl"QOJslyorrenlllin stabl ... Appr,,~imBlely 0 ..... thl"td ",quite evacuo tion and tying ofl" orshunt (teml"'rllrily "r permanently), Risk may he mlucW by gradual mObUiJation pll5H'P 2. s hunt in fection 3. inLropan'nchymal hemorrhage in the brain 4_ J;l"i~urell : IN pagf 188 5. delayed INlmplkatioo& H.dude: Bhu nt ob$truction or disconnection
O UTCOME
li kely symptom to 'mllrove with shunting is inconLioe~ then gait dl$and )/lsUy dementia Slack et ai ," 81"'" th e following mark~rs for gOOd and,· dates for improvement with shun ting: clinical i presence of thO' cla6l!ic triad" (II" PClIJ6 2(0). Al ... 77... of patienta wilh gai t di stu rbance liS thl" prim ary sympwm imprllVed w.thsbunting. Patients with dementia and lUI gait dilitur bllnce rarel)' respond to .u.untin g LP: OP > 100 IIUJl H.o iSlltope eis lemOi,.,.,.m: t,ypical NPH pattern. The n,i>:ed nr normal pat~rn has no INlrrelation ..... ith responlle to ahunti ng C'(\nti llU OIl$ CSF presl un recording: ,""sure> ISO mm H.,o or frequent Lund berg B wa~ea (_ pagtt 653 ) CT Or MRI: large vl!ntrides with f)auened $ulci /little atrophy l Re!ponae is better whenlymptoms have been pruent (IIr a shllrter time. NB: patients with suspe 11 .4.
m05~
Blindness from hydrocephalus
A rare complication of hydrocephalus and/or shunt m.lfu n~t io n. Possible ca uses include: I . o«lusinn ofpost.eriorc. rebral ar(.eriH (p e A) CAused bydoWll",.rd IJ"Dnste ntori.1 herni ation 2. chro",[ pspill edemu causing inj ury to optic oerve at the nptic diK 3. di latation of the 3Td Yl'J'ltriele with tt1 mpn!MiOIl of optic w iasm Orular motility Or visual field defects are ",ore common with sh'JILt malfunction than is blindn~ l"'''. On"""'nes found 34 repMU!d c.se\l lIfpermsuenLb)iodne.s.ll in children attri but.ed to shunt malfunction with Mncomilant increased ICf»f' (thu e au thors w"re baMf 100 pa t ienl$ with tentori al herniation (mos t from acut.. EOH lind/or SOH) proven by CT; 48 patients opel'lloted: only 19 of 100 surv ived> 1 month \,U were in operated group): 9 of LOOdeveloped occipita llDbe infarct (2 d,ed, 3 vegetotin stat.., remaining 4 moderate to severe di8~bilityl'· TYPe s OF VISUAL OlSruRBAloICE
9 ofH had pre~c:ulll~ (IIJIt..ri Dl" vil;ull path ... ay ) bJindneo.s w,th markO!1i optic nerve atrophy {earlyl, 8nd reduced pupillary light rene~e •. S of 14 had postgeoic ul ate (cortical) hlindneu with nonnal Light reBponMI and minimal or nO opti~ nerve at'mphy (Dr atrophy late). 11 few pllti entl; had evideote of dam age in bolh s ite• . Cortical hliodnelll: due Lo leti 'ol\.S po.9t.erio r to lateral gerucu)8t.. bodiea (L e B), may also he """'" with hyV"'Oc illjuriea or trauma''', ()eao,sinnalty 8590ciated with AnIoD'Sllyndrome (denial ofYi!ual defidt) and with Ridoch'8 phenomenon (appreciation ofmllVing obj«UI wi thout jltin:eption of stlilion.ry u.imuli).
'"
It. !1yd rocephBl us
NEUROSURGERY
PATHOPHYSIOLOGY
In patie nts wit h occipital lobe infarctio n Ocdpitallobe infantioos (Q Ll) in PCA distribution areseen either hilaterally, Or if unilateral are associated with other injuries to optic pathways poIIterior to LGe. The mOl;t often cited me.:hanism is compreS!lion ofPCA .-esulting from brain herniating downward. Alternatively. upward ce.-eheUar herniation (e .g. from ventricular puncture in face of a p·fO$$a ma5'l ) may i,mpinge on PCA or branch"" with !,he !;arne re$ultll"'. OLb are mOre likely with a rapid riseio ICP (doesn't allow compensatory shifUI and collateral circulation to develop)' ''. Macular sparing is common. Reported ca uses ofOLI include: post traumatic edema, tumor, aba~, SDH, unshunted hydrocepba lU5, and shunt malfunction"" " ' . The occipital poles are also particularly vulnerable to diffuse hypoicia''': attested to by case.a of conical blindneM aner cardiac arrest"'. Hyp>tension s uperimposed on compromi$ed PCA circulatioo (from berniation Or elevated ICP) may thus increase the risk of postgeniculate blindn e"",'''''' "'. Both coup and contre8t 64) patient. developing symptoms of increased Ie? Ihould hve CT Or MRI A. ifnoehlng.. from pr_n.:..ption ttudy, pu rw:turt ,hunt 10 meu .... ICP and (UILUre CSF. Coruider radioilJOtope .hunt ...·cram B. if aU studies are aegalive. then phy.iologie chaagN may be reapollaible. Treatment is bed rest , nu(d .... trietioa, iltId in ....ere eua . teroi.u ... dfoT diuret i"'l. l f symptoms do notabate, then early delivery it recommended a. soon u fetallunK maturity ean be doeumented (aive prophylactie antibioties for 48 h .. bef~re d..!iv.. ry) C. ifv .. ntricl ... have enla'lled and/or ahunt mal function I, d tmon.tI'a~ on testin,. shunt revision is poI'rforO'led I . in first two t rimute .. : VP shunt is prefen-ed (do not 11M poI'ritoneal tl'ocar O'Ietho:)d afte r Ilrst trimester ) and i. tolerated well 2. in third trimester. VA Or venLl'ieulop]eural .hunt i. u8ed toavoid utero ine tl'auma Or induction ofl,bor
lnt r aparlum manageme nt prophylacticantibiotico af .. recommtDded dUMKlabor and o..livery ton!lluoe the indden.:.. orshunt infection. Since coliform. are the most common patnog..n in L&D. Wisoff et al . recommend aO'l picilli n 2 gm IV II 6 hrs, and xentamicin 1.5 mgl'kg rv II 8 hrs in labor a nd a 48 hrs post pa rtum'" 2. in pa tien~ without symptoms: a vagina l deliver)' is performed ifobst.etrieaUy . ible (lower risk of forminX adhes ions or inf«tion of distal shunt). A shortened second stage i, pre fe n"N since the inerease in CSF pressure in this Btage i, probably greater than duri", other valsaJvl maneuve.. '" 3. in tbe patien t wbo bea:omes symptomatic near term or durinKlaoor, after stabi· li ~i.ng the patient. C-oteCtion under &:enera] anesthesia (epidurals arecontraindi· cated wi th elevated ICP) is performed with careful nuid moni~"II (LK. PA ca theter) and, in ..,vere cases. steroids and diuretiet 1.
re,-
11.6.
,.
, • ,
'"
References bo-""f"".
~",iIo" I· _ol .. J,uu,15,,"," 62,19" $ootIoo L N • . . - D A.S .,...._1111 onI W K... ~I_ Tbe ... .,ott Mo "'...,. ;" , h;kbn wi.h ....;o,y ........ i•. N... ""u'l'"l")' 19: 691·9. 1996. SccIi"" of P '".."Io ..... _ r y ......"_.,.,oI_lOI_ It>«plulu' . r«l",ri 7', 7'9I'. 1m. DMdy W E: E.>.r;rpotionof,ho 0I0000''' pluu.of,1I< 1>1l>al ••. Ano S ufl6i; 569·19.19 18. Gnlfi'" K B. bmjoom A B; The I~.,.",or .hild· hoooe-rtducoq de.ice> ;. ""'. pa,lo.", willi _. .... •..... ourt hydtoll.Iu •. Ikoch I..' '" .... ~ •• ''''' ..·"h 0.11•• al .... J N. S" f1I "' ,,"'"The
pnmaryoo,,,.1 ...,..ou • .,. ...m ",,,,,,,,. J N... ",. • ... 1.: In·7.1991 . Ji ........ D f. J.: w'n~ R. GoOOne!o I T: Silicon< al· I"iY in ""nu~.lopf>l!llic-0Ii"'" or .. nu\culop .. ,i •• i.,..,. Oo.;c., ("'SO) in "'un,
""''''P~ I poll;' .~2. ,98"
.. ri< ~)'Wt< '" ", ... « aO>d /1> .... I>,,,,,,,h,p '0 oho: """'U,,,,.,., 01 • ....,...111I ........ · .... liocolua.rn... f pos..n.unti"1 "",, ...1""nl.'u"",, Su ... Nt. n>! • ,: 81. 1996 Oit'tI! NaI""" ... Ps,e/lill ' y 33, 9s.\t. 1911'l. Po.idoff l M. F.irinl E II. Sub¢Uf" III.: Mil rtnl ......... Clln N _ .. 3l: 6n·S!, 1935. Mpo"'"' hyd/o>«ph.l", in d 0,b0_601 . ..·n .w.o. A B. M>ro_ I' .W,' .... E. ....... """'""•11d by food. Av .. ilable fonna are lurom"rized in Tllbl~ ]2· 1. & Adult; 250 or 500 m8 PO q 8 hr. or 875 mg BiD INB use the apptopriaLe tablet to aWlid uetlUive cl.vul.nll~; the lower total dose of clavula nat.c with the 875 mg tablet may pn;>duoe ft.w". GI , ide eO'ec\.I). PNa: 20·40 mslkgld II.moxicilli" d ivldl!l! q 8 hr..
P ARENTERAL CEPHALOSPORINS
Higher leoontion _,enu hive pro~~ely reduced uti-ity ag:aiJYt .tnptococci and pen;c;Uin• • producin, S aul't'US
First gener ation ce pb aJolponn ll
~
eefll:tolin (Anee"'. Kerzol®)
\
I
DRJQ IO!FO
\,
Good for auJ1ica1 ptophylU». c:-llltVeb in h.-.i.n !.loue hne bIom documented. Poor CSF penet•• !.ion ( !hila nllt good lOr mminciCU), Adv.ntage over otheno ~h.lo6pot h igher !leNm level. {80 !'&o·mll ..... ac:hievable,.00:1 h.lfli!"te {I .a h..) i. Ionger {.!low8 II 8 hr dOlin,J. Ib: Adult: I em IV II a hnI. Peda: 0-7 days - 40 mNkrld dlvldedq 12 hno; infanl 80 mll\lild d,,,,ded II child - 80 mef\Lgfd divido: sa..:
Third genention eephall>lIpori n . Potency ofthne dNP are . equivalent 10 .mino,lyro.id. for: E. coli, kleb.lieUlI • .. nd I'rou .... Onlycel\a:lidimei.itdequale for~. Cood fo r .... riou. · infecIlOu. (..... OO",;t;.. endocarditis .ad OIteomyeliU8). 8U)11.1:f"RCT11: dian-hell (peeOOomelllb"nOLl' coliti.). Weediordiathuu••nd may allow lu~rlnrection. {e"te~r, resJsUnt WI
12. Infectiou
l'IEfJROSfJRQE;RY
pseudomonas. enterococcus. fungus).
,--/
cel\azidime(FortaZ®)
\
/
DRUG INf O
\
l
Good for nosocomial infectionll. One of the b!:il drugs for Pseudt:>mcfWs (J('ruginQSO infectiolUl (large doses tole.rat.xl well), but doesn't rover staph well. Good eNS penetra· tion . tis) .
Good penetration into CSF. Useful for CNS infections involving CNR IIond for late stage Lyme di!!ease. Long half·life allows q 12-24 hrdosing, U nlike mostcephalosprins, excretion i9 largely de~endent on liver, therefore 8110me dosage in renllol flloilun!. May he synergistic with aminoglycoside&. SIDE ~ may CIiouse bilillory sludging. R.r Adult: 1·2 gm qd (may be given q 12 hrs for meningitis). Totol daily doee < 4 gm. P eds (for meningitit): 15 mgfkgld initiol dose, then 100 mgfkgld divided q 12 hr •.
MACR OLIOES, VANCOMYCIN, CHLORAMPHENICOL
,--/
vancomyci n (Vancocin®)
\
/
DRUG INFO
\
1
Arent of choice for S. aU,""" r infections that are either methicillin resistant (if not MRSA, better resulto are obtained with PRSP), or thot occu r in patients allergic to pen· icillin or de rivative/:l. Multiply resistantS. llunU$ infec1iollll may require co--trellotment with rifampin . Poor for Cram negatives. Loog half. life. R.r Adult : start I gm rv q 8 h Tl'! for serious infection. check levels before and after 3rd dose, and aim for peaksof20-40 Jlglml (to~ic > 50; ototoxici ty and nephrotoxicity that are usually reversible occur with peaks> 200 I-'g/ml), and troughs of 5-10 (toxic: > 10). PO dose for !l§eudgmembraOOUB colitis: 125 mg PO QID . 7-10 days ($Orne referenc· es re 7 days - 45 mgfkgld divided q 8 h r5. - 0
ch loramphenicol (C hloromycetin®)
\ /
\
Good for: Cram ( +) and Gram (_) cocci. Excellent CSF penetration (even without in· named meningesl. 1t i~ hard to find the oral forro in the U.S. R.r Adult: PO: 250·750 mgq 6 h" (m ay be very difficult t.;> find in retail pharrnacie. in the U.s_). rv: 50 mgikgld divided q 6 hI1l. Ped ,,: 0_7 days old - 25 mgfkgld PO or rv q d . Infant - 50 mgfkgld PO or rv divided q 12 hrs. Child (for meningitis) - 100 mglkgfd IV dividMi 8 days) . More rapid kill than H-Iactams and may thus may be used initially for sepsis and then changed to a cephalosprin art..r ~ 2·3 days. Increased activity in alkaline pH. Reduced activity in acidic pH,lIond in presence ofpusandiOT anaerobes (therefore may be pOOr for wou.od infections, nuoroquinolones may be better here ). J:lol!ages baaed on llIW body weight. Obtain serum l"vell! afte r 3rd dose and adjust appropriately. Dosage, of all MUST be reduced. in renal failure.
NEUROSURGERY
12. Infections
~
gen t amicin (G arnmycin®1
\!
ORUG lM'O
\,
Ibt Ad u lt l non llal ...""1 N!lction) I V; 2 ms'kg fV lopding dOH tllen 1-1.6 mgr'kJ q 8 > ~ .,ghnl, t rough < 2). & In tril lh ecaJ - 4
It... mllin l.eoance, follow I.v,,-Is \deai red peRk
rna q 12.24 II",.
wbramyci n (Nebcin®)
\ I
\
'I'he be$l aminog lyrotide for pleullomonu (but not n good u oe l\ezid imel. Ib: A dl.l1t (normal Tilns ' rUJ>dionl: 2 mg/k,& I V loadi ng dOile t hen 1.6-2 mlVkg I V q 8 hra maintMlnoe. For PI:' > 60, &arne do.e q 12 h rl, Follow lowet, and &dj u.t (or peak 7.S10 " glml , t n;llli h < 2. Rs 10Iro l h «:lI.l: I) mill initial d')$e, t hen 2· 4 m, q 24 h .... 1lJ< PP!dr. 6-1.5 mgIkWd divkled It 6·8 hrl.
12.1 .2.
Antibiotics for specific organisms
PSEUDOMONAS AERUGINOSA
Cef\nidlrue (Fortad>l;s the dm" OrChOH'. (1ftpa6f.209). GoodCNSpeMl.f1Ilion, large doses tol ora u-d we ll. Amon,amioogl)'«>lldu, tobramicin illhe best an tip-
u udomonal. Aa\inoglywoidH gi •• 1J\OU .. pid ki ll and tb.refcml wll..., tM'" i, •• trona sll~pition of poe ud omonali atMt wi lt! orit nid ime ph.. tobramidn ;n ltioUyand then atop th e tob ro..ni om alter a reo. d oys(redutel risk Iltaminog!y)' IIf t h~ a1)ov" IV medieatlon~ hat. JYne rgi.Uc effec l lbul illnadeq ... te by iUlelffor lOft· tiHue in fection,. auch 118 woond infKl.:OJ1& ). STAPHYlOCOCCUS ... URfliS VanOO(llyd n unti l it is dete rmmed that it i. not MRSA, then use PRSP (10.1. n,rcill io) 'I- gen!.a mici n (Ilm in.o,lyc:e.ld8 give !DOre rapid inltiallr..iU a nd are .yner(i!! lic .ga,nst lta ph ). 5tDp the gentamicin . n.. r. few doYJ (l'ftIUCQ risk ofaminog\yon of phen.ytoin, l id/l¥udi""', and oralll ntLcoa.(\l·
1..,1.1, amons nth"" •. Can QU6O! live, dy.fww:tlon. which roay not be ...... enibll!. IU Ad u lu ' (or orOphllrynreal c:andid,"ia. 200 m.r PO the fi ..ldsy li nd then 100 mil POqd for 2 weeks. lJUpp' .IllO: tabl4!Le of~. 100 or 200 m.. Powd'!r for Itn Ll"Ip"n&ion
which Un be mind to. voIoI ..... ot3S mt 0{ ei lh.r 10 0/' ~O m"ml. AI.o.Ylli lllblfr in IV fonn, which i. ~ry npenalve. and 1UI" .Uy not n«NUIry d". III eXteUent GI nbsorpt;.o n.
'"
1'2 InrKuonl
NSUROSURGERY
12.2.
Prophylactic antibiotics
GENERAL PRI NCIPLES'
1.
anti biotia mUlL be;o tissuea at tilDe of contamination (!hue, ",void "on-caU" a ntibiGtiu; give 60 minute. prior to il'l(:u ion) rept.~ adm inistration y;U] in prolonged procedure. typical infect inG organism. are usuaUy predictable. Co~erage for the.. O'llaniam, i, IId~UBte (broadeflinG .pe L.
ftlec~
:I.
PLUS
I ~)
AnLibouUI1 pt!ra· !.ive wound ;nf"dionS were rer mening;ti.'. Othe", su p port. the notion that cessation (pDilsi bly with the Ill;s;stam:e oflun,bar s pinal drainage) is a~teptable eontinu e antibiotiC!! f"r I we .. k afWr CSF i, st.uilind. Ir rhln"nbea perlliau at thiR ume, ~urgital [epa;, i5 f"8CGm1T\eJ1ded
12.3.3.
Recurrent meningitis
Patie11 \.11 ....·jlh re 12.4.
Shun11nfec1ion
Ri. k orearl)' infection lIf\er .hunt . u.rgery; ~pOrted range i. 3-20'li0 pe r procedure (typic..,ly - 7%). Acceptable infection ,111.41 ": < ~7" (although mal\,)' published aerie. have I r'lA! near 20"", poe.sibJy due to differel'lt patient popu lation).
Ri"k factors ror s h unt infec t ion Meny facto .. have been bl~med; $Om, that teem to be better doc:ument.ed include: I. youn g age of plltient" : in fD,elomening«eLe (MM ) p.tienta. wailiD, until the child ia 2 weelul old may signifioantly lower the infection •• I.e 2. length of procedur8 3, Opell neuraJ tube deJect
Mo rbi dity of . hunt infections in c hildren Chi ldren with shWll infectioM have. increased moruHt)' rat.e and risk ofseizull! than those without shunt in fection. ThOIIe with myelomeningO 8 mon ths alter procedure) Risk: 2.7-31" per palieDt (typiCIIlly 6'1t). Almost aU S. fpidermidu . Tends 1.0 be internal t.YJlf. 3.~'It ofpatien u. aCCQunt for 27'1t ofinfectiollS'". 'w.te" shunt lnffttions lUay be d~ 1.0: 1. M iodo]ent inf~on due 1.0 SlOph. fp;.u,m/di. 2. aeeding ofa vuevllr Ihunt during epiaode of septio;:em ia (probably very ten) 3. ooloniution from an epilg of merungitis PRESENTATION
NOII" peemc . yndrome: fevet. NN, letbvg)', anomia, irritability; may mimicarote abdomen. May 11]10 present II malfunction; 29'l. ofpatienta preMntu,g.nth .t>Wlt mal. function bad potitive euILu.ra. In neooat.. may mllnifa t lIS apneic episodes. anemia, hepato.plenolnts. ly, and . tiffneck". S. 'pidumidi. iofectlona tend 1.0 be indolent (s lIIOl. denn,). GNS inffttion. Uluallyeauae mOil! severe ilIne..; abdnminal fmdingo more COmmon; main clinical ma nifestation ~ fever, .... u.lly iOl.euoitt.ent and low grade. Erythem. and tendema •• long Ihuot tubing OCCW"Ioccuionalty. Sbun t ne pbritl, ,,: may OOI:Ur with chronic low Iev1tI infection ofa ventrieulovlllCu, Iar , hunt ea .... in, immune complex depotition in tenal , Iomenoli, chsracteriled by proteinu.ria lind hem.tu.rill.
Blood
t e_I.II
:ilIK:: < 10K in one fourth ofahunt infectiOM. It
i,,. 20K in one third.
&liB: tately nonnalln sbu.ot InfectiolUl. IUCI!!d cyltural" poeitive in lui than ooe thlrd of cues.
CSf; WBC i, ul uaUy oot '" lOOc:ellllmm'. Gram .taiN may be pcNIitive .
'"
12. Infections
~O'III(yie]d
NEUROSURGERY
with S. tpitWrm,dl,;. milch lower!. Protein 11 oll:"n elevatOO, glucose may below or norma\.. Rapid antigen tu tAUIHId ror«ln.muni\)' aequlred meningi!"'" ulually not "-Ipflll folr tht OTpnl.ffil th~l w nd to cauH shunt i!lrldion • . CSf' cu ltu rea are negative in 4()1l, of CIltH (higher culture yi eld Ir CS F' WBC count i. > 20Kl. EVALUATION OF SHUNT FOR lN~cnON
1. hiswry and phy.ieal direcc.ed at IMunuinilli Jlre!lenoe of.hove ligns and aymptonll with emphul. on A. hittory 11l!:!!ut!"e ofinffCuon at another lite I . eXlIOI ure to Dlhe,.. with "iral lyudromK. includil\l fick • •blinp 2. GI ao uroe(e" . acutl gMtroenle ritia). Of\en _i,ted WIth diant.ea. orarrhu ii II I YDlpt.om tbat "..Ilftlly elU)llIrl'I tGf, , hunt Inftctlon S, ollt ;1 medi, C OllICk tymp&.olC mtmbr.net)
8. pneumonia B. physitlll!:UlIl to RIO mC/l;ngi. mu. I,tiffll«k. photophobi • •,) Hrom wac COWl! wi~h liifferenti.l . hunt tap, ,hou ld ~dcmf in Q.li4!' of.utpe of th_ had pc!IIili"" G " III·lIa i n~ CSF .mea r (oniy. few C,am 'poIiti¥e inrectio ... hay!!. po!Iilin l"ESull.l). GNS hllYe higher protfoin . nd low1M' g1u.:.o.e, .nd oeutrophjl~ prKom inat. in differ.mual (unpubli~bed d!ll''') CT, UJlu.fIlly not helpful for dj~~lng mfection. Epeodymal enhan~lMnt wben it aero,.. hi diagnostic orvenlnculhla. CT m.), de_tr.te .hun t malfunction .lxIominal UfS or C'I': abdominal paoludOC)'it i& l uueotiWl of inr~tion • LP; usuaUy NOT re.oommended. May be hnardo ... in obItructi¥e hydrocephalus (HCP) with a oonfunctionillJl.hunL onen does not ,;eld the PIIlhoc.n
TREATMENT
Antibioties alont! (whbout removal of8bu.nt hardware) Although tudieat.ion of shunt infection. wi!.hout remo¥.l ofhrdwal't! has bem
"'P'lrWd""-"" ", IIUI has a lower 1ucr:etS rale than wi\.h .hUllt remov.I". mlY require
protracted treatment (up to of!> d.ys in some) • .-i6ks probb.m& 1S,0dated with dra;ninl infected CSF into the pentoneum (reduced CSf' abso'l'ti ...., IIbdnminlll "ignohymp\.Omil irn:l"din, '.mdemO!ll.l! to f,,/I· blown p"nlnr,i!.il'· '. ...') or vascular . )'Stem (shunt nephrit;" 18ft fHJIIt 2141. KPl-I.5 ... 1, IUld onen requ.i rel.t leut pe rliallhunt revilion al somepoint in CMSt e.Hfi 'l'T~IItmf!nl wilh .ttlibloties witbout . huntl"l!1llOVal b then-fori! recom· mended only in caietlwn.,re th ""t;ent:. " ~nnirn>lIy ill,;' a ptoOr .ne:IRmal velltrlculardrain {EVD) ...r by int.P. nnittent "eotric:III., tip. or LPa (...itb comlllunifltllll HCPl. EVD .lIo..... ~.u,. m.. nilnnnl of CSF flo..... conl rol of ICP, .nd re~ted " mplinl for WBC derAtVllnat;oni and eu1\IIrn,ln ' ymptomatic pltiwUl or Ih_ with . ~;li",CSFcult"l't!", 'ny hlfr:lw!lrel"llmoYed Ihou ld be cultl>~ 15 only - 6~ .. l terile on shlln! ,,,recl;orl" Skin orpni8IDI ""' rutOdioul and m.y taka .¥Rral d.)'II to 1JrOW. Irtherl! i. an .bdolDilUll PMUOocy.I, the nuid .hol>ld be. dJ".ined \.hl"OUlh the pentanul Q.thRte. befo.. n!OO¥inj: it.
E mpirie antibioticlil I. IV va n comycin u.ted init iall), Ipenetr.!Joninl.oCSF .-ull.l in co....,.,nt.. tIOnl181f> 2. 3.
tha t oheN m). PO nralllpm m." 1M added for incruted «overlge (10 mgIII"d.y PO q 12 h($) when rulturu rl'.tum . change ¥'noDmycin to tV o.afi:illw I>nle&l p.llienl itI PeN allergk Or colltl>'" ahow MRSA (cood pellelral;on or inflamed >II.millj"' lower lnlticit,. thin nMlhlcillin\, Ifbact.l!ricid.lact i¥;ty i. < I·S. _pin c,)!Isi tr adding
NEUROSURCERY
12. tnf"«1.ionl
US
4.
"fampin intl'aventncula. injection ofpreservat.iv.·free antibiotics fIIay be UlIed in a ddi tion to TV therapy. damp EVD x 30 mioutel after injection
Trea tme nt fo r . pecif'ic o"lla ni , m , Poeitiv. culturu from shunt hardwa re removed at the time of ahunt r~iBion in the abtell/>ll ofcHnical , ympl.Om a Or ' pwitive CSFcu lture may be due to contamination and do not require treatment'·, \ . S. Qu .. .... and S. epilk,m;di. A. if ~n.itive (MIC s 1.0 I'g/ml): IT geM" (TV n_ftillin, Or cef/lulin, orrephalothin. Or «phapinn) B. ifre, lltant tQ nllfcillin (i .l . MRSA), cephalothin, orOl!phapirin: PO rifampin • PO trimethoprill>" TV &. IT vancomycin 2. F.nte. oro«U . : !VIIT ampicillin .. IT giln! (If inlrlllVallCular s hunt: add IV gent ) 3. other It rept_d: either antil treptococcal or above enter«oCClll regimen 4 . ae robic GNR.; base on lu.5
12.7.
Cerebral abscess
EPiDEMiOL OOY
ApProJUmHtely 1500·2500 eases per year i1) Ihe U.S" wi~h a higher il\cidenee in de· velOping eoun lril!fl. Male:r"male ratio is 1./j..3: I. RISK FACTORS
Risk faC"tonl ioelud e: pulmonary abl)Ormalitieti (infection, AV·fiUulu .•.. ...,~ below). congenital oYlillQtic h ea rt disealH! lie 12. Infections
NEUROSURGERY
9. CT Kan. c.nnot be obtain.ed every 1·2 wee ks
SPECIFIC MAN AGEMENT
obblln blood ~ ... l: ... ~. (rarely helpful) initiate .ntibiotic then py (p~ferDbly aner bioP8Y l pec:i through a trajectory chosen to: 1. minimile the path length throuSh tha brain 2. avoid t raversins th~ ve ntricle!! or vital neural or vascular 1tnK:lul'flll 3. avoid t raveraing infected 8tnJ.CtUIU oUllide the intracranial compartment (in· rected bone, paranaul . inuse&, and scalp wound.) 4. in CllSes of multiples abscesses. tertle!"': A. the l arg~8t lesion or t he one eaUlin, the lDOIIt symJ>l.'.l<m B. One EPIDEMIOLOGY
"''''''1' ' ' In 8 hble 12·6 Finding_ on preaenlallon with SOe"
EVALUATION
CT: rv contTl\$l is usually helpful. CT may ,russ IIIn'e caselI (relatfld ~ early generution _nners, failure to give. rv colltralll. pooracan quality ... )' Ifnormal. r~~t tbeC1' at a loter tin,. ordo an MRI ifdi nlCIII , uapicion persi8\A Findinga: hypodl!frM! (but denser than CSF ) ~o.-n. ,"";""oftr\l>lllple atlleM Table 12-7 Qrglnl&ms In adull cases 01 SDE assoelaled witosinusili s
.""'.
TREATMENT
I . surgical dlllinllge: indkat.ed III 1\I.000t ClIl!e!l l nonsurgical managemoot hw; been reported·l . bUUhould onJy be cOll/iidert!d with minimal neurologic inv"lvement, limit.ed lI~tell5 ;On and rna&9 efTec~orSOE. snd early ravorable NIIPP!liHI to anubi. otiu) usually done relstlvelyewe'llenUy e..ly In the course, the pus tend. t mm deep specimen obtained from anterior portion of the inferior temporal gy"" w ith NO COAGlILATION' On specimen side (cut surface with ~II blade, then c/luteriu pial surface on rum-specimen aide) 3. 2nd specimen obtained from beneath surface spel. & Neon atal : 10 r.>glkg rv q 8 hn; for 10 days.
Outcome Six month mortality following tnatment wfth aeyda-·ir wos infl uenced by-. oge 16% under age 30. 36'11> \We T age 30) Glllsgow coma aoore IGCS) a~time ortreatment initi ation (25% for GCS .. 10.0% forGeS> 10) d uration of di~el,6f! before Ulllrapy (()'j& for initiatinll' therapy within" day~. 35% ifaner 4 days)
12.9.2.
Multifocal varicella-zoster leukoencephalitis
Ca uM!d by the herpt"s varice\la-ZOllt.er vi ruB(VZV) which i. responlible for varicdl9 herpes zoster (HZ) (shingles). ~nd po!llo-herpetic peu .... lgia ($"" fXlIJ/' 387). VZV is" " herpelvirus that is distinct from the ,",rp'" .imp/ex~_ Symptomatic_ler -related encephalitisoccUf"6 in < 5% ofimmunooompromised pIItfeot.05 (ineiuding AIDS patients) with cuUlneQu s zoster"'. It typ1C11lJy faU"",. cul.8neous AZ by 8 shon time (ave rage: 9 days) although ""su h8ve been reponed whre nlany Illonth.o hllve lap •• d ft , ManifeBtation ~ include: alterM level ofconsciousneu. he~~ sche, phot.ophobia. m~_ ingismus. Although focal neurologic defJcits m8y occ:ur. t hese liTe UnCOmmOn. MID m8y ~how multiple, di s'orete. round and oval lesion.'! .. ith minimol tdema (best se 12.10.
Creutzfeldt-Jakob disease
1 Key feotures
• on inv aria bly fatal Mtephlliopathy choracteriud tty rapidly progressive de men tia. ataxia and myodonull death u.8uaUy OCCUrl within I rr.0f onget of&ymptoma 3 form~ I I transmi~sible (pOs,uhly via prions), 2)"utoloroal dominant inheriten hr at room
~alur 8
Partlalfy eIfective procedures A. steam autoclll'ing II ei1he1121' C or 132' C lor
,. c.
.
15-30mifIs, or 'm:ne!s;on in lNNaOH for ISmins. or IoMr coni . Has been reported with: corneal transplsnts"'--, in tracerebral EEG electrodes st.erili~ed with 70% alcohol and fonnaldehyd~ vspo r after use On a CJD patient'"'. operations in neurosurgical O.R.s after procedures on CJD protienb,;o recipi· ~Ilts of pituitary-derivedA human growth hormone (hGm", and dural gT8.ft with cadav-
".
12. Infection s
NEUROSURGERY
eric dura mater CLyodura®J. Recommended stedli~at;on procedures for auspected CJD tissues and contaminated materials appear in Tol>I~ 12·12.
Pa th ology The typical fonn ofCJD produces the dassic histologic triad ofneuronalloss,lIstro. cytic proliferation, and cytoplllsruic vacuoles in neuron! and astrocyte. (sta l u. 8pon gi. oa'.), all in the ahsence ofan inflammatory response. There;s a predilection for cerebral cortex Dnd basal ganglia, but all parte of the CNS may be involved . In 5·10% of cases, these changes are accompanied by the depotition of amyloid plaques. Immun08t.aining f!IT PrP"" Is definitive.
Prese nt a tio n One third initiaUye:o:press vague foolingsoffatigue, .leep disordera. or reduced appetite. Another third have neurologic I ymptoms including memory loss. confusion, or uncharacteristic b;.havior. The Illst third have focal signs induding ~",beUar ataxia. aphasia , visual deficits (ind uding cortical blindne"'l J, Or bemiparl$ia, The typical course is ine"orable, progression of dementia, oA.en noticeably wone wook by week. with sub· sequent rapid development ofpyramidDI trDct findings (limb weakness and stiffness. pathologic reflexel), and late extrapyramidal findings (tremor, rigidity, dy ... r. thria, bradykinesia) and myocl(mu s (often stimulus trig· gered). Clinical sign. ohporadic CJO are .hown in Tol>lt 12·13. Supranuclear gaze palsy is an occasional finding, also usuaJly late"' . In utly stagea, CJD may rf!Bemble AJ,heimer's disease (SDAT). 10% of casea prellent as ataxia withouLdementia Or myoclonus . Cases with predominantspinal cord findings may be initially mistaken forALS. Myoclonus subsid"" in the tenninal phase •• lind ak· inetic mutism ensues. DIAGNOSIS
The complete "diag· Table 12·14 Olag nosllc cr1tena " 01 CJO" nostic triad" (dementia. PatllOloo;tk:aUy eonllrmed (WIth unequivQI.:aJ SI)OOgifolm d\ange$) myoclonus and periodic cWnicaty: requ~es bran biopsy lsee teJO EEG D~tivity) may be abo B. IoofICI at aulO9SY sent in up to 25% of cas· o...ration 1·2HlI* Clinle.1 crlterla "~~ ea. OiagoDStic criteria de\eoo. riodicEEG I'!1erII dOsorOel have been published" al ,""".~ 01 periodic (monlhs) shown in Tobit 12· 14. No EEG adiYity patienL! in their series ciinicaIIy delW1e with a diagnosis other than CJD fulfilled the OR dinic.atf probable • criteria for elio.ically deli· d1nieally possible nil CJD.1'h .. ",,,,,t oom' ., paUenlS ..... 1I> "",mal motIIIt>oIic: I18IU$ and spinal nuid. H thOle .re mon condition other than eM,.......tlooUa, or "",at $)'II'[)IOmS and Iht!1 mu"",'" tigidi\y, or iTat>CJD fulfilling the criteria oIher IIIm/1y member hair died 01 pall'lOlOgicalty"";fiIId"",.,..nt hGH 10 obtained I'm.. """.. bin ... t DNA te &tudif!8 are freQuen~ly normal, but are _ntial :on OIle .... ut othe. """dilions (e.g. " e.pel-simplex enoephali. tis. tecel, AKA periodic IIpike., AKA p8f!udoperiodjc .llarp·Wljve comple>Ce1 (0.~·2 per ilectou . atimulUI (may be ahBent In familial CJD" /lnd in the recent UK variant (""e
«bow»
-
S PECT scan! may be abnormal in vCJD even when EEG i.8 normal". howllver the findings a~ nolspecifie for vCJD b r aill blapay: see tk-Iow
II. otte. Iho Initiol. of 1M patient in who", it r. ...1 di.."....t"td. not ""nfused wi'" Jo.kobC..... IZ ~ ldt ( 1 prion d, .....,) nO. wi.., J . ",u taw" Caoyon >i",. (.Iao Olnfu.ill&ly •• II.d JC vi .... ) II .. nKI~ · . t ....dod R!>IA oi",. \hIt llO% oflhe. ge nera! popu latiWl have been inrKted by 1.00;0 and b.lve poI!itive tilers by ilge 6 yean, 80·9!l"7t ""iIl be positive \;Iy middle adulthood) . 7hechaneea-of to>eo Ilrehigh. er with seru m anUbodil'!l > 1:IGH (most are> !:258) 2. mUltiph! enlllillcingleaiun5 with baaai ganglion involvClment in a patien t Wh081! \.nxo t\tenr eh ang~ fn)m negative to poaitive have II high prlnilil n"urWI (especilllly thpt mi",jelLing Bell'. ""IllY: Lyme diaell"" is the mOSI common cause ofbi I8tunl ·Be Il'~ pal,y " in endemic areaa) meningilis radiculopathy B. other po!l.!libl" neU{ologic involvement includes: encephaliti,. mye litis. pt>ripherol niul';t1. Neurlliogic findlnp are frequ~nll, migratllry, and _ 60% IIfpat illfll.l have multiple neurologic [email protected]. In Europe. 81llUlwlOrth', 6yndNwe (chroruc Iymphocytk meningili., peripharpl neuropalhy, and rodiculopillhy) is tbe Il)0leflt'''. CSF findinll in lat41 dioe..e are usually wmpatible .... illl aseptic meninriu" OIit«lon,1 band, and increased ratio of IsO to albumin may occur''''.
TREATMENT m.'" 'If Antibiotic therapy i, more effective early in !be illne...
12.13.
Parasitic infections of the eNS
Th .. followinl it a list of lOme of the many p.o.raaitk infectiolll that involve the neTyousayitero. Thoae that poteotillity invol~e neurosurgical int41rvention have _ daggerCf). 1. cysti«rcosi.,: lee Nfu'WY'lk~rr:o.i. below 2. toxoplu roOllist : msy Ottu r .. , ronlen;t,1 TORCH infection. or in t.he adult u.su.lIy with AIDS (1M NfuroloRk mani(ufC.tion. 0( AiDS. pal" 230). Toroplcuma gond;; i. an oblilate int racellul. r proto_n thtt il ubiquitous butdoe. ooteau" c1inkal infection except in immunocompromiaed hOllt41. Histologic feature.: necro.i, containinl2-3 run tachyz.oitelllcytblll 3. echi nocoo:cust: He pogt 238 Odenum of man alld pil, the Ibell of \.be ov. diuolves and the thu.sly hitched larvae burrow throop th. Im,lI bowel Will to enler thl lyD'\phatics Or .y.lemiccin,:ulation and lain aeee.. to: brain: atiro.ted to be involved in 60-92% ofcaset of CYBticel'COllis .kelettl mu.de
.,. ' Uhcutaneoul tiMue
The IlIOIIt roromon routeJ ofinplltion ofviable egp I re : 1. food (Ulually veletablea) Or water contaminated with egp from human f/!Ceil 12. Infection.
NEUROSURGERY
f & rology CYlliten:oai. IlIlliborly lite ... delenwDII!'d by I::USA Ire ~ 1I.idered . ignltltant.t1'601 in lerum , and 1'8 II the CS F ill nlCll'e specH;" for cy5tia;r. co.iI, FI'" lI.,auve rata Ira hither In case. without nwningit:il 0. with leu sentitive IUU, Tha "",wer flUyme-linJo:ed im munoell'Ctrvtranaftlr blot i. _ 100'10 lpeci fic and hiKt.ly alLboulh MI\I.ti ¥lt)' il l..- in eaMI with a "'lita!')' (:)'lIt,
se.,.iuve"·,
R.4OcGAAPl-/IC EVALIJATK»I Sol\..tip"l .......,. mllY . how .,.ldfit.ltJ,gnt In lUbc:utllroeoU1 n«lulu. a nd in thigh and lbou lde r muadH, S hlll · rn' tbow caleifieationa iu 1 3' 1 ~ of ellllea with """urOl.")'~tiCl!r, ofchoioe fo r intntio.al.tage infestation. . A1bendazole (Zenl@latI) 15 mglkg per day divided in 2·3 dOBeS, taken with a (Itty meal to enhance absorption (.am .. dose ror pediatrics). given ror 3 month . ... "I"!. Niclosamide (NiclocidelS.nd others) may be given orally to trea~ adult tapewOnn.l in the Gl tract (not@: pruiquantel is drug ofchoieel. R.x I gro (2 tableta) chewed PO . ... peated io I hour (total ~ 2 gm;. Intra ve ntricular disease: The,e is no COnHtlluS 00 the efficacy ofmedicall.reallm'nt for intraventritu lar cysta'·.
5u.rge ry SufKeT)' may IOmeti "'" be n_ al")' to establish the diagnosi •. Stereotactic biopsy may be weil l uited for lOme casel. es pecially wilh deep l...iII.... CSF diversion i. nec: ...... ry ror patient.. with ' YlI'lptomatic hydrocephalus. although tubin, may become obstructed by jp1lnWomltelJ& innammatoT)' d.. b"s.... SurgeT)' mtly be indica ted ror lpinal ."..Ia"· and for intraventricular c)'IIla whim may be Ie.. respons ive te med ica l therapy. The latter may IIOmItimes be dult with ul in, ltereotactiC IA!C:hniqufll andlor endoscopic inatrurnentation''' .
Contact. Both .. uenta with cy. t icercosis end their personal contact.. Ihould be K Tftned for tapewo rm infection ai~ a l il"l(le d _ ofnidOlllllide or praziquantelwm l liminate the tapeworm '-. CION conlld.l of persont with tapew(Wml ahou lei h.ve acreerung by medical hi l toT)' I nd H rologic ~ti.n1I for cy.ticerentis; if lUu " tive of cy.ticercosi, a lI ECHINOCOCCOSIS AKA hydal id (cyll ) d ifUM. Ca uHd by Incysted la rv.e ofth l!oc tapeworm &hi· nococc:u. grntUdOIO in endemic are• • (Uruguay, .... ua tr. li. , New Zea land ... ). The dot!: i. the prim' ry definiti ve hnIItofilil ad ult won:a . lnterme CNS involvemeol i. diagnosed more frequenO,y in living patient.ll thin Iny othe r fungal distill 12.15.1.
Spinal epidural abscess
, Key feature.; should be eon~;dered in "pelieat wi t h back pIIin, fe~er, Rnd 8"pine tendern"," major ri5 k factol1l; diobetell , IV drug abuse. throni~ rena! foil ...... alcoha1iam mllY pr-odu«! p~.i .. ~ m"jf.!opalb.y. ,ometimes with precipi\DuS det.o.riorotioo. therefore ellriy su rgery is rt!CpenUure CIIn OCCUr dllSJlital pres£ntat ion oh skin boil (furuncle) DCCUI1l in only - IS~
EPIDEMIOLOGY Incid ente: 0.2-1 .2 per I O.GOOhospital ftdmiesionumnuo liy'''. po~!libly pn the ~'''. Average age: 57.5:t 16.6 ye;)rs'·'. Thoracle level ill the mos t commOn site (_ ~). follow~d by lumbar (351'» then .cervical ( 15%)"J, 82')1> ~re pOl:l, rior loUIe cord. and 1$'11> Interiorlnone1tl!ries''', SEA may span &oro I to 13 levels"' , Spinal ,pidurlll abscen (S EAl is ollen u~inted with vertebroJ o$teomyelitis (In one fl(!riea of40 cases. OIteomyelitis Otturr~ in all cases of anteriar SEA. in 85"" af elT' cum fe renLial SEA. a nd no CaSl'S or poste rior SEA) and ;nl.ef\Oerl.eb,.,.1 di.. post-op SEA may appear simi la r to pHudomeningocele'" SOURCE SITE OF INFECTION
hl!mato(enoul spread is the most common lIOurft ( 2~ of cases) either to the epidural ' paCl! or to the vertebr8 with extension tol!pidural space. Reponed foc:i include: A. akin infections (mOllt common): furonde may be bund m 15'Ao of cases B. parenters] injections, especi ally with IV dNg .00M'" C. bIIete:riall!ndoearditis D. lITl E. ~.pirat.ory infection (including otitis media. 'muaitis, Or pneumoni.) F. pharyniNl or deotal ab",,", direct extellIion from.: A. decubitua ulcl!r B. psoa' abloe.n: peoas O1ajor muacle .ttaches to ttan.verse PrDCelSeJ. ~rle bral bodieJ (VB) . nd in tl!rvl!rtebral diaQ ofapinalcolwnn .tartingfrom the inferior m.rgin ofTl2 VB, e~tendill( to the uppe~ pa rt of 1.5 VB C. peoel ratinr t rauma, including: abdominll wound., neck wound., GSW D. pllllrynre.1 in fectiont E. mediastinitis F, pyeloneph:itls with perinephric abKeQ followi", . pin al ~uru (3 ore ofthne pIltitnta h.d readily identified perio· perative infectiQIlI of periodonta, UTI, or AV. r;, tula ''') A. open prootduru: Hpecially lumbll r diIotetomy linc:idl!no:e'" _ 0.67'1» B, t:loeed prooedU'H: e.g. epidural catheter inae rtion for 'pin al epidural anesthesi."'·!», lumbar p"nctu ... ' ..... • hiWlry ofrecen t back trauma is common (in u p to 3Q9l.) no IOlIrct un be identified in up to so-. of patienll in l ome 'eriel'"
NEUROSURGERY
12. lofections
".
ORGANISMS
Operative cultures are most useful in iden tifYing the responsible organism, lhese cultures may be nega tive (polISibLy more common in patie,,1.$ previously on antibiotics) and in tbese Catell blood cultures may be positive. No organism mlly be identified in 2950% of cases. I. S taph. aunluS: the most com mon Qrxan ism (cultured in > 50 CBC: leukocytosis common in acuu group (average wnC. 16,1001mm3), but usuaUy norenal in chronic (ave. "'DC .. 9.8O cr Bea n: Intraspinal gas has been described on plain CT' ''. Post-myelograpbic CT is more 5eruoitive. TREATMENT
Early surgical evacustiorl combined with antibiotiC8 is the treal.ment of choice. AI-
'"
12. Infectio""
NEUROSURGERY
though the.r. ate teporu of patienla managed with antibiotial alone'" '''' J: immobi1i:uo t ioro' '', ",pid and irnversible det.erioration has ocOlIIursical management be reserved for !.he following plItienti (reference'" modified"';: L thoM with "rohi':liti~ operative ris k factors 2. i.nvolvement of lUI ext.el'lSivtl length of the .pinal canal 3. oomp lete Pl' rll lys i. for> 3 doy,
Sur,ery Go.b are u l.oblis hin, diagnQl;' and causative o rganism, drainage of pus and debri. dementofJTllnulation tluue, and oony stablHution ifneeeal3ry. MoslSEAare posterior todura and are app/'OlOched with. e.xtena ive laminectomy. For posteriorly located SEA and no l8\Iidence of vertebral Oftoeom~li li l. instability will u~lIally not follow simple laminectomy and appropriate po.ttope rati~ antibiotiu'M,
Specir,c ant ibiotics If organis m and .our« unkoown , S. 010"", mos t likely. Empiric antibiotics: I . 3rd generation cepha lOllporin. e.g. cefot.axime (Clafora"')
PLUS
2. vancomycin: until methicillin rel il:tant S. 0""'" (l'IfRSA) ean be ruled out. Once MRSA i, ru led out , witc h to syn thetic penicillin (e.g. n.fdllin or oueillin)
PLUS
3. rifa nlllin PO Modify antibiotie& bated 010 cultu re ..... u!~ or knowledr of 800m! (e., . IV drug abUlle.. have s higber incidence ofCram·neptive organiarm).
Du ration of t r ea tme n t For SEA, 3·4 week. oflV antibiotiQ followed by" weelUl of oral ... tibioticl .... ua.lly suffices. 6·8 weeg of IV antibiOlict are l uggftlted if there is documented concomitant vertcbTIII OOIIt.eomyeli til:'" (although .orne argue that o.t.eomytlitil is pTflent I"'thoJoci· N£UROSURG£RY
12. InfectiollB
2. diabete$ ~llitu8: sU$Ceptible to un""ual bacterial infections and even fungal osteomyelitis 3. hemodialysis: II diagnostic challenge since radiogTaphic changes o[osteomyelitis can occur even in the absen~ of infection (see 1Jf!'lrucli~ le.io"" o{the 'pine , psge 939 ) 4. illlmunosupprusion A. AIDS .8. chronic oortioosteroid use C. ethanol abuse 5. infectious endoenrditi£ 6. following spinal su rg'l.."'Y or invu;ve diagnostic or therapeutic procedures 7. may OCCu r in elderly patient.. with no other identifiable risk !"aetors'" Complications that my IIttru&: 1. s pi nal epidural absce .... 2. 6ubdural ebscess 3. meningitis 4 . bony instebility 5. progressive neurologic impairment 6. un ique toCl!rvicalspine involvement: pharyngeal abacells 7. unique to tha."dc spine involvement: mediastinitis CLINICAL
Signslsymptom.· localiZ@dpain (90%), fever (52~, with rever spike. and chilli being rare), weigh t lOS!!. paraspinal muscle spasm, radicular symptoms (5()·93'70) or myelopa· th y. VO somet;mN produces few systemicei'fecta (e.g. wac end/or ESR may be normal). ~ 17% ofpetients with VO heve neurologic symptom s. The risk ofpe ralyais mey be high· er in the older patient, in cervical VO (vs. tboracic or lumbor). in tbose with DM or rbeumatoid orthritill, and in those with VOdue to S. aureu. "' . NeurolOgIC finding>lare UllCO mmOn initially, which mllY deley tbe diagnosis"'. Sensory inval"vement is less COm· mon than motor and long-tract Signs because compression is primarily anterior. SOURCE OF INFECTION Sou~s of spontaneous VO: UTI (the mos~ oommon ), respiratory trnc~, so ft.ti""ues (e.g. skin boils , IV drug abuse ... ), dental 110r&, blunt tralUl"la to th~ spine. In 37%of ceses a ""u~ is never identified "" Potentie l routesofspr~ad: arterial , spinal epidural ,..,nous plexus (Bateon·s plexus). or by direct extension (e.g. foDowing surgery). Spontaneous spondylod isciti1 in adult.. usually involves bone primarily. and once infection i8 esteb1iahed in the subchondral space. spread ill to the adjacent disc and thence to the adja~ent VB"'.
EVALI.JATION
Imaging A comparison of the sensitivi. ties and specificities of various im· aging modalities is shown in Tabk
Table 12-18 Accuracy of various imag ing modalities for venebfal osteomyelitis''''
,
12·18. Pia ;" .. -ray: ral or epidurol'P1nolabacen. and il better for aueS8inll bony fllaion . With the addition of wllter IOlublli tntrath eocel enn trast. ~Lao a9~~ iJle spinol tanal for colnpromi.se. pOI$IefIOI" aMU£us hbrosus
12 rnfection9
"
NEUROSURGeRY
Dia gnos tic c rite ria
ThrH bas.icd\lwges on CT'· Of all 3 are present, pathognaJTlOllic for discitis; ifonly the lot 2 are present , then only 87% specific for discit'.): 1. end plate fragmentation 2, paravertebral soft-tissue _e ll ing with oblituatioll of fat planes 3. paravertebral a~88 SPINE POL YTOMOGRAMS
For P:n significant bone loll!! and Inatability, then an anterior d~tomy and (uII.10n through II retroperitoneal apPl"'IIAI:h !My be rnquired. SUrg 9 )'rs ace Low grade- fever may\)Ol presenL ESR Is uSllally 2·3 II nonnal . WBC i3 ~mel;mes elevated. H. fI .. Is a lJIore Ctlmmonly IM!f!n pathol!"1m in W. grllUp . In mo.gt tMef, there is c:wnple1.e resolu!.ioCi in 9·22 w~kP.with EPIDEMIOt..OGY
Indden"" after IUr:>bar di~lOmy' '': 0.2-4% (realistic utimate iR probably at the lower end ofthia range ). May lllao OCCur after l.P. myelogram. tervical lamineclOmy, lumbar sympathectomy, cilemonucleolysis'''. discography (U t paIJe 296). Fusions lind othe r proOl!durea. Very rare after ACDF. Risk factors include: advall(»d age. obe~i ty, immu nosuppression. syatemic infection at the time oflurgery. PA THQPHYSIOLOGY
There is Klme controversy as to whether 80me cases of p 3S· C in 9 patients: literature reports only 30-50% are febrile) and chill s C. pain radiating t(l hip, leg, scrotum, gnoin, abdomtn Or perineum (t1"\.le sciatica is uncommon) 3. signs: all had para"ertebral muscle !pasm and limited range of motion of the spine. 13 were virtually immobiliud by pain. Poiut tendemes>lover infected spine occurred in 9, upressible pus in 2 (literature repor\.8 0-8%). No new neurologic deficits were no~ . Only 10-12% have MSociated wound in fection '" 4. lab findings: ES.R: 261'27 had ESR > 20 mmlhr (60 .. ave .: > 40 in 17 pa tients; > 100 in 5 patients; the si ngle patient -: 20 was On steroids). ESR ;ncrf!S/!ea .. fter un· oomplicated discectomy. P':'alOng at 2-5 days, and can fluctuate for 3-6 w~k.oi bef\lre normalizing'· '. An elevated ESR that never decreases after surgery i. a strong indicat(lr of discitUi C-reactive protein (C RP)'"': aD acute phase prot~in synthesized by hepato· cyteg that may be a mOre specific indieatorofpos1.- All potienb eventuaUy becnrnoe pain freoe (or Iignil\cantly ImprOVe). Thi. i. not the in aU .erin, where lKlIlle n!port 6O'ltl were pain free It FlU. IIlhe ... found ,light welt pain in mo.t patient.l, and yetothe ... report Iflvereduooic LaPin 75'.4". 67-88'" return to OIelr previa", work. and 12·2':;'" received diaability pltllliGn; 01_ n ... mbers art .imi· lar to the outcome fram dilc ':.II"Iery in general. Na difference io outcomoe wU found for the variGul activit)' reltrictionl.pecined, 1\)1 • .:.opt for earlier plin relief with first two typt1i lilted .t.ov.. taM
12. Infections
NEUROSURGERY
12.16.
,.
References
,,"i.., A II. A""mlC.w;., prop!lyw.i, ...w,...,..",
, r..&1 J ,. K.",,,,II. of
Mod lU, 11,9·31. 1986. F.."", PT. W'UO>Jo;WI>,Un, C.Meko< ....... ond ",1«""" Child. N.",S)'" l. 106-9. J9I7. M,Lon< O. C.~"",w"'i D. ~ .. mo"m1ll"";"C ", ... In i.I..... ' ImJ)H>"td PORti ... ..,..1..1_yorofnJtl J M«I797' lS~. 19n. s.: .. ,iKMJ.M""' ...... R B.IIo<wl;.1 M,,,"": II .. ,. biopoy for ""'pII&l~". C~ . ,.. .." .... ,~ )): ~l · 602.1936. WIt"",, R J.e_cc. ""fon:I C ".ll"'", ....... iN>n .... ,1I< on· I "' .... dl.. ppo&"not oHnt.Sd« I~ . 94l-60. 1977. KIi....... R. T I>< " .... blJ o ~ """, nLoI •. A po ...... . , Ko,,,,,
1l8O-4. 1991 .
" n.
1\0,,,
.. "
190-). 1931 .
MUI'Jli. A J.CooO";. W I. AmO:dJ J ...... 61 '
"
"".ral.",·
~.
" ".
n."" •
Itooenl>,.".,..... 1pt".";n, in'" ..,"' ......pi .., noid 01 "..ito .. wim CIC l.tlQI.: Tbe 1. ·3-3 """tin in .... t>fO:Ip; .., o.id ... mut...- for ' ..........ibl •• ..,..,ph%pI>loi t'i Erp wave tornp ... · to ,. C... "f.Id'-I...., d ... .. A.-.h ro-.., .... ' l' '6Z·6.19%. -. R, p,,'moo I. Hll «Ii,..... «I., 199~. Vol ~'PI' loIIX)-6. Knopp !. B.Up"" . 8.S .... d..,.. '" \...,1: ""'"
1.m,,"", I PfWLI'Id".I;li . ( !I"' n,.. nh ~ J I .. f, .. Ok 1}1: JOoI·ro . 1986. II}. H... il ......... u.l H.C",. 1>1 ... 1, 1"""",.,,10""lin t\:InDtm.I li, ... ln "'n:bpOn,1 ~" id or-.! blood ovy .""'_ri« < l _.....y ,.,"' _ " ..... ,,.. " "".1...........:0 .-...I,.m, J 1'«....... 55. 9007. SI. 1'/1' , L I .D-otoIoS.~ w.l«C ....... u .. tmlJ i.lan:I ..... A ,omp"i""" ofM>o 0f>In< 3nI «I .• W.8. SOUndc".I'hiIod!IpI1io. 1m. Ki"';"' W s ... , ,toINrcolI..II • ...J,pi"'. I" Nc .... """'. E 'R
i. ",i.," .pid.ri! .......... . Nturolo&l' »:
8"·~.'95().
' S1.
,~.
,~.
"".""",.; . 19a5 . 186. Wf...,1 M. w•• ",,~. HNlkt E H: Tul>o"," ",",
112.
I. I.,.".,.,"...
j,
f""" _ ... ,.,.""",>1, ..
NEUROSURGERY
sponlti .. di"'~i ..... ",'_ I~ ...... yof 'I' U1U . ... ct. SIC;Ouane&1 only) b. with automatismll c. partJ~l6eixure ",ith secondary gE"rlcralization I. .imple pIlnial evolving to li:en~ ralized 2. complu partial evolving to generalized 3 . simple partial evo],'; ng to compl"" panial evol~ine to generslized 3. unclassified epileptic 6('izures ; ~ 3% ofal1.se'tures
ClaSllificlltioD by et io logy (an d so me e pil e ptic ..yndrome9) Thi5 liat t. nnt " lllnclu$;ve
( ~rererenc:e'·ll.
13. Seizures
NEUROSURGERY
s ymptomllotic {AKA 'secondary1: seiwres of known etiology (e .g. CVA, tumor .. ) A. temporal lobe epilepBies: 1. mesiallemporal .der(lsis: fI'" bfolow 2. id io pathi c (AKA 'primary"): no Wlderlyingeause. Includes: A. juvenile myoclonic epilepsy: Me IH:low 3. c ryptogeni c : Seizures presumed to be Bymptomatic but. with unknown etiology A. West syndrome OnfantHe spasms. BJitz·Nick·Sala3m Krampfe): sl!e below B. Lennox·Gastaut "yndrome: """ bfolow ~ . special syndromes: . ituatico-related seizures A. febrile seizures: u" p<J&t 264 B. seizures occurring only with acut1l metabolic Or to~;c event: e.g. alcohol I.
KEY dist inc tions (having thera peutic implications) In generalized tonic-don;c seizures, primary generalized generalization (o ften_le..., l On!let may not be observed)" In ,ta rjllg spells: abJlence VB_ complex partial..
V3.
pa rt ial with serondary
EPILEPSY
~: '~.~";-~'~~~~~'~'~_~.;.~'~"~m~-
leriu-d by recurrent ( 2 01 more). unprovoked A disorder, not a . 'ogle disease. Characseizures.
••
ABSENCE SEIZURE
Fonnerly called petit-mal sei~ure. Impaired conaciouSJIe8s with mild or no motor involvement (automatislIU occur more commonly .... ith bursl.8lasting > 7 secs).l:'ill post-ictal Ccnfusjon. Aura rare_ May be induced by hyperven tilation x 2-3 mins. EEG shows spike and wave at exaotly 3 pe r sec_ ond.
,.
UNCINATE SEIZURES
Obsolete term : ' u"cal fol.8". SeilUre50riginati"i in the inferior medial temporal loblinne.ata wu 44 per 100.000 penon yean'. E tl oloai"' In ~tienta pretet>tifl( wltb a firtHi ...... ltu re. etiologiell incl1.lde (modified'): ]. following IWurolOJic luult: .i!.her acutely HA. < I week) or remotely I> I week • • nd ulually < 3 ~ from illfull) A. cenbrcrvQCu lar .ecidenI ICVA. or I tl'Ol<e): 4.2'" had. leilUre wi thin 14 dllY' of a CVA. IUak incnNed with MYerily of , t rok.'· 8. heed trau.rna: cksed head i!\iul")', penetrat;na trauma Uft ptJIft 260) C. CNS inr~tion: meningiiQ. ce",bra] .b_n•• ubdural empyem& O. febrile .ilUra: _ po,. 264 13. s..i1UI'ft
NEUROSURGBRY
E. birtb 8!iphyxi.) In II prospeetive ~tudy of244 patientf with II ne"·~D.~et unprovuked Hi!ure, /IDly 2770 had further ~ei%,," a d uMng follow-up"- ". Recum!nt Hi zUJ eI wera more common In
",th
a family $l!"i~u .. lU! tllry. ~ pikl!:-and- .. ave!llln EEG, or . hist.ory ofa eNS poIuentf insul~ (eVA. head iojw:y •.. ). No p•.tfenneizur.... rree for 3 yeaMi h ad a recurrentt'oFollow· ing a second seilure . the risk offurLher seirurM W84 high. EVALUATION
Ad ul ts A new-oosel &fIlilllre in an w1Jd.I in the absen ce of obvious cause (e. g. olcQhol with · drawal) ~hllu ld llrompt a search for an u.nderlying bull/ the OnMlt ofldiupat hir ooituru. i.e. ~pileJ.lIY, i. most common bero"! or durinsad ole8cence) . A C1' or MRt (withnul a nd wilb e nhance!oent) ohoold be perfnnned. A sY"t~mi Ear ly PTS
(:0;
7 day. a fte r he ad t r a uma )
30% incidence in IeVere heed injury ("leve.e" defined .. : ux: > 24 hra, amnHi. > 24 h.-s, focal neurodelieit, documented oontuaion, or in t rlcuni. l hematoma).nd in mild to moderat.e injuri... O«un in 2.6% of children < 15 yr. Ig' with Mid injury cauI;ng at least bri efLOC or amnesia". F..arly PTS may precipitate .dverse evenu . . .. rNult of .I.vation oftCP, .It.e .. tio ... in BP, ch.nge. in oxygenation, .nd U«Aa neurolranamitt.er r.leale".
-I"
Late o o set PTS (> 7 d a y s afte r bead lra\lma l Estimated incidene.. 10·13'1> within 2 Y"' aner · signif,c.n t" head trauma (includ.. LOC > 2 mini, GCS < 8 on admiuion , epidura l hemat.om •... ) for alL.ge ftO<J(»"" ' . ReL. ative ri . k: 3.6 times control pIl pulation. Incidence in.leYt!ll! head injury» moderat.e > mild". The incidence of early PTS i. higher in children than adulu, but lat.e "';&urea are much Ie.. frequent in children (in children who have PI'S, 94.adevelop them within 2~ h ... oftha injury"). Most patieou who have not had a seizure within 3 yr. ofpenetrltinc head injury will not develop leizures"'. Risk of lat.e PTS in children don not appear related to the occurrence of earl,. PTS (in adulta: only true for mild injunea). Riak of devel· oping late PTS mly be higher after repeated head injurie •.
PeDe trating tra uma The incidence ofPl'S;1 higher herewith penet ra t ing head injuries than with closed head injuri" (occura in ~ ofpenet rating trauma C86e5 follo.... ed 15 )""Sf').
T AEATMENT
A prosPfICI.ivedouble blind . tudy ofpal~nlS at high risk ofPTS(exduding penetratinc trau ma llbowed a 731110 reduction ofriskofu.w PI'S byadministerinc 20 mglkg load· ingdoseofPHTwilhin Z. hraonnjury and mainlaininghigh lhe .... pel::tic level.; but aner 1 wef:k there wn no benelit in continuing the dT\1g {bued on in"'ntion to treat)2". Car· bamazepine (T~to~) hn also been ahown to be effective in mucing the risk of urly PTS, . nd yalproic acid i, currently bei"i Itudied '·. PhenytOin ha . advene cognitive effectl when given long''''= as prophyluia &(ail\.lt~.
TAEArMENr CWIOELINES
Bated on .vailabla inforTl\.ltion It« obouel it appeara that: I . no treatDlent lIudied effectiyely impede. epilep~nes;1 2. in hilh·ri,k patienta!He' Toblf 13·2), AEOS mUCH the incidence ofurl:t PTS 3. however. no .. tudy h.. ,hown thlt reducing early PTS lmpro-.·es outcome 4. on« epi!e!»y h .. developed , continued AED. re c. o. c
,
...
,"'"
G.
DIFFERENTIATING NES FROM EPILEPTIC SEIZURES
Distinguisbing between epileptic seizures (ES) and NES is a common clinical di · lemma. There are unusual ~ei~uru that may fool experU". SC:ed by administering diaz&pam 3.3 mglkg PO q 8 h ... dunn&. rebriillepiaode ltemp >38.1 "C).nd continuing until 24 hN th e r. .....r lu\)sided"
f_"""
.n...
13.3.
Status epilepticus
Derio!tion: More than 30 min ... t" onUtontin~ se.z ... re aeu"';ty , or (:&) multiple se..
~uros
without full _ery oftonscioulneK
beI."Weetl
H1"luree"
Types of atatu s e pile pli c u 5 gene ra lized SliltU. clHlvulsive, geMTIliud conv ... lei_ toni~lonic statu. epileptu:Ul lSE) i. lbe moe! frequent type". A medical .""''VIllCy
I.
2.
lIh$ence"
3. 8etcUldllrily ge ne"li;u,d: accounts fOf _ 75'1> ofgenerelized SE 4. myoclonit ~. ltonie (drop lIl .... ck): Npeeiaily in l#.nllOl(-G ......... t Iyndrome (In" ~ 2581 partlaIIUlt .... (""","Lly relllled t.o In /IIlllwmic abnormill ty) 1. simple (AKA e pil e pl)' pl rt l.li, cont ln u RII.) 2. compJu A: mo.!. often from frocllllllobe foo:ua. Urpnt t~.tnwnl" r.q ... ,red 3. .;econd. rily leneraliu-d Ep id e miology Incidenc. il _ 100,000 Ciau/yNr In the U.S. Most ea_ ooeur 1n)'O\itIII ehildtcm (amon« ehlldNn, 7S'Ao were < $ 'J"I"I old" ), th e nlllt n_t a rrtcted rrOJp il pauenla over Ige 60 yr!. In " 5O'Jo OrCl\it'Il, SE t. the !)flUent'. lil"$t aei . ... reM. EUo lo gl ea 1. febrrle ¥ellUl'll': ' rorr.nwn pT~iplUto.in youn« I"'tlenlil. o5-&'lIo IIf palrenla pre-
13. Se;z". ..
NltUROSURG£Rt'
teoling with S[ ha~ a hi¥tory of prior febrile M'izures 2. ~erebrovJ;lJculu accideot.t: the m Olll~ commonly identified c8uIIe in the elderly 3. e NS infection : in children, mo.! Ire bacterial , the rnOtit O. The high..t mortality occur. in elderly patien1.land th_ with SE .-ulting from a noxia Or CVA". 19& of patientl die during the epi.ooe i~lf. Morbidity and mortality is due to": 1 . CNS injury from repetitive electric di K hargea: irreversible ch.... ges begin to IJ)' pea. in neurona .ner IS little as 20 minutft of convul,ive activity. 13.3.2.
Medications for generalized convulsive status epilepticus
Table 13·6 shows a summary of medications for 8t8tus epilepticuJI that are outlined in further detail be- Table 13-6 Summary of medications for 6tatul eplleptlcusln average size Il.l!.Y.!t low (modifIed management scheme"· (see IMIo< ~tails) ..... ). Items below in boxes are(on8~· A. lorazepam (Ativan ) 4 mg IV slowly OVer 2 ered treatment of rnoice. · Peds· reo mlM, may repeal ancr 5 mini fel"$ to patients" 16 )'fa of age. Dru gs should be given IV (do not use 1M B. simultaneously loa with phenytoin' rou te). If IV aroeS!! is impossible, di· 1200 mg ifnot slready On phenytoin uepam solution (not suppository] 500 mg if on phenytoin (check levell can be given rectally. C. phenobarbital rv (@ 30 miM, intubaUl and begin pentobarbital (Ut tut) "",,",m...., rat, tor pMnytOin IV i. $0 mcPnIn ; t", lot· p/"ItnyIOin
~ ~ t5()
mg PEImIn
1_~2'2)
diazepam (Valium®) 0.2 mglk,g (10 mg aversge adult dose) IV @Smg/min, repeat ,fineffective q 5 mins up to 3 additional d08e$. Pfds: 0.2·0.5 mglkgldose with max 6 mg if < 5 )'fS, max 10 mg if .. 5 y/1!. Diazepam should routinely be followed by phenytoin to pnvent recurrenee. Rectal dOile is 0.5 mglkg of diazepam solution up to 20 mg max, and is usu' ally absorbed in ~]O mins OR
2.
in pediatric patients with freq uent seizures or prophylaxis of reb rile sei. zures and no IV al:Ce!;S, vaJproic acid is well absorbed ....,tally, a nd is admi n· istered at 20 mgfkg d iluted in water or vegetable oil" simultaneously W with phenytoin (Dilantifi®) (P HT ) as fol!ows (do not won)' about acutely overoosing, follow dosing.rJlJ.tl, monitor BP for hypotension and EKG for arrhythrniaa). Conven t ional phenytoin must be given only in NS to pre· ven t precipitation. ~r giving the following loading dose, start on maintenan~'1 (lfffpagt 271)
'"
13. Seizures
NEUROSURGERY
3.
for 70 kC adult) (use 15 mg/kl for elderly pat.ien~). maximum rau tor phenytOio < 50 mglmin (max rate for rOllphenyt(l' n ia 150 rna PE/min), P~.: 20 mgI'II.g, ral.e < 1·3 mw'klVmin B. ifoo fliT and ...... nt levI' i. known: a rule ofthu.mb il (ivinS 0.74 mglka: to an adult .... i.es the Iflvel by ~ 1 .. glm] C. if Oil PHT and 1eVi!1 Im known: -, ... It: (ivI500 mtO < 5(1 m"mln pnxeed to each :oIlowin,ltep iC seiturft continue: A.
PHT.dditional ~or5 mg!ka O< 60 mg/min up to_ toul of SO mglkg
8
either"
. o>th phtnoborbi"ta1 on benzoc!iazepines.lora,.epam (LZP) is preferred (diazepam (DZP ) ~istribute5 rapidly in fatty ti" ues~, and sei~ure. may recur within 10·20 minutcll), but ~a\lses longer sedation. LZP aborts SE in 97% of cases, " •. 68% for OZI"". Abo. leSli respiratory depre.osion than with OZP. As with all benu.>diazepines: 1. respiratory depression and hypoumsi on are exacerbated when used with other depressants (including barbitu rates ... ) 2. effectiveness in SE is reduced by prior maintenance on other benzodiuepines (e ,g. dona>.epam), but.s not affected by the presence of other anticonvulsants 3. tachyphylaxis may deVl;!lop 10 that .ubsequent d NEUR OSURGERY
13.
Sti%u r~
'"
1. 2.
etholux imide val proie acid (VA) 3. cJonazepllm 4. methsuximide: I a page 276 C. myoclonic _ benzodiazepines D. (.Onic or atonic : 1. benzodiazepines 2. (eJoomate:!Ie;> po.8~ 276
3. 2.
vigabe.trio,seefHllIe279
portial (aimple Or complex , with Or without secondary generalization)" (VA may compare fav"nhly with CBZ for secondarily GTC, but is less tiredive forcompiex partial se izures"): A. carbamuep ine (CBZ) most eHeetive, leaS! slde efteets
B. phenytoin (PHT) C. phenobarbital (PB) D. p!imidone (PRM)
3.
I
slightly less elfeclive. more slde eHects se<Xlnd line drugs for anJ of the above seizure types: A. valprOllte B. lamotrigineA: lMe p~t 278
C. topiramate': _ pllf;e 279
13.4.3.
Anticonvulsant pharmacology..
G ENERAL GUIDELINES
Monother a p y versu s polyth e rapy 1. in~rease a given medication until !!eiuTes are controlled or side elTeets he<XIme in2.
3.
tolerahle(do notrely$Olelyon theTal)eutic levels, wh.ich is only the ran8fin which most pa.lients have seizu re control without side effect$) try tno!lQtherapy with a dlfferent drugs befwe resorting to two drugs together. 8O'l& of epileptics can be colltrolled on monotherapy, however, failure of monotherapy indicates an 8O'l&chan~ that the seizures will not he controllahle phermaelogically . Only. 109'0 hellefit significantly fTom the addition of. second drog". When> 2 AED. are requ.i.red, conaider nonepileptic seizures fXJ8~ 262) ",ben first evaluating patients on multiple drugs, withdraw the most sedating ones first (usually barbiturates MId donnepam)
etics are 1st order (elimination proporti(lRal tQ concentration), metabolism saturates near the therapeutic leYel resulting in uro-orde r kinetics (e limination at a constant rate). ~ 90% ofleck: CBC &. platelet count (consider retku )ocyle count) & Mrum Pe. Package insert &ay. · recl"leck at frequent inte rvals, perha!'S q week I( 3 mos, then q
month I( 3 yrs." Do not sur!. CBZ (or diseont inue it if patient already on CBZ) if: WBC < 4K . RBC < 3 x 10'. Hct < 32%. platelets < lOO K. reticulocyte. < 0.3%, Pe > ISO pg%. SUrt Inw and increment slowly: 200 mg PO q d I( I wk. BID I( 1 wk, 'I'ID It 1 wk . ..u an inpatient, d(>Sage changes lI"lay be made every 3 days, munitoring for signs of side ef· fects. As. an outpatient, changes should be made unly _ weekI)', with levels after each change. SlIPPLtEo: oral fonn . Scored tabs 200 mg. Chewable scored tabs 100 mg. Su~pension 100 tnglS-ml. IV form' Dot ava ilable in t he U.s. at the time oftNs writing. Carbohy_ dra te (exte nded releu1! CBZ): 200 & 300 mg tablets. Caveats with oral fonns: oral abso'lltion i$erra ti~, and smaJJermorefrequent dose$ are preferred". Oral suspension is ab~rbed more readily , and also lit should JJ.l!l be ad ministered simulta neously with othe r liquid medicinal agents as it may resull in the precipitation ofa rubbery, orange mass. lit May aggravate hyponatremia by SlAD H·Jike
effect. PHARMACOKINETICS
l,l'i (ha~.fiIe) single do:l$e: 20-55 '"" alte! cIIIoruc therapy: 10-30 h's (&duIts), ~.~ ~~ (peds) may be mi_rng """"'" lhe _ ""'tabOide Clllbemelic. ... Iinea r !i. mOllle" pelllli..e
005E R.r. tUrti ng d_
for ptIIin con t rol iI 1)() ms PO BID, fo r ",~urat It i, 300 mg PO 810. Muimwn dOle 2400 mlP'day lOUt . !Jt..'I'I'UM: \50, 300 &.600 1111 Kored ta ble"., 300 ms/5 m! anol UlptllIlIion.
Effective in primary GTC. Abo uw l\.o\ in ASS with GTC, jllvenile myoc]onicepilep..y, and PIInial ~izu r. (not FDA appf'O'led for 1Mtel'). AJiCI FDA IPPI"O""'" for migr.>ine
propbylu iL Note; 1e"1"" GI upset and shOf'\ halrlife ~kl " .Iproit acid much leas useful than Oe~kotme ol'''-peru recommt:nd dtangi nalmm BI.o w TID d.:..in" " Pltiol'nts s 2yrs 'I'! (n. k of hepel.OlmrJcityl,
la , ~jlUIU
NEUROSURGeRY
\ I
phenobarbital
\
INDICA T/ONS
Uled u .Uemllt;Vi! in GTC a nd partl.ll nol DOC). Had b,e.en DOC fill' febrile tel· ures, dub.ioUIIlH!neCil" . About 41 en'clive I.-' PHT, but very &&datinl' AI.'IQ lI~ed fll~ sUo· I", "pilllpl;(U' (Me Pf18t 2611,
DoSE &1m" do8e PO, TV, or 1M. MDP . q d" " . Start .Iowly 1.0 minim it. tedation . Nor AduJt lOlldini: 20 m"" ~ IV (.dmini,ter at rate < 100 ,""min), Ma.iuk: .nlIJltt; 3()'250 mgld (ullulillyd;vidM BID·TID) Pcdlll!adin&; 1!>·2{j mg/llg. 1!'I..wt.tlW1r/l! 2·61t1i/'kg/d (uIU$1I)' divided BID}. 8U1"I...:0: Uoba 15 ml, 30 mi, 60 mil. 100 mr. Mi.w;ir '20 mKf5.-mt PHARMACOKINeTICS
~~ l (pHiI""') ISIH!1f5lll.)
1m
(h.itII-ife)
iiiilf5C1 (fBI'9&: SO::160 tnf IN rnr:f:'6
(~)
f&:2raiYi1/i11, IiQ F
'!!
PIds:~':7onca
~ 11M! &:a-..¥{,ecII.a 1s.30 iijmI
iDe
up 10 3OdI'I!) - 25,;,perWMll} _ Phel'lobllrbital i& II potent induee. ofhepahe In:qmu that rfletaboli .. ollier.u:o..
SIDE EFFECTS Coen ,live impairment (mill' be . ubtle .nd ltUIy oull ... 1 ..tmintstn.tioo ofo.e drug by It leul several m(lnlhr'), o.ua Rvmd in ~!; &&dlhOn: pa..-wal hypuac:tlV;ty I~
dllUy In ped!l; may CIlUM! Mmorrhage m newborn ifmother
primidone (Mysoline®)
~
on phenobarb.tal.
\ I
\
IND~T/ONS
Sime a! phenobartJital (not DOC). NB: when II&ed in combination therlp"lowda.es{50·125 mg/dayl ma,lld d $igniliant6\lin"'\I! conlTollOthe pnInllry AED with rew,ide elfec:"'.
SIDE EFFECTS Ataxia: drowl incU; behavior d>llnR"C!.
\ I
zonisftmide (Zonegran®)
\
INOICA TlONS AJijunctiVl! therapy for JWlrtlaJ Sl in ndulu..
~
\ !
acelazol8l11ide (D iamoX®)
DfIUOItlFO
\
,
Tho anli~pilcptie errC'Cl mfty Ire e.iUu:r due to dln:.d iMibltfon ofCNS carboni~ 1111hydraS\! (.J~ l1!duces CSF production) o r due. to thc ~ light CNS acidosis that T1!sull!l. INDICA noNS CentrkephaHe cpileps;~'S (ebsence, non focal
NEUROSURGERY
&ei~u r"g).
13. & ilurea
Best ,"""u)u. are in ab<sen~
m
aeizuru : how_ r benefit h""ellKl b!!en obllcrved In GTC. myocloni,jerk. SIOEEF~CJS Do no~ UII in fi"'t lrime$:er of prfllUan cy 11I111y
btl ~rll~nk). n,e diu r.tic ~fTect Ul ullU ,,!nallo... t>fHCO~ which mllY lead to lW .ddotic I\.III.!! with lon,-I.!!nn Ihe •• py , A aulfonamld., Ih,,,,fo", eny typi~lI.l reAClion \0 \hI , clll61 mlly OC'C ur (anaphylaxii, fevi!r, ruh. Stevens.JohnlIOn syndrome, \o~;c ~pi di':rmal n8Croly~il .. ,l. P. . . thui": medica· tion should be. diMOntlnufld.
DosE Rx Adult; 8·30 I1Ilt1'kgfd In divided dOJt!alma.x I amid, hiKher dlllla donot Il1Ijlf'OVI control). When given with another lIED, theluggelted lurtingdOlllll250 mgoocedaily. lin d thi! ilJTld uolly Inere.aeC.. 8IJ1'PULD: t.ableu 125. 2SO",.. Dilmo:~ ilf!qudalll ..... au.· tainl'd reJea'e 500 I1Igcapolulet. Ste rile cryodeN;csted powd~r ;,alllO ..... il.ble in 600 mg viala for p...... nle •• 1 frv) use.
,---/
gabapentin (N eurontin®)
I
\
IlfIUOII'FQ
\
,
Although developed w be. GABA .coru&t, ildoes nllt inlll rlol:t.t .ny known GASA Em""cioul far pril1lary generaliuod sellUl"e1 and P/lrtlallei.uru (with 0. with· out ~onclal')' generalization). Ineffective for 'WoeI1Cc. ,---/
laffiot rigine
(Lamit:ta~)
\
I
~ I!*O
\
,
A.ntleonyultant effect .... y t,. dw.1.o prayn.ptic. inhibition ofllu tUUlte relealJe". E1fiCicioua.1 .dlu!)I:Uyt thenpy for pnrtiallCizuretl (with 01" without seoond.ry gener.hJ.aUnnla nd .... nnox·O.. taul IyridroOle. Pnlim;ruory daUl I"'llBul it mly IllIo be us.ru! In .~ltRct (/)r refractlit)' lellenl lized KizUIU. or n IIIOllIIthe .. py for newly di.",~ part ial or ¥t'nl!flj li Uld aeizum .... Alia fDA Ipp«I¥ed for bipolar clillllm r.
a.
SlOE EFFECrS
Somnol.noe, d.iazlllQl:, diplopi • • II Serio..- .... b" requirillC hOll~itaJiElltion and di. cnnunuation O(WOIpy hllye been reported I,...h ....,ally bt!gi1Ul2 week. afte r inili.ling therapy and lilly t,. HVete and potenUllly lire-tb,,,,t'!lung, inclw\lnC Stewcna·JohlUOn l)'I1drome lmore of, concern " 'ith ~muJtaneou. UM o1valp. oale),'rod 'Irely. tolUf epi· deMl"l.1 necTOlyllia ITEN )). lncideru:. or'icni1kanlep;dennal rNction may bot dec:reBted by. alow ramping-u p lI( dataCe, MIY increaH MilU'" (requenQ' in severe myoclonic epileplly orin/"allcy"". Metabolilm or lamolri"ne ;1 arrec:t.ed by Q\her ABO.. 13. SeI1.U,et
NEUROSURGERY
DosE
Rx Adu lt : In aduJta receiving enzyme-inducing ABOs (PET. caz, or phenobarbi tal). start. wjth 5(1 mg PDq d lI 2 ",lui, then 50 mg BID 1 2 WQ, then I b.l' 100 mgldq "'...,1< until the UlIual maintenance dose of2oo· 700 m(id (divided 'nt.:- 2 dose,) is reached. For patients on .... lproic acid (VA) oJone, the maintenance dO&e Will! 1(1()-2oo mgld (divided into 2 dOlI". ), Rnd VA level. drop by _ 25'1> wilhin II faw weeks ofsLilrtinlllamolrigina. Por patien ts on both enQrne-inducing AEDs.a.rul VA. the starting dO& 1I.5-5r..s
naw·I~';s 15 II)' f'HT .".,. CBZ, _
•• VIIIproo 600 mgld i '. SIII"''''" ;II: 25. 100, &: 200 mg !.!llls PHARMACOiange to II 3rd drug WIlS required ~. EW cias. (Iele J3.12 j: cl81~ ~ had wo rst progn08;1 for reiap!ll!. Epileptiform discharges on EEG se rv" to di/ICoursge AED withdrawal"' In" larger random;~ed ewdy·. the.m06t ;'oI'pstimts ... ith med ically refractory CPS (dOH not t how actu , 1 W of origin). u""rul whl!n MRI and EEC uonnot localize.
SPEcr SCAN ($INOI.£ PHOTON EMISSION TC)t,f()OIW'HYJ U~ t.o demonstrate increased blood now during a Itilure to help localize lite of on· !Jet. 199m) Technetiu m (Te) hexam.l.byl· propy~ne·amine-oEime (HMPAO) ;. u~ually ad . mini.te~ immediately a~r O:l!Jet Or lei~ r• • and thoe lean may be obcaiJled within "-'Tal hou .....,
WADA TES7'AKA intTliurotid .mytal toeSt. Loca lizea dominant hemisphere (aide of language fu nction l .nd _ _ ability orhemi. phere witl\out Ie, ion 1.0 mainttin memory when ;aolstad. U. ua lly rHeT'Yed for candidate, for large resectionl'"'. Start witlla!1fiocr1m (m.)' uH IV digittl . ulXraetion angiotrrt-m (DSA» t.o as_ frOQ now ud to RIO persiltent trige minalartery. Signi licantcTOQ·n .... ia a re!ativeeontraindiuo tion to anelth.tiling ttl. lide or dominan t supply (patient roe' t.o I leep). Wilda Uat may be r - i y matc:unol4l with hip flow AVM , Alao, poTtiOlll of hippoCampUl may be aupplied by po.tenor circulation (not anestheti1ed by lCA injection ). EEe monil.orina: it uau.llr perfonned durine the teat wlltn it i. bein, done for Iti. zure I u.ra:ery , Pat",nl will lllO"N deltt "'''_ duri", deepen I."el or anesthesia.
T echn iq ue inl truct palient as to what it opectad tatheteri1e ICA: ul u. lI)" l tart on aide of lei ion
'"
NE.UROSURGERY
have patient hGI':: contralateral ann in air, and instruct them to hGld it there inject 100·125 mS"sodium amGbarbjtaJ (Amytal®) rapidly into internal carotid ar· tery (effect start8 almost instantanl!J~ 13· 16. If Tsble 13-16 Senlngs lor cona voltage based unit is used , start at I vOllt and in · . Ienl CUfTenlge n&ralor crease.
CORPUS CALL OSOTOMY
Psrtialor total section may be most elTective for gene raljzed mitior moto r seizures. or little benl'fit for simple or complex seizures. Benefit has been supported for: L frequent episodes of~.lI..WI ("drop at· tacu") where loss ofpr;>sturallone - falls and injuries '" (70% reduction with callosotomy) 2, jlO:Ssibly for generalized seizure disorder with unilate ral hemisphere da mage (e.g, iD fantile hemiplegia syndrome): hemicortical resection may be better for this type, whereas callosotomy may promote partial sei zures. Note: Q "functional hemispherectomy" i8 recommend...! OVl'r "anatomically complete- hem;~ pherectomy to reduce morbidity ond mortali ty'" S. some patients with geoeralized seizures without identifiable. resectable focu! Division of the anterior two thirds of the corpus callosum (CC) (minimize. the risk of disconnection syndrome. su b«low) may be advsntageous over complete collosotomy (controversial). Some advocate sectioning the CC with intraoperative EEG un t il the typical bisynchronous discharges that are usually ~n become asynchrOIlous "'. No need to """tion anterior oom misure. Can u.ually be pe rformed via a bi fronta l craniotomy utiliz_ ing a bicoronal skin incision. May produce post-op I v.. rbalization or akinet ic mutism that usua lly resolves in w~u. ~ Contraindication: major behaviora l andlo r language deficits may occur even with partial division in patients with speech and dominant handedness located in .Qjlj& W hemispheres ("crossed dominance"l. Thus. Wada test i$ reeonlmended in aU len handed patients. MRI sagitta l cuts are superb for assessing extent ofdhtlsion of the CC''',
,,
DISCONNECTION SYNDROME
In a patient with a dominant left hemisphere, conaisl8 orlen tactile anomia , left sid ed dys prax.ia (may resemble hemiparesis). pseudohemianopais, right sided anomia for smell, impaired spatial synthosis of right hand resulting in difJicultytopyingcomplex lig· ures. decreased spontaneity of speech, incontinence , More COmmon with larger surgical """tions oftbe CC. Risk is le~s if the anterior commisure is spared. Patients usually adapt af\er2-3 DloDths, with final function normsl for most daily activities (deficits may show up on neuropsychologicsl testing).
TeMPORAL LOBECTOMV
80% or patients with medically intractable sei~ures with demoTl6trsble (ocus have foci in anterior temporal lobe. Most patients have neuronal 1088 a nd gliosis of mesial temporal struot ures. Thus, a standard resect io n of temporal ti p (often wi th Bmygdalo- hippoc_ 13. Sei1ure$
.VEUROSURGERY
ampectomy) may be performed.
Limi ts of re"ootion (without aia-nificllnt oe urologic deficit) Not.:! tbat th~e valu" an! generally con.idered .afe, howe'>'e., variat ion. occur from patient to pIItient and only inll'tloperlltive mapping can reliably determine the location oflanguas-e «nle .."'. Some ~ot.:! rt .pare the superior tempore.llQ'ruI"·, Tlte following measure menta aN! made along the m.illlI.lt. temporal gyrol dQmioan1 temporal lobe: up to 4·6 em may be removed , Ove"'n!OJe(:!ion may injUn! ,peec h ~n t.:!ra. which caOOOI be reHably loc:ali1ed vilually non.dominant t.:!mpo.el lobe: 6·7 em mey be rue NEUROSURGERY
13. Seizu ...
'"
3. 4.
anticanvulsants aTe continued X 1·2 years even ifno po8t-p sei zures OCCur before discharge: A. neuropsychia tric evaluation B. serum anticonvulsant level C. EEG OUTCOME (VYrrn A£S;;:CTION OF SEIZUf'lE fOCUS)
The greatest effect of seizure surgery is adJ.ltli9n o(lICi= frt'9uency'''. however. any surgical procedure way fai l to have a beneficial effect. Sf.izure con trol is usually assessed at 1.3 & 6 most post ap. and then annually. A POSt..OP MRI i$ usually obtained at 3 most post..op to assess extent oflurgical resection. Most patien ts take anti-epileptie dru gs (AEDa) far 2 years post-op, and then may be dis· con tinu ed in those free ofsazurea. Recurrent seizures: although late seizures may occur, 90% of sei1u re8 that recu r da sa within 2 yearll. 2yeano post-ap in patients maintained on AEDs: 5O'Ji> are seizur .... froo, and 80% have ove r 50% reduction ofiaz"re frequency . Far temporallobectomies ,n the dom inant hemisphere without intrllaperat;ve mon· itoring, there is a 6% risk af mild dysphasis. Significant memory deficits occur in ~ 2%.
References
13.6.
c.""...."'" "" CI."if",..... ..., Tcmli .. ",,~ of
II>< I.,."..,,,,,,,", Lel , ,,,, A""o" Ep;k",~ , Prop • ,.. "r", ..1oo 0( : ~ R.a...n" R M.CI;I'\oo(; • .,..,.; (;.ioIe11... 1.. 'ht "'t ".rt""" 01 ....... ~...:IIQj.'l' . The 0", .. r",um' f ou""",ion(l'/<w YM.,l. Tho: A... of 1«"""",ic:0/ S.'r. S, A..---yA M, ., proI""".
,.l._
p1" F't>.
1""'' '' ........
,W;
NEUROSURGERY
". •
" o.
N... """",.'HSoppi 2t. 9-1). 1990. I'I"I)I,..S A,ShaUIIM R I. EI>oJocyond ...,.,,111)" of ..... , ,pikpoi<us ;" ,,,IIII,en, "I"pby< U,,,',,,,OI of" .. ", q>i"'. pt>1 ",""..,1001< ..1...... III F.... 'I J 101«1 lll' 1.~5'. I'lSS. M.. _R H.C- I A.CoIIi ... ' F. .. _ _ of'll_"'''~ap1:4·90. 1996. Pr....... M,FiDCh""'It.~')' J. ... MI · Topi ....... pl .. doO-...'"., dru,. ,. Ir« of for J Mod lIS' 9-lH,. ,9$1. 1\-.0. T. 6 ... ,,,..C.I' 1'0.""
law block pain is comm"". and in _ SSilomc:ues, no 'pe:rminolac h.. been conl.erlt.io". and n"".Ulndardised . Many diagnOllt ic Jabel. a re II.ed inoollli.len\.ly le.l . IpondyLo.i • • • pr.;r.. ' t rain . mUICU)OIkeltW p.tIl/l. ,"YeNaKial pain .. •). A l ubM:t ofnomenelature proPQlld by a task {orca" i. alIown in Tobl. 14·1. which il IIHful pri ma rily fDt oonai&lent "'-rrninolOO related to radiocn>phl Nf.'UROSURGERY
'U/f'). Ilmay be difficu lt t.o distin(Uish thi. on currenuy av.ilabl e imaging . Iudies from an unconll ined he rn iation wllieh i. undern",alh Ihe pO$lerior longitudinal lig.menl. Ruptured di..,: coJloquiallerm uaua lly in tended to be equivalent to herniated dille. Table 14-1
"""'"
Reco mm en ded classifies-
tion
Table 14-2 AHCPR classification
It i, I'fl RADIOGRAPHIC EVA LUATION
Diagn()l;ing lumb, apinPl ~tenO/iis or t..miated inurverub.-al dise;' ... ually helpful only in pOl.ential,urrieal o:andidaU$'·. Thi& inc:ludes ~ti~o~ with appropriale. dinj· cel.yudroroe5 who have not respOnded satisfactorily to adequall non-au.rrieal treatment over a iufficient pe riod of time. end who have no medical CIHItnlindic:aobon. to IUrger)'. RIIdiologk confirmation of these diagnoaa uluaUy requi res CT, myelography. MRl. or $Orne MRI
MRl hill! supplanted cr and myelography for diagnosing moetdiac herniations and also in most cases of apinalatenoai • . n.e test of choice for patienta who have had previoul badliUrpry. Specificity and ~.iti";ty ror HLO are on the allme order ... CTlruyelOjlTa· phy, which is better than myelography alone,· .. • . Advaotages: I. provides inf(N"mation in ..gittal plane lea n easily eva luate ca uda equina) 2. provides information re,an:iing tissue o utaide ofth•• pinal ca"al (e.g. extreme late..al disc herniation (Me paiJf 311), turno ...... ) 3. non·invasive alld does not in""lve ionizing ."diation Dildyan la,.*, I. plltienu io.evue pIIin or with claustrophobia may have difficul~ holding It,ll '2. d .... not vi.ualiu bone w~1I 3. poor for etudying blood early (e.g . • pm..lepidu,al hematoma) 4 . eJ:J)@IlI'ye(nOl.l:maybemoreCOlte ffl!divethanmyelographyi f pon·myelogram overnipt hospitatiution i. avoided. and especially if a rln complic. tion from myelographyoccun) 5. difficult to inlt'llre t in ct._ ofltQliosi • . Myel~ may be luperior 6. a numberofcontraindi:atiooa: 1M COII lraiiVlieoliOlll to MRI, ~ge 135 Findiogs: In addition todemonltnti", herniated lumber dise 1l{lJ)) outaidaofth, dill-" are similsr toCT for detection ofHLD. When combined with polIt-myelographic CT ~can (my. elogrsmJCT), the sensitivity and ""peelally specificity increase>'. A herniated disk in the large spate between thecal $BC and posterior border of vertebral bodi"" at U·SI (insen· s itive apace) may DOt be seen on myelography (CT Or MRI are usually better at detectingthis). Advantages: I. provides information;o sagittal plaoe (unlike plain CT) 2 evaluaUls cauda equina (unlike routine CT) 3. provides "functiooaJ " ioformation about degree ofswnosis (a high-degree block will allow now of dye only aller certain position changes) Disadvant.ages: I , occuionally requires overnight hospitaliution 2. may mies pathology outside of the dura (including far laterally herniated disc). ~n$i~ivity i$ improved with pOSt·myelogr"phi~ (,'T 3. lIlVaslVe A. dru!1:S e_f(. wanarin must be stopped, and sometin:.es converted to heparin B. with occasional side effects (pOSt LP HlA, NN, raN! seituru) 4 . iodine allergic patients A. requires iodine allergy prep B. may still be risky (especially in severely iodine allergic patients) Findings: HLD produces extradurallilling defect at the level of the inUJrvertebral disc_ Mas· sive disc herniation or severe lumba r stenosis may produce a total or near tot.al block. In some c8Ses ofHLD, the finding may be very subtle and may consist ofa cut-ff of the fill. ing (with controst) of the nerve root sleeve (compared to nOrma I nerve(s) on contralaUJrIIl side or st other levels). Another subtle finding may be a ' dual shadow· on lateral view. BONE SCAN
Seepage 293
NEUROSURGERY
14. Spine and spinal cord
'"
DISCOGRAPHY
Injection of water· soluble contrallt agent directly into the nucleus pulposua of the in· tervertebral disc being atudied. Re~ults of the test depend on volume of dye accepted into the disc. tile pressure needed to iQject.the dye. tile configuration of the dye (i ncluding leakage from tile confines of t he disc space) on radiographic imaging (plain x·rays produce the so·called "discOf(ram" , CT scan may also be utilized), a nd reproduction of the pa· tient', pain on injection. Someoft he basis for performing a discogTam is to identify levels that may produce -discogenic pa in" or-painful disc ~yndrome", II CQntro~ersial point (see PRACTICf; PARAMf;Tf;R 14·5. page 300). Critique: Invltsive. Interpretation is equi'·ocal. and complications mayoccur (dillC8pace infection. disc Ilemiation , and significant radiation expo.ure with CT-disCQgr8phy ). May be ahnonnal in asymptomlllic patients""'· (as any of the above tests may be) IIlthougll tile false poaitive rllte may not beq uite this Iligh"'. SeePRACTICf; PARAMETf;R 14·9. page 301 for recommendations.
P SYCHOSOCIAL FACTORS
Although SOme patienta with chronic LPB (> 3 montha durlltio n) may h9ve started offwith a di agn0$8ble condition. PlIychological and socioeconomic factolll (su~h a8 depression. secondary gain ... ) may come to playa lignificant role in perpetua ting Or amplifying pain. Psychological factolli. especially elevated Ilyateria or hypodiondriasis seal.... on the Minnesota Mult iphasic Personality Inventory (MMPI) were found to be a better predictor ofouk:ome than finding-s on radiographic imaging in one study'. A .creening lICIlle of 5 factors has been proposed'" (positive findings in any 3 suggests psyehological distress): I . pain on simulated axiltllOllding: press on top of head 2. inconsistent performance: e.g. difficulty tole rating $traight leg ra ising (SLR) while supine, hut no diffi culty when silting 3. ove~action during th e physical exam 4. inappropriate tenderness tbat is superficial Or widespread these two items may not be rf!liable. 5. motor o.r sensory abnormaliti"" not the others an potentially reliable'" co~sponding 1.0 anatomic confines However. the usefulness o.fthis info.nnation is limited. and no effective interven· tions have been identified \.0 address tllese factors, Therefore the AHC PR panel was un· able to recommend specific as:senment tool . or intervention.' .
I
T REATMENT An initial period of nonsurgical management ("conservative" treatment. /IU b This term has regrett.ably come to be u.sed for non·surgical management. With slightmodilication. simila r spproaches can be used for mechanicsllo ... back pain. as well as for acute radiculepathy from disc herniation . Recommendations (based on AHCPR findings' in the sheen"", of "red Oag-s-A): II . OOm. kty liten"u", 1. activity modirkationa; nn studies were found thal met the panels review criteria for adequate evidence. However. tbe foUowing information WaS felt to be useful ; A. bed rest; 1. the theoretkal objective is to reduce symptoms by reducing pressure on the nerve roots and/or intradiscal pressu1"l!$ which is lowest in the s upine seOli·Fowler position" , and also 10 reduce movements which are e,,~ienced 88 painful by the patient 2. deactivation from prolonged bed rest (> 4 days) appe8.1""8 to be worse ror patien,", (producing weakneS8, stiffness, and increased pain ) than a gradusl return to O(lnnalactivities" 3. recororoenda tio",,: the majority of patients with low back problems will [lot require bed rest. Bed reat ror 2-4 days may be an option ror thOSl' with severe initial rodU;warsymptoms, however, this may be nO bett.,.. than watchful ....·aiti ng a.
NEUROSURGERY
14. Spine and .pinal cord
8 . illluffidfn~ .... i.t.ne« to Il'IlOmmend 8 MT in th., p~flCt! oInodi~u\(lp'lh)' C 8MT .hould not be u:M!d in 1111. face ofuvere or progresSM! neuroLogiedef;CII until "!.iou. CQllditionl hllve b _tcnos,. ...
PRAC TICE PA R AMETER 14 1 INJtC"ON
' H ~~AP'
.0" ,OW'PACK PAl"
OplI0n5" : lumba r epidural ifl,ie.:tionl or t n@r point in,j.etionilrelllll.-n· I!,lend ed for long·tenn relido(chronkL8P. These techniqu.. o. fa~1 il\ject,olUl may be ,,1\I!d to provid e ItIOJIONII)' relief in sel""t pl tientlo
DiiiftoiUO DptiDII5 "~
lu",ba r faeel lojecllons
may pndict the respllnf;e to ndiDfrequency facet ablltion
• nm rewmmende:i ba r (usion
11&
a diagn*tic tool to predJtI the n-s,onte to lum·
~Ql. recommended by lhe AHCPR pan .. I' for trealmenl of aeu\.e low back pl'llblems In the a~ of"red nap" (."" TaMs 14·5, page 2921: L medieationl A. or.1 lte roids: 110 dIfference was found alone Wefl: and 1 year Biter .andom· iUtiOn to receive I WNk therapy "'lUI oral den.nwthuone or plMebol" B, wlc:hicln,; sI .....
01 -.1IDgeI'* _
~
Th e u.. ofin.tNmenlilion increa_ the fusion ratel">. Hardwlre used in the It;.. een« affusion will eventually fatigue, especially in the region of the lumbar IordOlis. Therefore , iMlTumenlalion mU$t be viewed ala temporary internalltlbili1inl" mellure while ,waitinl" tha ruaion prooeu to complete.
S urgica l fu lion option ! Eerly experien« with midline f .... ion. rel"lted in luroNr ' pinal ltel108il as 8 late complication. Then.foT1!, CUlTftDt fUlion t.echniquel ..... pamro-llteral rusiQIra). Relatively contreindicated with well PT1!urved dille_ ' pI« height. Mlny PI.I Fa when atudied ~ 1 ,.."Ialolr Ihow re-)(yapa tite and lrio:l!ci wn phOlphate may be lubLtitut.ed fot lutorran;n lOme callet of ~Olterol~ter~1 fU l ion n!cium ph.. phate il recom.men ded AI I bona vanaxtendar, upacia lly when comhined with autoiogoul bone Aase5Ioi n, auryic:&i lumbar fI.Il lo D: See PRACTICE PARAMETER 14·8. PRACTICE :>ARAMETER 14·8 R.. O!()(.R"~M'C ASSfSS"'HH Of fUS'O"
StandardS" : It.lIlit ". rays
~lone
a ..
rua r_mended
Guidelines "": in t he illwln£f or riJid inl tn.oml1\tation.lack o/'mOlion between verU· brae on laten' n"".o.vuleo.ion x-raya i. high ly au.ggeltive Or,uCff$$_ rul fu.ion • tedlnetium·99 bone Ka nnin, il DIll recommended Options", radiOfTaphlc techniquel, onen in «Imbination, mlY be used when failed lumbar fuaio:l il IUl pected. including: atalic and nelmel ICPS) ",fer to their problems with af_ fecl iveOl"emotional terma with a higher frequency than th .... with acute pain". The amQUnt of time that a pa· tient hal bHn out of work due to low back problams i, related Ul the ch llncea of the patient getting back to work II.i Bhown in Table 14·7.
14.2.
Table 14-7 Chances ot pao lien1a going back to work TlIM out Chancel at gettilT9 at -..:oB bitt to-..:oB < 6 mol 50'4
"
""
Intervertebral disc herniation
INTERVERTEBRAL DISC
The funttion of tile intervertebral dill; is to pennit stable motion of the spine while l upporli.ng an d di stribu t ing loadl under movement. ANATOMY
Aoul ua fibl'Otlua (IInulu l may alte rnatively be lpelled ann ulUl, but r,b rotul it the only correct "pe ll ing and II distinct /'rom!ib,.,,'-,'!': the mu ltit.. minsted I.,a"",nl that en· rom pa .."" the periphert of the di$e apace. AtLach"" \.0 the end·plate ca rtas"" and ring opophYllul bone, Bland s ce ntrally .... itb th e nueleul pulJlOlU.l. Nu c:le ull pu lpo.u l : the centra l portion of the disc. A remnant ofUlI! notocord. C ap. w e': combined fibers ofthe anulul fibror;Ullllld the po5terior longitudina l li(. ament \th is term i, useful b«au~ th ese 2 stn.oetur"" may not be diltlnguilhable on im' ft,; ng st ud'''''l.
NEUROSURGERY
14 . Spine lind I pinal «Ird
'"
14.2.1.
Lumbar disc herniation
C LlN!CAL ASPECTS
The posterior longitudinal ligament is atro~at in the midline, and the posterolateral annulus may bear II disproportionate portion of the load . The .... rore , most hemi. at.ed lumbar disca (HLD) OCCur posterior ly. slightly "ffto one aide, compressing a nerve root, characteri~tically c81l3ing .""".e radicula r poin . (j.{, Importan t fatUI in lumbardis: diaease: !. in the lumbar region, the Mrve root exiUl ~ ~ a nd in dose pm,,_ imity to the pedicle or iUllike_numberll(! verJen.1msl' .... rene. is unr .. latlle IfoOl a/Wavs PI". 1.51, ma1 space is located well . '00 S1imulare eadUClGrS when . ' ia~ng below the pedicle I _WE.AKNESSIn TaOle tH/. P"!Ie303lot br/la~CIOWIl 3. in t he modal (most ~ MMOrYOnpeir~"I1IO$I_inlloedlSlal."' r_oIlh. common ) human spine, .,.,malo....... the", are 24 prtS9cral vertebme, however some individuals have 23 and others have 25"'. Thus, a HLOat the ultima te disc space(usu ally L5-SlJrnnstn!ten impin.ges on t he 25th nerve rootlhowever, in the varia nt cases, it may actuaJly impinge on the 24th or 26th rootJ"'
R ADIOGRAPHIC EVALUATION See Radiographj,c f uoluolion On page 293 under Low back poi" .
N ONSURGICAL TREATMENT
For nonsurgical trea tment, se.e "Conurooli",,·In!lIlmerll. p88e 296.
SURGICAL TREATMENT
INOICA TlONS In s pite of multiple attempts, nO One has been able to detennine which patients are likely to improve on their own and which wouJd be better served with $Urgery. 1 r~ il" .... of non_.".gic81 m''''~geme'' t' <Wr 85% of pr>ti .. nt.!l with 9~l"e diS C h e mo Duc\eoiYlli 9 Ac NEUROSURGERY
Percutaneoull e ndosco pi c lumbar dil",ectQmy (PELD): Thi$ ~nn refers to a n essentially intradi5"81 procedure indicated primarily for contained disc hemiations, although some small "nooconwined" fragroenUl may be treatabl~'>I. No large randomiud study has been done to compare the technique to the accepted s tandard, open discectomy (wilh or without microsco~). [n One report ...... of326 patients ",ith LA·S HLD. only 8 ap· propriate candidates for PELD (i.e. 2.4% ofHLD at L3-4) W~re found. Of these 8 , only 3 were reported as havinga good result. This study is not adequate forevaluating the technique. Intradi scal endotbermal therapy (lDt.'T): AKA intradiscal electrothennalanuloplasty (lDTA). Efficacy: 23·60% at I yea r for treating"internal discdisroption"'" (radial fissures in th e nucleus j:>ulposus extending into the anulus fibrosus) which is purported to account for 40% ofpatienUl with chronic low back pain of unknown etiology'·. A DJUNCTIVe TREATMEIflIN LUMBAR LAMINECTOMY
Epi dural steroids following di scecto my
In a single-blinded non-randomized study oflhe u~ or fJ).i.!1luaI steroids (methylprednisolone acetale (Depo- Medrol®). dor;e not specified) irriga t ion of the thecal sac and nerve root following discectomy prior to wound closure found no statist ically s ignific. of dressing: look for l ign. of nreSlive bleeding. CSF leak ... a s . • ign. of Clludll equilu. syndrome (1ft /KIIJc 305). e.,. by post-op .pin.1 epiduTlI hematelm.: A. loti of peri nul "nution (' •• ddle .neslhtli.· ) 8 . inability to void : may not be not unulu.1 .f\.er lumbar laminectomy. more C(Once ming ifa(:((l:npanied by ION ofperiM/l I "filiation C. pain out of the ordi nlry ror the post-()p period D. wealmesl of multiple m....,l. graup$ Any new Murologi( deficit should prompT. rapid euluation for . pin.l epidural hemotoma' " (EDH) . ~Iayed defi";uo may be due to EOH or epidural Post.-op films in the recovery room can rul e out graft or hlrdwan! malpotitioo for fuaio ... or in. slrumentlltion procedures, orchangM in sUgrunent. The dilgno.tic!at oI'ehoice i. MR1. Ifcontraindicated or oot available. CTlmyelography may be indic:llt.ed. An extradural defect immediately post.-op .uggesta ECH .
.hKa,.
OUTCOME OF SURGICAL TREATMENT
In a stries of 100 patientB undergoing diseectomy, at I year post-op 73.. hid com· plete relief oneil' pain and 63% had complete relief of back pain;.t S-10 yea .. the numbef1!l were62% for each ~ategory". At 5-10 yeart post-op. o nly 14% felt that the ~in will the seme Or worse than pre-op (i.e. 86% felt improved), and S" qualified .. h.vinll' a fai led back aynd r .. me (not returned I.() w"Tk , requiring analge\lio:., receiving w.. rlter'1 compenaation, see Fu.ilt!d bac.\ ~"dromt!. page 314). In the only random ized atudy comparing standard diMJect.omy with COIlSe ... Bt.Ve treatment, two groups of. SO patienta with a document.ed hemi.ted d isc that failed tel improve afl.er 14 d.y. of rut (without strong indicstions for SU~I)', e.g. csud. equin. syndrome, unbearable pain ... } were randomized to surgeI)' or cootinl>l.' lA-S &. 1.5-S1 herniated lumbar disc. (RW) account for most C8SC$ of HLD (up 100 24'111 ofPl"tienta wilh HL.D at L3-4 frequently have a PlSt history of. HLD It lAo S or L,5·SI. IU"estinll' a reneraliztc! tendency taward, dill( hern •• tion . ln. te ri« of 1.395 HLDt, there W.'I 4.t L I·2 (O. ~ incidence), 18 It L2-3 (I .3'iIlo). Ind SI at LS-4
98~);
(3 .6")101. PRESENfATION
Typiully presenU; wilh LBP,onselfoIlQWingll'tiumt.or at .. in in SI". With prove.· .ion. paresthesia.,nd pain in 1.he anuriorthillh occur. with complain:.. oriel .... alme.. (Clpecially 0ij~tiv. SLR). "h"""" ~'" _ g to I tha I it i, an ext remel,. late ... 1 HLD rather than an upper lumbn disc he rniation ,
..
• " " .,. '" • ' ,
.
PRESENTATION
Quadric'!ps ",·ea kn eBl. reduction ofpa te Uar reflex. and d,minisht'd sen8 Dlion in the L3 or U der(091.Onle are tbe most comnlon ('ndings. DifJ~ rent , al d;agn~i. ind udes: 1 late ral reces.'~nosi$ or superior artic ular face! hl'per1TGphy 2. relropj!ritoneal heOlatomft or tumor 3. diabf!tlc neuropath,. ( .m,.orTDph,.~ Uf p<JIJ;e fjij6 'I. spin all.uWOT A. benign (schwannoma or neurofibromal 8 . malignant (umOI'1l C. lymphoma S. i,,(oid r"OI>t . 1..... dilatation : """ Sp,nlJ/ mfn'tIjJf"ll/ cy.If, page 3.8 b. I ..chnoid cyit (from 8llchnoid he rniation through. d ... NlI defect); notauoci.ted with feat joint. ma!"(inl thinner th an JFC'" 6. perineurill cysta (Tl rlov'l cflt): .rite in l pace between perineurium and endane ... ri ... m....,ulllyon ucrll roou' •. a«al;onllly &how delayed filling on myelog· ra phy comp~ssive
PatbololY Cyst walla are compased Gf fibfO\.l' cono~tiva ti.,ue of varying thiekn..... and ceHu· lari ty. There is \l5ulHy no aigtll ori nCection or inflammation. There lIIay be a synovial lining'. (sync,...al (flt) 0 . it (I"IIY be Iblent'" (ganglion cflt). The distinction between the two me.y be dil'rlC:,,]t ''", pouiblyowingin part to the flct thst fibmblllJltJ in ganglion cYIU mly fonn In incomplete ')'1IO"ill.like lining'·. Prol,ferltion of .maU .. en ... les is lee n in !.he eonneclive liMue. He~iderin Itl ining may be p ~e llt. and lJIay Or may not be as· toci.ted with a history oft •• " ml'''. Evaluation Id enlifyingl J FC pN>oOp helps the . ...rreon, I' the approach differs slightly from that for HLD, end !.he qat mi~t otherwise be missed 0 ' ... n kn .... ingly deflated and un· nKelaery time wuted afterwards t.r)'iDg to find I oompreuive lesion. 0,.. the unwitting may mi sinterpret the cyst as a "tran5(\" ••1 disc extrusion" Il"d needll!tily open the d ...... ~pdi"",_ were ;nrolTe '"
tA . Spin. and spinal cord
NEUROSURGERY
1Urvery (ror hemiatoed in~rvertebral disc. iarninect.omy for atenOllil .•. ). Thea" pat;en'" often require analgHiallnd • .., unable toNltum to work . The fai lure rate for lumbar di .... c;ectomy to provide M tidsctory long_term pain ",Iieri, ~ 8·251b" ' . Pending legal or work· er'. eompen98tioa claim. we.. the mO$t frequeM dete rrents to a good out.rome"', r a(ton thllt may CBuM or wntribut4: to th e fa iled back syndrom e: I. iTlC'O~ initial diagnOlii. A. inadequate pre-op imagil'l¥ B. dinic.J findi ngs not correlet.ed with abnormaHty demon. t. atoed (In imagi o, C. othe r ao utea oflymptorlll (Iometim e. in the prelM!:nce orwhat was coDsid· ered to be an appropriate Inion on imaging . tud i" wh ic h may h.~ been ..ymptomatic): e.g. trochanteric bursitis, diabetic amyotrophy ... 2. continued nerve root or cauda equ ina com p ~ion caused by: A. f'Midual disc material B. Teeu,",,,! d isc hemlation at th.tame level: u l ual :)' ha"e pain-free interval :>
6 ..... post-op (I« ~ 317)
C. disc herniation at another levtl D. oompreMion or nerve root by perid uralllCar (il'"", lation)tlnue (lfe belowl
E. pseuoorwningooele F. e pidural hematomll
3. 4. 5.
6. 1.
8. 9.
G. aegmenta l instability: 3 pattem.e-, ) ) latera) >"OU tional in,tability, 2) poIIt. op . pOndylolil the, il , 3} poIt- ,r
Radiographic findings in .rachnoiditi. NB: RadiOjl'aphic evidence ofa rlchnolditis m.y ,lao be foww:! in umptDm ltk patitnta-. A.. chnoiditis must be diffel't'nti. ttd from tumor: the oentrtl . dhet.ive type (.,.., bel/)W ) may rll$f!mbie CSF Heding oftumor, and myelOfnlphic block may mimic in_ tr.th eca l tumor. Mye logl'IIOl' May demonltrlte completa block, Ol'clu mpi ogofnel'V' roou. On. of m.ny myel0jl'8phic cI.Mif,cation ' ystam, - for arachnoid ili. i. Jho,!; n in Tabk 14· 13. MRI, 3 pfItUrllt On Mltl ...· ..:
NEUROSURCERY
14. Spine.n d l pin.lcord
lOS
central adhesion of the nerve roots into lor 2 central ~C(lrds· "empty thecal aoc"pattern: roots adhere to meninges a round periphery, only CSP . ignal i. visible intl'llthecally 3. thecal sac filled with innammatory tissue, nO CSP signal. Correspond. with myelographic block end tlndlt·dripping appearance Arachnoiditis will usually not enhance with gadolinium WI much as tumor (In MRL 1.
2.
PERIDURAL SCAR
Table 14-13 Myelographic claasillcalio n 01 anlchnoidil is
Wllu'ldoboJlaf MOO·sac with loss oJ A1thougb peridural scar i8 frequently radicular strialicr1s blamed for causing recurrent 8ymptoms.... "·, there hou bee n no proofof corre lation"'. Peri· dUTIII fibrosis is an inevitable sequelae to lumbar disc Bu rgery. Even pat ients who are reo lieved of their pain following di$Cectomy develop some sca r tissue post·op'''. Although it has been shown that if a patient has recurrent redieul ar pain following a lumbar discec· tomy there is a 70% chance that extensive peridural sca r willllf! found on MRltl'. this Bt udy also Bhowed !haton post-op MR ls nt6 months, 43% of patients will have extensive star. but 84% of the time this willllf!~"·. Th us, One must U5e clinicsl grounds to detennlne if a patient with extensive scar on MR I ia in t..'e 16% mioori ty of patients with radicula r symptoms a ttributable to sea""". For a discuuion of mea&urflS to reduce peridu ral scarring, see polJe 307.
RADIOLOGIC EVALUATION
Patien14 with ollly persistent low back or hip pain withou t a strOllg radicu lar com· ponent, with a neurologic exa m that is Ilor mal Or unchanged from pre-op,should llf! treated symptomatica lly. Patielli.!l with ~ igns or symptoms of recurrent rndiculopathy (positive SLR is a sensitive test for nerve root compreeaion), especially if these follow a period of apparent recovery, should undergo fu rther evnluation. It is critical to d ilTerentiate UlIiduallreclll"re nt disc herniation from scor tissue and adhesive arachnoidi t iB as surgical t reatment has gellerally poor results with the hitter t wo (..,.. T~olmel1l o{{ailw back syndrome llf! lowl. MRI wmlOur AND WITH IV r;ADO/.lNlUM
DiagnostiC ~1".d.tl!kl:. The llf!st exam for detecting residual 01" recurrent dis 14. Spine and spinal cord
NEUROSURGERY
enhWlee.s, and may be mOre pronounced when it is distorted by disc material, but the mO'l'hology i~ easily differ ential.ed from 3C8r tinue in the8e U!IU, On /(;.tt (> 30 mi n9 post-contrast) T I W1: scar enhllll Gene rally helpful only iu eases ofi'Ultability, malaHgnm~nt, or sp•.mdyIOllis:" . Flexion/extension views sre most helpful when trying to demonstrate instability.
T REATMENT OF FAILED BACK SYNOROME
For treatment ofinte,..,·ert.ebral disc-a pace infectil)fl, oee Discitis, page 245. S y mptomatic trea tme nt Recommended for patients who do not havII radicular $igns and symptoms. or for m06t patients demonst~ated to have scar tissue or adhesive anochnoiditis on imol,:ing. As in other case. of non ·~pecific LBP treatment includes: short-term bed rest, analgesics (non-narcotic in most cases), anti ·inflammatory medication (n(ln-steroidal, and occasion· ally a short course of steroids), and ph)'!lieal therapy. S urge ry Reserved for thOK with recttrrentor re sidua l disc herniation, segmental instability, or patients with a pseudoroeniogoeele. Patiente with post-op spinal in~tability should be " repair di"",tly. Place fascial gran beneath bone plug. Keep HOB elevated post op. Consider fibrin glue, lumbar drain F. Horoer's syndrome: sympathetic plexus lies withLn longua coli, thus do not extend dissection far laterally int..> these muscles G. thoracic dud injury: in exposing lower cervical spine spinal cord or nerve root injurie$ A. spinal cord injury: especisUy risky in myelopathy due to narrowed canal. Minimize risk by penetrating the 08leophyte at the lateral margin of inter. apace /increases risk to nerve root) B. avoid hyperextell$ion during intubation: anesthe.iologist may need to d~ tennine patient's tolerance pre-op. Consider fi~l optic guided or awake mo· &otracheal intubation in extreme stenosis C. bone gTan must ~ shorter than interspace dept~ . Exercise cau t ion in tap. piog graft into position D. rare but serious complicationa ofC3-4 operation. : sleep induo::ed spnea031 (possi bly due to di sruption ofthe afTerentcompor.ent of the central respira· tory control me 14.3.
Degenerative disc/spine disease
Since structures outside of the disc are usually in¥lndyLoli~thesis: AKA apondyloly sis: a failure of t he neural arch manifesti ng as a defect in Ihe pa.rs ioterarticuiaris (the ne 14.3.1.1 .
lumbar spinal stenosis
Unless indicated otherwise, this discussion refer1l primari Iy to central cansistenosis t Key points caused by hypertrophy offaceu and ligamentum Oa"um, may be exacerbated by dii!cult)' avoiding nerve mob). M.R.I: demonstrates ioopi"lement 0" neural structures and lou ofCS," signal on '!'2WI at leverel)' stenotic level s. MR I i. poor ror vi.ua)izill8 bo.ne which '"IIntributell . il· nifka.nt ly to the patholcgy (ooay be helpful for l urgical plannir.g). Good for evaluating nervi impingement due to lpondylolilthnis {pt*.ibly better thin myelogramlCT) snd jwrtafleet e,.~ . ....ymptomatic abnortnaliti ... are demo ... tca~d in up to 33"10 ofasymptoroatic patienl.a 50.70 yean: old"'.
'UI'f'IM,,"
NORMAl. LS SPINE MEASUREMENTS
Nonnll dimension. o/'the lumbar "pine Ire . hown in Table 14.19 for plain film and TQblf 14,20 ror CT,
T, bl. 14-19 Normal AP dlam.t., on I,t."l AIaIO..UInl (110m spio'loIamInar line 10
rio< ~ebrai
pasle·
llodv)-
"'-
~doD'a(U>O) QnaI~lra ~
_...., thodInnIa ,
~ fA 1IIerJI_ ($lit
NEUROSURGeRY
bf/oIoj
a 11.5mm a 16II1II a U5an1 " •• $II1II .3~
wte.-pcdiculate diat a nce (l]' 0 ): The t..ansverse d iameter of the s pinal canal. On plain AP x-ray of lumbar spine , an [PO < 25 mm suggests ate nos is. Average normallPDs in the lumbar and 10""er thoraci c spine appears in Table 14·2J . An approximation for the lumba r spine il given in Sq J4.J.
Table 14-21 No rmal in lerpedlcula~ dIslance (lPO) on AP L.S
"Bicycle test"; patien ts ..... ith NC can usually tolerate longer periods of exercise on a bicycle than patients with in· termittent (vascularj ciaudication becaus-e the p 1.0 is nonnal : mean of 0.59 in patients with intennittent claudication; 0.26 in patientB with res t pain: < 0.05 indicates impending gangnme. Vascular lab studies (e.g. Doppler) may assist in identifying vosculor insufficiency . EMG ..... ith NCV may allow multiple nerve· root abnormalitie!l bilaterally.
9O'Ii.1OIerance range lOr ~dul " IlIrge1 !O lilm eros-
urnc 3 months, Bnd moalpatiellt.li who have surgery for this have symptoms of > 1 year du ra tion.
Surgical techn iq u e Undercutting the superior articular facet is often neeeuary to decompre$& the nervea in the foramen (see wluu/ ,.,.,.,so ttyndrome, page 330). TTeal.meM of onodetate a te nOl'lis at adjacent levels appeS/"8 warranted as these level$ have been shown to have a significant likelihood of becoming symptomatic late r""'.
Progression of spondylolist he~ a Moy "",-,u r without deco"'pre3sion. but is more common followin g sU'1!"eryllf . However. IW"Oba r instability follo .... ing decompressive laminectomy ill ra re (only - 1% of all laminectomies fOT stenOl'lis will develop progressive subluxation ). Fusion is rarely reQuired to prevent progre.!lsion o( subluxation with degenerative 6ter.osis"'. Stability (witbout nood for instrumentation) is thought to he maintained if> 50-66% of the facets are pre.erved during surgery and the di sc s pace is not violated (mainLRins integrity of anterior and middle column). Younger or more active plItien ta are at higher r;.k ur~ubl"-,,inll ' Ooe approach is to obtain nexion/extension x·rays pre-op, and follow patients aner decompression . Those who develop sy mptomatic slippage po$t--p are treated by fusion, possibly in conjunction with spinal instrumentation.
'"
14.
S~ine
and spinal con.!
NEUROSURGERY
INSTRUMENTA TION ANO!OR FUSION PRACTICE PIIRA MtTER 14 11) FUSH)N '" PA,1,,"'TS "''''' I UMUA" ~lHIQ~I~ \\1">nlidered ' n plltienta with .tena.illind s pondyloli. the, i. in CasH where then!. is p_p ... identli Qf . pinO\I inallibi tity'" or kypboori~· at the l""'el of l he. l paMylnlil th..iI Dr when iattogenlc: insUbility is anticiplted ""otfWo\oI~_~_"'
..
_IIIISIOOI_.'~
Fusion on.y " _leU,," degetleratiYe Chll l\fe511t IIdjllcerlt I~vel&. Some ~ u.rgeonl""'" o mmo:>nd fUlion at levels m s poldyklh5the5tie lWl nosi.- ". Patients wi th CQI1'\bined degenerative .pondyloli.the.;s, 'limosi!, and rlidieulopllthy IDlly be reasonable candidl!e! (or fusion !, BRACE THERAPY PRAc n CE PARAMETER
I~
12 BRaCE T> 6 manu." duntion fs 1Wl....,.,."rnem!ed becau~ 1\ has not beeo ahown to be" 1000-terTU beoefit Op tions"': lumbar b ....Cft DlIY reduce the nllIDbero(.itk d.,.. due to LHP emons ...'Ortenr Wi th II pre¥1oo. PRESENTATION Patienude"e lop unilateral or bila teral l~ p"i n pr«lomioa oLly whe ll "'alking or sta nding, and usu· pJ ly obtain relierby sq uatting,8ilt ing with tl,e waist fleKed, or lfing in lhe reLaI position. PSinful burning paresthe· sif.8 of the l.:.wer u · lTC!mities are also described. ValslOlva mOneuv,,", usually
IiIltral recess heiglI
.,
do flot u., 14.3.1.2.
,,
on CT (bone windows)
Cervical spinal stenosis
·Ce ..... ic al 8poodyIOtlis" ;5 ~B8 i onally used synonymously with o:ernca! spinal st.&nosia. However, spondylosis usually impli es a more widespread degenerative condi· tion of the ceJ"Yiealspin e inclu ding varioua combinatioos ofth following: 1. oongenilal spinal stenosis (Ihe "shal low cervical can81-'"") 2. degenetlltion ofthe int.eJ"Yertebral disc producing a focal &t.enos;s due \.0 a ·c.a .... i. cal b ar whic:h is usually 8 oombination of: A. osteophytic spu,.,. ("bard disc· in neurosurgical jargcm) B. aod/or pro'rusion of int.e ....·ertebral disc material ("110ft di sc") 3. hypertrophy of any of the fol1owing (which also con tribut" to canaI8t.enosis): A. lamina du ra C. artiC"Ular faceta D. ligaments. including 1. ligamentum navum: neck extension may cause an incr ease ofinfold· ing oftha ligament from the posterior spinal canal 2. poIIu-rior longitudinal ligament: may include ossification of the posterior longitudinal ligament (OPLL)'" (U'O' ~ 345). May be segmen· tal or diffu9l!. Often adherent to dura 4. "ubhu;ation: due to disc and facet joint degene ration 5. alt.ered mobility: severely 8pondylotic levels may be fused and are usually slable. however there is often hyp NEUROSURGERY
14. Spine and spinal cord
'"
30% natTowing "fthe eroas·ae of ofCSM, lhere is progrusion either in a stepwise f~shioo (in one third) or gradually progressive (two IhirdsP"". In lOme series. lhe most oommon pattern WS8 that ofsn initial pha se of deterioration followed by a stabilization that typically lasLs for years and may not change therealar.... ,., _ln these oases, the degree ofdisabi rity may be established early in the coUrse ofCSM. Others disagree with ~uoh a "benign" outlook lind cite that over 50% of C-8ses continue to de· teriorate with conservative treatment.... Sustained spontaneous improvem NEUROSURGERY
14. Spine lind spinsl cord
3"
ed to 1 or 25 pinal cord segments_ Inevitably, some casn of demyelinating di$ease will be misdiagnosed initially as CS M until ~ome featurea suggestive of the former OCCur. EVALUATION
Plain ,.-rays Plain cp.rvical spine lI;·rays demonstrate s o!teophytiand clution mu~t be e>:e rcilled in hyperlordosis (_ btl"",) Anterior . pp ro.ch, Another controveny here i8 whether Or ~ to use Interior oerviof patienu af\er anterior deoompreP;on.... " ·( inl r.operative SSE P monitoring may reduce thi. IlIte''') .nd C5 111· d itulopllthy m.y occur (.eo! boolow ). P o, te r io r I ppr oach : For a decompretllive oervieallaminectonly, common practice dictates removal of 10mina extend ing one Or ~ levela beyond the , ,"",n.. il .bove and 1w1~ "'J". A C3·7 laDlInectomy ill olUn eon.idered I · . tandard" laminectomy. An "extended l.minectomy'" inclu-, with nO radiographically apparent uplanation in u p to 20~ofthese ca!le!l .... In othel"$, degeneration at levels adjacent to the operated segments may be demonstrated .
14,3,1_3_
Coincident cervical and lumbar spinal stenosis
Coincident symptomatic lumbar and cervicalspinalstenosia i8 usually managed by fin;tdecompreSlling thecervi~al region, and later operating on the Iwnbar region (unless severe ne ll rogenic claudication dominates the picture). [t is also possible, in sele 14. SpiM and spinal cord
NEUROSURGERY
A. Chiari ma lformaticms S. Klippel.Feil syndrome: u~ po~ 119 C. Oown'ssyndrome D. atlantoaxial disloc.a.tion (AAD) E. occipitalitation nfthe atlas: seen in 40% of congenital AAO't2 F. Morquio syndrome (a mucopolysal'charid()$is): atlanto,&xialsubluxation occurs due to hypoplasia of the odontoid process and joint la~ity 5. neoplasms: primary or metastatic 6. infection 7. following surgical prOral reo section of the odontoid Abnormalities include: 1. basilar impression 2. atlanto-occipital dislocation 3. atlantoaxial dislocation 4 . occipitalization of the atlas, or thin Or deficient posten"T arch ofatla .... TRcA TMf!l'iT
Fracturesofthe occipital condyles, atlas or axis are usually adequately treated with external immobilizatioo. ~cause traumatic occipitocervical dislocations are usually falal, optimal treatment is not well defined. Occipitalization ofthe atlas may be treated by creating an "artificial atlas" from the base of the occiput and wiring to that.... Indications are outlined inAlloi\lO(Uial (usion (Cl·2 orlhrOiksis) on page 623.
14.5.
Rheumatoid arthritis
More than 85'1> of patients with moderate Or8evere rheumatoid arthritis (RAJ have radio· graphic evidence ofC·spine involvement"". For involvement in the upperCo$pine. 8« btlow. In the s ubaxial C-spine, tbe m()$t commO n pathology is su bluxation. A grllding system fur neural deficit devised by Ranawatet al.:I2< is;;hown in Tobie 14·24l1nd has also found use in other types ofspinal cord injury.
14.5. 1.
Table 14-24 Ranawal classification lor s pInal cord deltclt Clan no r.eurailleflCil sLtljec!ive weakness. hyperrellena • dyses:besia objllClove ~kness • klng tracl signs '" III A. amboJlalOfy III !I" qUlidripare\ic & non ambulatory
, "
"'''''-
Upper cervical spine involvement
The upper oervica~ spi ne may be involved in RA in up to .:w.!I8% ofoase,..... Mala lignment and instobility result from destruction of bone and supporting ligaments by synovial proliferation. Theinvolvement may rane:e from mild (a few mm of asymptomatic subl uxation ) to seve ... I•. B. b•• in .t~m compr... ion"'). Two commOn types ofC·spine involvement in RA (often found together): I . anterior at la n tou i al s "bhu:ation : the most COmmOn manif~l3tion orRA in the cerviealapine, fouod in up to 25% of patienl3 with RA (U~ btlow) 2. basil a r Impression (RI ): upward tran slocation of the odontoid process, found in - 8% ofpatieot> with RA (m' pagt .3.39) Less common involvement of the cervical spine in RA: 1. posterior subluxa tion of the atlantoaxialjaint: must have either associated fracture of or near total arth ritio erosion of odontoid 2. 8u baxial s ublux.ations (su bluxations below C2) 3. vertebral artery insufficiency secondary to changes 8t cranio-cervical junction""'
NeUROSURGeRY
14. Spine and spinal cord
ATLANTOAXIAL SUBLUXATION IN RA
Inflammatory involvement of the atlantoaxial synovial joints causes ermpression first allows some patienta toavoid a second operation. and permit.s the remainder to undergo the anUlrior opprooch without becoming deBtabili.ed . Still. &Ome surgeons do the odon· toidectomy first"'. Reminder: the patient must be able to open the mouth greater than _ 25 mm in Or· der to perform tran80ral odontoidectomy without splitting the mandible. PoSTERIOR FUSION
SeeAllantoax;al {"sw" (Cl·2 aFthrad"si,) on page 623 for te NEUROSURGICAL INVOLVEMENT
PD may pres"nt to the neu"",urgeon as a reBult of: hack pain: usually not as a direct result of vertebral bone involvement 1_ ~Iwl spi nal cord andior nerve root symptoms • compref;Sion of tile spi nal cord or cauda equina (relatively rare) • spinal nerve-root compression • vascular steal due to reactive vasodilatation adjacent to involved areas with skull invol>'emenl, compression of crania l nerves as they wtit through bony foramina (Bth nerve is most common, producing deafnesa Or ataxia): see page 918 10 ascertain diagnosis in unclear bone lesions of the spine Or skull
E VALUATION I . lab work (marke", may be normal in monostotic involvement):
A. se rum alkeline pllospllatase: usually elevaud (tlli, enzyme is involved in bone syntllesis and so may nol- be eleval.ed in purely lytic Paget's disease""
2. 3.
4.
" "",); mean 380" 318 lUlL (norma l range: 9_44?",. 8one-speo:ific alkaline pllospllatsse may be mOre sensitive and may be useful in monostatic involvement"" B. calciu m: us ually nor mallif elevaud. one should RIO lIyperparatbyroidi.m) C. urina ry lIydro~yproline: found almos ~exclusively in cartilage. Due to tbe lIigll tuTtloverofbone, urinary bydroxyproline is often increased in PD with a mean of 28O.t: 262 m!(l24 bTII (normal range 18-3B)"" bone scan: lights up in areas of involvement in most, but not all~ cases plain x-raya: A. localized enlargement of bone: 8 finding unique to PO (not seen in other osteoclastic diseases, sucll as pl'1ll!tatie bone meta) B. cortical tb.ickening C. sclerotic changea D. GSteolytic oreas (in .kull- osteoporoolis circum.cripta; in long bone~ _ "V" sbaped lesions) E. spinal Paget'sdise&seoften involves severa l C!!ntiC'lICU/sleyels. Pedides and lamina are thickened, vertebral bodies ore usua lly dense and oompressed with increased width. Intervening disca are replaced by bone CT: lIypenroph ic cllanges at tile facetjoint.ll with coarse tJ"abeculation$
14.6.1.
Paget's disease of the spine
PRE SENTAnON
Theove rwllelming majority Q/"pagetic lesions are asympt.:>matic" " '·'" with lesions det.> SURGICAL rR£ArMENr In gene'III, _lQl'Yatj"e t realmen! oflTadoW1!:l in PO h8~e II hilh raUl cfdelayw uoion.
Surgi cllI ind icat i OClo II tor s pina l Page t 'll d ille a lle 1_ nlpid P"'.... -'o'I; indl~tiog pOIIr,ible O\a1iJ'llant challie or -r>inal ioatabiHty 2. .pioal inatllbili'r: nvere kypho&i. orcompromile of eao,tl by booe f'8(1l1eobl from patbolock fract-.ne. AlthauKil the «lila.,... i. ul ually Il"adual, ...ddeo cornprettaion mayoteObl.!lst ie lesioM) 4 . failu .... to ;mpro~ .. wi!.b medieatiQnll SurwiCllI _ aideration$: L profuu b\fttldiol;1 eoannon; ,'significaot bleedillg ~u ld pOllent an un usu81 problem, Irellt bt u long u l"e8.ib1e PI"lHlP with 0 bipbNphollatol Ot ulcitonin (M~
2.
caboorc )
post·op medical trMtrMnt _1 be ne«sury to p .... veot r~rnncesN
:I. IIOteogenic Un:(IlIIa A. aurgf:ry and chf:lDOthuapy ani! UMd, WI"&> bu~ may b .. helpful for monitorinl: angoing pby! icai therapy. Neurosurgica l inv(llvemen~ u.ually N!!Iul!.S from the rollowing : I . cauda equilUl syndrome (C ES): et..iolagy is frequently Ul>Clea., but is uBull!l,y rurJ. due to sten08is (lr rompres6ive lui(ln. In t he ab94!nce of com pressi (In. Burcica l in· tervention is not indicated 2_ ro18tory .ublultation: nt ocd p;\.O-atlanLIII.l.l1d atlamo-axialjoints. Ma,y oc~ur ~8 these OTt! typically the last mobile ""K'"IIDtS of the spin ... Incidence i5 much Ius than with rh~uou.toida"hriti, . Leaiorus that mightbe stable in otbuwiee narmaJ spinea are often n(lt ,table in AS 3. myelopa thy aecondary w bow·strinaing oCthe cord: laminl!(tomy may aggravate 4. acute I plnal cord injury [Scn: riak (lfSe) orCES dut to fracture is increased in AS, wnd may O(cue following minimal trauma . Injuries are ITKIre rommon in the lower cerviove with slup arRD, 4. othe r varianU: itJ.ludes a /'lire t~ oF OPLL that is eont.ig>lou, with the endplald li nd is confiDed t.o thedi5C$p!leI! llO vDlvl!!; focal hypert.rophy of tile PLLwi\.h punctate "alcifi"lItion) EVALUATION
P lain lr-ray. OftEn fail to demo:15trot.e OPLL. NEUROSURGERY
1(, Spille and s pinal cord
,,,
MRl
OPLL appear& as II hypomloenae area and iI dlffieuJt to IIpptecia1.8 unlil it reQcheli _ 5 mm thickness. On T1WI it blends in with the hypointerudty oCthe ventrnl eubal'8ohn4id I;JIII.ce: on T2WI it remflLPa hypoin tenR white I.h~ CSF beOOJl'\ei bri,ht. Sagiu..ol images mllY be vu)' helpful in providing an O\~",il!w of the utent ofinvo)vement, and T2Wl m9,Y demonstrate intrin~ic~pinalcon:l .. bnonnal;ti"" which may bea.\ll!IIL-myelographic CT (especlllily with 3D ~1UIt.ructio(6) is ptObably beJ ! n demolllltrating and II~Ur8t.e[y diagnosing OPLL. TREATMENT
T rO:!Btment deci sion s Bas"", on cliniCII! grade"'" as {ollow.: Clol;3 l: flIdlogr9phk eViden~ without clinical gjgnll or symptOms. Mod petienU with OPLL are asymptonUllie"'". Con"",."at;v!! Il>lUlagemtnl unlellS severe C I&IlI II : patient.s with myelopathy o~ ... dicuIOJlllthy, Minima l or sl..8ble deficit may be followed expectantly. Significant deficit Or evidence of p~ftsion w&T-
rant. t urgical intervelltioll Clasa ilLA: model'flte to &evere myalopllthy_UeusUy ~uires surgical intervenLion CIQ81 ms: $4'.vera "'complete quadriplegia . SW"/Ier)' isconaidered for incomplete qundriplegir.s ahow;lIg p~ressive slow wor~ ... ning . Rapid i:leteriorstloll or complet.e quadriplegia. advana!d ag~ or poDr mtidica! C01Idition 8rt-all lIBSOCiated with "'0"", outcome
Tecb);licaJ cons id eratio l18 for surgery' SSEP moni"'rin, tuu~ ~n rel>C)ftlmen(!ed by some.... P06t.-erie~ there "'U ' 10% incidenceoftrall3ient wo~ningllfneu rologk function follow 'ng anterior ...,rgery"1 which truly hllve been related U> distraction . The risk ofdW"1l1 teal wilh CSF leak fo\lowin( an IInterior 'pproach depends on tile aggressiveness with which oo"e ill removed from the dura, Mnd ran(!!tI . 16·25%. O!.her ri!1Ia of a nterior 1ll'I'\'OiIehes (e.g. esophageal i:1jur)', !lee ACVP compfU:oliollr, pIIile 320) a1so pertain.
14.9.
Ossification of the anterior longitudinal ligament (OALL)
OALL or th .. ee .... iealsplM andlor hypertrophie IillteriO' cerviea l 08teoph}'1M may prodllcl!-drlirllBti~
rod iograpluc finding6 atld minimal clinicai llyroptoml. SeparllU! from Foru tler'! disease{," tn/ow). MJiy present with !lytphagio)OlJ.
14.10.
Diffuse idiopathic skeletal hyperostosis
AKA "DISH ", AKA ! pOndyliti~ OSIIificans IipmentO!lll, AKA /lfIkylO11;ng hypero&t.osi8, ~mQllil others. A condition cbaract.e.riud by Oo....-ing osteophytic formation of the IIpine in the Rbsen"", of degen",ratiVOJ, traumatic, or post_infectious changes. Affects Call· cuiana a.nd males mOn! wuunonly, and ususlly....en in petien,"" in !.he;" wid fiOs. 97% ofcaJ;e11 occur in tbe thoracic Ipine. also in tbe lumbar .plne in 90%. cervical spine in 78", .and aU Ihre! segments in 70%. Sacrol1iaejointll are ape.re;:l (unl iU snky·
....
14, Spine and spinal cord
NEUROSURGERY
lo5ing spondyliU., _ PIlP !N31. 11lually~ not prodUte dinicslsymptoml. PatienUllllllY hllYClenrly momln, I tif!'· n_ and lIlild limil.atlt.... a(' ...:tiviti". r.by p"-n~ with d,..phllgia or Glob ... (I unu· tion a(' I lump in Ihe UIo'OIt. u.u.liy attribULed to hysteri.) due to eompre.aiOll of the elOphat:WI betweM the osteophytH and t~ rigid IIryc181111 .t."'Uet ul1! .... (port of F'orul.ier'ld i_ - t In CllMSofdysphal'a. av.luati<m ,hould lndU'da b.rhun.",al1ow!.l> help 101;.11" the III'" ofobol.rUl;l.ion. and esopbl8OllllOPY to ru'I'Qut Intrinsic IlIQPb1veal di,Oi"'IWI. Plain ~ • ...,. and CT KIn hel .. demoo.trl'" r.hI p/ltholoc'. c.... tbat do nOt reapond ... 1.i~fIlC: torily to dietary mooiiflCatioOl.hould be eGruoldered for lurgery. An ant.enor cervlulapprtNl(h. and ",uliUltion of. hilh .• peed drill with ta rtf",1 pro!.«tooo of .., n.·ti..."". I1.rUCtu rn (_phagu •• eatol.id sheath) without need 10 Iwbiliu apine nor diacel:t(lm,r hnl been recommended ....
Spinal AVM
14.11 .
Inddor_ ofllpin.l AVM (8-Avtot. ) is .. bout4 .. tof'primary intrupln ..1 mU/tH.8O'Io oecur bIt .... etn 11'1 20 -.d 60 yun*" - "'. MI,ior daM iftaltiona"" - ' Type ,. dural A'IM, Fed by I dural artery.nd drain in!: {nto a.pinal Wlin vi" an AV .bW'lt in Ih,lnwrvertebral for.men", The moon common type ofS·AVM in tn. Idul t"', GentaoeoW! lhrombnm oflhe AVM t.aWline: lubarullL necroti1inc m~lopIltbr" which would be ilT8>'ersible, However, more ret>enl""ldene. wRnta that Ole rl\)'8lopa. thy mDy be due to YellOW! hypertension with oecondar)' ;Kbemil, Ind the~ rLlJIy ba 1m·
prn\lement with t reaUllfl'lt.. •• CLINICAl A" *c,,IULtion Ovtr .pin~ mlly reveal. bruit in U"" oreuel. Cutaneo... an~ over back i. preienL in 3·211"ofclIlI«. yaJllalvII mllMuv. Mlly ell""" redicu.!opathy by p~""'" on adjacent n"tva root. ("""Y ~r IIlII)' not UI""" aym p\.oD:la of nerve roat (rom .. hieh it act_H.y ...ue.). Symptom complex depend.. on a;:r.e ofSMC . and pro~imil)' toO I pinal Q.lrd and nerve rootI. Type I SM Ca: in t.horacIc&l:ld cervical~, may pr~t .. ith l ruit' myelopathy (lJIIIl8I.idty and ... nllOf)' lenl); lumbar region - LBP and rlKi iwlopathy; lUIcnll ...• (ion - aptUncter d bl h.rballce 1)"pe II SMC" ollt"n I!J)'mptolllatic. but ..."..lleaionl IIII)' - .aatiea indio. Bphirw::ter diawrban .. Type III SM Ca: IIIII.)' also be multiple and ..ymptocuttic: more common along paS. l.enor aubo;rachnoid.pfOC(! EVALUATION
M"RJ to identify the 01"', thea witer-eoillble leer tc:I.O 10 eval .... te o:omm\lJli(1OtiOtl of t,Yllt with II'IIhatachnoid SpA!.'l't'OI5ive oeurologic dewrioration ""er month t.o ),eaJ'li. U8uaU)' Rlreetin!: UE rlr~t diameter:> Ii mm .. Q3ocist.ed edema prndiclu more ra~id deti!riaration prefel'Tf!d tr ...atment i1 directed et ft.en associated With "bnonn.iiliell of Ille forRmen m~gnum, e.g. ChWri type I malfarnl.tion (~tpag~ 1(}4) Or builar Ir1I.chnnidili5 (pos(..;nf Mecb an iam o r rOI'TO Btion obstl"Uctive le8lon~: etiology probably lies in outlet ohstl"Uction (partial or rom_ pletel uffaurth ventricle (forBmina ofLuschka and Magendle). CoogenHa l «Indi· t;ano .u~h ~ Chiari malformation type I or type 2, ce~bellar e(;topia. ba$ilar iDlp",ssiaQ (wi th (006tri('\;on ofthe foramen magnum, ~e 1391. Oandy-W8lker synd roDle. and acquired tnndit ions ~uch as adbe~ ive arachnoiditis are ll8Sociated with high iDcldence of syrinx 2. primary intraspinal pathology: A. intrnDled ullary .pinal corti tumo", may sec .... le fluid , or m~)' ca .... e micrllcysts that eventually coalesce B. trlluma: 6« pa~ 351 1
NEUROSURGERY
14. Spine and spinlll cord
.
,
14.13.1.
Non-traumatic syringomyelia
E PIDEMIOLOGY'" Prevalence: 8.4 eallellllOO,OOO population.
Tabl.14-30 COI'Idlllona auoclaled wllh .~rlngomY llna Usually ~Mt8 bu ....een agel! 20-50. AuociatW cUnical syndromes are shown in Tabu 14-30. Major theorlt!ll of formation of t"" c)'llt.: hydrodynamic ("water-hammer") theory uC Gardnoer: .ysU,llic "ulBations are tran!mit. ~ with each heartbeat from the iutrtlCl"llptraonlo!ca"'01~ nial cavity to Ihe centnl ""ua t. Hils be"n eMenth!.lly di~proven ""i ni MRr""" Will ia ms' (-craniospina l diJo&ociation") theory: llIaneuver. thet rai." CSF pressure (vaJaalva, coughing... J C3W1e "hydtodislection" through !.he s pinal cord tis-
May be more common in nonC(lmmuni"llting syringomyelia Heiaa.-Oldlield theory: tcclusion a l the foramen magnum caUBe! CSF pu.lsu.unnr; during cardiac tyatole to be tnlll$mittt'd through the Vi~how·R obill S]larell which increases the extraeellulll r fluid which CGIII~ til form a BYTins'l'
BUI!.
CLINICAL
Pre.!entation, highly v"riable. UsulIHy progresaeti OVe r montha to )'ears, with a man rapid deterioration urly that fP1Idually .Iowot!" . ini tially, pain, weakness, atrophy and 10IIII of pain &. teDlpeTatureaen!lltion in the upper e xtremitiell i, oommon . A myelo pathy that p ~ slowly over yelln! .. naue,.
Characterist ic .!Iyndrome (nar:uopecifie for iDtramedllllary ! pinal cord pathology): seosory IORl (s imilar to tentrsl cord syndro"ne) wlt.h u.uapended (·eape") d i••..,ci· IIted aensory 10109 (\(1M orpain and temperatu re ""nsa lion witl1 presetved touch a nd joint position s enM! - pa inless ulce rotioM from ullperceived ir\iuriellllldior
bums) pain: Commmuy eervital and occipital, Dyaesthetic paill often occura in th , distribution of the sensory loss"'" w e.. b ...., tower motor neuron weakn""" of the hand Bnd arm palllll!6S (neurogenic) arthropath.iH (C haroot'l jo iIl Ul) esp«ially in the I h(>ulder &. nede due to lou ofpein & tempera ture &!Inla t ;Qn: seen in < 5%
E VAL.UATION
Prior to the. CT/MR I era, dlagnOBis relied (>n myelography or on autopay. MlU: defillu anatoEnY in aagittal Il!I well a8 wa] plan~ Teat of choice. Cervica l &. thoracic spine and brain MRl (without &. with contras(, to include C11Uliocervical jun.,.. tion) should be obtained.. .cI:; low a ttenulltlon a rea within cord If!"en on either plaiD CT or rnyelogromICT (with water aolubl!!contrut). Myel!!(IJ!m: rarely ",sed aJon~ (u~u!l.lly performed in conJunction with CT). When used a]one:ol"ten normal (fals~eglltive), some - complete block at level oreyrinz; iodine oont rut atudiu moy .,how ru~iform wid~ ning ofspUia l mrd, ...·herea. air contrallt stulliet may . how col]aplle of the rord·"". Dye lI1JIy slowly leach into the cyst.
MANAGEMENT SURGICAL TR&.TMEfoIT
Options include: 1. tuT1l'Dt philosophy is \(> tretlt the underlying ppthQphysiology (and to U58 . yrinx draining proeeduretll\ll 8li!cond choice) A. posterio.- decompresSion: protlliiure of choice whIm poaterior enomalie, (e.g.
'"
]4, Spioe a nd spinal cord
NEUROSURGERY
lllo1 ronu8liun ).~ p "",~nt
ChI_ri
2. . I'mne..: A. diudvant.DgeI, 1. C/,)lIIplicntlQn rtlU: I ~ 2. dinicel ~l.8biHlation fate: li4* .t 10)'111 3. may prodllDa traction on I PI,rIIJ ~ord wlth potential for rurthtr injury 4.
pront' to obit.n.o(tiQn ; SO ... lit 4 yelU1
5.
d~~ n ut oid .pact (~g. Ileyer-Schulte·Pudenl l ),5lem): rtijwrs nonna! CSF now; 1'I 1.. l>e rllCiuwid lpaoe , t huefora ~IUI.OI U~ In ....cl'.nl:liditill ~ . pt'r'CulJlneoul a.pintion oftha non or othe r materi,l
B. open ing U.e.ubal'3chnoid I lW'te'" /"tmonn, Ulferior ~iJI C . Iyringoltomy: WouaUy fall. to n;ma l" paten!, !hel'll!fl)l'e ... lnC8 nenl Dr a Ihun~
(lyrlngOlluoo.rael!no;d or .yringOptriloneal) iI recommend,,;!
OUTCOME
A&9e:11s,ng t~atment t1IIIulta i, difficult dua to ranty oltlla ""ndiuon, variahllity of nAt ural hiator:y Cwhieb Dlay 8trQt apont.an llOusiy) .• nd toll u,(I'" follow · up*
14.13.2.
Posttraumatic syringomyelia
A fOl'm of n lUI pot;t·.pinallillesl.he9ia or follow;nc m orlOCk dille hernia lioo an: not included). May rnllow' lig!li!ife;QI .pinal trauma (wilh 01" without clin ical , pina l (lOrd injury).
P ATHOPHYSIOLOGY
am""'"'.
Etiology Appell" dilJe...",t \ha n !haL ornon'poillraUlllati~ Prop """" qiJl!& _ ..... and ~..
MANAGEMENT
u,,,.r_ ..
~h
Many autbora- advocat e sarly
aurgical drainage of ey;.t a. a me ..... of re·/ hleing incrused del .. yt-d deficit" ' . Some authors feel that aside fMlII:! di~ll1rbing &enMiry sym pto,nl, th at motor 1O!!a WaS iJJfrequenl an d ther(!fore conHrvative manngelrn!nl is mdltated in most CII&es"". MEDICAL Ml'Inaged non-au rgicalJy 31%. table. 68\\0 progres.sed o~r y" \longM FlU in tatter) SURGICAL
Th" .... ill probably no hen~lit in Dp8l"11ti ng on '" patient wilh a 81!1aU ftyrilU'*. Surgic~loptionll:
As in Cl>mmu"i£nling "Y""Sc>myelin. witb the following differencu:
tord lun$ec:lion (cordectomy)"",: II" opl ion in.l:!/ltlJll!:l& il\iuri ..... onl, unlike congen.iW syrinx, plugging the obex ill fel t 00 to be indicated
O UTCOME In 9 F'I'SJC pali en l.S trl!l'lted wilh syriogosubarllchnoid shunt'*! pain reliuved in .1I 9 ( 1 onl, 81igl"llty), nwtor • __very in :;18, imprllYed tendOn renux in VI O. Some po91.j)P
com plication' in 9 ptllienta inc luded: I illCOlllple1.e IHion became com pJe\e , I Hnsonmotor deteriocll t ion. transient paln in 8, MO!Il rl'llulta are good for rad iculili aymptoms. with dubiou$ afficacy for autononlic aymptom . trr spnti clty.
14.13.3.
Syringobulbia
Central cavilAtion uf tha medulla. Ma, prelll!nt wilh (bit~lern l ) peri-ornl tingling and numbo """, due tooom pression of lhe .pinal trigeminal trac\JI I I th . fibers decUSl8 \.e .
'"
NeUROSURGERY
14.14.
Spinal epIdural hematoma
R. .... Ovu 200 caM. ofvatyioll etiollll)' have ~n report.ood .... aJth\ll.lllh One third of re8QO;Iaeed with better outrome .... High· risk pa l; enLS: for medieally high·mlt patieolll (e.&- IcUIII Mil (If\ .... UroI!g"O181.;00, .u rgical mortality Ind morbidilY iz elllremeLy high . 8l1(. thilt mu.l be COn$;dered when m.lting the deci.ion o(whethe. o:r JWIt tollJM'l'IlII. In J>l!tienLi notDperated, llnlioo81(\1Januthould be SlOpped, Rnd reverseQ if pcIiSliibl.1SEe Com!CfiOll o(CO/lJlu/oputlJiu« ffiirr$Ol o{onlkoai/ulor.t,. page 24). Ccmsidu use ofhi,h d<we methylprednisolone to minimi"" cord injury {~ M~hylpl"tC1f1ilJlJiDlW!.lW'ge 704 "nell'!' 'pinal rord inju..ry). PueU!.Ineou. needle lllpiration m.y be 11 CONJidl'1'atiao in high·rid; ptltieob.
14.15.
Spinal subdural hematoma
Ra .... M.y be poattraumllt~ (indudma illlroCen~ clluanl or m.y OOXUr .pontaN!DUIIly. Spin.l.ubdu,.l hem.tomas (ssm thatoccu. apant.aneously Or roUowing lwnbar pwldure .... u.Uy O«UT io pal!tnLl ....;th -.gulopllthiel (priDWD)" or ill.TOBenier"", Coo,ervative trell.tmenl;' po&!ib~ in nontr.um.tkSSH~ with millimtl neumlogie impAirml!nl"".
14.16.
Coccydynia
P.in .nd !eodem_ amund the 0IICe)'L II ')'1Dptom. ool. ditgnoai • • Typically. diecomfort i. up.enenced .. n .iU.in, or on ri.illl froID .itlinl , More wmmon io femllet., poll.illly due to. more pl'OO'linl'.ot"'""" Th. condition ~ .." ....... I .n.QUf!:h in n>II.~S IIuII in the .b.o!n« oftr ...."'••• lronl CIONider.t;~ Ahould be gi~ to an "nderlyinl condition.
Et iologie. For di~nll.1 dillrr-i •• _AeII"" lOUJ kd pain, pap 907. BeLt.u .~ pted ellulog;e.l.ndude"; I 1oc:.1 traum. (m,y lit _ _ .ted with fracture or dllltJeoltion): It. 25"III'J>I!UenIi give. histoo)' ..... filII
NEUROSURGERY
'"
2.
3.
4.
B. 12% had repetitive trauma (rowing ma.~hine. prolongoo bio:y~le riding.•. ) C, 1'2% st..arted will! parturitiOll D. 5% atarted followin g Dsurgical procedure (hair of which we re in the lithot· omy position) idiopDthi c: ududing traumati~ ~oes. no eUology ~an beldenl ifjEd in most Case CIIU ... ,
even
Evaluation 511ctoOOCCygeal libruo are often performed to rule- (1',0< S.B"",.,o. """'" G, "Q/~
,,,.,.10,, ba«1 IA ~ . II~·'. 19a 1 Mcdk- ""T. ~ w..ryt T. 8o"",,,,,rey F•., .H Lo,n' lou 1>Pfl~_ J\.J1t 1'7, nl-M. 1986. Joo"""" It p.Co" J 1;,1_ 1t... """ The ... """". dootork "'""""'" of l.rnboI ",,,,,.,,,,, """Ir .. pulII. A""..,..,_01",",,,,,,,,,, "'~ (cn.",)'pIIie. and _ ... ;,~ nod'.l.1n .....,.'" ,.i", ,,",,!Ojl ... lOCI h"'B"",I .S--" II. ,," ;. A . "~ .. ~i>oCI or ro""" r.. d;oe 1>do_I'" P. A,-. pori_ of ..., ....10.....,.
,_Or. _
J(J7·2 I, I'lU. S. "',"_ S. T""'00/1 M, .. I. A ....11ed ~ . I < n"_n mol _ '" .... J N.~ ... ..... tsplnn
~,. o. J I' oll·9,1911. w......... B .. w.... I~ ~. W.l"I"'~ M S, .. ..t. 1"tGt;... 'IOIt.>!U/Uol ......... ill ....:..""'" '" rho.owoid 0/1hrn" ~....u . lIodkllo&r 1:. LoflUoCM,Godem.yJ c: M"'"te'H. "","Iof tn'VIC01.pino;";un..;. _ ... ,UI a:.Iooins 'J'O"iIyl~iI. J 1'10_'1 12: llll-S. 19\10. f ....... S M ,~ ,IlC.G,.y l M, Troumatk opr..l.pdotal !o'~ 10;)0,.,., ;m'''''ln'h'' .... of
'"
""'I" pi_"
'I..-if """ ,p,"II,. Spln< I I 69)... I'IIJ t:,o .. io N E. HOIII.,." ... h M. OIoir........ rllII< ". ...1aI .... """1,,",, ••1 Ii ......... """"""'"" 1104. W,"y C c , £>w< oI~)'ltito7C_II:I_ I I no •• '",-~ '0 .. oA lilt
...,Il.i'
"",h,
.. IJopw. Aooo'I>ooIuk>D' U i 1)91 · 3 . 1m.
"0. PIara""R . II ........ C.I'o"R8 . .. III.: S _
1ot...1n"'Ii.,, ,.....1«0 pot;"'.., .Ie J Clln Prl Eloo.t ric aJ stimulation, Deep btl.in $umulation in thearen of the OPi" lind subthaJam. it n uoleu . ....n alII{) ",Iiev.. purltin. emiao symplems " without im!versibly de~tl"(lying tis. lue. LDdicatioDIii l. patlenta refmctory Ie medical therapy (inciudinglUultipl .. ngenlB). Ho_vu, IlOm" Inve.tlglllCnl feel the respolUle to pallidlltomy might btc better ildon" eatly 2. primary Indication (based on an opinion su rvey") : pati~nta .. ith levodopa-in· duced dys.kinesill8 (especially !.hose with B!aodated painful ml,lstle I pNlma). lni· tisl reswta indicate that Lh6e IlN! "ery responsive to pallidotomy 3. plitlenlB primarily with rigidity or bradykinellia (unilDr.o ral or hllar.oraJ). on.(ltf nU(tuation5 Or dyawnil. 'I'rl'mor may he preSl)nt. bllt ifit is the predolllillant ~ymptom. then uBing the ventral is intermediUII (VIM) nucleu.~orthe th8lamUR"" the llirget (for ~b18liDn (thalamoto my) or stimulat ion)" i, a better procedure Contraiodicatioo8 L. pati enta with significant dementia: further ~ognitive impairment hlUl been noted primarily in pIItienta wi th mgnitiva defidta prior to LrUlment 2. patienta ... ith risk ofintracerehraJ hemorrhnge, those with ctlBgulopathy. poorly controlled hrp!!l'tell8iOJl. th06e on ant i· pletelet drup that cannot be withheld (may oonsider atereotaetic radiosurgery lesions for th"H rare patient.sl 3. ]latients with ipsilaternl hemianopai a: due to the risk ofpost-o]l contralateral hemilUlOplia from optic tlll%, and tremor in 57%. Other are ... of improvement indude, ep""dl, gs it, pOlllu ~ . and Iilduction ofoo·ofl'ph~nomenon and rreetins . AI· tho",h symptoms may be ameliorated, ovenll fun~tionsl improvement may not be remarkahl"",, Although daaage.fl of enUparklnOKlnillO medication IU"e orten roouced. continued medical thera py is "ua1ly requi red, IlDd 00 chnnge Is Inade for 8t 1118&t 2 month s folioVI" ins pallidotomy. Indica tionill re lhat beneficial su.reical efrecl.Scan bat,. 5 yesrs. with early foilurlll poMibly due to produ~tion of too small ofa lesiOn. and late r.ilurl!l po$iIibly due to pl"OgTeIIIIion ofthe dise"le.
".
15. FW'lctlonaloeul'O$urgery
NEUROSURGERY
Ongoing studies ore investigating 10nKer tenn ,..,5ul lll, mieroelf,!Cl.rode rI!CordiJ\lf, aI. t.e.mal.e leaioning tugeta. the roleofellrty s u.rgery ... Until more informo tioo i. ovaUabie, OM cannot mak.e any ltal.emenbi about !.be optim~l target., 10000lhing method, ere. COMPLICATIONS Viaual fielrl deticit OOCUI'II in 2.!i'h due to pT 15.3.
Spasticity
Roelullll froll) lesions in u"per motor neuron pathw l Y. ca ... ing absenca ofinhibit.ory innuf!nu II.n alpha Dlotor-neUTODlI (oMN) lalpb.a ~po&tkity) as well ason gamma motor neuTOns (inLrefusal AbenI) (gamma allli~l;cily). CaulJe. uninhibited ren"" af Tabla t 5-1 Alh ... orth IC'O..I"
TREATMENT Depend. On utenl ofu~ef'ul funtUon (Of pol.en l hti fOT$amel pre!lent in arerulal and below Bp88ticity. Complete spinal cord iqjuries usually bave 1;:tll11 (unction. whl!J'ells pa· Lienl!! wilh MS m~y !lH.e s ignifkant function.
NEUROSURGERY
15, Functional neul"OlJUJ'1::'"Y
36'
MEOICAL TREATMENT 1. · prevention"; m~ll9urel too dOlCrease inciting I~imuli ( phy~ ic .1 tberapy to pre"~o\. join t damage, Suod alLin lind bla dder Ciln! , •. set:! Au/imtmt;c hy~r",(lu:w. page
2.
3.
''''
prolonged sU'et.ching (rno .... thanjuat cling.. of motion ): DOt only prevent8,ioiM and musel .. wntracwres, but modu]al.e.!l s pRstioity ol'1d medieations"(seeSurgirnl frt:amu",1 below for intr8theuil medlcationll)' few drop all! "lfe! af\erward. O. $ten:otactic thallUOOtomyotd.mllltotomy: m.y boo ,,"ful in ceTebral pel~"" Useful for unil.l.eral dyl \.(ln; .. butunnot be UIIed for bib\.tral d)'lltonia as bilat..erall""'0n6 would bereqWred wbichjeoperdi ... sPftCh. Effectiveon!y fot d.ylIwnia.d.WlLl to shouldefll or hips, "Jld should not be used ,rlhe coMi· tion l~ rupidJy progrelllrive 3. ablative proeed'..II"$, ... ith tacDfice of polClltial ror ambulat; Otb er t reatm e nts ror to rtico Ui, in clud e Bt"'reotacti~
I.
eJeclrol;oaguJaLioll of F'ore!'" H, field
TOR rICOLLIS OF 11TH NERVE ORIGIN 1. u.uallya h(}ri~()fItal type (m~ nifell l.s as hUrUonuol head mO"emen t) which 1111\1 be
exacerbated when s" pine
( un~ke
e xtrapy.-midlll torticollis)
2. CQntl'llctlon of SC M is " , uully acwmpanied by activity In contraillterill 9goni!t lIIu-.cles
ml\,Y be treated u lrgicIIUy. Procedures incl"ue
3.
A. III!Ctioninl!l llfthl! a nastcmotic. bmnchl!! ""'tween
I~.I!
11!.h ne rve a nd Lhe up_
iH"r cervieal J)O;tterior root (C, anllatolllOtic bnlncb ill $ensory ouly) B. microvascular decompr"""ion of th~ ll!.h n~J'Ye irr.osl.C8""S caueed by Vertebral artery. but PICA eompre!lsion is lIllIO de-ecribed"t Relief takes sever· al weeks J>('IIt-op
15.5.
Neurovascular compression syndromes
Thi s Mel.io n considera tbose synd romes due to comp l'u llion ofCl'anlal ne ........ at tbe root e n try:r.one (REZ) (or in the o:ase ofmotcr nO! ......... r\lOLait zona) other !.ban trigemin al ""ural ~a (see Tr!&m,i,,,JI "tUm /g;ll, psge 378 for a diK\I.'I.ion of u..tentity). The REZ (AKA Oberr.leine r·Radlich zone ) iB the poinl where celll.a l myelin (from oUgodeodroglial cella) cMnges to:. peripheral my~l;n (from Schwann mlbll. loci urles: hem ifads! spum. d i.!l abling po8 ilion8ol vertigo. '"'OlD COrmi of torticolli s of Uth nenre origin (""e TortU:.r>llis abm<e).
15.5.1.
Hemifacial spasm
Hemifacu.l l pum (HFS ) ia 8 condilioll ofillLe:rmittalll. painleu, in\'Olul:l18.1)'. SpIIII·
Qlodic contractions orm~ll!!l innervated by the lilcial nfrve in one ai da oflh'l f8 EnOLOGV HFS is UJl ulilly caused by oomp!"ell!lion gfthe facia.! Ilerveal the rootl!lli t wne( REZ) by 8 v_I. which is most on.m a ll artery (111061 rommonly A.I.Q,A" (e,ther pre· or poiltml!lltv.I"), but other vlIKular p08llibilillu include an elonga ted PICA, SeA. a tortu· ous VA. thecodl lear artery. a dolichoect" lie basilar artery. Al eA branches .. . ). a yascular malformation. Bnd rllr~ly. veins have b eaudel u pKt of the VllNlll nerve eomplex. in atyp ica l H F'S (~nning in the bueeal tnU.IICJee 8nd progreesing upws rd crvi!r tb e face) thecolllpression i8 I'08tnl or po5terior to
VII"". NEUROSURGERY
lfi. Functioolll neurosurgery
VeoJIela oonla~ting the REZ orthe veotihular n~rve may nus~ vertigo, where,.. tinD.itUi or heaon, 106& may reilUlt from cochlea r nerve REZ oompreuiol1. Infrequently. bewgn tlUll."."O. a cyst in the cerebeUopontine lingle. multiple !;Cle rGsu., adheaioOll, nr osseous skull dsform,itioll wijJ be. the caugo ofHfS. Evldenee indicates that there is not U089 lephaptlcl conduction at the compressed REZ. but that the fS(ial mou:nudeu. ill involved St!~darily 8$ a Teault of the REZ oomprf'Mion, V1.a a phenl)m~non aimiJ nr toO k.indling>". [n addition to thespBsm. a 2nd ell!ctl'Gpbysiological phllomenon all80ciaLed willi HFS i& .ynkioesia, wheM at imulation of oDe bn, hOlll"Mne.. or 0.3"" periorell>erpn in 3.,...'
15.6.
dYHph~ci.ln
14". CSP' rhinor rhe/l in
Hyperhidrosis.
Either _"tial (prial.a,.,., or idiopathicl or Ma)..ollty leli~lociN Indude: hyperthy-
f'IIidilm, di"betH mellil&is, pheoc:hrom«;rtomJI, .~mq'lIly. pe.rlUflllonilm, eNS tuum_, . )'riniOlJlyelia, hypothalamic lumors, menop"uae). 0\J,e to overacti"ity ofta:riIMlIW"'" t Iland. ifO\lPd over entire body. hi&1lest I.'(IDceal.l'lltion in jMolmi and ..,:l.. The)I produOlia hypotonic ~Iion wi"" ... Hne..,. the primaryl.'(lnltituent. an wldercon~1 ofth.y~pal.helic nerYaus ',al.o!rn, how_r, the DtW'OLr.,.witter;' par.doxically acetychalina (Le. they are ehalin@rgic, unlike mo.! aY~JIlII.h.bt ~nd 0fI_ ... hich .rll ad.eMrcic). M:lIIIlettrine ,..,ellt gland. IerY•• thennoregulatnry funClion, h _ .r, thOll. on tho! pa lmo. and ""I... re!!pOnd primarily 10 emotlantlstrMl. Ellentitl hypertudraeis ,. t ~neraliUld I'!JDdltlon that WiuaJly manifest.s mostly in lh .. pal ..... Trn. incident. ia unk"own, a ltboudo. it ..." • • , ,.in an braeli Mt\ldy (probabJy hl,h).
orr.t).
n,_ ,h,JId.
Treatmen t Mild u_ ..... t ....a~ ..-\1,..lIy ...ith: 1. topleaJ llien"': utringenl.l (pocaaaiulIl J)III'ma"ganll.te, "'link add ... ) 0' Ilmipu.pi~t.I (contact dHmatiti5 ulually Iimitll u~ ofth_ &gmLlj 2. or 'Y8temic.ally with IIntkholinergics: includingltropi"e, prob.nt:helin. bro· mide , • {.ide effects of dry mouth IUId bluf'ftd vUion Ulually IirniUl use ofthue} 3. Lap .... ler innUlphOTellia: to"y pl"Oduc:tllunltioiution of palmar ..,ith,liwlI SeYeH caIN refrllttory Ul IN!dieal therapy DUly be candida," for turpcal.ympal.h. l!ctOmy (.ff ,"Inw) .
15.7.
Tremor
ThalamoUlmy OT thalamic lIlimulation may be ulIO!ful for t .... ,...,rtll'lBtant ",-fractory to medlr,.al trulment {including parlt1ntotliao (1ft ~ 3661. ~_tiaJ. cerebellar and po!It,.traumatic)" ,
15.8.
Sympathectomy
Cardi ac s ympalh B.:tomy With tIllI advancel in plfi!Ut.al1eGUII. coron.ry af' tery t«:hnlqu~, c.rdlov ... ~laf wurs"'.,. and dro p,
cardiac .ytllPlltheclOmy ro. an';n. pecton.. hu fow:>d left. "pplicalion. How8vlr, it may ~tiJ1 be uHM in pa· lienq ... ho bllve no further l ' U llIIenl opt1olUl. 81Jat.cr. alllytllpat.hectomy ffom t he ltelJaU! ganlll10n tlvough th.1'1 HBnllli. i.I "'Iuire U PPER EX'TREMITV SYMPATliECTOMY
Vanou.
pa~boiogieB t.ha~
IIllly be wdi~lItiOD8 for upper ex~rewitr lympathecUlmy
M\! l hown in Tab!, 15,4.
ReDlOV.,) of the only se«Ind thurac:ic 1[8nl[iion is probably adequate, and IIvoid4 II. Homer'llyndrome in 1110111. Tecl\niqullII used include: an1.erior tr,ns~horllek, tboracic endoacopic"l, percutaneou lllIdio!Tequency, and lupraclavkular, All approach via 8 mid · line posterior incision wi~h 91'3 conouanl"eraedomy allows bilal.eral .C<e.... >. .., n'e ri~k of sil[flifiCll nt oomplkaticma is _ 5'" and indude plll!umothQrUJI , interoos!.&J Muralgia, 11Jina1 cord ir\iury, and Haruer's . yndrome.
U PPER TMORACIC SVMPATliECTOMY ApJll'llacheB include:
I . posl.erior paravertebral approBch 2. uiUary thoracotomy "'ilh transthoracic expollu .... of the sympa t betic chain 3. 8upracJavicular, Il!trop1eu.ral up06ure 4, pe rC'UtAneoul radiolTEquency ~bniq o eM," fi. vide(! endoocopic apprQach"
LUMB AR SVMPATliECTOMV
Primllt}' ,nd iC8tion is forCilusalgia major oftbe lower extremity. PTi!operat,ve lum_ bar sympll thetic blocks may be utilized to ovaloaw pa~ie nt for respell". Renloval ofthe L2 and 1.3 ~ympatbetic Hanglioo is usually adequaw LtI remove aympathe tic tone from the lower eJIOtremitill'8(oecasionally 1.1 and sometimea TI2 arealatlrelIloved for causalgia orthe thijlh). The CII.OIjt common ~ pproaeb Is II retroperitoneal approach tbriIllllh a nanl< incision. The patIent i. pJacedio a fateral oblique poaiUon, and t he slu n lod8i~n i8 modefmm tbe anterior , uperior iliac ~pine to the tip of the 12th rib. The peritoneum is diasected from the muscul.. wall aDd is retr~cted anterior ly. The kidney and ureter are reUllc\.ed ant&rior ly; injury w the ureter beitlg a DUijor risk of tha operation. The 8ympa thetic chain ia id(:otified on the lateral upect of the vertebrlll bodiu. The vena tav8 &< .. "b !no..,.,,1< Por\"""",·. ~ II OomcO.SIellb".. GT. JC .... uu H L." ...... "'IO! "... ...
lOne e"llOlure-lule TON that;. often poor ly responsl"e to microv8KuJar d@compression In oddition to the ~naory diVision ofthillrigeminal nerve, other pouible pain pathwnya " include the motor branch oftha 5th Derv~ (portio minor). Or the 7th or 8th nerve.
Tabla 16·2
Epidamlology'·'" age (years) W~!Iy"50 (avIIlII1B 63) 1.8:1 lemakt:maie
laterel'tr
~"
"
~. djy;sm invoI'Ied
.,.
.
'" """
""'" ."". V1 6 V2. V2& V3
." ."'" "
EVALUAnoN
MRI is often usedto evaluate theupatient.s for poI1!!ible intraoranlal tumor.. or MS plaque.c, especially in cases with atypiea,l featurell.
Differentia.! diagno.i, 1.
Mrpe~ toste r: pllin ill (:QntinuOUl (not p8TQJ:Y$rnal). CharllCtaiatic "ellides a nd ~rusting ualllllly follow pain, most often in distributio~ n(V I (isolated VI TGN i~
Tare). In unusual Caiel! without "eaiele. different.iation may bediffio:ult 2. dental disease 3. orbital diul"le •. giant cellarter\tis: tl'ndemess O"IIT STA lfi (:Qwnon 5. intracranid tUlllOr. primarily p·fo!lS8, usually cauBH sen~ory defidt ($I'f oolow)
Hi,tor:y and phyaical (in additioo to rou t ine) history
A, a.:cural. An anu""nvul8snt page 218). mil)' act synergi.ticaJly with carbamazepine find baclo ren . SUIt u",,-,,,, indudE atax.ia. &edatio" end rallh. Rx at.art with looml: po :I1D. litrslf' to S.7mglkglda.y (3600 m,tVd mox). M ISCEllANEOUS ORUGS Also polisibly elf~tlyt!: 1. capsaicin lZoatrU®): I gm applied TID IrpI!Cl.ar>ey « 5 Ynl) are ideal c"andidat.es for PTR". For "atypical faeial J}aill", del'lervatlng the palnful region ofth p, face bi'!nefitll < 20~ of patientll, and WOT$enll 20%'" . PI'R rnquirl!!la patient who i5 ~ble to eooperate. RecllrTflMeli ..... eaailyt,,,,ated by repelltprocedllre • . May be u~ed l Mic.rovascwar decomprell&ioD (MVD) ( For mOre details, He fKJ&~ 38.5 ).
~ommended for potitn'" with inadequate mediCl.I control of pain with" S }'i!are anticipated $lIrviviaJ and ablt to t.Q lerate a small eranio\Omy" (surgi(aJ morbidity in· creases witb age). Reti~ris allen Ions lived, perwvering 10 Ynl in 70'1>. lncid@n~ or facial wW!stheBia iii much IU8 thlln with PTR, and an""th""," dolorosa dOh Dot OCCUr . Mortal· ity , < 1%. Incidencs ofa""'Ptic mcningitia(AKA hcmogen icmeningiti.): 20% . 1· IO%m.l\ior neurologic morbidity . Failure ratf!; 20·26 .... 1 .2~ of patiMta with MS will hlv" a dp'lU)elin.ting plaque at th" root entry ~on"', tlU. lIi llaHydoe. not rlldpond to MVD. and one should attemp: a PI'R.
NEUROSURGERY
16. Pain
'"
Stereotactic radio8urgery (SRS) Initiall,)o,!.hi . .... ae reliel"Yed tar refractory calle!l fallowina mulLipl~ operations", noW ~mlng more "'idel)' prllttil.'OO. The leastlnvlL5ive prvceduN!. Gen.,f/,JI)' re.:ommended far patients .... i!h c:o-m01'hiditu, high-ri sk medical illne", pain refraftory to prior surgical procedures, ar Ih06e on a ntlroogulanl.l (lln ticoagulalion d OOll nat hove w be revened toha"e8RS). 1Teatmetr.t plan : 4 -$ mm isoeenter in the tnieDlioal nerve root entry zone identified Oil MRI . Us.. 70-80 G)' at th .. I:Iilll&L k eeping Ute 8091; lead"" .. CllJ"Vl> ouLsid .. ofth .. brailllltem, Resul t. : Significant pal .. reducuan after initial SRS: 80·96*""', hut far those h.viJ;Jg MVD {ol1aw. ing Pm"'\. RllpeotMVD may al.abe penarmed. with attention givt!n to pOSlible Bli ppage of tho! in.&ulatiD g 6P'W'. or the fact that the true aifeoding v_I lUy be. ' lU"Lifu:illly" mllYed a .... ay frow the Derve oerondary to the lurgielli positioning. SRS un be "'pe&ted, wing the M inI! do"", with "'JIOrted llignitieant reduction in pOlin In 89%, and oomplete nlliefin 58%".
Intradural r-etrogv.sserian trlgemioal oarve &ectiOD Mey be u.wd 1111 mfuureoflaat resorl. in patien t5 who hovOl recW'Tt'nt TeN follow. inS "ne or roore PTRe in lhe presence ofwLaI facial anesthl!8ia, a r in patients u.nde.gaingpOflteriDr-fosB8 cnllli4!Clomy in' the pu!'}>D5l! ofMVD when na impio(ing vessel ean be ideotified. In the lelteTcaat, '" partial rhi~tomy 1. pe rformed by sectianing'll3 ofne""", with rNulting perti81 anesthe3ia.lo the tale ofpalienlll ...;Ih faciel anesthesiA p",-op. ronBiderat;on .houJd begiveo to lecuoning the motor divioion (portin minor) as an elte .... nDte pain pothway".
PER CUTANEOUS TAIGEMINAL RAOIOFREQUfNCY RHIZOTOMY ( RFR)
Due to coace-rna about helDOlThage. th~k roaguJetion profile (PTIPTT. conaider bleedlng time), and di8conl.inue ASA and NSAlDI, pnlferahi.l' 10dll)'ll pr&-op . Proe«Iul"ll may be petfarmed io OR wit,'" nuarD, or io allgiOj(rephy luiU! in x·rllY depllrlmen~.
PRE·OP ORDERS (RFR) 1. Nl>Qafter MNexeepl.mtils 2. conl.iOlJil '['~lI"'tlwl®" pther med~ PO with 8ipi or water 3. AM of procedure: rv NS Ii KYO in 8m> con~ralateral to newalgia 4. atropine 04 mg 1M PRN (MfIIInt.railldio::etiQIIS incl udl! rapid , ·fib) 5. 6.
methahel'itol®) 500 mg to acoompnay pal.ient 1.0 O.R. (wrile "do nat ad· m;rusU!r") non-disposable LP 1:11), to accompany petient
gIll> IU,y ~ .... b"~a1i" ... lb' kirh ' ''''"'''' "Ie•• 0 the oral mu foremen I pjno.!i\lJn (middle meniogea] artery). Ifnece;ssary to Yieualize (e.g. wben there ie di ffi cult)! Mtering), tbeCarameo ayale iI optimally seen on e l ubmental sfay by hyperextending.neck 20' and rotating head 16·20" away from side. ofpain", Impedan ce D1 e.asununenta: fmm tm. tip oft.heelectrod. when aYllilable may help indicatt! locet ion orneedle lip.irupedam:e: CSF (onlllY flu;d ) low (~ 4.0-120 0); connective ti5$ue, D1U1itlt, or nerve i~ usually 200-.300 0 (may be up to 4.00 01; if,. 400 0 thit Ukely indicatas e.lectrnde is contacting penosteUJll or bone. After slartirog tbe.lesion, impedallco! often goea down by SO (I tranaienUy, and then Ill! the ]...nonilli continues it gradually ..... tumll to baseline Or ~ 20 Q aboye it. If char dO!Yelops on th., electrode tip, the impedence wi.11 Telld higher tba n .. here it ~tllrted.
StilDulatiOIl and repositioning Once th., f01"lllll1!ll Oyale ia enterlment (s£e lJt./ow). The goal ls an' al~iII (but Dot Uleathuia) in tbe-aress of tie pain and bypalp~ili in ar .,.u of trigger pointa. An average of three leaiool are ne or _ .. td ! . ...'.."..iM'06. compli..Li ... ""~
..,
NEUROSURGeRY
RESIA.TS (PTR) Relo.J til of Vii rio"" Yl'R t&:h _ NqU" compam to rniuovncuu. r
d8COalpru.lion (MVD) are , h_n In Table 16..'1, Rl'Curre~ cll nte.l, ru,her in ~till\~ w~1.h rnultlplll ,derw;' {60'110 at3yrt roun prol"' .
Table 1&·3 Comp.ri.o,.. 01 o",tcome. of 1'I8O",.lechnlque.'O MVD
~ul ..
MICROVASCULAR D£OQMPR£S. SlON ( MVO) FOR 'mIGE MlNAI.. NEURALGIA
Indicationa:
1. pII l lIell" unable.., .d\ieve . deqllate rneditalcootroll)/' trigu'lin.1 nl uralliP. with ." ~ yft .ntio:i~WI.urviv.
--
-.wo_", RFR .'_ ~'-cwI_~
micro-
a I, without l.iplilKlllIt mad· ital Or .urp:.l ,.;U tnIs a..1/Iof _ _ ~ 11IJuo.. 10 PTR tfI 2.
been report.eQna) rate: IN C. ....inS KapIRll·Mel~r eu .... e. elCpecI 70'Jl, to be either ptr.ic freeo. have minor r~UlTenee th~
by 8.3 yo." (or - 80\11> It5 )lei."')
risk for a the ri. k for •
mW recu rrence lifter MVD i.3.5'i1b u
nually
.mi.nlII.I"1l(:UrrM~ al\f!rv. (Uuw.I& b... ofl.ongl.le rnoalcomlPOnl), involved. radial&! toeu(otaleia). Ota.IIionall), to neck). occ:a.sionall)' .... ith ... livallOlIIltld co ... ghing. Rarely, hypoleoaion-, I)'llcoJll!"'. ca rdiac .moal and convul:liolll mI.)' aceompa· n),. Mlly be triggered by .wal:o.... ing, talk.:ing. W,=""inll. Trigger _ , .ra r .... TREATMENT Pain mlly be reduced byc:ocainillilion o(to .... l1IBr pUlatll and (0""... U.... U" lhe per~ li'len 16.3 . H erp ....
Postherpetic neuralgia
_I.e.
(H Z) (Greek:
aolter· girdleJ(sbiDgles in laytenBII): pa mful vesio;ullit o;:ul$.neous U\l plions c\lvsed by tbs herpes "arleell. to NEUROSURGERY
Table 16-4 Medical 'r"tmen~ lor PHN'
" mocIitIoKI WIth permMion Irom Allbin M. Aeller!of posIllerp.llic """'. . tleu, oIOI;IY
alen. 1 ; :13· ••
,."
_.....mons: o.".:oid derived from hot P"Ppen , 8vaiiable WIthOUt pl"f!scnplion rOt Vlp.ita! treatmeot orllle pain ofherpea zoster and diabetic neuropathy. Benp.fidal in IIOme patieut.l with either ohhellecondltions (reaponu rate at 8we.e.ka waa ~ for PHN , 71 % fot diabetic neuropathy. Yil. 50%with pla~bo in ejthergroup). although the high pl8ccbo response .... to. i. diBturt.-iog snd ",auy authoriti"" ....... ~l<epticsl". Erpen,iYIt. am~ 1>I"Tf;Ct"9, include bW'TIing and erythema at the applicalion l ite (usually subside! by 2-4 wee.1t.!l). Rx Manufacturer recommends m81iSliging the mi!dication into the affected area of the UJn TID·QID (apply 11 very thin coat). Some authorities recoll11m'nd q 2. hr applica. tion. AYoid ront.acl.wtth eyesordsmaged l kin . Supp lied &8 Zostrix® (O.:l5% capallu;in l or ZyBtrlx·HP® (0.75,/&).
,-I lidocaine patch 5% tLidodenn®) \ 1_
IlRIJOINFO
\
,
Often bette r Vller.ted by elderly pstiont.l thon 1'CA.$ (due \.0 pre-ex.isting ~itiva impoi rments, cardiac disease. or l Y"temic iJ1neu). ~ Apply up \.0 3 patches of 5* lidocaine(to coyer a maximulll of 420 cm1lJ Vl intact l kin q 12 h'" to cover 88 much of the a .... s of greatest pain ..... pot!~ible"".
Intratb ecalat.eroid a Over 90% of pati~lI ta r1!fW'e 10 Ii.
16.4.1.
Cordotomy
tOm
lntarruption Gfth~ latera lllplTU)th Hhun;e tmeL fibers in the Bpinal Cordotlomy i. the procedun!. ofchoice for IIDilQ\.e[11 jlIIin below the Cli derroatloma1level (_ nipple)", in" terminally ill pa tienL Better ror achin,. PhIDe] in the CS F to _ 1200-15000 as the spinal cord [s penetrated. Stimulat icm at 100 Hz. $I'lould produce contralateral tingling at" thresl101d of 0; I
rew
volt. No motor response.should be el!clted with 100 HI- in the.pinol/"lllUnic (tad, and If muscle tetaoy~urs, 1~9ioning must III!I,be perfDTToed. Utingling is in theanu, leRionillg will uJu aUy render from the ami and beJowMalgesic. /fUnll'iing is ill tholowerenrem ity it will render only thM limb an~lge!lk . Stimulation at 2 Hz. Ihould produee lpailllt.eral twitchi"g of U,e ann or neck M _ 1-3 yolu. RadiofTequency lesioning is perl"ormtod ror 30 !leoonda while the pntlent sust.i\illll C:O"~ tract ion of t he Ulsil(ljeral harvJ a"d the voltage is grad ually inctened from zero. ~ twitching of the hand ia indication to back .mwn on the vultaga. A wcood l"";on is performed in the same region and ill us ually less pamful. The oppropriate bodYI.I'f!8 i~ then cl\ec~ for a nlllgeaia 1n additional nan:otlo:s IIbould be withhoeld until the effect ortbe spinal non:otics hu been detemiinedl 2. 2 a mpuJ u (O.~ mg each) o(na]cJrolle (Narcan®) .. nd .yringe taped to palient.8 bed (for the first 24 hI"! after e lingle injection ; I~ all limn with continuous ,nfuaion ) 3 . h!'ad of bed elevated " 10" for 24 hn ~ . re-corn respiratory rate ql h.r for 2" hn; ifa.l~p and respi .... tory rate < 10 breath~min, .. "'aken patieDt. lrunahle to awaken, adctiniBter naloxone 0.4 mg rv and DOufy plly.icilllt. Re~at naloxone Q.4 109 IV q 2 min PRN optional: pulse oximeu.r for 24 hn 5. diphenhydramine (Benadryl®) 25 mG TV q 1 hr PRN it.ch;,ng 6. droperidol (InapBlne®) 0.625 mi: ( .... Wch i$ 0.25 ml of the 2.5 mglml itandarn ctln· ce ntrlltton lIyaiJ~b]e) IV q 30-fiO min . PRN nausea
OR B. k'ltnrolat tromelhamine (Toradol®) 15 mg IV Or 1M or30 mg 1M q Ii br1l(use lowo;rdosa for WlIigbt < 60 kg, age" 66 yr8, or redu~ Tenal fundion )
IMPLANTABLE DRIX1 DELIVERY PUMPS Although satillfactory pl.io motrol can b.. achieved with elthu epiduntl or intrnth. eeal narcotica (morphine dilfuMa eaally through the dure to the CSF where it pins ac· ce&I! I.e pain reoopl.etll), epidural (at hetel'S mmmonly develop problems with lICarring and may become leu effective lIOIJoer thin intrathecal etl1.bMtel'll. Pump.'! shoul d WIly be> im· planted if patients have sutce.ssful plin control with (wt injection oflpi nal epidurol (Sto mg)or inll1lthecal (0.5·2rng ) morphine. A lifee!CPI'C\. Following elecU'Clde pl.cemeM, a lrial with an exwrnal Benerlw (IIIer_ra l d ay. tletllrmines !fSCS I. efl"ectiu. The ell!CtnIdes a re I"I!~ unl~.. ~le,r Inlpnr._t 00Curti, III which calle .1I 1mplantabJ~ put .... ~..... e... to r Ja placld ! ubcutllrteQu.ly.
Co mplif: stioos With plllt1! el«trClciu, there ia iii 3.:;" in cidarn:e of wrer:ti~ which '"POlIti to 11_ lTode ~U\O'Ial alltl IV antibiotiC&. I.e.. COmlDDn OIImplicationa . 1et.trCld, mitrat!on I.,.u· ,lIy_o with lim few weeki), lead brealuge (leM eommon .... ith prMent rym ml). CSf' leak, radicular PloW, lnl.&mit~lIl iate. PtOglI06ticaton of. poor teapOlIM to SCS indl.lde: p.in i>!Nl tinc from "Pinal cord inJury, from luiona prwoiJn.1 to the pDglioo (e.g. I'OIIt avut.Mm), failed bac.k ayodroUWI ",ith back pain:> LE plUo ....d mwtiple ptft'Iiou.Iope..tio ..... pIllCho~J fKto ...uch aa liti,.. t.ion. wOr ken ODWpellS3Uon, lilIniliallmarital diacord OrdTUglleeking beh ariOT*',
16.4.6.
Deep brain stimulation
ladicatloDB ConLro" Symptom. Pain: affecting a limb, u5ually burning, and prominent in !he han d Ilrfoot. Onl!el in t he m'llority i. within 24 hTS ofit\.iury l unJess iJ1jury eauaes an ... thetia, tllen.llrs or days may intervene); however, CRPS msy take day~ to weeks to develop. Med ian, ".lnar and aciati( nel""1\8 "'" the-most commonly (ited iovolved nel""e • . However, it il not .Iway~ pl.>lI4ible 10 identify a ~pedfie nerve that bu been it\.iured. Alm:lSi any III':Psory .timulu~ worsens thl! pain (aUodytlla ill pain in(iuced by a nonnoxious atimulu1).
Sips ThO! ph)'llicaJ ftlIaID is oftan diffiCUlt due to pIIin. Vasc"lar ellangBl ! eithe r vlII",di13tor (wann and pink ) or '"II.!IOCOnstrictoTlcold. mm·
tled blue). Trophic changes (may '"' partly or wholly due to im."I"JObil ityl: d ry/&caly skin . u ifl'jllinti. tapering rmge .... ridged uncut nails, either loogicounle hair or loss of bair. sweating a ltenltion. rvari"" fro m anhidro1lja to lIyperhidrOljis). DIAGNOSTIC AIDS
In theabseoce of an agreed upon e tiology or pathophysiology, there can be no basl$ for fpecilk te~r... and the "'ck of . "gold·atandard" diagnOlltic mteris makes it im~ibl e to verify the autllenticity of any dia~'tie ma rker. Numerous tuts have been presented "" aid a to thl! diagn!ll!;a ofCRPS, and ~>l8eutially all ha"", eventually been refuted . Can· didale1l have incl uded : I. tllermogTaphy: dUcredited 11\ cliniad prllctice 2. three·plu"!" bo!l!i 1ICl1n! typieal eRPS (lIangl!S auo OC.ation: non~pe n. u.
chorQid plel<Us A. - (;horoid plexus papilloma (puge 479) B. - choroid plexus earcinoma (page 479)
6.
neuroelJltheJia! tumo,... of uncertaln origin A.. Il!ItrobJastolllB. B. polar spongioblMtoma
7.
De\lTOfLS
C. gliomatosis ~rebri (and mixed n(ouronal'glial tUIDOI'8) A. gangiiocytQma 6 . dysplastic gangliocytolllB. of cerebell um (LhenniHe-Ducloe)
C.
deamopLa~tic
infantile gangUoglioma (D IG)
D. dysembryoplastic neuroepithelial tumors (ONT) (Pl18e 409) E. gIIn,lio,l ioIDas (pase 466) induding anaplutic (malignant) gWlgliogl iomas P. central ne\ll'Oq'tQma: s« poge 425 G. paraganglioma rial and in fratenwriullutnorll . (68% ),
SUPRATENTORIAL TUMOR S'
Sign.. and symptom. indude: 1. thOle due 1(> inc,.."ased lCP (_lnfro~Morioll~mor~ belowJ: A. fT(>m. maa.. e ffect of tumor audlor edemll B. from hlodwge.ofCSF drainage (hydrocephs lus): leu common in itlpratentonal tUDlOT"li Unay occur e.g. with IlDlloid ryGt, entrapped lateralvent.ricle) 2 . progressive foesl d~ciu: includel wealtn88s, dysphuia (w hich oeo:un in 37-58% of patlenlli will, l"n....iiled braiu tumOl"¥' ): -'U bdow A. due to desttuetioo of brain pan!llfhyma hy tumor inVAsion B. dlle tocomp1"f!1l4ion orhrsin p!trenchyma by maS!! andlor peritumoralOOeTna andlor helllorrbace C. due to Q)ml'l1!S8ioo o(cranial nervels) 3. headacbe: XI bc/oo.l 4. ....ilUreS: UOt infrequently the fu·n Iymptom of. brain tumor. Twuor should be aggres!Uvely sought in an idiopathic fiT&t tim~ 6eUure in B patient"" 20 years (if n"gative, the patieot3hould be followed with ""peat stlldillll al laterdate.). Ran! with posterior fossa tumors or pItuitary tumon 5. m~ntal ,tatUI cl>",nges: depreuion, 1~lharl:Y , apathy, c:onfugion 6. syrcptoma suggesli"", ofo TlA (dubbed "tumor TrA"1 or atrok~ may be due to; A. occlusioo of a vess"l hy tumor ~ella B. hemorrhage Into the tumor: any IIltIlOr may hemorrhage. see HemfKrhojJ;c
bmi" IUmOt1, page 8M C. foall.eizunl 7. in th" apecial case of pituitai)' tumors (see Pitulu.ry A. aym ptoma due to endocrinediaturban(eS B. pituitary apoplexy: Uf})Ol:~ 438 C. CSFluk
ad.!/10100. ,
page 438):
INFRATENTORIAL TUMORS
Seizur" ara raTe (unlike sup ratentorial tumon, (scizW"H""'&& from irrita~ion of cerebral cortex). 1. most ]>Osterior f01li1i turoors present with ail"S and symptom; of increased intl"aeraw1I1 pre8lure (l Cp·, due to hyd rocephilluf {HCP ) ..Thu~ il:>Clude: A. headache: (ue bot/ow) B. nausealvo,niting: due either to inere.Bed ICP from HCP, or from direct. prouura on the \ragal nueleUli or the area po!Itrema (..... ~miting ~nter'") c . paplUeden'a: mon when the tllmor impai:!;: circulatiou) D. gait di 9tllrhllnwataxia E. veNgo F. diplopia! may be.dlle to VI nerve (abdllcena ) palsy which may occur with in. cr.!BlII!d ICP in the absenceordil1!Ct comp""",ion "rthe nerve (5H pe.g~ 5861 2. SIS indicative of map effect in variou.lotationll within the p-f__ a
csr
J7. Tumor
NEUROSURGERY
A. luiona in cerebellar bemilipheA may cause: at.uia or the ex~remities. dy,·
m~ tria. intention tremor 8 . luiona of cerebe llar ncniI may tlllliIft: broad balled gei!. lnU''':el at&.tcia. titubi t ion C. brain.tam involvement u~ually relu lt.s in multjpl~ craniel nBrve and long t ract abaormllllllel. end a hould be. 5UBpec:~ when nynegmua;1 present (upecially rotatory or vertical)
In addition to nonfooal ~igns Ind sympUlIlll (e.g. 8eizure$. intnased ICP ..• ). e. with eny delltnlttive bn1n ~lk;n tutMnmay produce progT1lSl!livedefldta related to the fun(>tioll of the involved brein . SomB ebllfllcteristic "ayndro.mea": !. front.&llobe : abulill. demen tia. pIll"llonal ity thanjl:"". Onen nonlate .... lizing. but II prllx.ie . hemipllrellia or dysphasia (witb dOlnina.nthCUr {lief! Clinkci 1/YrW.romes u{parit:tolloM diuau. pail' 87) ~ . oeI!IaIrtatic tumor(a ~ of pat len""). CIBNicaUy desaihed u being W NEUROSURGERY
p~ge
923 for differential diognQ.i, {intiudf:!! nOO ·neo-
17. Tumor
."
plfUltic lesions a. well), EVALUATION In pediatric pati~nt.s with a po8c.eno~ fOlsa tWllO~. an MRI orthe lumbu spine should be done pre-(lp to I"Ule ... ut drop met.li (.po6t.qp there llIay be artifacL from blood). In adulUl, most intraparenchymaJ P·(O...II tumor s will be meta.tatic, and wor kup for a prima", loould be undertaken (su pt trealJlMnt (ltRT. cho:mot~f1Ip)' ..• )" no benent. Mil.Ollllll or e ndotl'ltlial prolifef1ltilln, .eeo 00 O''';:''lOlI, do not affoct OUtl;OO\t.
or
Malignan t IrlllulQrmBt;On i .....'1 .......
PLEOMORPHIC J(ANfI(QAS TROCYTOMA (PXA) L:r. ge nenl, a compact, s uperficial tWDOr WIth IIIlrked Cll!llula r pieomIIrptU.m. abun· dant reticulin and frequ .. n~ perivQculu chronic infbllumal.(lry 0111'-. Variabl, lipidQ,a· (;eln . Abtenl>!! mn"""lar proHferatiOG and neo;roa;.-, tnCHIl but nClt aU lack mitol.it
figur ... . Some f'XAoIlUlde.ro .lllIplllltK chBne~ ~ o;yltk with tnhaoci n, mU,n l nodule, U.ually 0C'CUJ3 in )'tIune adu/I.S. Inclmlpltte ruectionllho..ld be foU_ed ~ th l'll" t UIIIOl"lmlY ifOW "ery .10,,'1), ow-r mao.)'~.... bef(lre trealJlMnt b I1«NIII')', and repelt "",ci~ioo . hou/ d be colllidered.
17.2.2.
Astrocytoma
'('he iDOI:It wmmon primary in tr .....I.1brain tumor, ~ 12.000 new l hu Unil.ed SUI,".
t._
per)'ill ' in
CLASSIFICAnON BY CEU TYPE 1'hfl domin~nt n il lYIl8 O(lItrocytom•• Illow. the ir cJassifiClotion intD OIIt of t he l ulx h vllions . hown in TObIt /7.[, . 1'bt rationale (0/' HpalBtlni"ordinBry" rrom "lpe'cial"
NEUROSURGERY
17. Tumor
Ntrocy1.Oma. ill baaed on a much d iffe~nt and 1II0~ ravOr1lb!~ beh.avior of the latter If'OUp ...hid! oo...111!1 d"pend "n pade within lhM ITQUP ~these alliO tend to oc 2(Wo of t\l1QOt cell' fot an utro- a troeytolllil tylOmli to be tonlid",.IKI, gemialOcytic n trO\:ylOmal. GelOistoc:ytit ..lrotyt.Omu Ilfl! tompri&ed pnm,rily of th eM ~U •. but ra rely O«lI t in pure form. Often _ t gradlU] (mal ignant "tl'OO;y\.omll.) muoria.
17.2.2.1.
' _ ...... ltrl:i1ry
~ilocytk:
I'lQoeystic oe<ebeDlr
" Ordinary" (fibrillary) astrocytomas
GRAOING ANO NeUROPATMOLOGV
"'f.ldlna of "ordinary" IItrucytOnlu hlf been hilwriCIIIl)' frllChL wilh diallogre..· ment, an "nunlber ofgradinl ayltem~ hive been propaoled oyer t\le)'tlr1- 11Ie littt Ira' tem ofBailty and Cushin( WlIIl 3·titred Iy.tern , the Kel'1lohlJl lyatcm Wif 4·tiue Low·grade 88trocytoma (WHO 11) '1'he.e tUWOIli teQ d to OCClU" in ~hiJdNn and young adlilbl. Mooot present with tehure8. There. ia. predilection for ternporal. p06t.rriOT
.....
Grd
,, 3
No. 01 tIileril
o 2
• 3.'
frontal aod anterior parietallobea"'. They demonltrllte low degree.!! oftel1ularity IlIId preservation ofnof"ll]aj brnio element.. within the tUWOT. CalcificaLion. liTd COIlscioll&n"" • • p"""",a)ity change. ai.gnifieant neW"O· NEUROSURGERY
17, 1'u.n:>or
'"
logicdeficiq-,ahort dilu tion oflymptoml before diag,,,,,i., and enhan~ment on imag. wSltudiea. Also"" pap 4OB. Dediffe .... nti . tiun ' The mlljor ClOuse of morbidity with low·,nde T.ble17-11 Dedifl'rendetlon rate utrocytomll i. dedifferentiation lor low grade . .trocytom.. to I mou malignant grade. Low P.UtntI dlagnoNd Pl tlen" dl"llnoMd grade fibrillary u t rocytomal tend • age ,,45 yrs .... Clioblastoma multifonne (WHO IV) Tha II"IOIIt con:a1YlOD prima" brain t ... mor, it i. allO the most mali~'M astrocytom •. Hiatologicalfindinp aeaoci.t«! with GBM (notall mly be present, and this list doe. not follow an, of the nandan! padina: .,.tema .bove): Simiat.ocytie ..t«-. MISCELlANEOUS PATHOlDGl'"'.AL FEATURES
Glial fibrilla ry . cidic prote1a (GPAP) , Suinm, for GFAP i, positiv~ with II"IOIIt aa· trocytomu !however, may not stain pnoitive in ... me poorl, differentiated ,Iiomll, and in gemiatocytie IStrocytOmas .ir>C4! fibrillary ast.roeytu af" required to be positive). Cyu,,: Gliomas may have C)'IItic C .Cl: /lCaD & MRl grading impree"e". bllt m.), be und II I preliminary.uealm"nl(1ft Tobie a·ln Of.dins gliom .. b, i, CTIIIIlapplica· or MRI i. NeuroradiolOfic p.,!inS bl~ to pediatric patienta. MOlllow-gr.d" ,liom .. eo not en· h.nceon CTor MRI (althoush up to4~ do". and rna)' have a poorer prognosis). Th ey are u . ....U)' hypod~nae on CT. Most ar~ hypointaDM on T t WI MRI.and 17. Tumor
f~'~"!~"~·f'~'~~~~~~~~~~~.~.~' NEUROSURGERY
tbat txUOnd Myoo.d the t umor vol 11m... 80m\!. maJignan l glinmat dn nnt.nhan"e...... Ana· pt.Atil:: Nt,.".,ytomu m~ (lr mll,y nCII. ~nhanoe:lJa a110 ul.eDd ill far N I~ mm beyollli the ring'"' . P o ai t r o l) emi &8lo D tomo gra pby (PET) RcaD Low fI.ade fib.ilIllQ Ritrocytomas appel. 19 hypnll)etllbolie-cold' lpobl with OllOm· deol ...
17. 'I'ulllOr
NEUROSURGERY
Recommendation . for : .. cllnnot be cured with !u'1!ery, and so the gorot should be to proloTl8 .Q.l.!lIl.iu a uTVival; this can u&ua.J1y be accompLiuhedwith t\lmor exo;.iaion ror lRha.rgllomll5 in patients in good neurolQgi~ condition. In ~ pIItieots (> 65 yrs age), the benelit eonferred by 8U'1!en' i& modest (""edian l ur· vival of 17 weeu atle. biopo;y '" XRT. Vru"lUS 30 weeks for surgery .. XRTJ'M Partilll resedioo of a GBM carries aignilic.mt risk of pSt..-operative hemorrhage andlor ed ema with risk. of herniation. Fwthermon!, the benelitof '\lbIotal me ,-I
teIllilzolomide (Temodar®)
\
/
IlftlGIHFO
\,
All O1"a l al k,yla ting l&enl Ulat it FDA app"",ed for un in 1Id"lts for the initial ra· la ~e of Alli pl u to.: a.'IlfOO:ytolllll. and ~jon oJ di.liellH ..,hUe on I regimen containlrq; • nitTQIIoureli (lee TIl~ n·4, page 4071and pror.arbaUne. l1 hllll a\$o ~n used(olf lal:lel) for nl!WI, dillgno&ed GaM and AAt' and for patient.. with miniollll poIIt ..urger)' tnlt· rn~ l III ..... eU ILl fOr ~I"~ low Il"ade ulTocytOmu'1 . Co.l; $1.300-1,500 per cyde. IU 150 lIIafm1ld PO q d 11 I') d. Ilr<wie for . ub5equeot I> dayqcles nvy 28 da,YII i. ad. juatalaceordin, to nadir neutn>phil and platelet allmta (whieh OCCW1l at doIy 21) during the prev.oue c:ycle and th e sta rt of the nNtcyd.e (thtJ'tfore ened.. cae on day. 21 " 29). iteoptl l"BtiOD f or reC\UTeDCe Leu tban 1() with KPS;t. 70 pre--op, VII. 3 .2';"0 for KPS < 700
With AA, smaller ~i u: and frontal location InJll.lence 51.U"V ,vai favorably. Su ..... ivlll dilfarPoeell between AA and OBM: Two large studies treated mnlignllnt .l:lioma~ by surgieal ~n.io'" 60 Gy Table 17-14 Malignant glioma lite table whole brain irradilltfoo, lind then vllriOllll $urvlva I SIaU, Uc$" cltemotheMlPY regimens (BCNU, ptOl:ar. buine, methJIl~niilolone ... ) I'flul~ in the . urvivalslatistics s hown in Table 17- 14 . STEREOTACTIC SlOPSI' SW"rfOlactiC biopsy lIlay Wlden!stlm8u, the IX'tU.Tunce orOI3M by n much 118 25%.
Indications for J.ttr:Wklctic biqpllY io IUIlpe ,
''
...
" I
17.2.2.2,
" "
xen
."
",.,
'"'"
Pllocytic astrocytomas
T Key (eatures m~ftn age Ij(oceu~nee it lower than rQr Iypi""l BatrocylOmaf " better p1'll os;s thaD inliltra.ting fibrillary or diITlUie IISI.N noatically favorable group described as "dorsally exophytic'" (su page 420) 4. cerebellum: fonnerly referTed to al cystic cerebeUar as trocytoma (se t below) 5, ~p illal oord : PCru. O"I8y also occur here, but little inform~tion i$ available on theM!. Again, patients tend to be younger than with spinal cord fibrillary astrocy"'~. PATHOLOGY
PCAs are compoeed on""""ly knit til.'lue romprilllld of stellate astrocyte! in micrQcystic regions containing eosinophilic granular bodiea intermixed with regions of compaot ti98ue consisting of elongated snd fibrillated cells often as&Ociated with R.:mentbal fiber" formation". These latter two distinctive features facilitate the diagnOllill . Another cluuacteristic finding ill that the tumOl"8easily break through the pia to fill tbeoverlying subarachnoid apace. PCAs ",ay also infiltrate into the perivascular apacea. VlIIICular pro. Iifel1ltion i. ronu:non. Multinucleated giant cells with peripherally located nudei are com · man. especially in PCAs of the cerebellum or cerebrum , Mitotic figure8 may be seen. but are not a~ omin()ll s as with fibril1lll)' Wiltro 8! troeytomll$-. P RESENTATION
Signs and symPtIK"~ ofpilocytie IlJItrocywma !peA) "'the cere bellum are U8u811y those ofjlny p-fClllli8 mass. Le. O,ose ofhydtocephalua orcerebellnr dysfundion (see POI"'ru.~ r~fIa (infro"'''UJrial) tUmoN!, page 405), UUllllly p~ntl during ~nd decade of life (agas 10·20).
PATHOLOGY The clasllC "juvenile pilocytic 1lJ;troqtoma~ of the l>ert'b(lCi.e. reaperation i, pre ferred i(pos~iblelor rOT recurrence with malign ant histology. CbelElo\.hera py i" prefefllhle to)CRT in younger patienl506 • Al$O. _ P~krior (os.a (infra/entorial) lumon. P/:Ige 405 for guidelines regArding hydrcephalu$, etc.
•
Y""".
PROGNOSIS
Chi ldren with Wi!uton TypeA cerebellar PCAs had 940k IO·yr survival , whereBfl Iho.o .. with Type B bad only 29% IO·yr survival. Tumor I'I!Clln"eDCII i.a relaUvely common, and al t hougb it haa Deo>Jl5llid that tD.!y geaerally OCI:Uf within ~ 3 ynr afsurgery 'OO, !.hi! iAc:onlrovenial Nnd vel')' lale r!!Cunence. (v;olating Co llin ,' Ie .., which 8ays that a tumo r ""By be c:on, idered curod if it doe. not recur with In I time period equal to the patient's age at diagnOllis + 9 mon ths) an well known". Also. BOme tumor8l!l1'ci5lld partially fnllta ahow further growth , .."present;n!: u
NF.UROSURGERY
17. Tumor
'"
forn'or""l'e. About 20% of Cli.Sl!S develop hydroc~phlll u8 requiring treatment follow;ng au '1t~'1'IO'. So,clIlled "drop metsatasu· atl! MlI'e with fICAs.
OPTIC GLIOMA Ac I '"
1.1. Tumor
NEUROSURGERY
I.
u~ul!.Jly
present8 wil.h multiple cranial nerveJ'"18i~8 a.r.d long t.rnct lindinp most "..., malignant. hav" pOOr proglU>l'U!, an are not . urgiCIII candidlltell Braina~m g liomo, (SSG) tend eo octurdu ring childhood and lIdoluceme l77% lite '" 20 yn old, they COmprise 1% of adll.lt tumors'''). They lire one ofl.he::l most I.'OmmOIl brain luman in pedatriu (1M PtdiolrlC brain '"""".... plIge 480), comprising . lO·2()dings. Due t.o their invasive. nuLW"e. li,lUI and ~ympto"'8 usu..uy do nOt OC (iv<e-yttar 8urviV81. Dorlany exophyli~ tumon comprised of pilocytie uL""'ytomao mayha ye a better pf'OgTlOllis. TECTAL GLI OMAS
A topically deflfl6d diagnosis generally consis ting of tow-vade 18trOCytomM. Cooi lde ttd a benign . ubgroup of brllin8tem glioma. 6eca""e ortocnion, !enda to pre9(lnl with hydrocepha!o.lI. Foeal n PrognO!li9 'rumor
progr~ion:
deacribed in l5·2:;'fo .
Follo,," up: no aa:epted guldeilnes. Serial neurolosi~ exam. and MRla every 6-12 months hu been s uggested" '.
17.2.3.
Oligodendroglioma
1 IV.>y f"" turea •
fl' ofcerebral glioma.''': bul recent evidence indicates these tumor. have beto OJlderdlagl106ed (CUlny lire mialnterpreted as fibrillory Ill!lrocytomae, eapeo:wUy lb. jofjtt'-'''ivepo,tiooof these t umOr1l) and ODGa may "'presen t up to25-33'11o or,U..! tumo"' '''' ,... Ratio of rnal8; female _ 3:2. Primarily . tumor of adults: avenge age~ "O yean (peak ~ t wet!o 28·46 yeai'll ), but with a smaller earlier peak in dlildhood btuw8i!n 6· 12 yean''' . CSF metastases reportedly occu r io up to 1O'7c, but 1% mlly 00, mo re real is tic .... ti m.te' ... Spinal 000. compriBe onty ~ 2.6'110 orinlrameduttory tumo ... orth8cord and filum.
CUNICAL CiUllic presentation
oroOG: e putieu l witl, Mizure~ for en.ny yea ... prior to th .. d.i. 'gnoI!ia being Olade when they would present with an apopleetic eventdu" til peri·tumoral inlr~erebral h.emor.rhog\l. Thill acenarlo is less ! inl;1.e~d afgllal fila· mel.ltll>'''' although ... me do'''', Also. in mixed gliomaR, the astrocytic componeot may atain forCFAP. GRADING
A work·in·pTllIfTe&8. Historieally" number ofatlempts atgmding OiXltJ haye been propo&ed and then obandooed because of lack ofprogno.lic .ignillcaru:e{(or a review, see N!fereoce'''). Far eu mple,!.h e '.fllem of Sm ith e t al,'l1 "'18 b!l8l!d 01\.:; histopat.hiJlogic feature. which lIeye beeo not to be independent detennlnant.& of tumor pTOgrell ' aiM (l'In1y pleomorphi~1D has bo!en IIhown to bil Btati$ti cally correlated "'1th $urviya]'''), NI!C1'OIIi ~ does not appear to ~Hahly predict a poor prognOllI~''', For prognOltiC pul'pO/!ea, it i. sUlllle.1ed thllt DOe:. be 6lralifiw into two group.s: low grad" and ltigh grade'LL,". Although there isnot urn· fOml agreement On the mean. for dif· ferentigting the tw, the ract£;rs ahown in Tobit 17·19shoWd be !.Iken into account ... they hay~ been demo onstrated to have pro(I1 oatic aignifi· cance. Ullillg the "patial grading 'Yll tem ror loW gradeghGmu (I« ~ 408), no ODCs areofthe 'J'yp@ I tumor (1M)1id tumor withou~ infiltra· tiye ~mponent).
""own
"",.
,,
TREATMENT
CHEMOTHERAPY
MO$t ODGs rl!llpond to chemotherapy, ~.ually in I Also Itud itd: ttmOUllomlde for rec" l'1lmt lUI"pl.alle olill"'lb"9r)'UIml .ho~!IOme
l'flkoey'"
SURGEAY Indica!iollll for IIlr,ery; I. 0001 wiLh 'ignilkant rna.. ~ffecll'l!gardleq of grade.: lutlel')' deere_Ill. nMd for (or1.ICOlI~roid •• red"cl'tl .ympl.Om llnd prolongs .umv.I'" 2 . hlluor. without tigniflcan l mas. elfeet: A. low.gr.d, Ooc. lind oU;outrvcytotua; aU1"F-1')' III: rKOcnmtllm llrachnoid ""p cell. (notdura}. May be multiple in up ta 8% ofcll.l!e(l"·, this findWg ill more common in neurofibromata.i•. Ottasionally fonn. a diffilae abe-e t of tumor (meoingio llla en p la qufl). • • • •
EPIDEM IO LOGY
As Olllny 8lI 3'J1. of lIuwpEies on plltienu" 60 yr"I age. te.vett.l. " ID&ni~oma"·. Menu.gion'lI' aCOO1JlIt for 14.3-19% Dtprimary intraCT.nia] neopluma'W. Inddence peaJu at 45 yeara age, FeroaJe:male ratio is 1.8:.1. l .&'i' occ:ur in childhood and adoleseeJI.ee , Wlually between 10·20 yeal"$lI~ I. JMII. 19-'l4'1f>of adolescent merungioma~ occur in patient.. with neurofibTOmlllmlis type I (von Recl Foramen m8g'DUIll meningi o mas A. with any (oramen mPgtlUIll (FM ) luion. t he neurologic .ytnptom~ and signs can be very co.nfusing and oft"" do oot initially auggut a tumor ill ~hia I~ation /RI': page 492). In the Fren:h CAlopl1rative Study, there were 106 I'M m(-ningiomlUl"', 31 e lI_t comm" ... Sh""", ofpolygonsl celli. Some use the term aoglom"lou. ior meningotheliometoUJI vllri~ty with dosely pa.cked blOl'ld vessele B. fibroua or fibrob last ic:' c~lJslleP8tall!d by eonr.e Rad ia t ion t berap y !XR"I') Generally regarded u ine ff('Ctive a. primary modality oftreatmenr. MIlRY prerer not to uee KRT for "berugn" leaiOfill. Efficacy ofXR'll in preventing Tetll.TTence is controversia l (tee b>tIOlo undu Rec"nv""",); some Burgeons .eae..." KRT for malignant (l nvllJive). va&cular. rapidly:recuTTing {"l\ggnl8.;Yfl"}. or mm_reserl.abJe meningioma • .
The extent or.u'glQl] tumor removal is lhe mOll!; importent (actor in the prevenlion ofrect1J'Teu.:e. The. Simpson grJding By&t.o!Kl for tbe ex· t.o!nt of meningioma removal i8 shown in Table J7-2J . Recurre nce aller gmB3 totalWnlOr ",moval QI:~WTI!d in 11 · 15% of CIllIl". but WIl8 29% when .... moval ia incom plete (length of follow· up not lp;!Cified)'''; 5-year recurrence ,aWII of37%'"-85%''' RIter partial re5('Ction area lso quoted. ThaovHall recurrence IlIte at 20 yellnl WU l~ in one i'>ries .... and $(). however 10,," report .udden hearin.&: lou (seeSlllklfn ".,..,in,g los. below). 70,. h"ve I hi... frequency lou pattern. and word discriw· inatiO'll is ul",,11j atr~ (es~aUy noDoeabL. in telepbime Q)nvena tion). The tlnnitul i" UluaUy hlgb pitt:hed. Suddeo h&ariD, 10M: The dilferential di.agnosis ror "udden hurir., leu (SIlW iI extell8ive"'. Jdiopa.lh~ S HL (i ,e. no identifted etiology: mu.1 nJillout oeoplulll_ iDlec:. tion •• utoimmWle, vucuiu and toxic ca.-a) ooe\1l8 in an liSb.maud 10 per 100,000 popUlltion' .... l ,.ofp.atientl with SHL will .. found to bavllan AN, and S tn. IMY be the p""lellting symptom iD 1·14'lo of p"tientl with ANI". SHL with AN i. pnIIUmably dWII to an infa",tion nftb e 1ItODustic: nerve. Or acuteoc:clusion orthe cochlu r Ilrtery. 1'relltmmt optiona for SHI.. indude: I. &teroids: e.g. prednilaDe 60 mg PO q d. 10 d then tapered'" 2. • heparin h u been shown.llQl. to be Or help 3. l'O~rv&tiye tN!atme:nt: rut and 1I&lL, akohoI and tobacco restriction i>! -4. elq)erimenr.al: throml:olytic tl1fr1l1')' (e.fI;. rt.PA) (Me fJ'¥f 768)
"til
Symptoms !:rom 6tb and 7th nerve cOlllpreu,oD OuIigill. raci~1 nUUlbnHI and "eilien , a nd Ulal.e ChaOE" occur .. the tUl1loren· b,rgel and compr~ses the fifth and HYiOnlh nerves. 'J'hese I)'ItIptoml 1mI.1Iy do noloocur until the tunlO. i.;.- 2 ~m. Thi, hqu,lightllUl inltre.tln, p.ar&d ~ fad.1 weakneu'. I ra~ or lille DCCUmtnc:e, evtJI th oogh the 7th nerve ill e.IIno5t al .....,. diatoned eaTly; wh e~o8 raciul numbn lllll ot.e,IrslIOOuer "nce trigemiDll comprltlllion na:ul'I(ol\en in the ~.Mn"" or normal facial m .... emenl), dtoIpil.e lba r,lrt thlll th, 5th nerve il farther aWRy'''. This mey be due to the reai!illo~y of motor nerve- re\ati¥e to Ie"..,ry Ml"YH'.
Symptom' from com pre88 ion o r bral n8tem and other crania l nerve. l.!Irpr tumors caUMl brains1.em compression ( .. ith .tM.i•. HI.... NN , diplopia, ""reo bellAr . igna, and I{ Ulleh"cI.e , ,
..
"., ,. : ::.:~!w"""
PATHOLOGY
. ~ ~~
Tuwors a.cecompoBed
of Antoni A fiben (narrow eLongated bipOlar cells: and Antoni B fiber. (IO;>M n!ticulated).
,
2.
20 Or 30 mrnlYT''''' '''. The
•
, ~"
.
,
mQjority oftumol'll (bu: notaWft) will , how ~Ule gt'O"tl h within 3 )'fW'&IIl .
E VALUATI ON
,
..
Gr o wt b ra te Growtb rate Us unpredictable , Usually quoud Tanll'" _ I . JO mm/yr, However, l ome ~hnw tID chang .... over many years, 69& BctuaUy oecn!a88 ill , ize''', whil 8 !KIn18 can in_ crease in diamelel' up to
,
, I:::""
T""'"
,
........
..... ~
..::. ... ,
.~
AUDIOMETRIC AND AUO/Of..OGIC STUDIES Most can be perfOmled onl)' ;fthe affected ear tllIJI usable hearing. FOT dill(f1011tic purposes, tbe eKpl!nae Intiy not hejWltitied l!XC<jpt In low . uspic:ion patient.s, 1& IDBny patienta will go"n to haVE-CT"r MRf r egardJ_"r audioroetrie.fllldiup. Howevar. haSf'Ulle aludiell ma)' be helpful ror later rompariMo" (to docwnent deterior8tion), Or ifaurgel")' is performed to prepare for intra~perativ8=onil.Qringend to(!OMpaTe to pcMt~p. M!IO rnay belp in treatment deci~ion -wak.ing_ The modifi&d Ga nJe lle r .Robe rtson $Y$tem fo r vading hearing i, shown in Tubk 17·25. Mnemoni C: ·~OI~O· tClaS! IJ) il II ~llIlCIl\llble cu((!fffor usefu l hearing (pure toM audiograru ~s hold" 1)0 dB Bnd s~h discrimination !\('Ore " :;(1'10). Clsss I patients may use. the phone on thaI side, cia" [J patientll tan loealize younds. The American Al:1Idamy ofOtola'Y"gology_Head 8nd Neclr. SUllieI")' Foundati(NJ hearing elanifiCllt;on !l.)'lltem' · ;8 .hown in Tobit J7·26.
NEUROSURGERY
17 Tum"r
All patic otlshould ha ve th e fol· lowing Pure Inn e lIudi OfJ' .. m (PTA), May b.
Table 11-25 Gerdener and Robertson modified heari,,!! CISlllIllCill lon'
USI!fi.J.J Ila r;~t-It.ep Icreenin, test. Air cooduetion aa$t'~ the. entire ey1't.em, bone conduction a!U 3 em dial mey .bow cYlltica ppeoring a rau OD CT'l"r MRI ; in aduality tbetlll! 01(1)"8 are uSUIIlly ..olid. Adjacent blOIlklld CS F cisterna mlly 111110 give cy&lic appearance . CT !lean wi th TV conlr&llt.: !I i._
TREATMENT OPTIONS
1. ....pecl.8 nl manllg>!menl: follow lymptoll1., hearing (audiometrit!l) ""d tumor grow\.h 00 seria l imaging ICT Or MRI q 6 mos ~ 2 yn. ttoen lirtDually iflitabll!). In· ter.'l!ntion ia perl"onned for progr8Sllion (Ii", fQQtnolAl to T"blt 17-21 for detail '). Growth ~tt... ms O~r.'ed ; A. litU. or no growth : usually thOlle contained with in the lAC B. $Iow growth _ 2 mmJyr C. rapid gro.... th· " 10 mmiyr 2 . radiation therapy (alone. Of in conjunction with lIurgery) A. utemal beam radia tion u.l'rapy.(EDRT) S, I!tereDtaw.c radi osurgery (_ page.539 for trealmMl detllils) 3. 8urg~ry: appl"O"olHl. indude the following \Sn Mlaw ror details) A. I'I!trosigmoid (AKA suboceipi llll): may be abll! t.o .pare hearing B. U"an.'llabyrinthine (and iu BeVeral variaUIm')' w.orifi"'lll Mllring, may b 3Q11~) choice for moot lwnots. lot post-q.. grCM1h Recom~ndatlonl for ASA _ ~n Society ot .......,~la palientr; without nelU"or,-.... _ ~ SRS ...n _table 0pII0r! (5ft"..'i39l bromll\.OiSis are shown in Tol>lt 17·21, Age W8' not a •• p.c..nt ""'nag. .... fIt ; ClOSe _al",". ' _ I factor in $urgicul WItt ........... uong /CT DO" MRI Q e """ ~ 2 y\dC.aliana), ,.\lt1III_ tJf glOW!!> II ooIls_ Tn mast ""_ , inee tumor growth raY, i. b\l11Of\g~",m mo-ulII" ant no! _o&IIablIIlO IuIb" ....... 1NI'''f'''U\Ic elli· v8riable arid moy be .i(lw Cky Iln6 c:omplicariorl 'ele ,11hi, \lme usMu! haring is un li k"ly to be pT_rvad po8l·op Ie
17. Tumor
NEUROSURC;ERY
A. prtI-op 'PHch dlsmm;nMion < 7(j'l. 8 . IIf pre-op Ulr.I!hold Ion, 25 dB
C.
I)f
-
Table 17 29 Approach lor AN
pre-op BSAER hu .bllorm.1
Tumor .Iu
.... v. rnor;>kolGp'
D. or tumor" 2-2~'; tfI'I di ameter 'arye tUmonI may be approeched by. tom bi~ Ir.II8I.b-.u~ipjt.a1 .pproa~h LO d. bulk tumor and pl'fterva facial ne!'WI:' two alall:a ap pro.cb (with 1·2 WHIu in belween) mlly improve rellll:. ... ith very ,,"',
lllge (> 4 ~ d,a)
medium t2..j em dla) ttM'ICil'lfl~'t)
wiIh poot ht... ~ -
,umor.'".
.lPPfOlCh'
''':_'' !:1,TL
'"""'" ""' T._ m,". SO Of Tl
..
1811111 )n lAC
5uRG~L CONSIOERA TIONS
Thelupel"iol" .... t.i:mJllrdivi~ion or VT! I ill til.
.... go:ld hearlnll-
.F
"'IAC
usual origin of the lwoor. The facial nerval. 8IIPIOK"'. MF .. . . . . 1cIua. SO._ pullhed r_ _rd by the tumor in ~ 76 ~ of CUM oocIDOI... T\..~""""" (n nll' : lIO-SCM). but m'l)' 0«. .100.11,1' be pulhed rwtraU"1_ olten lnferiol-Iy. and rarel, pGteri· 0I'"1y . It IUIIt be n..tt.e NEUROSURGERY
17. TUmor
"s
Most leaks ate d iagnosed within I wee k of su rgery.lIlthough I p r~nt.ed 4 yeal'll post.-op, .... Thoy aeem to be moTe common witb IIID", laterlll ullJ'OOting of tha IAC'-. Meningitis conrpllcal.t!s a CSF lull in 525\'0 ofr:aaes. and lIiIuolly de~(llop~ within days of thl! onset of leak"'. Hydrocephalus UIJIy promote thada~el· opmllnt oro CSF fistula . Trea tme nt, 25-3S'lo oflealJ..os ijtop SpOnt.aneoWlly (one 3E!rill$ reported ~)' • . Tnntulcnt Dption. include: 1. no"·surgic81: A elevate HOB B. ifleak p. eVA in o 7%. subsequent rl!Q ... i .~mem. ror C$ F .hun~( ror hydrOCflpha!u s or l 17.2.7.
Pituitary adenomas
Pituitary Lmno,.. (adenomfll) ari.a primarily from the alll.l'crior piwitary , land {ad_ 8l1oh,)'pophysii) (neurohypophyte.1 t UIl>Ol'll an rare) and may be cllllllilled by a IIl1ll1ber ofscbellJU. incJudini: by eodttaina funetion, by li,i'ht m;trweopy with rol,lt;1\II hi, tolori· cal staining method. (ut!~ 442). and by electroo miu~ appdlllnoe. MJ e ro a d eoowa Definition: a pituilary tumor < 1 CIII diameter Larger tumors ... re Ixm.idem macroadenOOlaB. Curnmlly. M>'iII uf pituitary tUD'lbnl ~ < 6 mm at timeofdiaer-ia. 'l'bese may be difficult to rind al un: Lime of a urgery. OIFFERENTlAl DIAGNOSIS SH ~ 927 which Inclu~1!II non-nec>pl.sllc c:lIAid ...... tioM IS w ..U.
EPIDEMIOLOGY Pituitary tumors repm;ent ~ 10% ofinl.¥al:raaiallumors, all hough itautopsy 1100' iM aTe uliliUld the iDcidenu ia highM. They an m*t I;OlIlmOtI in theard and 4th decad~ uf1ife • ..,d "'IuaUy Iffec;t both.ut!S. The i.nadwoe is incre8eed in mwtipltl endocrin.. nd"oo .... atlMria aT neopluia (MEA or MEN). CLINICAL PRESENTATION OF PITUITAR Y TUMORS
Pituitary twoon usually pr8leOl either due to endotrinolog>e disturbance.« due to \DaM eifec!.. Pituitary wac:ro/Ideooma~ may prod~ HlA. Seizurn llNJ'Uely attributable to pi tuitary adeoom ... A auall nU\Dber PreRnt with pituitary apoplS"lCY twlout). RIorely, iowlI';vt! Kenomal ~y pre""ot with CSF rhinorrhea~ «2). Clu.ieally, pituitary tUQlOI1I _ctivided into two pDUp!I: funetiooal (8lhetiQ within ~ ca~",_ .lnl/.l I'I\8y produce .. torm of Hom.. r'. l)'lIdl":w". with unil.tl:ral pt.-it, mia.iII, &: anhidroti.!iro· il.l)d to tbe fol't!helld 4, when hemorrhage breaka throuSh the tumor cap'ule.:I'. It is common rOT apo· plelQ' 10 be the Init ial presen tation ora pitllitru)' tumorU!. Eyaluat io n CT Or MRl l bow.llemorTbogit masal n sella and/or I liprofe lla r ""Oil, o/Wn di.ton. ins the PDterinr third ventricle. ClI'ebtlll a ngior nphyllhould be considered in caIN where di/Threnliatin£ pitullary lpaplny from aneurysmal SAH is difficu lL Ma n tlgem e nt of pitu.i tuy a pople s-y Pituitary function iI DO""'lItcntly c:amproDlileci, net:estll8tl"g ,~ pid IIdmin;ftl' "tion Di't'Ol'Iie08teroHl, and .ndoc:rin.e ..... aluation.. In the obsence of ... isuaL defieit.!il, prol .ttinol>~ nay be t reated with brwnocriptine. Rapid deeompreaaion is required for: l uddeD coo.trictHlII (If ... j,,,tJlield., HVU1! lIodIor .,.pid dK.. rim-alion or acu;ty, or neurologi.: delAlrioratio", due to hydmcel,h.lul. Surgery in s 7 ct.y. of pltllitllry lpopl"V rauJ~ in be(tu imp ...... emMI In oph t hal · moplegia (100'10), YlllII l rolty (88'i\) Pnd field rotl! (95'10) thin . urlery Illler 7 dllYI"". DecomPT"SIOD I, uswilly ... ie. a ll'lnupMnCl,dlll routeilnnIoCTlUli"I'ppl'OIIIch DIIy be IIIYlnlage(l... in JD(Il" o;atol.).. Coal. or .urgery: 1. to decomprna the foil_inc . truet", •• lf uDd.r p,.-ure: opt le IIpp8rat.u ~, pituitary gllnd, eayemol/.l sinu" third y~ntricl. (I"1!ti.... ing hydroeephalul ) l. obtain t i _ fCH pIIthology 3. ""nlpl..1e ~"""'A ' oftulDOr il ulu.lIy not n~.ry " . ror hydl'OOllph.lul' yentrieular drAlDOle I. g~ertll!y required
NEUROSURGERY
17, Tumor
'"
FUNCTIONAL PITUITARY TUMORS
The most rom man functiano.l pituitary tumoTII """rete one of the fallowing: 1. prolactin (PRL): prolactinomas o.re Ihe mOlit common !eUi s"pp",~siQ" on page 446. Prevslence: 40 cu· es/million population. Cushing's di.urue is 9 tima mOre commOo io women, wherease are Ja rlle enough tQ produce IIOme rna"" effe.;t, whicll may C8use enlO'llement of the iIeIla. visual field deficit, cranial ne!'Vl! ;nvolvement andlor hypopituitarism . C",ll. contain p roapiomelanocorttn (POMC), the precul'1lOJ' molerule which conu. iM amino acid lequences fOr ACTH. a lpha· MSH. J).lipotrop;n, O"'ndorphin and mel.-"nkephalin. ACROMEGALY Growth hormone (OU);, Wlrier dual hypothalamicrontNI ,';8 the hypoph)'lial pottal ByaLem. C U. ..,lea8inlC hormon e (G HRl1) stimulal.ft pituitary secrel.ion and _)'nl hui, ofCH and induoo. GH gene transcript ion. Sornatofltatin IUr-p'l!IoIIU OH ~ (mil'. SomatoTnf!llin·C (AKA insulin· like growth fB.ctQt·1 ClCF.[)j Illh" prGteinJ;t'le is variable, wi th IoOme tumors beinS more IIggTU' ,ivlI than otheT1l. (kcasiunaUy, these tumorl llTow to gigan ti c situ (> 4 tm dial, and the.;e are often very aggre5' lIive a nd follow II malignant course"' , At time!!, an adenoma may pu~h the medial wlill of the caV8J'I1DIR linu s IIhead of it witJlout IIctulllly ""rforating this duralsln.1ctuu..... Thi s i3 diffic"Jl to reliably identify on MR!. and th e roost de~nitivlI sign of cavernous sinus invasion i~ co ra tid artery I!nca..,ment''',
'-I: 6P'fid.,ja CSF OfbloQd.bobslMlCtion or CSF rhinoM'b ea, which occasional ly may be preclpi· tated by tumor shrinkage In ruponflll tobromocriptine>. E~ophthahuos may occur with orbital lnva.rion dUB UJ comprom.i6e oforbi tal v~nous drainage. Thos. that BCrete prolac· tin ofLtoo prellellt with tindin~' ofhyperprolactinemie (SH p~ 440)ll od with theso;,. the prolactin levela are Wlually > 1000 ngldI (caution: siRII ! invasive adenomas .... ith very high PRL production rna,!' have. falHly low PRl, level, ,,«pagr 446 ).
PATHOLOGICAL CLASSIFICATION OF PlTUITARV TUMORS
LIGHT MICROSCOPIC-APPEARANCE OF ADENOMAS Older da!l!!ification'lYstell1. Witb newer teehnlque& (EM, immllnotUslOChemis try, radio-immuno a$&3Y ... ) man) tumoTl; previous ly cmsidered noosecretory have been found to have ,oll th .. componenta nK:e/;$3ry to ~rete bormon es, This _)'Stem i. of limited
..,
u8ef'ul n~&5.
17. Tumor
NEUROSURGERY
' "'~~~~~~~~1Y~~E;~::~~~
w acidophil is ~-20:1), OIigprod ute prola~tin. OH, or TSH ,or ....uIlJI)' ® - m~ (i n
CLASSIFICATION OF /l,OENOMAS BASED ON SECR8"DR'r' PRODUCTS endoc:ri n e-"c~i"" tumor., _ 7~ofpituiQl'1' tumprt! produ< '2 hormonQ th"t lire menurable in the.lierum fiod uu&e defined dinical9yndromes. thesl\lI.re dns.ifi~d ba& L
E. glycoprotein-ucret ing adenoma E VALUATION
Table 17·35 Summary 01 workup lor pituitary lumor. I
HISTORY AND PHYSICAL Directed to look for signs and symptoms of: 1. endoorlne hyperfunction (eee FunctwncU pitr;.iI{Jry t .. mor~ above) 2. endocrine deficita (due to mU4 effect on pituitary ) V ISUAL FIELDS
Forma l visual field testiog b, perimetry with 11 t.8J)gentllC~D (U5;Og the small red stimulua !linea d""",tuTatioll ofcolor i~ an .... rl, sign ofchiumal compreasion) or by Gold-
NEUROSURGERY
17. Tumor
11111[1 or lIutomllted Humphrey pIItiMt to be ulld,.
~nm8l.f.r
(thelatler .@QW ..... IOOII """~a tlon rrom the
ViAUsJ fie.ld deficit pattern. Depend. ;11 part On IocaLion or chill!lm with reBpec~ to "II .. turcka: the chlllll'" i. located above thl' ..JI.. In 19':'. pootvior to the ~1l11 11.=1" (pn" n Jled ~hium) in 4"": In mill' orthe . IS ,,/Vdl .. llh .. n incl' 1 IlgidJ. Or .. pu,k '> 20 I'gldl regardl_ orthl increment a. 'Ilbnonnal rtlllpo..- indic8~ "d",oal in . .. Mdeoc,. In pri. mary o!lnnlll in.uffidlncy. pituit.tl)' ACTH _ lion Will be II· cva~. In _ d ill)' adrlnallnllllfficienc),. (/lron_lll..educed AC'l'H c...ae! adl' In,
,"p,
11. 1\,mor
NeUROSURGERY
2.
regular j""ulin 5 U rvP thyroid alii.: tile basi. for thyroid screening is sllown in Tobie /7-36 Table 17-36
· ·
·· ·
·· · · ··
ea,i, for thyroid Icreenlng
Prim..., hVPGlllyroidlsm cho"Qnk: primai"/ fflpolhyroidiwl mB'j p~CI! 8if.Ondai"/ pilullai"/ hyperpl~ (piru~al"f p66~U· t-=~ mor) irh:.kIil"!ljoisl\able 1r<Jn ildenoma 00 CT Of MRI. Must be con&ldared ill an\' ~lierrI wiI~ I pill/- I I ltarymaosu ,." pat/lOpl1y5ioklgy: kIs$ 0( ~t!Ye IeeWiICk lrom ihyrota homlonts ca\I$eS ooease(lTm1 rel6356 frO'!l"" h)'POlllil.1rrJl/S poodUClnll ~nyperpWja of U!yfo!,ophlecer.s irltle a~ sis (\royrolroph ~) , The Jllltien! may Pfesee W&~; give adret\31 hmoIS ~ IIlOSl (85·90'4) cases oj DMZ I "'6 PO @Ill P.M. and ec!opoc; A~ proOJdioll (tspeeidf torordUl dr .. w ""rum cortisol the next Cal IoiIll'lOlaupptess fI'<eI1 wiIh Jio:1I tollt: (NS: up 10 2011 Orplltianl' •
... ,th CDIio root I" pprti AcroOl elaly
IfTOwth bonaoae (GH): nannal bani fauiullevel .. < 5 ng/m1. tn pI:Itie nl.8 with aawnq:alyOH;. " 10 ndmL NOl'Ulal be.allevels do Dot dia;tinlrllillh nonnal pa_ li.nt mm OH defid~ncy·'. Furthenno..., dUd to pulaatiJ", selI"llIll!Jl~r CIItQtid Il.rteril!ff and to RIO IInNT)'IIW 811 II po$8ibility. Ga,lcium In pituitAry usually .ignifi... hemOfTholge or ior.n:lion within tumor.
Enhaocemfllt (WLth TV contrll l): I. normal pituitnry enhanc,", den6O!ly (m. 11118) 2. macroadenolUAB enhnnce mort than oormBl pituitary 3. mict'OadenomBll enhllr.ce ll$l l may JUSt be . lower). DiognO!tic citeri.: A. must h"vlI nttenuntion chlWgtI On CT
PLUS
B. 2 or 1Il0ttl ohhe roUowmg; 1. focal bone eroo;ion of ~eUa 2. fllIL, nightlUllrea pnd naaal congettion . Side efTecti are more troub/uome during the first few weeks or treiUment. Tolerance may be illLproved by bedtime dOlling with food, l low dOH eeca.lntion, ~ympathomimeliC5 Cor nas,,1 congestion. and Aceta.minoph~n 1·1l h.rs hefure dolling to red""e HlA. PsychOlLi. and vWIOi!:Lp!Lsm sre "'ttl Mid" effects that UHUMl!y neelS' lita tes discontinuation oftbe drug.
J
bromocriptine (Parlodel®l
\
/
01100 INFO
\
A JelIli·aynthetic ergot alkaloid that binds lo recept.on on normal and tumor I.~ totropha, inhibiting syntheais and s«~ion ofPRL and other proee9BI!IL regulntina eel! growth. Bromocriptine lowen. prolACtin level regardleu oftbe whether the lKIurce 1ft In Idlllloma or ILOTOIILI pituitary llO.g.lIlL . resultof BilLik effect) to < 10% ofpNtffatmentva,l· ues in most patie nta. lt s!$O frequently redu oe. the tumor .l ite in 6-8 w~ks in 7,5% ofpa. lienl$ witb IUlIcrOlldenO(Jl8B. but ollly ftl long,.. therapy it maintained and a nI)' for tumo", tbat attually produce prolactin . Only . 1':\ ofprolactinOnULIi ~ontinue to grow wh.te the plLtient;s on bromocriptine . BromMriptin e can teltore fertili ty. Continued therapy during pregnancy hoi beom flS8: 2.6 mg K OI'9d tab_: 6 Inl CllJllules.
,-I
pergolide (PermaX®)
\
I
DIUlIl*O
\.
A Ign8·lIcting ftrot .lk,lold dopami"" qonj.It that NdllCU PR L laYeI. for .. 24 h. l. Not FDA approved ru. hyperprolactinemill, O~d • •1y d",inll improYu eompl ilU'l~. "OII I;J'I"'ECTS: ~u
nboue.
Jb; Start with O .~ mg PO II 1>5. and ineNa .. by O.O:lS-O.Qli i!lO'j!mllnU\up 1O . max·
imum of - 0.25 m!Y'd l until dC!lllred PRL levfiJ an' aehievtd .
,-I
caber gol ine(DOlitinelC®)
\
I
~I;pond to bromocriptine. the us"al dOSllge is 20-60 mgld in divldoOd d~ (highe,doses lire uOWlllTllnted). The mloJ to"
,.
; I
,
, I
h
Increase UI' IlIl1roulmum of 1500 l'g1d (doses t"'lluil'til is 100(21)0 I'i SQ q 8 h.rs.
~
pegvisomant (Somavert®)
\
I
0lII.IO INI'O
\
,
A eompetitive GH.receptor " n!JIgonist. Tl'illltruent /'ot .. 12 mOIl "",,,Jill in nonnlll IGF. lleveLt in 97'lborpatienl~"'". Na ~hange in pituitary lumaraize hn bttea abll"rved"'. Slm:Erncrs: a ignlfiClint buI rflverBihle liver furn:tion abaormalities occur in < 1%. Serum GH ;ru:rea!t:tI, probably 6$ a result ofloll8 Qfnegaliv" feedbo~k on IGP.I production. Rr. 5-40 mgl'd SQ (dose muat be titnlted to keep IGF· I in the normal ronge.loavoid GH deficiency tllTlditipfI.!!IJ. CUSfiINO 'S OISe.SE T1-ans. ph-: an anti fungal s.geM that blocks adrenal steroid . yo thesi!s. Th e initial drug orchol ct-. Over 75% of potie nt. have nonnali:ution of urioary ~ eorti.801 and 17· hydroxYCQrtic:oeteroid levels. SID~ 1IPnCT"ll' Illvers.ihl" ele¥lIIioDll ofsenun hepatic Iranilaminll8e (io 159lt), Gl di_fort, edem A, gkin l$sh. SignifiCBllt hepa!Olo)(' Icily 0 becoming normal. TUmor ahrinkaB" o«u.,. in _ 33,.. lU: Start with 51).100 IIg SQ q 8 bra. Titrate to TSH, T, "nd Ts leveiB. NONFUNCTIONAL ADENOMAS Due to poor respOnse 1'8te9, ( urgery and/or XRT '-Ie uauaHy the initial treallnentof choice (..... lH!low for XRT).
Non-&ec reting adenoJllIU Bromocriptineha. been tried with mild reduct.iontl in tum~r 6me;" only _ 2O%of pp. tients, The pOOr resulta are pl"Obsblydue to the pauci~yofdopaminergic recaptoreon cell membrane. in these tumOnl . Od.reot.ide r tdUCe$ tumor volume in _ 10% ofc8lie5. Gooadotrcpin-secretiDg tumOl'1l Some non·l'unQtho.• .erin of 108 pitui1.aJ'y macroadenomal found the re u.
NeUROSURGERY
'"
oervwchia.sP'l and ca,rotid. Does not all11W tot.al removal ofintrnellar coml' 250 mVh.
2. acl.ivity:BRwithHO B @30". 3. 4.
'"
ice crups PRN . To a"";d aspirating flit groflfrom sphenoid $lnul, pilti~nt is lUll to drink through II Itraw and incentive I]ltrometry is nnl. used lYE: base IV D5 112 NS .. 20 mEq KCIIL III uppropriate rnUl (75·100 mVllrJ PLUS: ""place UO "" ~e IV rate roJ ror ml with JI2 NS. 17. Tumor
NEUROSURGERY
NB: ifpatient re.:eives s lgnifieant fluids InITH-operatively, Lhen they may hav •• ,.. Rl'prnpriat.e pos~-op d,uresit, in whkh e!liO! rom;;der ",placih!; only - 213 oruo '"
b.ise IV ute .. ,Ill V2 NS O. 1ll00s A. antibiot.i",, : continue thlorampheniool500 trlS fVPll q 6 hr (a]so oont;n ... & o;.mpicillin if used pre-"p), chlonge to PO when tolerated, DIe when nllSa. packing remoV1.'d 8 . steroirls (post-opateroirls8re required until the adequacy ofeodogellO'" ~te· roids is established , eKl"'cillJ]y wi\.b Cushing'5 di.ellae, Off! bela ... ). E ither: hyrlrooort.i!lOne 50 mg Iwrv q 6 h~. on POD f2 change Ie prednisone l> m~ PO q 6 hl1l" 1 day, then 5 IUJ!. PO 810, Die OIl POD Jl6
OR
hydnxorti!lOne r.o IDg IM/IVIPO BID IIBd ltIper 10 mwdOlllelday C. ";,,betea jnsjpjdu.e (OI ): ! u bt/ooJ for !ypieal pIiIt~l'1U'. Criteria ' U.O. '" 2W ml/ht II 1·2 hrs,.llllI SG < 1.00(; (usual ly < 1.(103). [(OJ develops, Btlllmptto keep upwith Ouid los! with rvF (..fioJ:.,.... ); ifrate Is too h iSh ror IV or PO replacement (> 300 cclhrll 4 hra or > 500 ttJhr It 2 hI'S), che.:kurine8.C. and if < 1.005 then give, vB.Mpressin preparation (6U bl!100J, or "'''' Tabl~ 1.9, PIlI!" t8l _Caution: dllnge. ofovertre&ting in ClOse of tr'ipbasic resJlOn~e (see below ), therefore u86 EITHER: 5 U .qul'Ow; V.lIIIpre5!lin (Pitres$in®) IVP/IMISQ q 6 h"" PRN
OR
desmopressin IDDAVP®) injection SQIIV tit;r&t.t A$elliuneot for- ACTB (corti cotropin) ~etlerve'" If patient was no\JlypOCQrt.i$OlclIlic pre·op. taper lind stop hydrocorti~ne 24. Table 17-40 /I,\!e rpretalloo of 6 AM c:orti,o l 48 hnl post-op. Theil, ~t.eck 6 AM llenun I."els t:Ortir.ol level 24 hl1i after dillContinuini hydroMrti!gldllhe IUlxL mom-
NEUROSURGERY
17. Tum\lr
in,. CAUT ION: in padenu with "'try little tKem!. ~he reduced cort...ol may p...,.,o!.e.d tvnl l itllulflClancy (thi. te.~ ito safer tha n the hlll'her dOlle' used rnr urinary 17.0HCS Ie.tln,), If do n~ do thi. tat .. an outpatient.
Postopcrdive c or .clln A Iludy e>f 12 plltiellu with m.~roadenornlll fol1owin, lranaspheno,m growtl1 honnone lev,,1 or < 5 npmJ,."d noronll.upp~on to < 2 "glmJ"fter Int\:etltin, 75 gm of giucose. Soml\.llmcc!in-C (ICF-I) level •• hoold "Iso normalize. Ttsnssphenoidal.urgery result" In biochemical cure w85"" of ea_ with adenom ... < 10 mm diameter, no .. vide""" o(locsl iovasiun , and ",ndom GH level. < 40 nglml preop . Overall, _ SO% of.oJ.1 ac:romeg"liu undergoin, tranuphenoidl' wrgery had I bio. chemical cure"'>. Only SQII, 01 wr;crosdeooml, and very f_ witl1 marked .uprll"UIT u, !.enlion have .urgical CUrl!. These tumors m., llan recur yean l.l.er.
17.2.8.
Craniopharyngioma
eranioph"ryngiom .. (C P ) laid \.II arise frou, anlerior IUperior margin of the pituitary. Dlld Ite lined with .tratified aquamous ~ithelium. Soma CP may aMAe primarily within 1.h.l.hird ventricle". Almoet.1L CPhavt.ol>d ami cysticeompooeo ...; fluid in lhe cysu v.riIIs, but uluaUYlX)nt.airuo cholestenll c:rystal&, CP do not undergo malignlUltd.,. Beneution; but difficulty ill cure m.u. tham mallpant in bahlvio'-"-"'_CP are distinct from RaLhlu!', elfofl. c,..t, but ah!lnllOlJIe ';'milaritiell (.- ~low). C.lciGC!!t jo,o. mktoe.coplcaJly 5091.. Pl,i " x-ray: 8M" in childhood, ~ in adultl/. EPIDEMIOLOGY Incidef\oe: 2.$·~\lo or.U bl1llll l u-.: "boUI ~oceur in childhood !9'Koof Mauon', seriea). Pm inc:ldence; 1I0'Il 5·10,... ANATOMY
Ar\,tri., . upp]y: ....... lIy , man feed(,rs From A.CA. and A.-comm, or from lCA -.nd Pcomm (do not re~lvI blood (10m PCA or 8A.-bifUlcttion unleu bloocl a upply oflloor 01 lhitd vllntride ia pano.itil!ed •.
SVRGlCAl TREATMENT PnI-op endoerioolocie .VlI h. at ion
M fot piluitaryll.olUOr~~. 444). H,ypoadreMII"m m.ybeoonw:ted rapidly. bu t hypothyroidiam I.tIIIM long;:r, ei thercondiQon CI.II in".,.8e utrgicJ.I.mor'lllh~y. It..
bued DO .... - " 108 _ _ m..-. ...
".
17. 1\"ooT
NBURQSUROBRY
Approacb
PQ5t·op
Corulider all or these pa~i~ts h,JlO.a d.-enal , add hydrocorUlIOne in phy!liologic d _ (mineralocorticoid activity ) u. additlOll to anti~ema denmet:,aoolll! \.Bp"'r(.tef'pag. 8). T9pe r steroids slow ly to avoid auptic (chemi~all meningitil. Dillbeu.s iDsipidua (01): often early, may de"elop inlO I "triph",;( Tespon&e~ taee Uri""l)'oulpu l: paller"" of PQttopttru.ti"" djU.bi>lfB ill1lipidul, P{lgIl 455), Best managed initially,..jth fluid replace me nl. If oll!Cel!8ary, use ahort actillg -u80pteMin (prevent.. ia· t.~nic renlll shutdown if a SIADH.like put lUinimize deleterious effect u.., IQ, .wt fJOIJe 53!.). rebluu.""'AlOI!, ......w ( R(;R) II.... hi Rtniogl""lly bnign tumor&. lntrAl:rsnjal_ Iy, they otCur almost exclusively in the p-foasa (tbe ilion occuraa pm orvon HJppel-Unda" dillea..(..... ~low). Retinal HGS andior angiomll5 occur in 6% of patienta ""itl! cerebellar HGlh.
VON HIPPEL-lINDAU DISUSE A rare ( I in 36.000 live birlbl) auLOsomaJ dominant multisyatem neoplaalicdisordcr with.90% penetrance"'" charflctf!rized by a telldency to develop .elinal angiomas, h'" rnangioblM tomu (11GB) ofUl e brain and . pinal PRESENTA7KJN
SIS ofce~l1Dr HOH are usually those-of&ny p./bssa u.a.s.!l (HiA. NN. ce r-
ebeJiar findinga ... __ Pomri"~ fouo. fj".
i~ rarely ducurnent.l':d "",a O1luseQ/'apopiexy due to Intracerebral hemorrhage (JClI) (lobar or cerebellar), however, IKI m~ I ludie! indiCllte Iha~ ucasu oflCH are earefully e!fa.:nineod, aboormal ve~I B t'OMiste nLw ith HGH (and occasion ally misidentifieod as AVMJ may be lOund with ~urpriliing frequency (in ~pite o f negative CT and/or &l1giograrJ!y)*. He!.inal HGBII tend ~ be located peripberally, and may hemorrhage and ca Ul;e ... ti· nal det.achmenl Ef)'throcytoi is may be doe t. erythropoietin liber8ted by the tumor.
(/"alcnforWl) l umfl., page 405 ). HO B
P~ THOt.OGY
No report of malign anI clIan~e. May ,pnoad thru CSF after s urger)" but n'JIlain benign . No:> lroe CllPIlule , but ulluaJly well cl"'\lm$llribed \ nalT1lW zone oflnfiltrat;oo). May be 101id, o r cyalic with 8 mural nodule (70% ofcenbf!lI&r le-sion! are cYBtle: DOdulu are very "'8& Radiatio n t reatme nt EffectiVeness 11 dubious . May be useful to reduce tUDlor si.e Or to retard growth. e.lI. in patients! woo are not l urgic..J candidatu, rDr multiple $1118 11 d~p 1e5ion~, or ror inopflr"bl e brflUat.em HGI;! . Doe, not prey!'.nt re!(TOwtll fonowing aU.b total excision.
· I
17.2.12.
eNS lymphoma
t Key
features may be primary Or secondary lp.atholojpCllIIy identical) • lU8pect.ed WIth hDmOi:ene (l/PCNSL''', II!lpeciaUy AlDS-nll.ted cateS'.~"I
PF/ESENTATION
?relentstioo is ,lmllsr with plimaJ)' 01" 5I'ctIodiry CNS Iymphom;o.; the tW of caSH! A. hemhnotor or hem~nlOr)' deficit. D. aph.aa C. visuoU field der>dt. 9. ctlmbiut;on of foc:al."d non .foc.al.ignl I.
Uncommon but c h .....cteristic .yadromu 1. I.Iveoc:ycliUa. coioeltienLw,th (La 6'i\ofl'll!ll!1l10r pn.cedine the di'i:Oosmof(in 1I'l1> ofeaas/ lymphoma 2. .ub.cu~ elKephaliti, \< hemorrhage), .pina l cord or ~rvt: r OQ11,'fmptom. Iindudins: cauda rqull1.ll syndrome,.u pog~305). ~n. dCHTIen Or lower I'lItremnitll, .nd theM CIllIe. ItUl,)' be diaglloted wi t.!> sltin bio.psy. Other. ""lie, thagno.i.oftc!n reql.lirn brain bio~y (open o r .u.rl!OtacticJ. in which involved are&/! on iRlllging l ludiu ar~ Ulrl~IOId . 'l'reat.m~nl with combinat1un o;h.math~rap)' ca.n resull in Ioni·~rm rtmluio n ;n IOm~ p"Q~n ta. bU l early djapOIIis bo.ro,.. pennantn l damage OII(:u,.. il are hyperdC!l).!t to bnlin. Gnly l~ aI1I hypod.nM. Cha""l.a'1lli~ly, ~ ~ot the .... tumors e.Mance; \.hill is deneely hom"lleneou. in over 7~ AIJ •• esull, whe" ra14l nOfl ·.,nhancinK _ O".
Rad iatiOD tb e r a p y The al8ndard t.... atment mller ti88ue biopay is whol ... bra;n radiation thenopy. DoIIes used teod to be lower than for other primary brein tumora. ~ 40-60 Gy total are UUH•.IJy given (n 1.8-3 Gy daily &actio"".
Ch e m o the rap y 10 'l~'i ~aS With no t .... atmellt. median surviyal i. 1.8.3.3 ruonlhs following dia&JWSis, With radiation t hern py"lO, mediDll ~Ilrvival is 10 montl" , witb 47% I·year roed.ian i urvival. Llnd 16~ 2-year median surviva1.S-year .urvival l. 8%, and S-year furvival ii 3·4%. With intT~ven tri~ulM MTX. meWing X.t:~T, tbe median l urvival;8 only 3·5 roontlW" ·.., Ulually related UI A1D5-relotl!'d opport un ifFt;"mfet'tion. However, neurologic funetion and quality of life improve in ~ 76%"' Although the~ are ind ividupI nudie~ lhot tihow trends.lhere are no prognOlltic featuru that eonlistenl ly correlate .... ith aurvivIII,
17.2.13.
· I
Chocdoma
t Key featu res primary mIIlignant tumor of the !pinll Or clivus with high rec!l/Tente rote • char& Tnlatm ent Wide.en·bl\lC excision with pO~!QP'lrative radia t ion IS usuauy the blM5 t option, al· though t his may also til! onlY tempoT9riJy effe P06Sibly \.he m06t oommon parag.lllg1ioma Ipheochcomoorytorna may be more «1m· mon). Appl"OltimateJy /;~ au bilateral; th~ incidence of bilau.rality i.ocreasel to 26'Ao in fa milial caJlll8 ( lJ;Ie"" a,," prooobly a utollOl118/ domiOlmt).
C LINICAL U~ lLI1tly pre5ent atI painle/l_, .low growing mUll tn upper nec-k. l.arge tumo~ mtlY -cranial ne ....ei n.. olvemen t (eapecially vagu.~d hypIlgloual). May a\soeaunste"03i. or le A - TtAo or $tcoke.
E VAL UATION
I. carotid angiogram: demollStratel predominant blood supply (usually extern"-\ carotid, with pnIISihlll oonlrihutiona from ..... rtebnll and thyroce .... ical trunk). May 0180 detect bilet>eraJ le8ionll. Characteristic. 6nding: splaying ofbifutaltion 2. MRl lor CT); ,",vaJllaWII extent. aod ass-e'l5e8 for intl"acnloial extension
NEUROSURGERY
11. Tumor
..,
TREATMENT
Re!!ectioo reported U\ CIU'T)' n high cornplkation ral~ , includi ng s!.f(Ike l8 ·20%J and oerve injllry (33·44'1to). MClrtfllity rale Lf ::;·13%.
~rllnlal
GLOMUS JUGULARE TUMORS
Some ill tbora conlljder glomus t umors as a caLegory that rnPy be l ubdivided io\.O giQmU$ju8u/al"f/ and glom I" fynlptJ";CUnI t umors . Glomus tu.r:non are rare (O.6 .... of all hefld and !leek l umOn), y@tthe,lomuS tympnnii:umia the m09t~D'lmon neoplasm orlhe
mIddle ear. Glomusjugulare tUlllon (GJTI arise from glom us bodi i!!!. uRually in the area ofl.hejugulAr bulb, and traok alOllg ve6 Imaging I. CT or MRI uU be performed.
to delim,ateloQltion and exU!nt oftu/t.or: CT 18 better for ..seasing booy involvelll~nt "rthe . kuJl angiography : c 17.2.16.
Ependymoma
Ependyrnomfll .ri•• from epandyroll celt. Hninjj: Ihe Cflrebnl 'Yf!nlride. Ind theCfln· tr.1 CIOoII o{the apinal tord. They lII ay \100 ... anyw~. alon8 the lIeurwtl.
£pidelllioIOfP'= In tracranial: tomprisel only _ &~ ofint.racranil l ,liom.. ~. 8 " o«u.r in ch ildren-, a)mpriH " of ped iat."K brain tUllIGr," 'pinal: ~ 8~o(apinal w rd ,Iiomll. 96~ otI:\Ir ill . dulta-, aspeeilily thoM of filum terminale (He myJ& pop C U NICAl
Symp to m, M&tly tllose or po&te nor rOS$ll IIlIlSI with u.crea&ed [C I- " ~ L headacbe.: 80$ 2. NN : 75'>\ S. aUUtia or vmig: 6OS\ 4, sei~ures: only in - 30% ofsupntenwrialles;ona: comprise only 1% ofpatienlB with intncranial t"mnra p.l!8O'ln ting with l ei2ures E VAlUAn ON
CT/M1U: usually presenta a~ Q mou in the noo. or fourtb ventricle, obn wit.It ob. Itructi ve hydrocephalu~. May be difficult.to diatingllish from medulloblaal.oma (MB8) radiographically. th~ following mllY help: 1. caldfic.. tlolUj are C'Omrnon ill ependynIOIlI..,.. but tltt.een;n OUTCOME
Op,!rative rnortality* '.rm'; 2G-5~ in early .Ieries, Il10 ........ c:ently. 5.W. . Totlll9W'gieal resection ofpr.m&f)' Intrncr~nill.l tumor followed byct/miOSplnal XR't as ouUined aboveyielda 41 %5·yt!ar . u ....;val. Survival !.eoda to be ...·orae tbeyounCer {he pat;ent(5-yeor l urvival of20-a0%in the pedialtic Croup'",. ;n ,. complr&d with up to 80% in adul~). Excluding- ep.mdymnbla.toma, malignant features in an ependymoma dQeB not neeB&SaTily pt)rtend a wOI'llfl proKflDfl;..... The nil ofrec urT'tnce III hiGhnt in pa tientl with pprtial ~til)fl . SPINAL EPENDYMOMAS 'fbI.' m~t common 6pinaJ «Ird g-liorna below !he mid· thorooic r 17. Tumor
NEUROSURGERY
""urobl"aloma, u lhuio"""roblutol'1lll, apendyrnobl"ltc>mll, an d polu ,ponglobl",toma, Th_ \umon mill' di Y eminllta vi. the CSF . ponlilnaQusly""', Or IlItnlgllnkaUy (rol. lowinS8w-geryorlhunting. tbe lattl!r i. n rlln! cauKOrtumo rdi'Mminalion"). Th ull, .11 patients wi th PNET. require I pi.nlli mlen/u.clon (a:~dolinium tnhanced MRI I, dlOu l
... MNiti,," .. wllt.er-SOllubl. myelography) and cytologic exam ination orCSf'. ProphyLa... tic: craniOlpi nal XRT I, indicated foUowll1. aurgicaJ re mQ'" .omelimuplUmDt.ed by shunling'" (a llhoug h thia ia Ilncommoll"l. EVALUATION U.ually appears all II aolid. midline. rv·conlrest_enhaneing lesion on CT or 1100. Most eob....,. (pno-Ontrasl may be hypo_til hyper-de ..selon CT. See l nJ.rurlllJ$ on pa~ 471 for companaon ofi tlMging clllullderislics. Spinal MID wi th gadolinium Or ...1IteJ'·",luhleconlrut ....yelogTllp/1ylbould be done to rWa-out -drop meu". Staglng;' doneeither pre-opot ... ith'n 2-3 ... eeks of au'1ery. TREATMENT Trea tment of choi"", surgle&l debuiki"i'of as much tumor as p!lIIIW1d "ital I trutt"'Q {lIr'Knial m!)"Vee, ICA ... ).
Din inetioQ from ebole8 ter ol granulomll Epiderttlllid cytt.ll h.". o~n bee" equa!oOd with cholw.erol rranuloftl~. HO'II'_r, these are diaund INion ..... : he 1~11.er uaually 00I:\I"" fuU_illcehnmie
Ta ble 17· 49 Charll'Cleril tlcs of epic:lermold III . cholesterol granuloma
innlltl'lmaUon.. Some dilferellCet • .-edelineated in Tcble 11-49. PriESEHTA.T1ON Ep~ennmdlnmyp~t.,
any ...... laiDn in the nme loca_ tion. Addition.lly, th,y may present ... ith I'flCWTeIlt epillOde!l of a.aeptk mwingilia caU'!l~ by rupture of the C)'ft ('c"'I~II~. SYIIIPIOmI indt.ode lever and menincul irritation. CSF shows plcoo;ytllSi,. hypoglyt'Ol'Thachia, .levated protein.lInd negati.." cultures. ClioIMterol crylta!. m.y be see.o and can be remgnlzad by their amorphoui blrel'ringe.otappearanct. Mollaret'. .... .,ingitia ill a .are ".rilUll or.""ptlc memngitia which I.ncludd the finUing of IIIJ'i'I! «!Ib in !.be CSF that rHembto. I!ndotbelilol «!ll!! (whkh may be lDecrophaB"""" tblll lOlly be seen Ifl.ome patienlll ""jlh epidf'rmoid cylle"· • •
EVA.l.1JA11ON C'I\ low delUily, lli,hliy paler tha., CSF, with 110 Hlhanctme"t*. The presence
er enh.loncemenlluH8lits the poM;bllityof. malignant epithelial component. 8oM..rolion;1 HeI\ in S~. MRJ : T1WI; ;nte,..ity ill ,li,1I11y > CSF. l'2Wll turner and CSP b.".lfrnilar hip illLeNlity.
TREA.TMENT CIo ... t lOll whel\ remoVinll ePld~rmoid qlta Jo avoid spilll", c:ontellta .. th..,. Ire q .. iu. Irrit.lltin, and may 1:11.... ,wv.r. chemical meninCltil {McUlret"1 menintili........6ow). Berg."- .dvoc:atetlntrlop....ltl". il'Tljflllc" with hydrocortilOMllOO mgIL. of LR.I to reo duce the n,k or poa~p oommunic. tin, Rydroe4oph.I .... P"n-ope ... ti". TV et.eroW" and gopious 1.I;n e il'Tlption durilli WIJCf)' m.y provide lirnilat res ... lta. f>l,e 10 th.d~n&el,
NEUROSURGERY
17. '1"t"nor
;t75
adh .. rent ~lIpeUJ .., it J~ on .." ne~essary to leave I"I'tIlIIanQ behind , ho..... ver, Ifthe!!11 are smaU this dGes not pnoclude .. ti~r.ac,,>ry outcome, In ! pi te of adequal.e. remo"a], it i! not un .... ulll to see peraistent brainlltem di.tortion on post-op Imaging"6'~ Post-op radiation Is nOl indicated as the tumor is be nign and XR'T d091 not pn!""m' reCUrT\!IICII-
17.2.19.
Pineal region tumors
Pinl'al regionl": the area ofthab.ain bounded Ta b]e 11-50 Cotldltlon,glvlng rl$e to pll'leat region tumors dorsally by the s pleoium orthe COI'JIU6 callo.um and the te]a choraidea, ventrally by tbe quadrigeminal plate and mid_ brain te Usually an incidental finding(i.I . not symptomatic), ...en on -
4'-" nfMRls'" or
2fi. 17. T umor
NEUROSURGERY
2. excision: rel iev... ,y mplom s ami establishe$ diegTICHIis. Lo .... morbidity 3. $tereotactie or endoscopic 3splration: may nOI get eno~gh tiss"",,, for di.goosi! 4 . "ndOllCsfti dmJlily W IoYfHros/JJ{;i$(){Irn. c"t."rl.o on pal((' 932for differential diagn08is. Patho logy ColUlis!.8 ofQlltcoid t.i""u~ within QOjteoblllllticti•• ue, .urroundl!d by r .... clive oonl. tlhrous dysplaBia.
Diffieult to distinguish
fro",
Radiographlc Olv aluatioo SklillkIP.X: well demarCllt.ed, hon'ogen~w; den,. projection. May arise from the in. ner Or oute. r.able. May he compact Or spongy. Unlike meningiomas, diplile are pre"erved and vll5l'ular channel.$ are not In~reased.
O$Leoma, are "ho~' on nudea r bone """n. TTOlldmeDt AIIymptomatic lelliOll8 may !rimply be ro1JoweCessible t lUnDl1I.
17.4.3.
Epidermoid and dermoid tumors of the skull
SIll'. aJ:oo po.ge 474 for epidennold& and den:uoid. in ~nI!raL SkuJl involve.nent oe· CUtll when KtodlM"mal resta are locluded in the developing skull. Usually tD'dliM. Arise NEUROSURGERY
17.1'Ilmor
481
within tho! dipltle and e~pand both inner nnd ou!>!r table •. Iden\;col clinically and radi .... logically, dennoids contahl skin appendages (de rmal eJemenla). Theile benign leaiorn; may involve underlying dural venoUl atructuT1!/l or brllm. They may b«ome infe{:ted.
Radi ographi c ev ajUu,tioD skull I' rBy: UI~ OfI~ lytie lemon. have welJ·defirK! PATHOLOGY Gros9: pin.k.i.sh gray to purple
l~"ion e~tendiD/t"
out of bone and involving pericrani·
wu. Dural involvementoc B. o_ity • . d,ffu" idiopathic s kelelsl b,ypt.rCI!Il.o3i. (DISH)
CUN/CAL HPI mly vr-nt without .ymptOml allIIn incide-ntal finding on radiorr.phic. .... I· uotiou for mher ",""or.. MODY sipanO sympl.Oms h."", bee" .llributed 1.0 HFI indud· Inc: hypsrten~ion, !e-ilUres, h.adllChe, eranieJ ne~ deficit.r. de~nti •• irrillbility, depresllion, h,...lerill, r.r.igllbfllty and ~ tal dullnI!N. The inciden~noect;ve tiMue (mllJigt,ant tralllfonnation occura in 0( I ...). Doe. nOl IppI!ar to be mont.blt, M... t
monOllto(.ie: most COmmon polYO'ltotlc: 25% with this fOI"1l\ IUlVe > 50~ oflhe skeleton involved with i05I!oci · aUMl fractures and ske letal deformili~, 3. as part of McCune-Albright syndron,e (endonino dysfunet.ion, cA fe, au hlit spotll which wnd to occur on one side of the midline and tend to be lllorejag~ thM Ib.-e seen in neurofibrOUlatos i& (_page 5(2). fibrous dyspluia. and pre«ICiou. pul>l"rty pri,"arily in fMn8l"l and ita variants C lin ical Clinieal Dl&nifulationa oithe fibrous dywsplu.ia frn) lesions include. 1. incide[}lal finding (Le. asymptomatic) 2. 10000I pain 3. local_elling (1"3tl!'ly marked diltonion r 3 forms orthe FD 1....;01\.1: I. cystic (the lesions are not actually cysts in thlt strict H Ils..): widoming of the di pille uGu~Uywith thinning ()ftheout.cr table and linl", in,'Olvl!men: of the inner table. Typically occurs high I,n ~lvaria 2. sclerOtic: usually involves skull but (especilllly Bphenoid boM) and facial bones 3. mixed: appearanCf! i8 ~imihlr t8 cystic type with pateha of in:rellsed density within the lucent les, METASTASES OF PRIMARY e N S TUMORS
Spread via CSF
pnthwaya T umo ... th at may spread viII CS F pathway. inc lude the following (whe n thl!li~ tu· mora 5p"'ad to th e spinal rord. they at"!! o'wn call~d "drop milts"), I. high grude gliomaa (l().. 25'11i1 (ue ~ -113 ) 2. primitive neuroectodermal tumor~ (PNET). espe,i a)ly .IlIedullohla~torna (sa paSt 472) 3. epeady/llQ/TlII \II~)(_ ~4 70)
" 5.
pinull'9gion t umo ...
A. germ ~ell lumon. {sa paGe 476) B. pineoeyt.oJ:la Md pineoblastoma (~e ptlflt- 4771 ranly, t\. oligodend rogliomas (_ 1%) (Be B. hemansiob lasUlmas (let p Radi"""nsitive. Alth"", .. SCLC C(l ",pri~8 on ly - ~. of primary lung ~lInCeTII, it ill mol"tl lika(y to produce cerebral meta than ot"er bronchogenic cell typu {brain metl are found in 8O'I>ofplltienta who survive 2 YI1l afl.er dillgn.;s ofSr:l.C),,'. The""for. prophyloctk brain XRT;" W!i!d even in patlentll without hrain metl. Treatment of primary: uauaUy not re/!ect.OO; treated with XRT t ehemothllrapy.
-
..,.,., kidney (reoal)
.-,' '",n
""" M"
""""",mor>. but_:)I;Ur
Non·smal l c~lIluog can cer (NSCLC) Indudes: large cell, aqu&lDOua cell, adenoxarcinnma, bronct>oalveolnr. R 1'1""""""'1")'1
3. bone scan 4. brain: CT Or MRI When mataatlllW: lung c;ancer is the aUBpected aaumi ofa newl, diegnosed brain lesion, the lung lesion should be biopSie MELANOMA Onte cerebral r:nel.!l of melanoma Gredetec~, lIleCUl1lr. 01 flIucolal. RENAL .cELL. CARCIND:'M U5Ua Uy auocia\e~ with aprwad to lungs, lymph M>dtNI, Ii-e' , bone, ad/'llnrtUi', and CI'lnu.latenl kKllley bdnre in~ad inl the CNS (thus, thi. tumn~ rarely presenLl a8 i",l al.td ef-rebrJ,1 m.WbM,}.l.aok fo, hem.turia, abdomlnBI poin, andlor lIbdnmiDal m'8JI On palpation orC'!'. CU NICAL. PRESENTATION
Nwith moIIl bf1lin tumon, lignnnd &)'IIIptorns a/'ll \>.iUIIJly~ln .. ly pl"Dl:ll'uive"","' pDrlld I1' hewQrTharie infarcts ) wlUch lend 1 IIr CNIN with nelltive Ca hiltory. nfIIU" , CXR.nd ~ptive IvP l~u mftbly. this would a III apply it a cheatlabdomin.llpelvicCl' wu nagative}; 7'1: or..,lltafJI Je. liona are lUtts. 5'7'1' .... primary bra in tumo .... and 6'JI. &1'1 nonneoplastic. Yi,ld orrurthe. wo, kvp to find pnm.". ;' 1_ ( r~m_ndl.tioa: follow teria l CX&) with hie tory "rt'""!.cd CII: 93" nfllollliUU"f I.inns aN! f11 1U
MR'
M~ Mn,iti"" thu! CT, .p«iaUy in th e p-(OSIIII Uncludin8 bn.ln , (11m). DetecU lDultiple metl in ~ 20'lIo o( . ill(le mell 011 Multiple p ....je~"'n~ may alao .... iSl in
l u"';Gtll plan",nl.
c-r-.
L UMBAR PUtfCT1JRE
May be ind kt.ted OIl", m ... ltlion h&IF befn ruled 11\1 1.. M.y be IlIDSt uileful in d ill8nnaln, tI:Ircinoma\tlul It,eniogiti l (ae COrcinomof_ me"i'Y:it~, pap49l).
NEUROSURGER Y
17. Tumor
METASTATIC WORKUP
Prior 10 obla inlng tillS " '" (rn m br .. in lelloo : When mellstatlc dillea"" i~ 11I5pected based 011 imlginll: or on 5ucgiell tlli!lut, II ""arch fur I primary ~Ite ft nd a&'le&!lment for other Inion. rna)' lie oollsidered and should indud ..: 1. CXR: to rule oullung primary ar olhar ~t& ta lung 2. C'T of the .-hilS( (mortlllen MEDICAL MANAGEMENT
Initial t"""\JD~nt: I. InticonvulsaulII: e.g, phenywin , G.-n .. ra Ill' not. n.....d.-..l p - 2·4 mg PO TID '''' lot,&, as ~ymptom8do not WOT'l,len H1 M!.IIgonjgta ie.g. nnitidine 1&0 mg PO mpl~tely resolve ..•. Thje doe-s nOlo result in load oontrol for tM osens1tlve" :;;','Ho;"C-;';O~"--' mlljority of the ... palier, t./Illnd they fTequeolly IU Natural hiMWry
By t11e time thllt neurologic findings devel. op, median sun;.,,,] """"'luntl'elllM ""bent.!; i. ~ 1 m'mlh....
ou. .. bIIMd t&'l-IJ' on p..-.(." ... d" ... and 1/>0 ......... w .... ~ro ... Pf'OI>oWyt • .,...1.Iu>a
'. 1> and appelll' 10 boo 5imilar t papilledema and sYlllpwm",;c ICP elevation" 20 em. ill the absente of ;ntratTAnial mass Or inftet ion more rommon ill obese fe mn J,,~ of childhnring age thlUl general popullli ion imaging 6tudiell (CTor routin e MRJ)ofbrain are rwmnnl (aUowtd e¥ception: alitlike v~otriclu ). csr is norma! .... oept for increased pr..,sure usuallY sel f_limited, rvt.-urrente is commOll , chron ic , n IIOme t'Dtient.. p prevetltabk cl\UlIe of (ofl.eu pennRnent) blindness fro rn OptiC aLrophy risk of blindness iI; ROC rt'liably corr~ltted to duration Ilf symptoms, p8 pil19d~mll, HlA , Snellen vi9 ·~ DI8cui~y, or number ofrec:urnmee!l for PRti"r>ts fai li'll m~ital mllnagement (weight Ices, O'Qmox .. .) • optic nel"'ltllhe8th felle.tration is best for v;lual loss without HlA • csr 5hunting is betkr fo r lilA than ONSF
Idiopi\thic lntrac'lInial hypeortenaion (IIH), AKA pse udo tullIor cer"i!bri , AKA be· n ign intracra nlnJ hypert.enaill n , (plllll numerous othllr obl!olete U>m>s....) Is a heterogeneoua group oFoondilioo. chancterized by iocrOlll",d intracranial prell8ure with no evidence of intracranial mllSII, hydl"1!Cl.lphalua, infection (e.g. chronic runpl menlngiti..,), or bypenens;'·e enceph aloplltby Some. but not an ,.8uthonl txclude JlIItients with intm. eranill hypertension io the presence Ilfdu,.,.1 ~inus thromboolil. lIH i. thu.o a diagnosis of mh!.illl!n. TheT~ it "juvenile pod An adult form .
EPIDEMIOLOGY
female:male mtlo reported rlnges rrOOl 2:1 to 8:1 (;nJuvenile form) obeaitY ;1 n!JlOrtoBd in 11·90'lI0 of case~. lind ill not 8.! pravalenl in men'" incidenca amons: obese women ofch!ldbellring yeart-''': 19-2Ul00.000. (whereaJI incidence in geoen l popu lation'"": 1-21100,000) 4. pe.u. ,,,,,iden.,, in 3rd deeacl" (. .. ng~: I-M l'6$nl t 31~ .. f "'."8 ...... in child."n , 90% of these a ..... age 5-15 yePni. Very rare in infancy S. fnlquently sel f l\mited (recurrence rllte : 9-43'iL) 6 . " Vere visulIl deficits develop in 4_ 12"'-, ullrelated to du ration oraymptonul, dogree ofpapilll!d.. ma. head .. che. v;Qual obscuration. alld number of~n",,"·". Perimetry il thE beat ruNnS to detect and follc!w vi6uallQ!;' I. 2. 3.
PATHOGENESIS
Not fully undenltood . ln 20 em litO (P""'Sun!fl > sy'ln an oche-rwise awake and alen patient ~o are not uncommon!". Some recOlO ' increaSild CSf pn!5SUI"t wiCtlou! d1emka1 mend that the press ure ahould be> 25 to CI cyIOIoglee!~ exclude rmnnal ..... r'iOrnIiIIlO·sma\I 'IOOIricles and flO intraera2. CSFo:ompoEition: nonlla] gl<W6!ieand cell count. Pro~jn i5 nom,al, or-in ~ two thirds ofCIIsl!8 it i8low « 2(1 m~) 3. .ymptoOUl &. signa are. thOSf! oreZevated ICP alone, i.e. papilledema & HlA. with no focal findings(whh the. allowed exception of abduce"" nerve palay whiw may be due to im:reased ICP, Ii« pa~ 586) 4. nonnal radiologic ~tudil!8 oCthe brain (CTor MRI) with tho aUowed uc~ptiOll!lo(! A. tha oc:casionttlly Seen aut ventridu (the inddenee may be no higher in UH than in age·mAtched controls"') Or empt)' sella B. infsntile fonn may haveKt'neroUli ventricles and luge fluid -llplleea over brain C. ;ntra-orbiwllbnonnaJitiu mll)' "" seen: ~N below
"'-
CT Usually adequllteto RIO intracranial maU8l'I1 posaible I I I diif.... UL 1.I.... I"d.,I11., "" .-qui"!
...
11. T\Jmor
NEUROSURGERY
CLINICAL
PRESENTIH.,O N··..· ~ lIymploml A. cJauic ("",jor) Iymptoros 1. WA ': Ihe mOlilt D;lmltMHllymptQm ); 94·99'Jlo. Typleally refl'!HM:ular and pUl8lllile MlY' with ~ye movemenL Se... rity doe.1 no~I'(IrT8law with dearee fCS F Il ..... ure alavet in . OccIl.iOUDHy WOtaf! In A.M.
nlultl. 3~ (loClull vornillng '. le a eomn>(lnj ",1",,11081' (1M Vi.ruaJ /Qu III 1111 btlow t: I . ItBOI'mt mU1l1o~cu rlltion (TVQ) b. IXnnllnwt alTer.lIt vil ual pll lhwll,)' ''liu/')' 4. dlP'op\8lmoN eolI'lmon in adwt. \l.!ulIlly due tQ VI ntrvt pal5yl: 3O'Jto B. mino' lymptoms'" I . neck 'ti"neN , 30·~ 2. tinniILIr', ",p 10 6O'fr, UI"ally ptJ~ Iynchrooo\l.! . Described'i I"IlI h. ing nolae. ~t.y be "nilale,al (in th_. mly be red UC"ed by iplllmtenJ 2.
3.
j"'rulftr vein ...,rnpreaion + ipli late ra l h e.d ",union) 3_ .tau., 4-ll'Jlo
A. pIIpilled.,ma, I. preeent io almoal ~ I~ 2. id;"pathic inlrllCl"aniQ hypertelllliQn ... ithou t papill edema CITH. WOP)"': a ""rian t of IIH . Vilul ll..,. tend s ~ tQ OttL1r 3. usually bilateral. ocrasionally unilal.eraJ .... 4. irnIy be mild fsubt.le nerve fiber elevatio n) B abduce-na.....,..1 (Cr. N. Vn paily: ~(. raise lac:lining lign . •e ~586J. 'nIfl e!JOtmpia Mlnge5 from < 5 prism diopiHll dyx:mjU(llte 8 n,le in primal')' pH to > 50"""' C. mlln,es in. visual """'ity: I"flativelr inaeMitjv~ IIn_ment of vil",,,1 fum:-
"""
D. u1l1rred blirld .p;>L (66~) and roncenlrie ~MtriOrmnliti~. oft.he dural.joust's , including thronlbost., stenoail"·. oba tnlction , Or eleva ted preuu l't! have been dBmOllll~... U!d. While thl'lle
_..
m~
''''1'0'''' !O.\ef; ~'"""" (in hip dOltiI: 1(,.1 parealhe· ala", naUI('I, IDetabolic aCIdlY;" litered taite, riOnll etlleuU, drowllneel. Rlre: St.ven.Johnson I,)'"Odroml, \o~ie I!pidenntll oecrolyail, al!'"3ou!acyt05i l . • Conlrain.d.ic.ut.ed with Il1eri:J' \0 lul r. or I hiltol)' orl"@nl!calculi b. IIM!t"'zolamidl! INeptnane®): betW IOleratftl buI1IIIS effecc· NEUROSURGERY
17. 'I'UUWlT
li,,~. 1b: 50- 100 III8 PO BID-TID. SlUE EITEC'I'S: similar to 800\.8zolBJl1 ide topj r81l1stto (Topamru0): 8J,ti~onvulu,nt with SO!condary inh ibi _ \.ion ~fCA. R:c 200 mit PO BID. Smli: IIFI"U:T$: Similar t.o .,et a· zolamide, but CIIn be used in ul(a all~l!ic pat.ienlA 2. IJ.u:g,~ (Laa iX®) 8. ~ \.8r1 : 160 mg per day in .dull.l!, adj~t pe.r .ymptolIUr and eye
c,
e:own (wa to CS F prusure) b.
if ineffem"tive; IWllbopleural ahunt 2. o!.her shu nur may be used, especially ", hen aradUloidiLilr precludes use or lumbar 8ubarachnllid ip!l"", e.g.: • VP Mune often dimcu lt slnoo the ventric)u are frequently BII)$I! or Illit.-hk .... ' . Ste",.,tactic teonaJ pf'OCWed a l leUl two yean , ...ith nlpell t imaging. ""g. MR.[) to RIO IlCe lJlt tumor
Opt ic n e rve Rbeatb fenestrat ion (ONSF) ....... C 300 111m Hp I1H with ";'u':' I..1 AND HlA, for pat ;enlll with l u.rJirallndklltlalll, 1I1tlu!. IU.· gical proc:edu~ i8 approptieta. Sbuntin, mIIy I'1!lieve both problems .imult • • n/lOluly. ONSY may be men reliable to relieve the viluel probllllllS (!he (ajlur • ...I.e may be towe.!han the.hunt m.lruncUon ... ~) but il not III good far tbe HIli. IIHWOP; .ym ptoma'ie tnlliment ron tL'A.diurtl.,,:. [lH in thikfA,n and adolet«nlll; A. may be Hen with withdra",al of Il.eroidl uled fct a~ thmll B. ~h f~ aod enrn:et;"'n ofunde.lyina etiology (offlndlnl drup lilted lhove, hyJle"lIkemia. clnn!r... ) Co IIOI!l.uolamidt h .. been used with lueoe.. IIH in prqnency. A. women who fi ..1. preHnl ",jlh IIH duri", pregn~ncy: resolution of IIH rol. lowin&: dlli ...1)' il cornman women who bec»me prfl1l1lanLducinltberapy. I. III trlm4!Sle.: obH .... alloo , Jin,jt.l l inn ofwlilhllain, Rrial LP.. • Awazoiamide lIhou ld be aYoilied beuu .... Orlt'ra~n.icity 2. 2nd 10 3 n1 uimnlt'r: acetalOlllmida has been used .... fely. but involvement orhieb·rilll obilt'uician IpKiaiitt it ad..i$ed pleudopapilledl!ma (associtlled ",ith df\llln. etc., in lloe ab5enee of IIH): no inllrventJo~. Re&lI'l,Iran~ and tL'A maolliemenl are ernplo~
a
7.
17.9.
Empty sella syndrome
Empty aeUa "yndl'l:>me reSS) Clln be ' primary" or "aecood.."... l'rilll lU')' empty .eLla eyncl.ro me Hamiation of l h arachnnidlll~mbrane into the sail. turticll ... which can act .. a m.... , probably as a f1'Sullofrt .... t.ed CSF pul&llUon. Tt,.. "..lIa QUI booc:ooneenlllrwed 1_ Sdla lurei.M, page IS8 for normal dimension.,and the pituitaI)' glaDC! may beroroerol'll ' prelsed againl l the tI_. Most of these patl.ent.l are obese WOO"lIIn. 'The i'l"'equency g(inr.neeUar ar.~hnoid her. oillic," is higher in patient8 with pituitary tumo ... and in thCllf! with increased int.-atta· nla.l prtl.'illure for any relillOll (;Dduding idiopathie intunanllll hypertemion, _ page 493) than in the re-nnlll popuJ.tioo, T~se palienta usually prelJenl with symptoms thBt do mollUaest RD intnNl1ar abnonnaHty including; hea.da~he (the moot COmmon symptOm), din..i,-. aeizulU ... 0.:C'lsicmaUy patien", IDay develop CSt' rhinorrhea, deteriortlliOll or"i.ion (&eII;t.)' 0' field delitil miul tiQg from lI.inking or optictbium due 10 hemiation uuo the MIla). Ill" amen· arrhu·galactorrhea .ync!rome. Clinically evident endocnne di6lllrbance. a re rare with prima,.., ESS, up 1030';\ abnonnlll pilliitary function le51a. moatrommonl,y ,"1ICed (JlI'Wt.b hormone secretion fallowing stim ulation . Mild elention ofp-mlactin (PRL) and rtcIuction of AD H may ot"tU.t. probilbly ""om compression ofth, .tau... 'l'but pad,nlll _h_ a JIOmIal PRL MM .... ith TRH !It.imuletioortly thereafter.
nowevel"
h,,,,
Secondary erupty se Ua syn d r ome'" Oc:cun followinllaucassful Ira.nllllphenoidal t1ItnOval
orl PitUItaI)' I ... mor. Often
lI_nta with vi . ... ftl dellri()TaUon due 10 herniation ofthe optic cl!lum into Ure I Urgical · Iy evatu.l.td IIUa.
NElJROSURGERY
17. Tumor
'"
17.10.
Tumor markers
TUMOR MARI(ERS USED HISTOLOGICA LLY IN NEUROSURGERY
GLIAL FIBRIUARY ACIDiC PROTEIN (GFAP )
Polypeptide. MW .. 49,000 Daltone , Allhough the
pr~lIce. o rGi"AP
u8ually indl -
caWl .ll8trogtial origin,;1 mlYO«l8;00ally beseen inoligodenriroglio!lllls, eptlnd)'llloma$, and chom>,d plCX\Lf papHlon".. s'.......". CFAP is op]y rarely /bund Dr,at.o;ide Ihe e NS (in nnnmyeHnated &bwllDn ~lls, epithelium ohhe lerut, bepatic Kupffe: cell •..• ). Thul, the p~8enceofCfAP in" !UffiQr (ound in theCNS;8 "",,,ally takl.'n 8BIQGd evidcnCl! for glial origin of the IUI1\O •. GPAl' .18(1 occu", in nonnal brain parenchyma. 5-100 PROTEIN
A low moierula. weigM (21,000 Oalt1ln.1 calcium,bindinB protem. U~ed on tiMue microeeopy fot pathology. May participate in regulation ofmicrotubule.18embly. ln eNS \1Iml>l'II. the di.ttribu tion ;6 similar to GFAP, but it is POI as ~pecilic u GJo'AP ( may be found in other cell types such S$ .tell.ne cellg ofl.he BdI!ne>hyp(lphy8i~. dwndrocyt.ea}'''· I....J. moIBlle>III38.ln the peripheral ne.rve>u~ ijystem. it i8 localized in Schwann Cffca rc'ine>ma. T"nerefore. may help distingu ilih metnu.tic tumol'll (!.hat 6.t ain positive) lrom primary CNS tumors.
MIB-/ (AKA MONOCLONAL .\fOUSE ANTI-HUMAN K ,.67 ANTIBODY) ImmWlohiltt.ea high mitotic Be· t ivity which e>/\an cOlTelBt.e& with degree ofms\ignllJlC)'. MOIIt a/\an used ill lymphomn ,.nd breaRtcance •• AJ.ge> used in Blltrl:yt.e>ma$whe.ea MlB_l ~ 7.~ 8Uggelltll an Inopl18. lic tumor. wbile MIS-l < filii> f8ve>fS 0 low' gTade tumar. Nr:URO€NDOCRIN£ STAINS Indudes' I . chromagTB nin: ata ins (e>. neural CTellt derivativ"", yi,. pituitE.ry ad_e namll5, pangonglie>m as, neuroendo!:rine turtlOta 2. .ynaptCIphyain: 5tainl n@uronalsndpineal tumOnl, PNET &. ml!dulloblll5tC1mlll 3. neuron specific .,nolase (NS"); very sensitive hut not lpecifi~ fe>r neuroI!ndClCrine
Mrwtaaes that are poa'ti" e for nlluroendocrine ,lain! ,ndude: .mllll reU I'IIrdnOmll of the lun8 . mlllignMt pheochrome>ma, Merkel cell tumor. MeLrutati< small cell tu· roora 10 the brain staining pooitiye for ul!uroendoc:rine 8ta'l"IS 8'@ allD05t ~11 due to 1"'ng_ STAINING PATTERNS' " An individual tume>r may lack II marker thal'e typiuJly r@j)ri!.entQtivee>rltstype. Therefore. 8 JIINIitivll l tain ill me>1ll .il'lilkllnt than I n egative ' !.aln. General Jtaining plIttenlS arlu ilown In Toble !O·63.
T UMOR MARKER S USED CUNI CALLY
HUMAN CHORIONIC GONADOTROPIN (HeG) Ai~proU!in, MW. 45 ,000. s.e.,reted by placental trophobI4lltic@piti«!lium . BeUl thalo ( ·hCG) is ne>nnally presen~ ouly in thl! fet\l& e>r il) gravid or p;IlIt.partwn females. othl!rwlse it indicate" di9llB!lI! . ClallSic.a!ly o8ioctat.ed with che>riOCll.rt.inOIllB (uterine Or t.e&ticu la r). IIJIO fe>und in patients with embryonal cell tumOT~. terBtocnr~il\Clma aftl!l t is. lind otb ..re. CSF fi_hCG is 0.5·2% oflll!rwn B-hCG in non-CNS lumOTll. Hi@herleY"'_aredis8_
17. 'T'umo;>r
NEUROSURGERY
GI)8\ic of tel'!!b ral meta from uterine Or teBtiwlar t:horiOCllrciDowa, or primal)' chorioea rdnoma or embryonal .~. GF~P
cy\OIo;ef8~ • '.'
QIl.
ALPHA-FETOPROTEIN
A1pha-fetoprotein (Af'P) ift a nDrUl1i1 fet.al giyeoprotein (MW '" 70,(00) initially produced hy the yolk ,ae, and later by the fetal ijver. It is found in the felal eireulotion throughout ~t.aLion, lind drupe rapidly durin( the fila! few "'eeU of life, readting normaiadulL levela by agt! I yr, It 18 det.et'table o;mly in trace amounts ill normaladulL IDlIII!II Dr nonpregnant Cemu • • lt ill! pAHnt in amniotic fluid in normal pregn&llcies, and il detectable in mllterulll .... rum starting aL ~ 12_14 w .... ks gelItatiDn, ;""rell.ing 8tm1dily I.hroughcmt PregTllUlCY UJ:Itii ~ 32 weelu; ..... Abnorm ally elev"ted serum AFP may occur in ClI of ovary, etomach, lu ng. 0010.11. pancreas, as ..ell III b drrh()llia or hepatitiJ and in the ml\Jortty IIf gravid women can)I' ins a faLu8 with lID open neu.ral tu be deroc~ \_ P,n.a.lol dtl«.tio" offltlJl.TfJl tu.6edtfecI8. pa«e 113). Se rum AF? > 500 nglrul UlIually means primM)' bepatic tu.mor. CSF-AfP ia el8YlIted in lome pineal region germ-cell hlmors (a« p~ 1 6·2li~ ofpaLienta with te$tieulartwnofll geteerebral meta and elevated CSF AFP level!i are reported in !lOme,
n n.
CARCINOEMBRYQNIC ANTIGEN (C£A)
A glycoprotein, MW . 200.000. Normally present in fet.1 endodermal ~l~. Origi nally described with toiDrectal adeno-Ca. now known to ba elevated ill mallY millignant and nonmallsnant condition. (;"'c1uding cholAO-9O% Dftennin41 patillnts having elevation). CSF CEA: level~ > 1 nglml are reported with leptom ..nlngea1l1prelld of IUDg Ca (8~). breut Ca (60-67'11). malignanL melanoma ( 25·33~), and blodder CIl. May b& norma! ann in CEI. IIIIICl'Olting cerebral meta if the,. don't IXIllllllurucnte with the auoo rllch· noid apau. Only carcimunat.o\l8 meningitis from lung or brelllt Ca coll6iaten tlyelevDtel CSF CEA in the mlljori ty ofpllt;enlB. 5,100 PROTEIN Serum S-IOO protein lavela rise after bud trauma, and pouibly af\(trother inaultli to the brain. Loewe1. m.y alao be elev . ted in CreutJ: feldWalrob ~ (_ pagf 230).
NEUROSURGERY
17. Tumor
..,
17.11 .
Neurocutaneous disorders
Forme rly C811i!d p h a k o m awaell, Nau rocul>on"l)us di!!(lrof!nl (NCO ) a rl! 8 gmup of conditions. each .... ith unique neurologic find ings and benign cut.aneolUl tl!lljon~ (both .kin and the CNS deriv" embrynlogieal1y from ect:lOle to tile a((euUon ofa neurosurgeon: neurofibrom81.o~is's«~10IlJ
Neumfibroma, !01Iis (N"FT) i~ the Table 17-64 1 and 2 IIlOIIt COm mOn or the N CDlI. Thereate a! m/lIly III 6 diltinct type8,\he tWQ most Cf l ymptarna. Kewt;(en (which ;1\l1;bitll his~mine ~leal!e from mut-«Us) Inay r. lieve some I'l'\IrituS and local tandemell1. 1U 2.~ mgld ror 3~O monlhll .... N e UROFI BROMATOSIS 1 (NFT'1 AKA voto R £CKLINOHAVS£N'S DlS£ ... SE""')
CLINICAL FEATURES More common th/lIl NF'f·2. nl" resel1ting" 9O'i\ of C3li(:S arneurofibromlltOlii.
malPloltic eriteri,,"": two Or mo.t orlhe following: 1. '" 6 c af EPIDEMIOLOGY [neldence: _ I in 178,OO(} penoon·yean. Point prBV1I.lence: 10.6 per 100,000 I'll"""'" {both figun'll from RociIester, MN-J. AulmomBI dominant inhe ritBnce, h"",ever 9ponteneoua mutetion i. oommon. 1"hB fIIlIpolIslble. ~ene mllY be on clu- DIAGNOSIS
III the infant, the ea,li8llt finding ill of "ash leaf" macules (hyporneIBnolic, leaf shaped) that are best 8CeD witb a Wood·, lamp. 3 or wore ofth~ mHule. > 1 em in length together with the presence ofin!ftntil~ myocloDus ill dillg"nOlltitofTS. In older ehildren or edullll, the m)'oclon'" is of\.e.n nlplaced b)' generalized tonic· donie or pIIttial complu aeizure •. Facial adeuo01IlS Bre not prellent Bl- birth. but appear in> 90"10 by "IlII 4 ynI (thea" au not re.llly adenomM oft be sebaceow glendJ, but are amaH hamart.omu of cuUl neoll.l ne~e elemenUl that are yellowiab.brown lind glilltening lind tend to arise in II butterllJllD8lar dis tribution u."ally sparing the upper lip). Retinal hamaM.omllJl OCCI:J in _ 50~ (central calcified hamartoma n.... r ~b.e disco, a more aub!.le periphenl Dal &almoa-colored lesion). A di~liDcti ... e depigmented iris Im ion lDJIy a.II!o oecur •
Plain skull :.:· ... Y8 May .ho.... calcified cerelirlll nodules.
CTacanln t.rDoerebMlII:lllci.lisW!w.i are ~he w()/;:t common (97"" oreasea) .nd characteristic finding. Pl"imarily loeated lubependymaU)' along the lateral WQIl. of the lateral ventri. 17. 'rumor
NEUROSURGERY
de. or naa r the fonmisuo or Monro. Low den~lly le.lona that do not eMllne. are _g ;0 61~ . Probably ""'p_nt Mterot(lpl~
tl .. ua ur d. ffHiye myeJinatltm. MOAt CClI:IIMM In oa:ipil.allobe. lIydrooephah•• (lt CP) may oecur even without ohBlnIction. tn the abeen« oftu.mor. He p;1 ~u.n1 mil d Moden'" He p u'\lally occur. only in the pruenee of tumor. Subeptoll(l,ylU.l nodu~. e ft lI'lIan)' c.lci/ied, and protrude into the veolml. ("cand ia CIo' tte rlng" the appelU'8/lC't1 0/\ pncurooelltepbalognphy ) Pt.... ",nLric...:J.r l UIDO . . (moIIt!y giant ce11IllltrvcytOm..,~, bc/(>W) are _nuelly It.. W eM.neing \""ion in 1'5.
Gi ... , eeU •• t rocyt.aml: a tnruformation Inion. A1mQ8t a!wHyalCK8!.ed lit !.he fO. IlImeD of Monro. O!r thM in vasion"'_ TYPES OF SPINAL TUMORS May be clll$5ifled in 3 groups. Although met8fit.a8t!8 may be round In nch otItegory.
th ey are uaually utradural , F..,quonc:~ qu,,~ below an! from .. genua l h08pital, U'-
lNIdurnJ leaions ate I...".. com lnOn ill neurO$urglca l dini.,. becal.lllJ' or rt:'Jative e xclusion of e.rtradurll.llymphoffia" rne1.allt.atic Ca . etc.: I. enrad ....a1 (ED) (55%): .. rUe oUll.ide cord in vertebra) bodiel!' or epidural t i4l;Ue!I
2. !rllradural edT.medullary (ID-EM) (.~); arise in leptotn DIFFEAEtmAL DIAGNOSIS OF
{e.g. I.
t issues
a.
....
Iympooma, mIl6Lc"ae81l!1'~en[ sp .... ad or.ystemkdi.""se
(lH!C 1 rr prior to diagnosis in 82~ of cases-. M yxopapill ary ependymoma Papillary, with micfO, di_turban"". di llSOClaW:l sensory losl: decreased pllin and tem$leratu"" preserved light toueh (liS In BrowJl·~uaNillyndrume._pap 1)6). Thero,l.i~ disllgrflement whether this is ~ommon'" Or uncommcmw in IMSCT. :0 non· rad icular dys· esthesiu (early), with upward e~WJ\!lion'" • P8 r .... th~illS: eir.ber radiclllar o. "medullary" diuribuLi on -'I . aphinctcellaugge&ts ED SCT. L.umbar punt1,urC; E11!Yat.e MANAGEMENT Su.rgery ,howd be performed l1.!1800n 8.1 posaible after diagnosis .ine.. ~"'lPcal1'"""",,,, 7411-(of 42) ali_ ... o.U 7 dun>clda. """ (ora) );1"' ...... ~.td I. t_ .... "2 . ..g .. borQh d.i~ c..ot .,r~ by Iouti"" t.. _tiGool a .. Ionriludi...J di1l>tI>rion of Be (""'''''" ........ 1..,,,,, . _ "' .... Iteq... atJy di • ..-..! '" 101 ....,.H. p~CT Iladf)
."
11. Tumor
NEUROSURGERY
blood v~l!4O!cl" and II.dhQions"'·), an attempt at total exci$ton it. an option· ... For high cnode atlUOqt.omu or for low-grade w;trocytOluu without'" plane ofa~paration, bio,,",y "lOll. or biop:ay plus limited exeisiotl is .... <X\mmeudtd.... For high'gTadeI1l8iolls , poot..,p RTX ('" chemothuapyl ill recommended''''. RTX is not supported following radical ..- Benigl1 I)!It.eoblastic Ieeion.• of bone mlly be divided inl.6 two types: osteoid ootolOo ma (00) and benign OlleOblastoma (BOB) (1UN1 'Thble 17·
T8ble 17-65
end
OS).
ChR ract.erislicall) caUSIl nigh t pain and PIIia l het Ui re-
lieved by aspirin (.ee.Cli/lical below). Thlltwn are indistin. guj8hable hi.tologkally, II.Od muat bediffcrenti"ted baaed. on 5ize and behavior. OUeohlul..Oma ill. r8r!!, berugn. locally """u~t tu· mor wi!.h a predilectioo ror epine.that may ra .... ly under_ go sarcometou. thangl«to 011.t_rcom a:P'. More Va.'M:uiu thanoa-. Differenlial diagn08is :
(for lesions with similllT aymptoOl& and increaaed up'.akeon rlldiolluciide bone ScaD) 1. osteoid .... teom,,; more pronounced BclI'$(!, often n)llCOllti«uoul. 'l1le lumbar a nd lo ..... r thorltClc spine anr tbt' m".1 eoo:u.mon I_Uon.. QelYicat te,iona IIrb ra",. lawns in"olveonJ,y lh. vertebr.l bodieJ in _ 26~. posterior epirtlll an:b in . 25'\\0. and both ..... In - 1>O'lt. Oc:o;.qiooll' _ or pw-ely eltlnodu ... lluioo, hive ~n dw;cr;ber! .... lotramedullru)" I"u.n • • re .... en 1.- fOmmon'" MII1ilnen~degmar.tinn h u ~ been reported. Blood "ea." rep"DIl oormal marrow, producing hypertrophie k lerot.ie bony Il1Ibecu lll;Onl orierrted in II IWtra L_u. riaL d.irad-ion in 0'" rrfLwo fnl"lM: cayemool or arpiUlryldi lflm!nce In .ubtypo! ClITia. no propnlllie lignUlcs nc.t). PRESENTATION L incidont.aJ: t:nOJt. VH ...,.."ym~tk, these ,.q",ire nn f(tllow'up ""' IH/OUI) 2. eymptomll lc: there may bo!., homronllintluenee (unPfO'l'm ) Ih, t may ar uM! l ymptOIl\l" to increue with pNgl"llnt)' (could .lfO be du~ 10 incre-sed blood vol· ume IndioI' p.-ur.\"" or to ""ry with th'in: _iorralb' VH 1IlII), pruwt wLt h pIIin IocIIIlized to 1!w11evel orin·
".rtOI.OI
511
17 Tumor
NEUROSURGERY
volvementwith no rad iculopath, Pain il m01'e.Onen dUIlLOolhcr pathology (herniated disc. spinal 9teDOIIi$ . •• ) father than th e VH
fl . progT'elJ!live neurologicdel'icit: thiso.:clll'II rarely, end usually takealhe IQrm ofthor,clc mytllopathy. Do!ficit may be CIIUiled by the. rollo .... ,,,g mecbaniaml 1 . IlUbperiDlltea\ (epiduNll) growth oItumor into th .. spinal can.l uptUll\iOll ofthf, bone (corti 2.
S urgic al treatmellt rabl. 17-67 fllIC .ft.er lublotal ..-tion, " , ,,"lIy within :I)'T1 Pal;ent$ with ."bt.oc.aJ r....ec:tion . bould have R'I'X wlUth n! 11. Tumor
N EUROSURGERY
1. Ipillai or ek\lU ilwol"lmentwiti) t l.l mor(_ obo~) with cornprellllioo ofbrlin. cra· nill nerve.. or ~pinll cord or fOOUi 2. d~poIIitlon of M"yloid within the flnor ratill,cul\ln! ofth •. wriJlt - eupe.l tunnel IJ'lIdrorn~ (the medillll lIe,....e iIMlfdou IIOt conUl;n a.l&)'loid. lind th~",ron . . lpolldl well too lIurgieal dl,,'.ion of the trlllll"UH clf1lI.l lilfllment . "" fI(J~ 668) 3 . dim...e Pl'OliTeeai". MlUIOrUnotoT polynl\lfOpeUly: OCCUni in 3-6'11oof pIItient. with
MM A. about half an due too Illl,)'loidotli. (Nf pGII' 660) B. polyneuropathy tIIn alao oc~u r WlthO\J ~ lImyloidOll". 8p«ia lly in the, ...... O$t.fOIclarotic VftriDllt ofMM 4. multifoclllieukooncapha lopilUly h.. been ducribed in MM"" 5. hYP,"uloolllia; may produce II dramalie entepha lopathy .. ilh confusion, del tri· \110 Greoma. Neurologic: Iymptoma orhype~lIlcemJa ailOeiat.ed with MM are more COII)1Il0n than in hypflUlcellli' of other etioiOgiN 6. vuy rllt$: int rapar(',ndlymal m~l.Ii.ta~
Evo'\LtMTION The cUagno.llc criterie fot MM i, . hown in Tobie 17·68. TNtI that n>IY be
Tibia 17.. Cfitwlllcw dl-.gnoal. 01"'"
~uspeo;t.ed
I. 2.
3.
•. Ii.
6.
7.
MM includ e: 24 hout urine for kIIppa Be"... Jon .... protein A preseot in 7591> bloodwork: ...,rum protein electrophoreeill (SPEP) and immune electrophoTteLI (lEP) (lOIIkinx fOl' IgO kllppa banc\~ B. "keJeta] radiolllgicft\lrvey. Cluu:ac~ic .·rly fir,duUr. multiple, round. "pwlI:hed-Qllt" (ahaqlly d ... mamoted) lytic ieliolll in the bon. lypieaJly iovolved C- ollow). Os~ ........"",I:" I' z."''''OI'1 AKA OI~..tom. ( ofprilnl,.,. llit ..., lung. brea.n, GJ, prosta~. melanoma and Iymp/lomtl DO t .... almeO! proJonp lIu,..,iva!. but may red*- pain and neu.rologic del1rit. Spin .. l epidUJ'sJ mtltast.lses {S EM) aa:ur in up to l~ oruncu palien"".I!IOme timll'"". IlIld am the mOIl! comlDon .pinal tumo r. ~.IK oflJlali&naneiel present initially with ~ord compreslrion. Table 17·69 show. primary tumor typel thlt rive ri N to SE -'1. Forotheretiologies gr$pinlll coni oompft!$IIIOn, _ itlll .... marked ..... tlIa dagger(t) under ~lopIJllI:J gn P!l(e 902. Route. ofmetlilsUosi.: ......... rial, veno ... !via _pinel epidunL "eina CB.toKIo'. p ie "".... ») and perine ..... ou. {Iii"""t aprlllld). U.""II"OULeoflpraod ~ hema~t>(I ... di.&. emLMtion to vertebral body With ero&lon ~k th,rllUgn pedjelfs and IUbHquent uIoI!n· siol.l into tht epidural_pace.. May.1&o mitlaUy I'IHItuLuil O! 140p"!letlts in their hands to sUbil i~ it\. I'oaibly ...aI\oIllCI_~..tr lor Dart ~~ as II re.ullofw upuioUi .pinal canal In _ 8 . 'VIfIer'" ft:IucIu Gl GU. _ . ENT. e NS at lhiIIl«vfi. only . li · \S'i\! ofpatienta prMen! with neurolociC'aymptOml. IS" develop l pina l coni romprus;on .... and quadriplqi. from atlllntoaxi., ,,,,blwtuion ooeurmi in 6'V'". Anln10r 'pproaeb.. for stabilization " I th;" Icnlion ..... difficult. Palhologic fral>lurudue to OIIteobluuc Iypea oflumon le.g. prostaw, some br(,8511 may hul with rad io ation tnIo lmenl and immobilization. for others, rood paio "!ier.nd ,t.abiliuol ion may be adUeved wilh radi.tion ro~ by po.!!ln1o, fu.ioo'"
eu ltloOtit up (_ ' will bold theirb.,.dt
M
EVALUATING SPINAL METASTASES
coou, leslon a Irom eeuda equlna'''''on....
...1
The re if no dilTeri;ou In GI,It(Ome betweeo iMionsabove or below the conue: UlIlIIIP" nal cord. eonUi med"lIllrit, or ,auda equina mef.ll are conuden:d topther here .. epulu.
ral'pinal cord compruaion (ESCC) GRAOING FUNCTION There i. progu OIIuc tlgnific.nc:t in the pnl!lOI!nllllg Deurolop roncbtion Gnd lng KHlu . uch ,.. thai ot8.~ ."d MeKi"",,1r; (... Tablr J 1.111 ba~ bwn propoMd
NEUROSURGERY
17. '!'umor
'"
MAnAGEMENT Patientf .r~ Ul.o!gori~ U,UI oue orthe three foHowins gmVpll below hued on the rspidity and BeriOWlntlll oftl1e neurologic fiDdingIJ"f. Follow the reed for patienlll in Group I, wb~ more complete workup mn bedooe atben).
,n
GRQVp/ ign sympWm,,( )" new Or pnlgftSSive (hou", 1.0 ay.)oord:ompreuioo (e.g. ouinery ur~nc:y. 8IICeJlding ou",bnea). TheIle JlIItieotII h"ve a rugh ri~1r. orupid dete.
rloflltion IUId require immediatot .v.luati'"I.
III,
Manage llle"t I.
2.
dexymethuone (DMZ) (Oel:lldron®): 100 lVSTAT (redut'Q pain in 86'1., may produce tnw!lient neurologic impI"O'oIeruent) radiographic evaluatiOIl A.. plajn ,.mY' ofentil1! Ipine'. 67-85"" will be aboormal. P_ible findings: pedideer06ion (defect in ·owl'l! eYell" on LS ~pine AP view) of widening, petholoFcal oom f>re!ls;on frQctlU'e, vertebr1l1 body (VB) tiCalioping, VB Kll::: DllIA'oblutic change. (mayottUrw;th p.Qfitate Ca, Hodgki.n'. disease, ON.alIiollBUy .nth breast Cs, and l'tU'ely with mu ltiple my SO% bl(l neum eigmlayroptotnl. Can be av.lu.t.ed SJI olltp'Hient over several daya (modify tm.ed 011 ability ofpali.mt to trsyel, rali . bility , etc.).
Managemellt 1.
plll;n ' pille ~·rllJ' (AP, lat, oblique! A. iffocal bor.y patbologydemonslra!.ed: obtain MRI or myalogram (66% ofpa· l;en ... with i.olatve Ind bilLow the uteu l or tha lelioQ,
n.
17. TUDlor
NEUROSURGERY
The", i$ II lheol'11tlClll risk orradilltion indueed ed~ma ClIUlling or p~l"""tin, "e,,rologk dewno •• tion. This hI'S not been bum out by uperimenealituditsli with the ,,,,,"l small daily fractions utilized . Det.erioraboo ;9 olOT1IlikcJy to be due tu tumor progru!ion>O',
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ere via photoos) and particulate radiotion . The goal of XRT in trea ti..,g tumOri ill to
causeeell death OT to $top cell replication. Photonl impurt cri tic:al energy to ac:bi ..we thill re.wL by the pbotoelect.rie effect (at lower eoergie>!, < 0.05 MeV). by Compt.on _tteriDg (aL higher energies orO. I_IO MeV, e.g . in linear 1I<X>e.lerat.o", .lDd Grunrna Im!vul, Or hy pair-production (il Ltbe higheu energies)' . [0 the COlDpton effect, the miLial collision of tbe photon with lID atom creates 0 fTee elecLron which then ion ius other atoms and breaka cllemiea.l bonds. Theabsorption orradia\.inn by indirect ionization in Lha pref'ence nfwal.er produces fTee radical. (cont>linin g a n IU'pllir<eon rn
children 4. 5.
6. 7.
primary hfllOth.rroidilial (e:l pecia!ly rn children) Ul.IIy rnduu formation of"" ... tumor. tumor. moIItcowmonly identified as bavWc illC",alied 'ncide Ra! foll_ing ndiation IrBIItment are gliomu (lncludioll glloIlIallOma ",uJtirormt'), mmlngiomu". and nu"" she.nh lwDor:!". Skull base t ... • mon hay. been ....pon.ed followinll EBRT'" malignant tl"all!Ifurmalion : ,,-g . • bc SRS Ibt _ .... tK: nruromaa ""'~ fXJ«"' 431) IIJukoanoephlllop8t11y: profolllld deml"'linaUng/nlKl"Otizing re.ction 4·12 moe at· teroombined RXT lind ",elhoi.rtnte, " pKially rn child,,,o with acul.e iympbo-
blilltic leukemia (ALL) and adult. with primary eNS tllmorl E"VALU,IIncw
CT&MR1
Cannot reliably differentia te lOme cue. of radl '~icm necrosis from wmor (e.p«cially ut.roeytnma: RN ~aJlY r_mbh!ll gll(lblutowa mullirarm.,) even w;th QI)lI~ Orten tel! peri.-.ntric:ular luc:e-ncy and ventricular cWaUltion (diffieult 1.0 disllnCWl'b from IIydroeellhalul). Dynamic f"LASH ·MRJ . howalOme prowl... MR lpeetroKopy (_ pDIJ'Il 131)""AI ,-eli_bli In dl. tinplahlng ttlfDO. from RN when eithu pure tu.m., or p ..... R.N . .. p..... nt. but w.. lea ddlflitiye with mi.l:~d tu· morlnec:roais" Nuclear brain &e1lUl Some re-poru of a UC«lll wllh thailium.Wl &nd techDl!!tiwn.99m bra in
KILN..
Comp uterized rtl d ionuclide atudie. fIrr (poIitron tini.BsiM I.omO(rl pnyl.can. '-11M poIll..on emi~ti.nIIIOtope. have
NEUROSURGERY
sho .... half1;v." PET sea nn ;n8 requi .... u nellrny cycLotron to generar... lila radiophannllCfl ut k a !' at , renexpenlt, Utl lil ing t'-F!·n uorodeoK),lI'lueote. (YOG), reglOnlll gh.cote l1Ieu.holiam It imaged lind it g.:naraliy ilJ~reall8d .... ith rlfl'Ul'Rot tu mor, and i8 d~used with RN . S(lftl lici t)' furd'l lirl6Uis bing RN (rum tumor recu rrenc,", is,. 9()'J" but sensitivity Ully be 1.00 low to make; t .eIJlIllle". Amino ac,d traeer. such ., l"Cjmethionifle and 1"F]tyroalne lIre taken up by man "rain UUDO,.II, Npecially goomas, lind rnl)' . 110 be UMd to help differentiate tumorl'rom nec:rOlli • . Accuracy may belncnased by fU$ing PET scln willi MRI" . S1!£C1 hingle f>O'itron t miu ion com puted to mOfnphy): 'poor man', PET _no, u_ radil>-labeled 8mphetillnill\l. UpUlkedeJHmd. on ptlHn.etI o(inl.lod neul'OOIIlUld the ronditiDfl ofCflrlbral hk>od
".uti, tlndudin. blood
brain I'Ilaniu}.
~ ....... Md
,..dkmu-
dide upWike India t" neetO.IIi, where". tumor 18.2.
Stereotactic radiosurgery
· I
1 Key ftaJ.u.rea
stereotactic i(>CIliutioo to pteei8l!Jy roo"~ II large dO>ll! or radiation 00\.0 a """,al ly in a ail:l&le t"!IIt ment • be.t lICd_ faU· otr.:vrvaofS RS, the ... Is 'till .igni6canl radia tion deliv~ witbin a rew ,,,iI\ imeteo ... or the me'lPJ'll of the II':5;on. 'fhil , togil ther .... ith the slight .we!lin~ ofll!Ollon. Ihat commonJy fullows SRS creates significant ri.k of nourologic ul,iury , esptfClalty over the 10flI: t.erm (whicb i. even mre lik"ly with benign leoio". in ,I'Ou"g individual , ).
COMPAAISON OF S RS TECHNOLOGIeS
VOrioulI me.th0d.6 6.1'1\. AVllil able, differine I1IO!I tly on the IIOUI'Ce of lhe radiation .,.d tbe t.echnique fOr incr ..., in" tbe dOli! delive~ to Ihe IHion, A photo" beam Ihat il pto: d"ced by electron acceleration i. cal led an K-rDy, whe...,11.1 if it il produotd by natu",1 radl oaeli". deciljl It II called II: ,a mm. rajl. AlthO Co nformatio nal p lanning The Ih8pe orthe treatment volume ean be influenCOld to some degree by covering OIUDle soun:l'lliwith gamma·knife Wlita} or by cl>OOlIing arc5 with nly 6 t e pol'Wd mali(nant lUmOn in n,'n 80,000 I'lId iOllurgical pf'OClld...,.. for ben;~ diseaM" . Sallm.ted inc,dllfllll. < 1 in 1000 5. nonn'" perfu.ion PfUlIlltlI brukl.b ro","'" ch. ulcelly O«Ufll followinl convent ional micrOllllTfel')' for AVM. (_ po,. &19), it 10.. ']110 been dll$ftibed foUowln, SRS lo ...... "' ~EI'ono .. ""'"""" ""1)4.SI.XlOO, laMlS Io.SIorIIooO!: ........ ....." .... _ . . . . . . . . . . . .. ' _ - . 6 6 1-12. IW .
.,."J-. .....
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•.
S.
A deeply loe-ated c"",bral1es;una: eapecially nelU'eWquent brain B. brain stem lesions: may be approached through the cerebral hemiHpher'" C. multiple utlaU lellona (e.g . in some AlOS petientll, Ue pag. 234) D. patient medically unablll to toler8~ gene ra l anesth~ia ro r open biopsy calh. te. pl aoa meot A, drainage IIr deep leilioTUl: colloid cy&t, ablice,;s B. iodwelliDg catheter plecement rOT intratumural chemotherapy C. ",dio.. cl;ve impl/lnlo for interstitial radiation br!lch)'lher/lpyf D. &hunt pllllCeme1lt: for hyd rocllphalus (rarely usec:) or to drain ey!l electrode placement A. depth ellll:trudea fur epile]lSy B. "deep brain stimulation" for clu'vnic pein (requueS' eledrophys iologje atim ' ulptlun ) lesitm generation A. movement d;!OIlrdeTll: P,rkinaonism (see pact 366). dystoni~ . hemlball i&mu8 B. treatment uf chronie pain C. tr-eatmoot of epilep;y (rarely uliOO ) evacuation ofitltra cerebral hemurrhage A. uSlog an Archimed es' &Crew device'.' B. with adjunctive uroltinase".' or recombinant tissult-p1aaminogen activ~to"
NEUROSURGERY
19. StereolJlctlc surgery
'"
(ao, Jl(lljt 860)
8. s tetllOtactK "Tadiotut~ry" (_ SltlWJlaclic rad;o.urlltry, pap 537) 7. 1.0 locaIilUl s I..lon for o~ craniotomy (e.g. AVM', d8&p tumor) A. uti", a ventriet.>...·type ca UleteT B. ...ing a blunt blopy ..-lIe or introducer" C. 'Y'u.m. Yitlibte liibl lau,r beam for guida nce 8. tnnaQrll blo~ of C2 ( uit) vertebral body l"",iolJ4" 9. "experimental- or unC(.nventional applications A. W!reotact.k. dippin, oraneuryam." B. .tereot.actlc ItJII!;. aUfrel')' C. CNS tfllN plsllt.e tion": '.,. for p.,.ltintonil .. tMc p<J.BC 365) 0 , foreIgn body removlll"
u.In,
STEREOTACTIC BIOPSV
Th;' M!d.ion presentl information regarding ltel"l!Otadie brain b:.apl)' (8BB) in pII' enl. For SS D in specific conditioN,", tM ifldu entry for WI eondit.lon. Ma,. be per. formed under local Or pneralllD"",tMli • • For Indieation., . . "bout.
Con tra lndica tioD I l.
COJIguilition dilordere A. co8gu lopath iu : bleed.iq diath-.llttopnic (hep"rin or COWIadIn) B. low plat.elet coun t (PC): PC < 5O,OOOImi i, an abtolute contnolndication, it ;. deeirabl. to get t M PC a 100,000 2. inllbility to tolerate ",Derataneeth""i • ."d 1.0 COOlM'rtl te for t_ l a/lel!lthesia Yie ld The yield rllte(j .• . the ability to make a dlagnoail from a SBB) .eported In I.... Nne. in t he literature rang... from 82-~ ID ntmlmm unocompro.ru..d (NIC) JMltienu, and illillightly lowe r in AIDS petientl a t 506-96.... Higlm- yield ral.N in AIDS may .... ult from improved turgical technlqueand hiuologic evaluation". The yield rate is higher £Or lesiana thatenbance with contrul on CT IN' MRI (99'JIo in NIC patient.e) Ulan wiLlI leaions tL.t do notenhanc:e (14'llo)" .
Cornplica t io!38 The monfrequenlcompheatioD is hetnolTbage, although moat.re Lao . mall to have clinical impll(:l. The riBk ofa """jor complication (mOlltl,. due 1.0 h.,...onhagel in NIC !)e· tient.e r&nIH from 0-3'i1. (with UHlIIt < 1"), aDd o.J.2'JIo;" AIDS" . Hlgbe. complid to havll c.lfleer aI\lIr .. ~n intllrval o£28 montlu. '. ALCOHOl NEUROPATHY Charactl!riaticaUy prodUCft a diffuse se ...... ry neuropath,.. with ablleDt achillee reflexu.
BRACHIAL PlEXUS NEOAOPATlfY' \I''''
Differential diagaoaie ofetiologi", o( bradLial plempathy: 1. tumor: e.g. PllDCDIllIt'a eyndrame hdmost alwl,.)'tluwer plex""leflion) 2. (idiopathic) bra! U>O$t common in oULer surface of upper aftn (circumflex nerve distribution) and rad iallUlPf!Ct orfore"rm, Reflexel WeN! vllriabl ... Overall distribution judged to predominantly involve.wmtl: plexu& in 66'l1>, di ffuse plnua In 38%. and 1()'oO,'M" in 61,\-.
Outcum e Functional recovery i. better in plltiMti with primarily upp"r plexlI4 iovolVt\mMt. Aller I year, 60% ofuppe.r plexu s leaion!! ..... ere functioning-normally, ..... herea.s none with Io ..... er involvemenl ..... ue (Iatl.er I.ook 1.5-3 yell",!. Ra~ orreeo~erye!ltinl .. ted 1.0 be 36'1< within I year, 7~% w·.thin 2, and 89\\0 by 3 years. Recu rrence "'a~ 6een in only 6'>\-. No evidence that B!.eroidi alwred the oourse of the disea.se . RAOIATION NEUROPA1HV O~n follawl ex!.emal beam irr!ldiRl;on In the region of the axillA fo. breDS! carci· noma. Produces $MllO~ 1081 with or without WenMllI!s. CT or MRI may be needed to rule·out tumor invasion of!he brachilll plexul.
LU MBOSACRAL PLEXUS NEUROPATHY'
AnAloJOul \.Q idiopathic brachial plexltisl_ about). It i8controveMlilli wtlether thi~ "",tuaUy Ul/Itli in isolation withaut diBi:>ete!l . Often st8rt1i with LE pain of abrup~onsf!t. follo .....10 in dill'S or a fe ..... "'eeu by weaMasa ..... ith Or without mu&ele otrop/ly. Se~ry B}'lI\ptoms a.e leN prorr,inant., aocl 115"ally uwolve plIl"9attlesiaa. Objective .eMory 1055 is only OCgre8sive. Often with a«elera.ted J[)6lj of difltaI vi· bratory 11106& (normal loss with aging is ~ 1% pe r year alter age40). I'resenta as pain, pate/ltbe.ias, and dyseatheaiu. Sole. offect may be Leader to pressure 2. autonomic neuropathy: inVn 3.
d ui pr.... lne !NorpramiD®): mont .elective blocker OfnOftlllinephrina I'VI.IpLllke (wlUch IftIDll more tffect.i.-e forthl8 condition than III!l'(lltN\in reuptake block' .... ). £ffl!Ctiv • . . - at ~an d",," of JIO mgfdl\Y ...ame ... amitriptylin e and c.he...,for. may be uwfuJ fur petient.. unable to tolerlte amitr iplylirle" . awe ..,.,....,.,., incl ude
u..olllilUt. 48 haunt poeo~. Rilk may t. rtoduc:ed by PfKIdrn, the ann at. a.nd ... pedal1y d ittal tQ, the elbow, and ne~ion of the . Ibow (.void;> 110" Ilellion wbich tighten& !.he cubital tunn.1 retinaculum) Ind by ...dudnS the amount oflilP8 conwalllklng in th~ rKumbent poIS, t lon2. brae.b.iaJ plexu. a.lU"Opailiy: .....,."" w.t.lUn for neuropathy. 000. not IPpea.. to be related ~.rm poIition or I*fdina. M.y be ..1OCi.ted wilb: A. medIIlllIIUllOl.omY(mo&~colDlDOa .... ith inl.c'mallllltmlD&T)'di'lo!d.ioo), PIIII_ terior IlUTIal retrtdion diSplal:.. the\lp~r ribe and lillY .trf!teh or com· pnru the C6 throush TI roote (m~ contributo ... tQ tAt "'tllir oe,.,..) B. h$ad-GowlI ~itiON w","re lbe pati8t)t ".t.lbili1ed with. lho u'd~r bra~e. The bt ..... lhouJd be placed over lbe acromiocl.viculAr jointll). and nl)n-.lip mll1reue, IlIld flexion oft.he knO!H mly be ulled .. lMijuTIC~ C. pr_ poIi; l ion (I"IIre): "pl'Cia'ljIwl\.h Iboulder-Ibduc:tion lind fllbow 0..1I.1on with con trll.~ .. l bead rolItlo ....
nv"lJ'
,w"'dine
"In.,
20. Pa riphe..l n ......
NEUROSURGF:nY
S.
median ne urnpillby; periopellllive median nerve i/lJury may ret;uJl from i.n middl .·aled llIutcu ler male.!. PGddin • • hould be plB!led under Lhe (ol"llarma Il1Id handt to llIainlA;n mild elbow
Ill'9tcb oflh. ne ..... . SeeI1lll 10 occur primarily nuion~
4.
lowl r utl"l!m ity neuropotrue.: 'no.~o«:ur in patien t.. undersoil1l procedU TI!5 ,0 the lithol4rny poIition~. F l'f'qutnQ of involvement in II LaTre HriM ofpationtt WlderlOing procedurea in Lh , lithotorQy poaition" ; c:ommoll peron.alBl .... ad mlie 1:)'1>... nd f.moroI4~. Rilk ("ctOt. other then poailiOll: prolollied duration of pro. oedure. ex l remely Ihin body habitue. Dod dprette emok.inc In the' pr«>perati .... period A. commou peron~a l n~u ropal hy- , uaCltlptible 14 injury in Lh. poIterior popli teal f()Me whel' tI,,,
Moougemcot On«! e neuropathy is detected. delftrmine if it ill ""'IIt>I)', motor. or both. Pu~ MOllOI)' neuropathi... ere mwe ofte " temporary than mntnrM, end ezpeo;tent Dlan$gemeot ror . :) day. i. luggest.ed (ha~e the patient avoid poetUI'e' or et'tivitiell that lDay rurther injure the nerv1l). Neurologl""""suLtetinn Mhould be ""'IueBl.ed (o..u;uen,ll to include EMG eval uation ] ror . U mow. neurop. thiH end ror ROSOry neuropath;", pel'Silllog :> 5 day.".
OTHER NEUROPATHIES
NellNlpa t by ofter c llrdiac cath .... te riUltioD In. u.rio. of _ 10,000 patien'" foJ .
lowed I fter (elnora! ,rwry ta theteriutinn"' ("I . for coroolty llIJIi"l' raph,y Or engiopluty). neuropilLhJ oe. "urn NEUROSURGeRY
20. Periph"ral neT""'!'
'"
Outcome: Group I p. titnU aU bad ...-olution In < IS~. in group II , 5O'lo had oomplf!~ te80lulion in 2~. 6 pe.lian'" had pe.l'lliatent'}'UIptolllll, 5 hd mild femanll KnI!ta .... uropalby (1 ofwholll felt It .. u u leu ! Io(lmewhat dlaabllng), 1 had mild pel'lilten! quadri«pe. ... _ _ and ooeu\on&lly ... alb ",ith a Gall.,
Am yloid n europathy Amyloid is an insoluble utn« llvlar protein .greg.", that ean. be depo&ited in periphe. ...1 nlH"Yes. Iwlyloi(\olw OCCUnl in. n"'mberm conditions, a., . in ~ 15$ of p .. tientl with mul tjpl.. mnLQml C,I.o. _
~
514). The neuropathy predomin&nL\y proc:lUeH'
JH"OfP'e8IIivI autonomic neuropathy and .yrometric diuotiat.ed .eenlKlrt l.OIIII (redu~ pain and I4mperature, preilf!mICI vib ... tory H,,"j. There is uaualJy lell8 prominent motor involV1lmtlnt. May prediJ:poM til p.... uno UUIll)' ar Derve. (upedll.lb' CU'pIll tunnellyTl· drtIme, 1ft ~ 561 for Jaboratol")' teata). U~", lc
neuropathy
0.:.:...,.. in drrorw: ..,n&l !a.ilu ..... Early II}'mptomA include ealfcra.ml* ("Chulie homu"), dy_thetic pain In feet (almilat' to painJ\il diabetic neuropathy) and "ret!ltll'SIIlegll", Acb.ill. relle~ .... JOIt. It. ltockiog .eMOry lou i. rollowecll.ter by LE weakneu that ,Larq di~tally .nd u~d,. The offending Imowledi;_ ofl.nltouur) iOr guick II to how Inn, to wlit before _iderina: fail\lnl of therapy (eitlwr operativi or OOD-ilpenouve). H,,_ver, thill rule may not be Ipplicable to lona di8t.aMM (> _ 12 inches) IIJId thera ro-y be flbrwia " rthe mu.d. beyond u.lv.... PERIPHERAL NERVE INJURy CLASSIFICA TJON (_
Tllbk 1U).10)
nble 20-10 C....IIQtJon of pet1 pOera1
20.
Peripheral~ ...
~
Inlury"
NEUROSURGERY
Table 20·10 CI .. ,ljleallon 01 per!ph.,.J nerve Injury' (eonllnu.-d)
KJumpke'a palsy Tf1.iury to lowerbrlChlll piuWl(C8" TI, eome luthof'lwclud. C7l, from trlCllon o( Ibducted anu in catchln, an_I( clUJ" n,. fbU £row a hei&ht, or by P...-.t'llyn. drnmo QUill IIJIM tur:nor · check CXR ... ith IIpic/ll lordotic vie ...J. Ch"IICten.tic de ... (Ie. funni ly/allG wen with ulnar ne ....a injury) ",jib ..... aMeil and wutin, oflmall hand
byPBPP One mechanism;" low ering oCthe shoulder with oppo.aitl! incLination orthe cervical spine"'.
MADag" m e nt of PBPP: Unless .. Croup" U!JW'}' ill clearly p ...... ot at birth, ..onHerv,,tive treatmen t ia auggellted. lrthere i, no spontaneous recovery by 3 mont1a, the electrophysiologieal evaluation should be performed, and ifn~" root avulsion ill confirmed, surgical treatment .mould be conaidered", Surgery Is Mt favored in the presence of signs ofpreglU'glionic injury C- ..00",,). When EMG ahO'Ra aigM or reinoervation, 80me also rec:omnand continued npect.ant management. MANAGEMENT OF 8RACHliU PLEXUS INJURIES I. w(l6tinjuri",aabow muimaldeficit at onset. Progrea,oive deficit ill WlullUy dua to
vaacular injuries (p.eeudoMew"Y'lIll, A·V fistulo , or e:.cpam ... 2·5 mootl"ll$ functiOIl usually r eeo-·er B. deflCil ... 6$l1ilotiDn lhal . ~ 10 SU!gltlY (e.\I. sponlanoously: t bose with (;5, C6. C1, uppeiOl micIde~. ~11II;JI OI'pWerior complete dyBfwu:tion TIIrecords 0I\tlei" OIJIIlows) I)' do so. Surgery i, oflittle C. 'rfJrieS wilh loss onI'f" tow.l!iemllnlsare llII!.opere!' bendit for diacreta iI\.Iuriea ad to the lower trunk, medial 2. ~181oss wiIh failure IoCOllllOl ~ meticdy cord,orClVJ' I roou. M oat 3. ~doIOIlIsIufalrriaMngpielUt aN! manaSed eon""rvativ". ,.,.-, .",~",~"" . .",_"" . . "'"_"''''''''''--_ _ _ _--' Iy ror 2·5 months. mdica· . t;ollll (or.ursery are "hown in Tob/e2Q./2
'"
20. Peripheraloerves
NEUROSURGERY
I.tactiun injuri": inoomplet.e pottga.Dglionic iQjuriu tend to impfO'Il! Bpontaneoua ly. Injuries in adults withouLutisf$cWry recovery at 3.... month. arl!e~plor$l 6. neuromll~ in continuity: th06e that conduct .. SNAP an managed by neurolysil. Those thatdo notoonduet II SNAP baveoomplete intern.al dillruptioo and rllqWre ...",,,,,tion Ilnd grafting
s.
20.2.1.
Missile injuries of peripheral nerves
Most il1,juri"" from II aingle bullet arl! due to shOl;k and cavitation from the millei1e caUlinB alumotmesi l or Deurotmellil, IUld Ire not from direct nerve transection. Appnaimately 7()';t.will recover with I!XpolI:t4nt IDanllgemen i. However, If there ~ a llltk oiimprovement on aerial UlImi.nlltions inc!udingelectrodiagnostic nudiell, intervention ahould be undertaken by about 5-6 mont.he to avoid fur· ther difficultiell due to !I.e""" fibrwi8 and muac!e Iltrophy. See Tabllt 20·/2 for iDdications for surgery f!lf miMile injuries !If the brachial pluWl.
20.2.2.
Entrapment neuropathies
Entrapu'ent neuropathy ill a peripheral nerve iQjury resulting troD! wmpl!'9Uiou ei. ther by ell1.emRI fortetl Or from nearby anatomic IItrue]ur"". M""hanillnl can vlIrj from eme or two .ignifiunt compreN;ve inault.l to many localiwd, I"I!petitive mild oomprel· . iona of a nervI!. Certain neJ"\'811 ate particularly vulnf'rable at $pKific locations by virtue of being 8ul)t'rficial, fi~ird in poIIitioo, traversing II confined "Pace, or in proximity to II joint. The most oommon s ympl.om is pain {frequently at teIIt, more severe at night, often with retrograde radiation causing more proximallel!ion to bes~l with w.dern_ at the point of entnlpment. May be SII8OC;sted with: 1. dillbete~ mellitUH 2. hypothyroidiem: due to glycogen depo!lition in Schwann cells 3. acromegaly 4. lIn elln produ,," iaehemia which can lead to edema. ouu,ide the axona l .heath which mllY further el NEUROSURGERY'
20, Peripherlll ne ....""
""
gull,
inject in line with wurth digit)
C. median ne ..... e injuriea have been reported with !.hill te~hnique". primarily due to inLTa-neural irljel1n inLTllfpRQw , and nar nerve InJury the m.ajority oornmena' over 10 yUtol fala,lde Dlscflplton lowing the original injury. The elbow is the mOllt vul.nenble poiut of ulnar nerve: 1 lIUIety subjective symplamsl mild hypfslhesol here the nerve t. .uperficial, filted, and 2 ~ ~ l weakrleu at i'lI.tIsic hMld muscroeoell a joint. MOIItca.'lelJ are idiopathj~, cles 1 sIi\tIl wasMg although then! may be a hi5tory ofeibow 3 Ul'&le se NON-SURGICAL TREATMENT Avoid elbow t.reuma (patient education, DJl elbow pad may help). Resuitl! are often ""tier when definl~ trawastic et.ioloc can he identified and eliminated. SURGICAL TREATMENT
Surgical options primarily OJruillt of: 1. .'mple nerve rlewn'J:D"e$Sion "",tbou! t.ran/,l~itiQn· (_ ~bw ) 2. nerve d«ompres.ion and \.I"W\lipoaition (extentof.urgery differa becaUJle degree of.nt.apm~ot v.riell; all (orme ortran~p(MIition '"""lui", fe,hionina B . !.ina: 10 ..... U\l.n the nel"le in il8 new location ). Transpollition may be to: A. subeutan(!(lu s t'"lIe: Lb"'leav~ the ne .... e fairly superfici.l"ad vulnarable B. withi" the flexor ""'lIi uhuuili mUijde (intr amuscular ~ran~JIOI!ition): ~me contend thill actually worsena the condition due t.o intrnn)tureulllr fibrollis C. a $ubmustu lar poaition: lie"- below 3. m1 lyear, with only 3~ of these IymptolnaticaUy im proved in one r.eri es". Lower 8Uceess ra l.f! is also &een in older patitnta and those with certain medital ronditions (diabete!l. alcoholism.. .\. Pain and &(!1!8OT)' changu respond betler than mU5tlo~ wllakneoos lind ,trophy. ENTRAPMENT IN THE FOREARM
JIIBtdist.(llto thll clbow. the uln ll.l" ne rve pa~ frorn thOlgToove between the ~iI1I epicondyle and the olecranon process to enter bet ..... een Ihe (WII heads o£. t he flexor carpi ulnaris (Fe U] Wlder the faacial band connecting the two heads (the cubital tunnel) but s uperlicia! to the flUllr superlkialil. Entrallmenl in the cubital t unnai litor jW1t distal \0 tile elbow producea the cubi ta l tWlnel l yudrom .. , wruch ie very rare. Findings are simila r 1.0 I8n1y ulnar !\eNOl palsy (1Fo!~ IJ~). Surgical tre atmen t cont islS of steps outl illfll f9~ nerve dut,,1 to the alOOw in ulnar nervt deo:ompres&ion. A u,eh,nique for localing the c:ourMofthe ulnar Derve di~tal to the elbow;' to IIlllte !.he pro~imal phahullt of the surg~n's little tinge r (using the hand oontralate ral \0 th~ palienc'5 beingdecompreS!ledI in the ulnar groove aiming it toward !.he ulnar side of the wrist" I. - . ENTRAPMENT IN THE WRJ ST OR HANO
At the wrist., th .. terminal ulnar Derve enters G uyoo '. C A OIII, the roof of which ill r..acia IUld paltDar1a brllY;S. the Oour i8 the nel'o~ retinllculum of the pllim the canal is ~ the l1ansvaree carpal ligament that produces carpal lunnal BYOdrome]ltnd the pi~oh/llDate llg1lment. Th e canal has no If'ndllM. oniyUle.ulnarnervalind artery . At tha middle or Lhecanal th e nerve divides into deep and superflcial br\lJ>ch. Tbe 8u perficial branch ii mostly senlOry (Utellt for the branch to ::oal marls brevis), and supplies hypothenaremiMoce lind ulnar hllif of ring linger. Tha deep (mU$Cuiar] bntnch ;n_ nel"'IILes hypotheMr muselea, lumbrical IS &. 4. lind 1111 interossei. Occasionally the abductor digiti minlmi bronch ari ses from the mo in trunk or superfIcial branch. Shea and McClai nlt divided lesion,l orthe ulnar nel"Y(! in Cuyon'sl:D nal into 3 typoil s hown in Table 20·15. l r\i ury to the distal IIlOW bl1lncb can a llO oa:u r in !he palm lUld producel lindinp einnlar to II Type 11 illjury.
th palmar
(k~y;
01 "Ins r nerve lu lon, In
, depetldltlQ.",
!~
1oca1Dn. mav ."..re
!lyPOO>on.' m~"""
Injury is most often due to a ganglion oflha wrist". but alllll may be due !.II t rauma (poeumaticdril1. pli ers. repetitively 11anuninll a stapler.leMing-OD palm while rid ingbieyelet Symptom. are lIl mi iar 1(1 !hose Drulnar nerve In''ol'''e,,,,,nl til the elbow, e:.cept t hen will never be sensQry loss in the dw:nI.w ofu,e band in the ulna r nerve lanitory OOc:lluse thedors/ll cutaneous hranch IlIIIvl!I the nerv;)m II thoracic hamel>8 3_ irijertiOll injury in lb. high poBt4\rior aspect oflhtl shoulder ~ . Imtrapment of the ne rve in tha quadril8te~al splice (bounded by !J,e tere!! ml\ior e nd minorrnuscl es.long head ortriceps, lIod nedo: ofhumero-\lwhich rontains til", axilhll")' nerve IWd Ute po. i~
usually due 1 Sigiis and symploCDa Burning dyse&theaiu in t.he 1 . ~TaJ lI8pect of the upper tbigh, DCell~ionaJ ly just above the knee, UBUIlUy with im:re88ed senaitivity t.o dothing (hyperpa thl a ). There may be decreased sen,",uon in lbi. diltribution. Sponlaneo\lfl rubbing Or ma.uagio(! thll a rea in oroer to obtain rel iefi. very choracteristic". MP Illl\Y be bilaleral(n up to 2O'JI, orc:.a!le8. Sitting or lying prone uiluBlly IlDleUorel.el: the ~pt.om •. There may be point teodeme611 8t t.he . i le of ell u.pment (where prellllu", may reproduCf! the pain l. which is often located .... hera the nerve exit.s the pelvis m~i. 1 t.o the
NEUROSURGERY
ASIS. Hlp HtenilOr> mloy III.., CollUM pain .
Oceun-en ee Usuftlly _n in obelle pati~nts, m.y be lIucerbal.ed by walllnna tiaht belu or ain:Uea, ~I)tly found in Iona dlstanQ'l nmn- . Higher in dilbeli($. lollY IIIt.o o«u r poet.-op in I len derpa t ieDu poei!.lO!1ed prOne. lend. 10 be ltiliM:W (aUptJl, 559). Poo.Ilblo! e l iolcltiu a re too Dumermll to lilt, morll o;ommon 0'"" includ~ liabt doth · In, or belu, IW'aicaJ l /1 dinl",1 iII"Ound • . Wbenta. .. ) 2. In obelli: palients: weiab~IC*llltId exetciae5 to ' l l'l!ln,gthen!.he ebdomin.l UlldClei ii Wlually effective, but ill ,..",Iy achieved by the ptluelll 3 elimination of IIClivi!.le:s involving hip utenllion 4. application ofice to th e area of presumed conetrietion x 80 minute. TID &. NSA1D nftboi~ x7- 10day. 6. CIIOpsaicin o;n\.ruent appLied TID tIIu tJOIIf- 389) 7. if tb~ above mea,u,," rail, ;~Il of &-10 JlIl ollocallllle:sthe*, (with or wi t hout steroid.) a t tJ... point oI'tendemess, or medial to th CompoHd of1.2-4 roota. CoWHIalong pelvic wall to provide HI:Utioo. to the inner thigh. and I'OOlOr to the thiah;uiduct.or1r; (a'tacili, aod a dductol'tllonpa, brevia, and matIO~). It m", be comprueed'"by pelvic tumon, aleo from \.he preYW's :lfl.he fetal hMd or {umopol duriz:lt, parturition. The reflu lt it numbo,*, ,,{the Itledilll thlah and weak thigh addu ction. FEMORAL NERVE EHTRAPMENT
Compoaed o{roota 1.2.... Ent"'pmen t l, I rare ca~ oIfemoral :If-uropathy. See FlrMr.d nluropolhy on pqeM7.
20. Peripbf-1'tI1
oe~
NE:UROSURGE:RY
COMMON PERONEAL NER VE PALSY
The peronell] nerve is the mOlll common neNe lo develO)pnc"u cornpl'Mllion palsy.
The ...,iatic nerve 1.iA.s3) conta' n8 2 ""1"' .lIle lIerv~8 within II <XImlD(ln she-ath. the
common peroneal nerve (C P N) IAKA lateral popHleal nerve) and the po.hllio. libilll o el"'l1! (AKA medial popliualnerve ). AI a variable Iocat'or. in the thillh. the two h.~nches sep8ra\.e ""Ole Tobk 32·4. page 9 101, the CPN p""ses behind the nbu.Jar head where it i8 JuperficiaJ IUld fixed . makiDi il vuinernbJe 1.0 p"""I!1,I..e 0. trPu.m8 (11.11. from uOSIIing the lep at the M," l. Just distal t.n iliirI. i~ d ivide.! inlO: d eep per o neal n e rve (AKA Il l'teriO' ubiol nervel: primarily motor A. mowr: f_ and toe extenso", (EHL. tibialis anterior. e~wn80r digitorum longuor) B. sen80 ry; very .mall BI'ilIl bel"'~n grea ltoe 811d ile«)nd toe • a upel'l'io:ial perone ... berve (AKA mUlICUJoeutanooue nerval A. motor: foot everton {peroneullongus and brevi s} B. l ensory; laleral di$ulleg and donum of root
Findi ngs In peroneallle ...... e palsy r.eIUlOry thllDIletl (uncommon}: involve! laleral aspect. of lower haU'O)f leg llIu6ele involvement: sa raw.. 20. 16 Common peroneal oerve pelsy Imost com· mOIl ) prodUC85 weak ankle dorsinexion (foot drop) due to anterior tibiplis plIlsy. w~sk foot evenion, a nd '"'lIftor)' impeinnen t in areaR innervlOted by deep and superficial peroneal uerve (loleral calf and dors"':JI "ffoot). The", may be 11 Tinel's etgn with perculISion oftb e nerv e near the nbularneek . Occ85ionally, only the deep peroneal nerve is in· volved. re\lu!t.ing in foo~ drop with minimal R nllOry 1061. Must dilfen!ut.iale from other cauae, of fOOl drop (8ee Fool drop. poge 909).
Ca uses or common perDlleaJ nenoe injury 1. entrapment .. it crOll.~ the fibular ned< or 8S
i~ per'l8tl'8tes the peroneus 10"8'l'l diabet.es mellilu5 and other me1.8bolic peripherAl neuropathies innammalllry o eurO(>llthy: including Hllnsen', diseaSfl (leprosy) 4. traumatic: e.i'. dippinfj' in"iw:y in roolball playe rs, Itrttch injury dUEl to dislocating foru a pplied to the. MH. fibull\t l.lIeture 5 mss""" in the a res of the fibular headlprOltimllJlower Jo.g: poplileal fosta C}'1its. snlerior tibial &rtery lUleurySm>t (rare) 6. presaul'fl at fibu lar head : e.g. From CtOllSing the legs a~the knl!i!, ca~ta. obstetrical
MclC_ H C. Vooot.i ita ,ize with COIIM1mlaI1'ft1lO1lH), !.bia i. In atr... renl pU pillAry d eff'JCt (W, 1M/ow)
21 .3.1.
Alterations in pupillary diameter
MARCUS GUNN PUP.. AKA .tfere n t pllpilluy del"t (AP O), AKA amAu,.,lie pupiL Findini! tho! aide dlb. impaired di.ec~ rei1u:, .1t11eriorJCt the thjum: 1. either in tlla retina (a.a. ~tiaal det.Khment, NUDAI inflll'Ct e.g. from embolu.) 2. or (IJrtil: (I ....... e ... may""""" in: A. opticor relrObulbar Dellritia: commanl,.!letn ill MS, but ClII}' alao occur afte. vaccinaliolUl Or viral inft'Ctiona, and usually improvH ",dually B. trauml to tha opli~ '"'"'t! indinct (.ou page tH5) ordj~t
ANISOCORIA Key pooine an alfH'elll pupillary deC.,.,.. (AP O) togeth .... _itll loiaocoria indi .... tea two !Hiool Ii.e. an APD alone does lUll prodLla! aWlO(Oria) (Me ~/D/.II).
RpeTllA:
Differential eliaanneia: 1. ph,..ioI~ aniaoalria : '" 1m'll diJfereoctlln pupil.in that it the lI.lOe in a lighl and dark roDIlI • .5ef,n iII - 2~ofpopnlatloll (moM! common in peopla with al.ht iria) 2. Ii orner••yndrome: in~rTUpOon ofa)'mplltheti.... to pupilodih.tor (Hcpage 683). I"ere 1M ahllOnllal pupil it the IIIIa.IIu: pupil (mio$ia) 3. Adie', pupil (AKA toni: pupil)-. I« belOUl 4. thirdn ....... palsy fKJlJI 685) A. CKlilolDotor neur.Plthy (a -peripheral'" nl!UfDpllthy of \he third n~e l. ally ,pare. pupil Etiologiea: O:.1 lUlllaU,. ._lve. in - 1_Ju), EtOH •.. B. colDpreaioll: ilX'llidina by the fol1owil1l (tend. nQl. to ~I*re pupil) I . aneu.,..ln: a . ~"'''': \he llloal COUUIlOD anau.,..", to CAUM til .. b. baailar bifuraotion: OOCIIIIionall,. (omp'- ~rior III fit",' 2. uncal bemi.lioo; _ OeodC)rMlt)r" Ill"'" comprnfion below 5. local \nwoa to It. ayl . -'led ~.uma.tk iridoplegia ean oo:cur in ;.olalion 1m.., result in traumalic mydrilllia Of miOl;I) 6. pblnnaco!ocic pupil; ... tw/0Ul 7. light.-near di.IJoXi"lion : ' " (lbow A. Argyll RoberU>On pupil: cla/ilic:.ally detcrib.d In .yphil~ {_ pD6f 582) 8 . Pannaud'. syndrome: doraaI midbrain leaion (1ft pagel6) C. flCUlomotor nl!W1lpll\.ltr (u'ually ... . . - a tonic pupil .. ito flCU loll\Otor com-
IL (TONIC PUPIL) An iri. peJay IWIuitinll in 1 dil.tOO pupil, due to Imp.INd posl4l;anglionle p....)'01pIIthetica. Thought to be dlle to. vi rol lnreetlon ofth. ciliary pngUon. When u.oeiated with lou or 1111 mualIry.m, K()Wlver, within 2-t boun, ' -t oIth_oc:a... wiU ,lIIo develop an oc:ulomotor .,.IIY (witb down and ,,\It deviation oflhe eye.nd ptOSis). n.- p\lpib ~ .pond to mydrilltia.nd too mIOtic IIgenta (the lalte, help" difTen!ntiatft this fro", a pharmal.'Oiogie pupil. _ obow). Althoush it i. pouible for I W\iiatml ilydilitecl puptlalooe to be the iwtlal pr_tIIt.lon in IIncal i"M!miIlUon , in lew. lily .Ira.,., all oftben pIIt>enta wiLl h~ """" Other findinll , e., . • Ite"tion in mental ate lu. (COlI fuaion, a&itation. etc.) before midbrain com~ioo Ottun(i.e. it would be nore f(l, a ~r$OfI ..... derzaillfl' •• ' ly ..... caI hernietion toile
aWllke, talk.io" .ppropriete soo neuroioci:oept ill Ilrpd_ .... her. IOII\oI!: of the rir.t.nod I«'Dnd order nl!Ul"'OhI mlly bI hloo:ked H ORNER ' S SYNDROME
Homer'a .ynd.roaoe (HS) i. cauaed by lotelT1.lption ofa}'DIpathetiQ to the eye and fsee .nywhere .Iona: t~elr pBth tsee PupiltJdil,,/()#" (lY"'polillJ,q, pilip 581). Unil,ter.l fi"dinp on 0.. involV4KI ai-· oblique (10), . nd tOll lraJa;.l'r1lI .llupeno. rect"" (S RI. C. N IV (the vnlytranial ne.rv1l
that dlleuaa.t.u; mlY decu ... ", internilly' inne,... ..... the oontrala"" rl ! aupenM oblique (SOl (depre_ the Idducted ~). Cr. N. V1 inne,....w the Ipllilat.er.l b."'ral /'eClU5 ( LK ).
lateral uocadlt~ llIOWmentll!·pre-pt'OlI"rtImml!!l". rapid, ball;,llt)to U,.~5ide. and iJl loc:ated in Brodmlnn'..... 8 (in lb. frontal lobe, intenor to the prim. ry m01.Ol" cortex, aeeFilu", 3·1, pap 68). , , _ tuttitobulbar fibe ... puc through tM ,enu of tM inu,rna' ~I~ ule \0 the pe •• medil n pontine . aticular ronnuion (PPHF). which swda fi· be" to th e lpeilll'!",1 IIbducemfpll ..·.bdu.:.l!Ilt{V1) nuclear complu , and viII ltlt medial lonritudin.l fl.$t'~lI tu. (MLP) lathe contra/ater . 11I1 nucm.1 to fnll' tvaLl Lhe t;Onlrtllat. 21 NturopMhalmolop
NEUROSURGt'RY
eral MR. InhIbitor')' libets,o to the Ipaihuer.1 third ne""e to Inhibit the anLagonln MR muoclt. ThuI, the righl PPRf' controb lall!raleye mOYem~"t. to ri,hl,
IWTERNUCtEAR oPHTHALMOPI..EG'.... lnll!muclea r ophthalmoplegia ((N O) ia due to. luioD o((h. M r. F (U. /Ibow) rOil ral Ihe abductn, nucleu .. Dod produ~a the rOllCIWillff. 1. the eytl Wlilall!nl to the I•• ",n rail. to ADDuct completely on attempting to look to the oppCqlite aide 2. addu~tiOD oy ..... amu. in the DOotralatera l l!l'e (rn.)I)«t.l., 1I)'1t.;>rm .... ) afUtn wi th lOme weaJm.:u of ABD...,tion (toct-thar with I' produeell .lall!ral pu pallY; 3 converren«! il nol impaired in ;&Ol,tee! MLF Il!Clion~ (lNO i. not 1U\ EOM pal.y) The IJI(J$t DOtnmon cau_ of INO: 1 MS: tIw mOitcornmon "UM 0' bilateraJ INO in )'Ounr. adulta 2 brainltem ,Irvke: the mottcommon Quae of W\ilateral INO in the elderly \.0
OCULOMOTOR (CR , N, III) NERVI! PALSY (OMP) May illd",de pmpherally Iocall!d fibelll mediating p"pillary eIOllIItrictlon (pal'RJlym. pathetical, end /I10t0r ribera to the folltrwinjJ EO~: SR, MR, IR, 10 . Qe".JomalOr oervt IIIQIQr: pal,y ~a", ... pt.o.ill with aye de> ;ated "dawn'" out". N ud .... ulnvolvement oI3rd MrYe ia 1"Itt. NB: 31'd nnve pallY alone cao cauae up to:l mm exophlh.tmOl l proptOli,l from AI .... boo of the nctUI mUlICles. Alto ... Painful op/tlluJ/1fU}p/qJw .od Pdinkf. ophlloiJlmcplqia below For br-iD· Item . yndro",", _ &~iltl" .,lIlIronu:. pap 86 and WrW~ ondronut O"n page 85. AlIII. _AniMxloriD, paJl'C! 5-82.
The rule (ltthe pupil in tbird n e l"Ve palsy Elucldf,ted in 1968 by RUttve ullpain pupillary coru;tridioo.· However it 1& o~n overlooked that in 3-' the pupil wu ,pared and there Willi alirht;rnPl'inneot of the ~noeulat 1IIU5C1es'. Pu pil ' pa rinl oc ",lornolor p all)' (pupil. ~act to l ight): UIUally rrolllln~rin.ic '18.1. cular leNoru ocdudinr VI_""onutI cau.,; .... y: inelud;r.&: di.bel.el! and &:ian! ceUatl.eritia. MOI~t._ ~lIOlve with· In 3 lJ1(IDlhl (altemaliY.callllf1l h(mld be .,ught In ea... l.,;tin, lon~r) 2. inerellM.'d intr ae:ran1 11.1 prouur.; pallY may ocrur with increa&ed ICP • ...., iu the abMOI;I ordi~t COID preuion of t.he nol .... (. · ral.. loeali~ina" . ip In Lhil...tling). Pottulated to occu r dua to the ran that the VI De .... hal a loog lnuacrao.i.1 roul'H which 1UIIr. render it more lenlltJYe to Increased pTN!luu. MIIy be bilater· al. EtiolOlin We uda: A. traumaLiCli lly In=n!~ ICP: Me ~ 637 B. lncreaaed ICP due to hldrocephalu. (I.&:. from p·foua twnor~ f« ~ 404 C. idiopathie intraeuo.ial bypertanaioo (paeudotwnor oeubri): 1ft JJOllfl493 3. cavemom IlnUllllllions: caYsmo"a clrotid aneury&al U. ~ 818), neupl..m (menin,;om •.. .). carotid cavsm ou, Ibtu)1 (au p(Jft 8 46) 4. inO&mmatory: A. C radenigo', 'ylldrome (involvement at Dorella'a canal): .,ePQ6'l688 S. sphenoid ,[nUlio.: (iuyoJvUIIMt.t Oot'eUo', caoaJl 6. InL.acraniaJ oeoplum: •. g. cliVUI chordoma, ehondl'OlanlGlDl. 6. p&elldtulbdl«lelUl pelt,: may be dill to A. i.hyToid eye diHqe: tbllDOlt common CIIu-.e of chronic VI pA1&y. Will h.... po!litive fon:ed ductkm ~t (eye e&nIIot be moved ~ eu.miner) 8 . mYlltbenil gra,i.: ""pond. to adropboniwn (Thnaflon3) lelt C. long·.atanding Itnobl&lJlu.
O.
Duane'l.yndrolll~
E. fracture of th e wedialwall 0( the orbit with medi.1 rKlu.entrspment 7. fo!lowing l ... mbar pun.lllre: almOlt in"llriably unilateral (we~ 616) 8. fractll N! Ihrough cli"\lI: $ft palft 665 9. idiOpAthic
MULTI PlE EXTRAOCULAR MOTOR NERVE IKYOLVEMENT LesiolWl in cayem oUl . ious(aft 6.:1oc..) in,ol"e tranieJ ne ... es ill. IV, vt.nd VI &. V, (op hthalmicllId ma:o;ilhu)' divisiona of~m.i"'1 nervi}, and apaN! II and V.. S uperim- orbital flM ure a;yndJoo me: dysfunction ofnerYellU, IV, VI pd. VI' OrbiW .p"" a;yndrome: involvf!S n. m. IV , VI and putiaJ VI ~th n.,...e paI.y IDlY rMUlt from. COIIbtIDOup U1.iwy in fron\.lli hud Inurn ... P AINFUL OPKntALMOPLEO.... o.flnitJon: pain and dys!i..mtion o! ocular motility (may be due to involvement of one or more of eranial nerves m. IV. V &. Vl ). EnOl..OGI~S
1.
Intraorbilli A. lnflll.\llmatory ~I1UlO' UdiOj)llthieorbital inflammation}; see Mlow B. ronti«uoUl .inulit~ C. invaei ... funpl tinUl inledicm producingorbiLII] apex .yndroma. RhinocerebnI mLI(QDDYCQllia (AKA zygom)'Ulllit~ IlOUIIltill "';th painleu black p.latal or toaaaIaepcai ulcer CI. eschar with h,ypbal invNion or blood vellll"l, by funci of the order M......"."w, NpecIalJy r/Uzop,"" . Uaually _n in diabe!.ic or lmmunocomprOUli.ad IN-tiellLa. oo;:cMionally in othe ...... ;... hHlthy petJecLa u• Of\ero lnYQiv. dw--.J ain.... and may ClIUM [awmoul ~UI thrombosill
0. _ 2.
...
E. lytDphoma IUperior orbil.alll..umlanterio. c.",moUl.inul A. ToIoN·HunleYJld.rom.: .u Mlow B. meg
21. NeurophthalmolollY
NEUROSURGERY
C. nasopbar.fllgeal Ca D. Iympboma E. berpes wster F. carotid-cavH'D(lIl~ fiIItula G. cavem(IU~ ainUI thrombc...ia H . intnlC3va:-noua aneuryam 3. PllrwoeliRT ~on A. pituitary adenoUJ8 B. meta C. nasapbartngeal Ca D. • phel\l)id ";nU5 mucocele E. meningioma/chordoma 1". apical petrositis (G radenigo's syndrome): ..... ~k>w 4. po!lterior fOlllla A. p-comm aneurysm B. builar artery BnBurysm (rare) 5. crUsullaneoul A. d iabetic aphth.olmopleBia B. migrain0U8 ophthalmoylegia C. trlUliai arteritis D. tuben:ulal.!1 meningitiS: may cau~ophth4ImopJegia. u.uaUy incomplete, mOlt ofteo primarily ocuIomotornerva
P AIN LESS OPHTHALMOPLEGIA
Differential diagnOli.: I . chronic prgre!!llive ophthalmoplegia: pupil eparing, WluaUy bilJlterll.l, . lowly progreu;ve 2. myuthenja grl.v;.: pupil . paring, reapgnds to edropban;um (Teniil on®) teat 3. myollitia: UlIually alllO produce. .ymptoltlll in other argao I y$terns (hean.. g0nads ... )
PSEUOOTUMOR (OF '!liE ORalT) AKA ·chronic If"LOuJom,,· (a """nomn, since true epithelioid granulomal am rarely fOWld). An Idiopathic in llamroltof)' disea8e confined to the orbi t thet mill" mimic a true n\!Oplum . Utuo.lly Wlilatenl. Typically pNlHnl.8 with repid onHt ofpropt.ollil, pain. and EOM dysfunction (paiD, ful ophthalmoplegia WLth diplopia). M08t rommonly involve. lhe aupf!rmrorbitallilllluell. DilTerentiaJ diagnoeiB: See Orbi/QI.ltt,w,u on page 929 ror lisL Key point.e far G ... ve·1 diaeMe (GO ): Lhe hi.toJogic apptArance of GO (hypertllynlid· iBm) may be iDdiBLinguishable from p!M!lIdotumor. lDvoiveIlJll.IIL with GO iI uluaUy bUnt""I. TREATMENT
Surgery tende to C3Ul1e II. Ilan up , eDd is tho. \18uaJ.1Y bc>wL avoided. ~Ulro'd.iI are the tte4tmeu~ of choice. HJ:: &0-80 mg predni80ne q d.. Sev1!re cases may ntU8lliUlte treatment with 30-40 mgld ror .evera! months. Radiatio" trell~ment with 1000·2000 rad$ may be needed fur CUM of rellCtiv,,- Iyro· phoeytic hypel')llasia.
TOLOSA·HuNT SYNDROME
Nonspecific iDllammRLion in the regicm ofthe superiororbil.8J 1i8llure, often with u· l.en8ion into the .... vemol1ll SiDU~, . omelirn"" with granuJomat.ol1ll featW'f'!l . A diagnOBi. Of Ol1c1usion. May be a lopograpJUcel varipntoforbital po!IeudotultlOr(ru obow). Clinical diaKDOI!tk criteria: 1. pa.infulophthalmoplegia 2. involvement of any nerve tmvenlng the cavemoWl l inu8. The pupil Is usually spared (frequently not the cue with aneuryllmll. apecific infl8.DlIDa.tion . etc.) 3. symp tom .laBt days 1.0 weeka 4 . spontaneous remisaion, aometimes with residual delicit
NEUROSURGERY
21. Neurophu)lllmology
.Ii. reculTf!nt attacks with remiS6ioM of mon!.h a Or y68J'S 6, nO syetemic involvement (~uional NN, due to pain?) 7. draOlltti Surgica l app roac bel for SVN l. r etrolabyri nth ine, AKA pOlluur1culu approach: IIn\erior to sigmoid s inu •. Pri, mary choi~ in p 8tien~ wilh Meniere', djaellR who hlv~ oot h&d previou.o endolymph.tic a8.C (FLS) proeedures . inee it permiu iioult.aneou8 SVN and deo:om p reMion orthe endolymphat/c IIIIt. Require,; ma.otoid...:tolllY wilh s keletoniUltion or .... ~ Mmieircu.larcaollb and ELS. Thedurlll opl!ning ill boWlder! anteri. orly by lhe poIItenor semici",uJar canal, P()llteriorty b)' the sigmoid Bi""s. Water_ tight dural closure is difficult '2. retros igmoi d , AKA pDl!tatior fossa, AKA auboc EPIDEMIOLOGY InddenCll _ J per 100,000 popullltion'. Most oases hllv" o~t bo-lwet:'1l 30-00 },>,"I'II or oge, ra"!ly in youth or in th e el,leriy. May beco me bilateral in 20%. DIFFERENTIAL OIAGNOSIS (AlM &ell DifftreMia/ diOJ/~il: DluinU8 and ~r'i£o On p>' 590 f~r mo~del.aila) 1. benign (paroxys,n"J) l'OO 't iolllll vertigo: AKA cunnlgHtb lMjll. Seltlimit.,.j (mOllt case. Jut'< t yur ). No bearing lOS! 2. disabling po~itionfti v"rtill: ~ disabling positional verti/ll.> Or dyscqullibri· urn, _ COQuant nauaell, no vestibular d.nfunYeO bioi non·.orvIcoabio IINIini lil!VeiI o(vertigo, 5% Ilochani~ WOI"!H!; 9'l> incidenx· imal to this point produce 8 dry eye. The nen braoch il the bran.h !.lI the atapediua mua' de; leaiona pronmal to thie point produce hyperaou.ia. Next. the chDtda tympanijo'nl t.be facial nerve bringing t.ut.6 "Illation from the anterior two thIrds ofthe tongue. Bas.a I .kull fractW1! &may lnjUI1l t.b~ ne ..... ejUllt pro.runaJ to JJ,i , point. Trav! Jliog with the chorda tympani are fibe!1l to the aubmendibular8lld ,ublinguai glandl. Thefacial nenoB e:tit!! the akull at the al.ylornMw id foran'ef1. It then entara the parot.id gl!tlld. where il l pl''''' into the followingbranchl!lJ to the facial mU l cl tlll(crsnial to",",udhIJ: temporal, zygomatic, buceal , mlllldibuler, and oenoiw. Leaions within the parotid gland (e.g. perutid tumoral may involv e IIOme b ranches but . pare other •. ETIOLOGIES These Itiologiea produee primarily facial n"noe pel,y. 1100 s"' M.. lltple cmllia! n~",~ pa!sie. (crllnialllfll.l1vpothiuJ, JWlge 917. I . Bell'. palsy: IU lHlow , 90-95% of all CIU!etl of ra ciy] 2. herpetl z06ter OIieua (Iwil); aoet! pogI! 1;96 3. trauma; b!l8Bl , kull fr&c:tUTB 4. birth; A. congenital I . bilot.cul {Galli pmiay (rmdal wplcgial cr M6b' U5 ayndromo" ......;q ... o it> that it aff""ta upper faCIO J\HIte thall lower face (oft page 917 ) 2. coogenil81 fedal dIplegia may be pert off""i08Cll ~u.lohumeral or myo. tonic muscular dystrophy" B. traulOatic 5. otiti. media: with acu:e otiti , media, fatial pal$y uausJly improves with anlibiotics; with chroPic e uppuraliV1! oliti. ~urgi",,1 intervention ill required 6. c""tral facial parolysis and nuclear facial peralyBiI; see Lo«J1ui"IJ $/te oflfl~n above 7. neopLaam: usually cau.e. hellring 1011$. a nd (unlike BeU'. polJiyl slDwly urogrn. W f.cial paralysis A. m06lare either benign 8chwannom.. ofthll fetia1 or auditory lie ....... 0' mo· lignancies meLa'Latie to tbe tempore1 bone. Facial nel1fOmaa 8COOunt for ~ 5% ofperipheral fHeial n"noe palsies'·; the peralysi. Lend, w be slowly progtll$llive /IIH PIII1e 922) D. parotid tumon may inYDlve IIOme bnwchea bul spa re lIthera C. MaMon'a vegetant IIItraYIlJlcu1ar hemangioendotheliolOli (...... /Xl&fl- 491)
.....,.
'"
2'2. Neurotology
NEUROSURGERY
8 . neurt\&t\Tcoidoal. - : Vll i5 the w06teolllmonly effected cronilll ne ...... (.u pQ&e 66) 9. disbo;te.I : 17% ofpstienlll > 40}'B old wi th peripheral fecilll plIlBY (,PFP) have libnorlllal glutoee toleraoce len.•. Diabetic!! bllv~ 4.5 timea the r.!lotive risk I)fdevel· oping PFP than nondiabetics " 10. Lym~ dilltaBe-·;' 6piroeh~ tal diseaee", fadal diplegia i811 hallmark II. GulUo;o·Barri.ayndrowe-; facial dipl",,_ 0«W'8 in ~ S{)% orr.UlJ cales 12. occui'lIlo11y BIlen in Klipp ileJna w,th 10 uteriok art o~o .. oodato S ......ca l dec:Qmp reuinn: controve rtlial. The Rarely utilized.. Indh:aliOlls may indude: NEUROSURGERY
defin;ti~
22. N"urotology
aludy has nOl been done,
'"
1. 2. 3.
complete facial nerve degeneTllti<m without respon&e 1.11 ne ...... uimulalion (altbough this absence i. a 1!!O ""e(\ 115 an tul[U1Dent agaillHt aw-gery") progreulvely deteriorati ng response 1.11 nerve stimulation no clinical nOr objefl-
2,
mobilizing tbe intratempoTliI portion ofVlloul "fthe faLlopian canal (lUI previoU.!!· Iy describ.-d" ) and t hem anutam05ing it UlIing II bevelled ( ut O u toollle Reault.ll 111: beuer ;f performed early, although good resulu can ~ur up til 18 CJ10':1 good t"e5ll1!.5, 14'i1 fair. 18% poor, and I pati~nt had no
A~r i!'\jllry. l n 22cMe!I. 64% h ~d
~yjdence of reinnervation . In 59'1>of ~asa, evidence of rt!innervatioo WPl! £OOn by 3·6 mos. in th~ rt!main ing patienlJ; with re;nnervatiun improvement ... 91 noted by 8 mM'". &!ooyery ofrorehead movementoc:cursin only ~ 30%. &!tum tone preced~s mOvement by ~ 3 lIIo ntlu;.
or
SPINAL ACCESSOR'I IlJERVE· FACIAL NERVE (X/·VII) ANASTIWOSIS firat detitribed in 1895 by S,r Cbarles Ballance"". Sacrifices l orne shollidar mov(t· lDent rAther tJ,an uHf! of tongue. Ini ti al Mncems "bollt signifiesnt shollider disability IUId pain r"," ulted in the techniqlle (If,,,,ingonly the SCM brnnch ofXJ". h"",,,,, .. r these problems- have not occurred in tbe nlajority orpntient.B even with use ofth .. major divi.ion".
22.4.
Hearing loss
1'woanat.omic typell: COndllct.lvt and &el\.5Orinell1al. I . CQndllctiv~ h .. &ring 1!lI!JI A. patienUltend to apeak .... ith normal Or low vol u me B. 6ndi ngs: L Rlnn~ !elit with 256 Hz tuning f(lrk w ill beneptiVf) (i.e. sbnnrnlal, air conduction < bone conduction) 2. Weber klIt wIU laWlruUze to .ide of heoring 10ft 3, abnormal middle eI. ;nlpedanee m~lIIIurOlDenUl C. eti(llogi",,: anything \.llnt in wrie ...... with nsaicular 1T}{}Vement. indudinr. oti . tis media with middle"o r effusion. ot",kerOllir 2. Mllaorineural h earing loss A. pa t ient.! tend to epeak wi~h loud voice B. clinical finding!! : 1. Riru\6 ~t w[iJ be positive (i.e. nortI'UIl. ronduo:tfon > boneconduc~OI1)
,i,
2. Weber Jeat "'ill h.ten.li%e tn Kide IIfbeUerbearing C. furtller divided intn sensory Or neural. Distinguis hed by oto~COUlItic emiitsionB Conly produotd by D Mchlell .. ith fu.nctioning hair >0 .
No rilhl o{operator', except in the followi"" I. U"al1llllpbenoid,lllIrgtry (whe" thllaureean ltan- (e,g. HemoClipa®) 3. chemical hemaaLluit ; >H btlow CHEMICAl. HEMOSTASIS
Su ...."iew' {or mOre infonna\ion . Some kel' pointa:
I . gelatin sponge (Ge lfoam®;' no intrinsic coagulating ,,«""t. Allsom. 45 times ita wO!ight in blood which C8UI!ft it to@qmndand tamponade blt>eding. Absorbable 2. OXIdi~ cellul_ (OxycekKl) IlJ\d ol location of the lorta b, level orpatbGlogy il poIIitionoed ov~the brenk in table(to get I.h. iliac (;l1l:IIt 0\1.1. ofth .. ",a), durinc initi.l appl"Ollch . ",member to unbruk table bo!;rore fuI.1 imtmroent.lltion!). Key: .h ouJd~.
and pelvi, U\MI vertlall f.... lI"·ray locali211tl0\"l t . axillary roll d. nell" the upper thillb (til relax pIIOll nlUKle) '" knee eo stabilized ",i\h tN..,. bag (keep pal.ien1.upoHd from midl ine ant.lrior to posterior) and wid. acihuivi tap" over p.w. It , houlder I.!Id thii/l (keep illic ~r6llt el<poeI!d for dono. bone) f. UN II fluoro ..,mPl'tible tIIbll, or plaoe pat,en1.on table fe'lI!TMd 10 ptl"Ulit &«eM by C.arm 4, ir>cieion" II. to aeee» L2 IIl1d below: obl.ique ioci8ian . tarting anteroin feriorI), It the edge ofUlI~"" mutc:l. utt'ndlnl cephalad and poll. II::riorl)' through thebed ofthe 12th rib,ending paste riOlIy 11.the vertebral mUlINllwre b. I'poa:~ of l.l: I ., . for iftlltnlmental.ion. ,..qui .... utendi1lg the indlion c:ephal-.d through the bed of the lou. ri b, and ma)' reqWre radiullakedown of the disphngm for full expoll~
6.
approlllch~
ao the 12tb rib c:an be divided "' diAtt.iculated poIItenorly. I nd mey boI uud ... I ~ 01 bone for bll)l1 b. the kidnll"Y and \IllItII"r .. ,.. retracted acte-riorly, the pAOU lIl"..cle .. . IU-de 23.5.
Craniotomies C RANIOTOMY PRE-
& POST-DP MANAGEMENT
RISKS Many rilkl eanrtQl lie genernlized for all o;raniotornieillUld are ape~ilie to varioualumon, AIle IlT)'IJI1lS, etc. General in formAtioo! 1. po9t.-operative hemorri1Bgl! A. oveNln ri ak ofpoal-op8rstive hemorrhage'>1: 0$-1.1%. 43-60% nflhe hemato,ru" we ", intrllpaTllndlymai. 28-33'lo epidW1l!. f>. 71{o . ubelural , 5% In· 1,lIl8l1a. , 8~ mi>oed. 11% confined to euperfidal w OWld . Ovarnl! mortality wo! 32%
a . i1emotoma may 1l«U1 at
the.urgical li~
or In remot.. Jocat.ion'. e.g. Int rae-
!'crab.llar helnDrri1age all4lr p\erillnalllUld temPQral ' craJliotom lu (ue pase
852) 2, in CTW)iotoo\y ror brain tumor'"; A. risk ofanellLhetie oon,plkatin".: 0.2% B. tnc!'e8led neurologic delicit in hi 24 hoW"ll post-op: ~ 10% C. wOWld infeet.ion: 2% 3. poat.opetBtive headache (see ptlgf 6(1.1 )
P RE-oP ORDERS
for t umor: if patient Ol: steroicU, give ~ 50% higher dose 6 hili before aDd on-calJ t.o O.R. (atress doses); if not On steroids givC! de.r8met b8sone 10 mg PO 6 hr8 before IlI1d on ce ll to OB. {in A.M., give with aip waUlr) 2. ifalready OD IUltiepilept ic dru.g!I (AEDa) CODtinUe l ame d06e6. IfnOl on AEIb, and cortical iDCu.ion u li cipaUld, load with raJ PHT(may givfo 300 IDi PO q 41u11 Ir 3 d-... (total 900 n'i) to load orally) 3, prophylactic antibiotiCll on call to O,a (oplionnl) 4. recommended : pneumatic comp",""';on boot.ll or knee-h igh TEDII ho!W! 1.
P OST-QP OADERS ~ddiIwi (Individ ualize 811
appropriate)
1. admit PACU, tranafe. t.o ICU (neuro unit ifavailable) when l table 2. VS; q 15 min x 4 bra, UlIUl q 1 hr. Temperature-q 4 hralr 3 d. then q 8 hrH , Neu.o check. 'I 1 hr 3. activity: bed real (BK) with HOB elevat.ed 20-80" 4. rem<We elastie leg wrap4(jfpre!W!ntl IlI1d repillre with ~ high TED hose or me pn eumatic compretlli C. l ubciu.rll D.e ..... tolD8l 2. oorebral infarction A. arterial B.
'«!!lOu.
infan:Uoa: apedall)' with lu.rgeryon or around che VenDue ai n UM.
!.... -'P'l4~61J)
3. • •
Ii.
6.
1.
8.
poortDpUati"" wizure, III.Y bot due til modeq",,~ ant!oonvulun t levels, and mIIy bot exaeerb.t.ed by any of che.bovt. bIN M low (or l7UUIaf'ed drainage of blood Bod CSF out of!IUrgiealsite enbaowd venQ\U drairlage wb ich belps redu~e venous bleeding aod sl 80 ICP eBiy vontilation d ue to unenculllbered chat patient's helld rna)' be kept exactly rnidline. lliding ope-flltor ~rienuu.ioo, and reducing risk of kinking ofvertebralarl.eriel
23. Opera ti onll and procllliun!II
NEUROSURGERY
DisadvRDtage.!!l'n sks I. possIble ai r e rubol i~m!.H1 bdow) 2, 3. 4.
A CSF leak may occur thfOOgb ; 1. the skin in~illi~n 2.
S.
via tha euatllctJaD tube (Ull /XWr 435 for poNibla rouUIf; of egreM followu.,.ub-occipil.Dl acoustic neuroma fl'lIlova l); A. through the I\OBe (CSF rhinorrhea ) B. doWD the back of Ow tlm>at tbe ear «:SF otorrhea) in c811!!8 with perlOfat.ed TM
l'Teatm.mL; IniLialtfeatlDBD1 meuunllllo temporiza in tha hope thot CSF hydrodynamiell ... m normalize andlor that th$lelk aite will ~Iir dOlled within a few daYl ; I. e levalAl tha HOB 2 . hlJllbar .uballchooid drains,. 3 If the leRO«urs thrtIugb the , 1rJn ulI;I.lon: A. ra;DfOm! the ind,ion wlth ...m'fN, aoJ. n.ulnLDj'loI:kfKi s.o nylon after prepanlim of thl Uio with antimiCl'Pbialaod local lUl.ellthetic
B. altermoli·,ely. the inasion may be paint.td with "vllral COIla or«lllodion If perailrleul. II. CSF rlltu l" Il!qulrea 'ursiea! cornc:tioo, _ CSF ,btu/fl, pag' 174 for po,n l intonnatioo. '"'~ 436 for CS F fistul" fulJowinll .uboecipi tal ~oYa l of II.COWItic neumma.
Fiftb o r lleVt'-Dtb De ......" illjurie.
C.~ diminiebed rorntoll ~nn with potential conH!a] u]~entiun; initla]ly man· .ged with ;lUtonie eyedroPl' \a .lI. Natural 1'e~t4) q 2·4 hn &. PRN, or with a ruuilLuri2· Insert (B.,. l.Icrioertlt) q dlY. IfId III nlibt with an eye ploteh or csping eyelid ahl.lL
in,
M.isct'-Uan e()u, s...pntenl.Orill
in~",bn]
Inn.jent hyperten"ian" .
ft!EUROSVRG£KY
hemanna", has
*" dH '"
Ad vantages Spartl!l8U\UI ami owlogic IIPPlLnltU''''. traction.
Mlnimi:r.e~
rerebellar and temporllilobe re-
TECHHIQUE
See reference""
23 ,5,7,
Approaches 10 the lateral ventricle
Classic ~view" '. "'·101 aummariU!d: 1. a tnum (AKA trigonel; PUrneroUB approache.! ipdude: A. middle temporal jlVt'US: through the dilated tempol'1l11wro B. lowral t .. mporlll parietal C. ,u~riOT Pllrietal occipital D. trllJl5Callosal (sa ""low ) E. traru;t.emporol hon:>: .~te" to temporal horn i. via lobecl wan. otu.,rd ven COMPLICATIONS L vpnous infarction, WIly be due to;
A. 8acrifice ofrntiCliI cortical d.aining veins: plan !.he nap to avoid thia witb pnooJH!t ative llIIgiography, Or ";Ih sagiual T2WI MRI images'" B. liuperior wgilt.sllinua (SSS) Lhrvmboais". Factor& that may oonlribute t.o .in ul injury indude.": 1. ,!\jury from retndor: ."aid placing I1!t.ador (\I:l BinW! (deformation IIf midline .hauld oo~ exoeed:; mm) 2. over·tetnction of the d.unllrinUB nap or on ssg ilselrnal '"
aytw m, '!'hila, with norma l ailed vtntricl el, the third ve ntricle and region orth t~n of Monro II'!! bet~r apVrwcl!.=d Irl" u,enllo-a1ly (n ApPROAC HES ] ~"'rior PI' netal a. rohldl, t.emporll] ')'1"UIt! u.&llrul whllO ~mporlll h4m ort.l.4nJ ~ntriclt hi dilated due to hy d roce ph ll.1u.1 caU$ed by the llUllor; IIQCft.I; I. tlml",h tha telllporlll hom 3. m iddle tron l al lYT\l.ll a pproa-ch : a 4 em inddon illlUlde pDrIlU~ 10 the am of lhll. middle fronl.ll1 gyrul,II.b411i1and anterior to the exVreui ....peed. fflIter (flrocR'.al'l!a) Md anteri 4r to tha motor . trip"(lboul the .a n~ pointa. used ror frontul ventricuhlBtomy, ~ KI1CMr'. poinl,P"Ce 620 )
23.5.9.
Interhemispheric approach
INDICATIONS
For le~iolUl abutting DO midline, deep to .,uface, but .uperlicial '" CU'l'''' c.llQf" m (les[orw that can "raU a way" from midline). Simila. to Im llSC8UII6&! approach above, D eep! thai the pathology 23. OpetlllioD. and procedUre8
infedion in rqioa dftired for puncture: choose Mother aill! if pouiblll eoa""loptI\.h.y A. plat.tlet IlO\lDllhou)d t.. ~ 5O.OOOImm l t-: pop la) 8 . ~limt.bou,Id DOLbe OD IIlItieoaculaot. beeauae of riel!: of epldu,al hemato-m a (_ J1GIJe-35J ) Or l ubat'llc:hlloid hemorrhage'- With RC.IIIldarycord eon>-
prMSion
5. ao,,!.ion ill I UlpecWd _u.,......a1 SAM: uo.uivl! 1o"'Inn,oI'thI!CSF p~ure in· cree_ ilia traaamw-.1 p""uu.-. (preulln! eaoM the 8JlNZ)'lm waU) and may pmc:ipitllt41
",",pl.......
6. cauUon in patitmte with ..,mplele . pinal blodl.: 14" will deurioraUi after Lf"I
"Iev.ted l CP lIIIdIor paplJledem. by th"mHIVH In liQI coutl'llindicatioM te.g. LP i. ,dually II~ diagno.:tiaoUy and at II trulmotnt in Hiiop;athic intnc",nl,l bype"'.nliOll, _ b&IouJ TECHNIQUE
Bflckg'round and anatomy The _pina l cord and column Ire tha Ultle lenllth in II 3-month fHue, after that the spinal column crow, fult:r than the cord . ..... reaUlt, the conWl medullIn. ia Located l'OItt,,1 to the Wrulinatioo OO"tha thec.1 sac In the "!ult. ~ eon .... is located behoeen the milkUe l him.ufthe verttbrIU bodill ofLi and L2 in 51-68'J1,oradultll (the mcucommon 100000tion,l, TI2-L I in _ ~, ao d L2-3 in _ 10'A0 (with 904'J.ofcords UInnina~ .".ithin the unitol')' otLllltld L2 vertebra l bodi • • )'". ThelhKal RBCa.M _ 52. ~iDtflreristal l l De (conneding lhe luperior border of the iU". e 173). Ir ,poreial ~ultuN!1 att reqlllN!d Ie-V. acid (un,aI, "irlll ) th"y ' " .Jso.~ itled oa the tube for tu)tu ra"'~liIJ"i ty
.""ty.....
T. bl.23-3 Ro ulln, ..II. for CSF
'"p.
r.t.
tC.t.S).
(fCSf' torrylOlOfrY ~ dn'red re., . to RJO u.cin!>mllto,u meninJ;lt" or CNS Iymphomal. then at leut 10 ml ofCSF mull be lenl in ooe tube to pIItbology.
COMPUCATIONS FOLLOWING LP Th, D¥m'all ri~k ofdiuhllnl or persi!tent aymptonu ( 1 days, c"n illllle"" pal,i." ~r tll.lcerb!l.tiOQ 0{ p~ellistino! M l.lrologice l disuse, prolonged bIIck pain._pUc meninriti., and n.",ato l>Eedl~ witbl>ul . lylet (~lanlinz a core ,f epKIennal tie...)"'" 7 . impingin, ne ...... 001 with IIftdJe: UlIuaUy tllUSK transiol!Ot •• dieu)" , pa in, may ClllSI! poennal'K!nt r1IdirulOplltby in lOGIe 8. iEUJ:a I
HEADACHE FOI.LOWI,'JG LUMBAR PUNCTURE For charncteristi::s orl.be HlA and treatment ""~ fXJ!~ 40. Risk af"""t·LP lilA (PI.PUA) is rela ted to II own~r of factors including:
Facto .... olltl; ide tile c<mtral ofthl! physician : 1. age; I In younge r patients 2. sex.: 1 in femal es 3. pOOl" headache hiatory (includ.inK previom Pl.PIIA) 4. body s;u: I with small body mass indu = weightlheight"'· 5. pregnancy Vlriable8 that Ita"" baan .hown to iflOucncc the incidence ofPLPHt\: I. ne.edle . i!Oj; larger needle/! I:IIJ"ry' ;n~Wled riak" 2. bevel orie ntation : orienting the beve l paroneJ to the lG;lgitud;mllly running tiben oft.he du ... reduces the risk of PI.Pl:IA" 3 . replac'ng the ~tyle' pdor 10 needl" removal lower. the illcid~nce" 4. the n umber afdural punet'll .... (may not be totally under the phYBician'1 control) Variables thst mayor may not Influence the incidence of PL?HA: I. needle typ" A. Quinckeneellle: bevelfed edge with cutt ing tip Ithe 8tandard LP needlel. l n. cidence cf PLPliA with 20 and 22 gauge Quincke needles: 36%") B. atraumotie needle~: a n!.lmberoftype8 are aVlilable . Most are "pencil poin~ !!d" and IIIay produce II hole with e lawer incidence of tranBdural leak" . UI'proven" Factors found Ml to affect the incidl'tnce ofPLPHA; I . the position of Lhe JlIjtienl al\er LP (doe$no~ see m to prevent PU'HA. but may delay the onset of symptom.... " I 2. .al ume or fl uid rem!lved at u's lime of loP 3. Ilydration rullowing Lpn
NEUROSURGERY
23. 0l"'rllti""5 and
pl"OC~UrES
'"
23.8.4.
C1-2 puncture and cisternal tap
lndlca tiooB Situatious wllereCSF s~imen i. required but I ccessvia L.P;. difficult or con train. diGlited !lumbar arachnoiditis, marked obesity ... ), or to inatill conlrUI to d"moMtmt.e the I"06tral utenl or. block dt'Curoented by dye ;l1,jected via LP. Spinal heada~he is le88 cammon with thue procedure. than with LP. Cl· 2 puncture is safer than cisternal tap. tI Con t raindicated; in patient with Chian malformation (o!\en present;n lDyelomeningoa!le) due to low lying cerebellar tonails and medullary kink. Normal CSF valu" for ,Juense end protein dilYe. only s light ly from CSF obtained by lumbor PUltctwe. Opening preMure8 averaged 18 tm arfluid with lateral puncture.
C1·2 PUNCTURE
AKA latersl ce.rvieal punCI.ure.
Equ.ipmeot: LPtIllY (useful for the epeeimen lube~, e> '"
23. Operat ions and ptooedllteS
NEUROSURGERY
Th ... needle must be supported more tbll n wi\.b 8 lumbar puncture. To hUec~ iod inated contrast, use e.g. _ (, ml of ISO mg% lohuol® for ce""';cal myel· ogram, watch dye on n~oro (6hould be able to ..... it in 8ubarat.hnoid apllce),
Ri8U
CllSelUI,."d "'AI often uud 10 Lip these) Dandy', poiDI; 2 CID &om mid1in~, a em above inion (m8ybt mOll! p«In' to dam3.
2. 3.
•
e.
age viaual pathways t han a!love) Roche r'. pelin l (eorc:naJ)! p18_ cathell. III f"",~1 hom. The rillht .id, I' IIIUIll, edi91 canth,. ofi",ilat~nl ey. a nd In AP plArte towlll'di EMI C. i ....rtion length: IdvillW:llClthet.erwithnylet unt.il csr i , obWlled (Illouid be c 6-7 em depth; thla ruay !:le3·" em with markedly dilat.cd ventr'iclea ). Ad· VIUl(lt ca~r without Uylat 1 em d~r. • CAl1t10N: irCSF iii not libtiline 23.9.2.
VentriculostomyllCP monitor
AKA ;lllra-..!ntric:ula r cath.t.et «(Ve) or utcmal ventncu/a. drainage ( E'VD).
INSERTION TECHNIQUE
Unleat conttalndiutad , ..,. rigbt .,muiewM bllM!d), thI! right r; make twist drill hole. Bont-waxedga to 5top bone bIeo!d. log; uutl!riudura with bipolar roaguLltor; incise dun La tnlcilte fasbion with ' ll !!CIIlpel blllde; caut..nM m.a:.ed dunt.1.,.q... a04 the .. piala.uhnoid ",itA bipolar For ventrieWo.tomy: i ...ert catheter lItrpc:gdirul .. to b .... i...Ufface"' 10 II depth or 5-7 CIII !most a thele .... are marked at 5 and t o em). With .ny wntrieulBf t nlaryement, CSF lhould!low at IeQt by 3-4 cm depth (with oormal ventriclh.lhi. m.y be 4-5 nnl. 1f DO CSF il enDDWIte/'ed here and theutheto/:. It ~ funhl. until CSF iii obtained, it il unlikely to bedu. tocathll.eriutian oIfrontBI bortl olnatel'lll vtntricle{in thi.I ca.M,.t. _ &-l l eru thoo Lip will often be in the pre-pontine Wtllrn. 'Iubllnochnoid spKe. which It undesirabla). lfunau_rulllfteJ' a mulllllUIi of three Ilterop&ed p. . - . thM placa. SUM",c:hnoMi. bolt Or inneparenchymal moaitor. Fol' nced period. (up to 4A de,.) with _"",bl)' low In feo:tion " ". The UlM!olr a one-wlY "alve, CDnt.inuou.t antibiotiCl (arupkillin end clolllciLlin) and met.ieulOid technlqulI wal n-edLted (or !.he ltd or infKtlOll.
23.9.3.
Ventricular shunts
Ventricular cathder .Qm:iailql.burtJude I, tilled ;n m..t eua!'or Insertion •• w ofventricul.oor atheto/:. l.ell V~"lricuIDr co liule,izal/on palJ 2.
3.
-)
lit 1l'!Ult 24 hra. due to i1eu!l !'rom manipulation ofperi tonl'um) ahuntserieB{AP & lateral fkull, and chest/abdomina l :t· noy) as baseline for future com pari&Ol\ (6oDle surgeons obtain these films immediately po&l-Op in csse some i,,"nediate revision is indicated, e .g. ventri~nlar C8thl!U!r tip in temp third ventrieJo:> using tl,e ,·entricul0800pe.
23.9.6.
lP shunt
T eCHNIQUE OF INSERTION
St!e referenee"". LP SHUNT EVALUATION
Do-a]uation offlmdloD ismore d ill",ult than with VP L!hunt s~ an aCOlWi devieeill not alw.}'II inserted. I . It is 8Ometim"" pDMible to tap the t u bing with e 27 C"suge bu,terlly needle 2. ·.hunt--o·~8 m· A. with il\l~tion ofwate' -80Juble cont.ut"; perform LP jUllt above 0' billow level of lumbar catheter. The presaw-e may beO 0. negelive, &lid it may be n~~saary to "Pll"lLte CSF to confirm. plaoement.lnj~t 10 1111 ofiohexol Or ) 250 mg meu1l.amjde aod monitor the flow of COlLt' ''"'t fluol"OllCOpica.lly a. the _____ pot-ieo t ill brought vertiCIII . Coughing or "alssl,"D. mll.lle.~ver will acoelerPt8 Ill .. 00'" of eootnut B. NEUROSURGERY
2. problema with ..ound healing; \he ~n.kJe; •• nowriou. eegion for poorcir.:ulation and the lose OfHnsatioo ifrom the diNaH Of biopsy) may !"i!ndu the BreD lubj«t to re~t.ed t .. WIlD without the IHotl~nt being .......... Furihennora. OI~n)' pIIuenta with 8n undiap~ .,.temic diiJtue r~ uirlnll. lural nervi biopoily will have poor wound healin, (a .ignifiC1lJl~ nWllber UI .110 diabetic) 3. failure t.l make. diq:nOllit; although boopa), may be able to anlude lOrna eon!;npnciH, It onen does not wake I spedrlC d !'lPIosi~
23.11 .
Surglcalluslon 01 the cervical spine
TeclIniquH mcLude: I.
2.
CI and C2 A. atlanlOUi.J fuatoa 1M! below), IIOmetimel with inCOrpOl'IIlIon oItho O 01" the follttwinr techniquu i,.-ed; I . Broou tu.loo techniq .... &lid ita modif_tion.: Jattral.-l ........ bl.roi· nar to both CI and C2, with wedgl bone IBn.. Cae bdow) 2. C.IS. ruaion teflow) Inlenpi"., ... fusion technlqu~ of Dld.m1Ul and SonnUlI: t«< ~ 624
«.
B oc~ipitoceO' ;e.1 fwion: btlow 2. C I·C'.l llltenl ..... sa fUlion : UlIIIful in caBell of incompetent C I when> nnly the pas. tenG/' . reh 11 C(lmpromlaed 3. H.lifllX dllllljlll with fusioo": th_elaml!' 8ueff Indicatkln for occi pi~rvical fu.ion (veri ... CI·2 flUionl ...· 1. wldespre~d bone dealru elion 2 . a~enu of II complete IIJtb orC I A. C(lngenltal B. poal.dl!COmprenion" C. posttra umatic: "bW1itinj( Cl frac ture (bUatenl o. mtilliplc Cl nOI fno~· tllrU)", NB: wme fe,el th.t t,hi , mlly be ..tiara Indi cations
R...tucible ooorllo;d Type J1 't.cture (.nd 'lYPe 111 r... ctnru wllere th s (ntt, ... e line i. in th"""phalad portion ofth. bodyofC2 in an elderly Pllt"!; t wllo mlly nOt flUe,. well wit h immobilil8tion ..... )'OlInger patien t ''') w;th . n inlI.~t ttaMverll"lig.menl. A con-
NEUROSURGERY
".
lrvcni.1 rvquif'lment i, age of fracture .. 6 month. ald.
Co ntralndica Uoo. 1. 2. S. 4.
5. 6. 1.
8.
f1'IIduI'N orlhe C2 vertebral body (eu.ept cephalad Type III fracture) d;lruplioll ofatlanlal tnnWf!I'!W! ligament: .. demoo~ITllted on MRI. A1!1O may be ',,""ie4 irthe RIm of tke 0¥8rh lDG of the IlIum.1 "'lIues ofCI On C2 Mceed,1 mm (Nie orSplnce. UfI fJOI~ 723) Ilrgl odontoid !'r..cturl liP i~uc;ble ITactur. ~me lIuthort con.ider I nOli union It 3 WQ old as D ronltaindi~tio n due to tns lower I UC1:t*l rlt.! pltienta with !!hort, thick n .... k. and/or bllT'Gl chut: mak ... it diffic:ult to acl>iIlve !.he properllnille. May be circumvented hy thl instrumentation di1;tributed by Richrd·Nephew which ati iin. cannulated flnihledrill. tap llrnllK:rewdrivet I'a!.halogic odontoid ITadute fracture liDe in oblique orienta t ion to tTonllll plane (ahelU'ing fol"Cfl C&.ll tause malalignml nt during acrl'" lillilteninll)
Poat-op POllto~ra t i"o ImmQbiliutiQD: the Inun.dilte poet-op IlnInsth of the odontoid ... serew i. only. 5O'Jo orth. n01'D111 odontoid. Tharefore, a ce .... IClIII brKe ia n.comlllended fot 6 w~h'" (although SO"'I autho", some don't un one'''). l h1le patient hQ aignilican t OlIteoporOlli •• a halo brace il reoommende(! .
Reault. Healing takes _ 9 mnnlha (or lnnger with chronic nonunion). With fradurH .. 6 montb . old. t he union rate wu 9MI•• Chronie nonunion. :> 6 month. aid hive ... ~[ft· tant risk ofhardw/ln f~i1ull! (~cr_ hNlaupor pull-ou ll. wlUi .. bony union ral.eof :U'IIo, and38110 rat.! ofprl!lumed fihroul u.nion'''.Thul,in _ofchronic noJnuoion:> 6mooth. old. C I·'l anhrnd.ll is prooo hly. bett.!r choice unle" U\.II need 1.0 maintain motion ia wonh the riek ofneeding I IftCllnd ap"noliol'l ifth .. ona r,,;I, . Thllavenagll kdmicaJ c,;u;lIpllcation note i• • 6\11o (~IeI'llW roalp051tion, 1.5'110 lCfew hreakout),
C1-2 TRANSARTICULAR FACET SCREWS (TAS) May be UMd III eo adjunct topwterior CI -2 wirinll and bona ,...nfa., . Ied!.,.quaof Dickmen and Sonnl.llll, _ ptJ6t 621) to achieve immediate 'labiliUlUon w;Uiout tbe need for posl 2. wound infection S, fracture 4. e""n'etic defonrnty !'>. increased time to pr-oeure 6.
MUr(lv88Cul .. tOmplie Te(:boiQu e \. olter r3;~;ng. skin wllula! the d"";red l!'Yet in the PAL, iluert a 22 Ga. or .rull. er needle directly .g.!>i nst the rib 2. walk the needle down the rih millimeter by millimetu until the needle jU"11i1» under the ri b: to avoid pleura.l punct,,-",. do not twlvlUlal!.be needle more !hili) o".,·eigllth inch deep to the "'nt.erio. lurf."" or the rib 3. aapilllt ....
"'.~0ItUrr0 l1, n .. T.IYJJ. e._1P.K...... HII O"""' ...... _ol _ _ out "''''' _ _ JA.,",A 114:~ . 1960. CMtMm"OTCE. _ _ .....ir o l - . . . - l ~ A ~.OI ......"'"'.....,..19
."" . . """G.,.. .qo ..
101· 1".
-
_ _ ............,.I.. I"~_"" U).J). I_
.... ,.,.,...., .......
("
". ".
MoIool., M S. WtollOo C.. "'....... 101. " . ' 101M" oI ...-tof ......... "'10.< .. _ _ .... ;... A.. J OIOI ...' .... I ~ . ~1.. ! , l Pn,
" t.I·»1 l('lboIH , l.lIIlola ............
).0·1. 1..... ' ....... IO.S,...,...OC. _ l f i -.,.,..;... _ : A ... _ _ , .... idt... U)6 • .J.
.l6-!6. 1....
_ J I ....... IIK. ........ A ... 011·~~ .... _ _ .........."A...........-........,. J
B_" 1 A. 6,... M .... NlIaQ 'l c: 1')';lomoJ . in. !O'yaI." ,. efIlkl .... ".h _ ''''''''''.10J''1or ' '."on.1l.11IIW. 1.1I . bl~'t 0/ "'''''''''.1 Nt~'""""" 77: ll~
:10.1991 0., ...... C A. ():o..-/.. ~ N R. 0 ..... lt1 A C U." "j lli_"..;OIIJ <JI.... ofb.otQo'aI ~ _1. 111 ........ IloIf>.' l.lm 1'/11 • • "" I "'1 . 11lJ. V H R.,001 .... Ifinol .. p"",,,,,,,I"'''''''_f ..."",lat_poeu,... J 1'Ic............ )oI.r7) .. , 1911. a .... S S. H.na- 0 A. v." U,l1i. A...... R D. V,,"" M, PrlDdpfo,o of lMUl"oIi>,u • lJlJod M~·Ki".,. • ..-Yo"'.I'I31 WI ... II, R... ON.Silo.,AL.I!QI.: u""",fI'I'""" '" .. iII"JII'P'0_'" tol< "","li', A" OIIllyoi. 0/ ,1o:. boI ....,..,. 101, 39Ii• • I/Jo')l
K.............. A, CIlnln' ..... _
of ~h •
III01mo1oc1.e; ........IioII.2NI 0/1.-0.- ..... !IW . ",",Ne,.. VOlt. 19111 .
VI,ldtt·SmlUI E. KodIbwtr-MOI'J'elltrl. UmmJo oIIil ..,; •• .." ]00I ...... l .. ~ fl 1''''''''''''' .rr""
--..l"}'
,98!I.
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... ~.
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_ . J ~............. 7S: i~Z·1.19I11 . GNijo ••
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••
Second impact aynd rom e (SIS) A rare condition that has been described primarily in athletes who sustain a se(ond head injury apparentl)' while still symptomatic from an earlie r injury, end in whom subsequent malignant cerebral edema develops which is refractory to esseoti.lly all treat_ ment efforts and carrin a SO-100% mortality. Classically, the athlete walks ofT the field under his or her owo p:.!wer "fulr the second injury, only to de:;eriorate 1.0 coma within I_ S minute.l and t hen. due to vascular engorl!'emellt, progresses to IK!ruiation . The uistenceofa syndrome compatible with SIS WaS first described by Schneider" in 1973, and wBa later called the "second impact syndrome of catastrophic head injury" in 1984". Even though it has been conterwled that SIS;s rare ( ifite~i9ta at al1) and may be overdiagnosed", its prediJedion for teenagers and children still warrants exira precaution following ooncussion.
Retu.rtl to play guidelines T/.blllJ:l:4-3 CerebrAt contr ..ln tttc"lion.;; l or , .. tum No sy.teo> of ..... t" rn 1.0 plAY (RTP) guidelines h88 been rigor10 contact sports nusly l -,. '"
Ma nagemenl opUons lor III s Ingle sport'-N!iI"'· ed concussion MlInagement Op~ons"
removefromconlesl •• exarrine q 5 mins for amnesia
remove from conleSl •• disallow return ilia! day examine OIHlite ItequenUy lor signs of evolving Imtarctanlaf ~..."
ree.am;.ation lhe •• CT
..
••
• ,.
e.
ne~ day
by a IraInrKI lnclividual symptomsworsenorlasl> 1 weekt return 10 jIfaC!ice aller 1 r~ week wiIhou1 SYfl1'lOOlS. arrbuIance uanspon from field 10 EIA ~ still unconscioos or lot concerning S;!JIS (~ 1lIe 1 minr.rtej Q/ade 3 OOrlCUSSlon, rell.lm 10 practice only aile< 2 M weeks wiIhou: CT or MRI ~ HlAOfoller~omsW!)(Sltl Qt IaSl> 1weekt orMRI ~WAoro/llel
syrr¢orns.,
evalIIot;on at ' e" and ... !~ e _ {see reXfl S&IlSOrl is Iem"inatIlCl lot tNlt play .. H CTIMRr _ ."...... tentially dangerous (su abo",,). ~mmendationsU for multiple concuSlliOll. in the same season are 8hown in Tobk 24·5. Also. see Ch"m;c IroutnlJl~ en""phal()p~lhy, page 683 for long·term effects of multiple concuuiona. N e uroim agi ng The nll'ed for neuroimoging (e_g, CT scan ) in the athlete with resolved or improying 5ymptorn. is cont roversial, and is felt to be beat lef\ to the judgement "fthe treating physician. Suggested indications; 1. a seyere concussion 2. symptoms persisting> 1
OJ,
"
wilhovl symPtoms .".or ,
11(1
with _ion 1_ r",,~
Hany 1IOJ!e abnoImal ~i M on CTMA! ; _ . 1NSon. Consid.. 8f>(!irog .. pMicrpalion in conrtc1 $pOrI.
24. Head trawna
NEUROSURGBRY
3.
week, even if mild before returning to competition aRer a 2nd Or 3rd concussion in the same season
GRADING HEAD INJURIES
Despite many (va lid) Minimal -
mO .
S tratific atio n' There are II n umber schemes to stratify the !leverily of head i.r1jury. Any such cate"",riUltion is a rOi· trary. A 8imple system baaed only on GCS score iS8S follows: CCS 14 · 15 ... mild,GCS9· 13. mode rate, and GCS ~ 8. se· vere. A mOre involved system" incorporates other focto"" in oddition to the CCS score as shown in Tobie 24·6. A classification Sys tem based on CT scan" is shcrwn in Tab/I N ·?
Moderate
__
_
•
( GCS ... 9-1S) OR ( LOC"Smln) OR ( Focal neurologic doIliciI)
s.,.. ----. ~ Ctilical
---_ (GCS~3 - o4)
all alteria In 811)1 oval musl be mel 10 quality in \hal category GCS . GI~ A. SSP < 90 mm K8 may imp. iT CBP ODd •• acorn. ... brain il\i~ry ODd oho" ld boo . " 60 mm HgM 2 . true cerebral edema: dllSllically IItllutopsy tbeS NEUROSURGERY
24. Head trauma
."
ati "" may be employed, howeve r, this will preclude following the pupils for a variable period of time, and should be undertaken advisedly (SI: NEUROSURGERY
CT scan is not usually indicated. Plain SX.Rs are 00 recommended: 99.6% ofSXRs in this group are normal. LiMar non-displaeed skull fractures in this g-roup require.llQ.. treatment, although in-hospital observation (a t least overnight) may be considered . 2.
MODERATE RISK FOR INTRACRANIAL INJURY
Possible findings are shown in
Tobl~
24·11 ,
Table 24-11 Findings with moderate risk oflCI
Table 24-12 CriterIa lor observallon at home
history 01 cMnge Of loss o! CO. significan1 subgaleal swe~inot"
1. normal cranial cr" 2. i'liIild GCS,,14 24-11) except loss of consciousness S. pallenl is now neurotogically inlal;$ of swelU", 5. skull fracturt!!l: A. bao;.al skull fract\lres (incil>ding temporal bone fraciu ra) B. orbita l blo ....··out fracture C. calvarial fracturt (CT may miss l ome linear nond ieplaced sk ull fracluru) I . linear VI. ltella~ 2. open vi. dOlled 3. diastatic (aeparation Of . ulu .... ) -es and paralyt-
«(>I in neu rotrauma patieo.w may lead toa high. er incidence of pneumonia. longe r leU stays, and possibly sepsis". These ag~n~ also impair neurologic assessment...... USf! should there· fore be reserved forcases with clinical evidence of in t racrani al hypertension (Sf~ Table 24·14), or where use is neresaary for traasport or to penn itev.luation oftke patient".
llems A-C. .-present CliniCal "'II"S 01 ..... niI· lion. The mosl 00"'"""'''9 o;IItIicaI_ 01 IC-HTN is !he wiln_eo evoMicn 011 or _ 01 these 0lil"" IC·HTN may jYOduCO an r..-d
'"
,.tho, tho"
24. Head trsuma
NEUROSURGERY
depreosed level ofconscio.usne$$ (patient ~flIIno.t proteccairway); usuolly GCS" 7 need fnr hyperventilation (HPV): 8U below 3. severe maxillnfacial trauma: patency of Dirway tenUo.u3 4. need fnr pha rmacnlo.gic paralysis fnr evaluatio.n Dr management Cautions regardi ng intubation: I. i{baSilI skull fraclure ia posIIible. avoid nasotrocheal intubatinn (to avoid intra· cranial entry of tube through cribrifonn plate). Use orotrscheal intubation 2. prevents aS8encnent of patieors ability to ve rbaliW" E".g. for determining Glasgow Coma Scale $COre 1.
2.
Hyperve l)til a tio n (HPV) Options": hyperventilation before ICP monitoring is established shnuld be reo served fur patients with signa nftranstentorial herniation (s« Tnble 24.14) nr progrenive neurologic deterinratinn nnt attributable toe~tracrllnial causes 1. due to possible e~a~rbat.inn nf cerebral ischemia, HPV sh'1lIid DoL be used prophylactiCAlly (s« pag~ 659) 2. prior to ICP IlInnitoring, HPV shnuld only be used brieny when CT nr clinicol signs Df IC-HT'" ore present" (su Table 24-14 for clinieal ,igns) A. when appropriat-e indicatinns are md: HPV to. ~ = 30'35 mm Hg B. HPV shnuld (I9j be used to the point that pCO:"" 30 mm Hg (this further reduce. C3F but does nnt ne.:es!lllrily reduce ICP) 3. acute alkal08is iocreQel protein binding of calcium (decreases ionited Ca"). Patients being hvqlryegtilated may develop ionized hypocalcemia with tetany (despite nDrmal total [Ca))
MANNITOL IN
E/R PR,IICTICE PARAMETER 24·6 EAlllV USE
C~
MANNITOL
Option s '": the use ofmannJtol befnre ICP moaitoriag is ~,tabli~hed should be reserved fOJr patients who. are adequately volume·resuscitated with signs nf tn'lnstentorial herniation (S"'" Table 24·14)nr progre~8ive neurolOJgic deterinra· tion not attributable to extmcranial Cau&es Indications io EIR (also 8U ~ 660 for mnre details): I. evideo 8. which implies at leastlocllizing) A. operative nelll'08l1rgical ;nU'rvention i. probably not required B. utUi ze good neuroanesthesia U'chniqU e8 (elevate head orbed. judicious ad· ministration of IV fluids. avoiding prophylldic hyperv«J tilation ... ) C. obtain a head C1' Kan immediatdy post·op 2. i(patient has focal neuGlogic deficit. an eltplorawry bUIT· hole shou ld be placed in t he O.R.si multaneously with the treatment or other i'\iuriu. Pll'Cemeilt is gulded by the pre-op defIci t INtI EzplGl'lJlory bur, hoi". 645) 3. if t here is seyere head i'\iuf)' (CCS" 8) without localiling l ip • . or ,f initi al burr hole is n""ative. or ifL"ere i. nO pre-op nauro eKam. then A. measu re the ICP: ifIHrt I ventricu lGitomy catheter (l fthe lateral ventnd e cannot be entered alter 3 paSsetl. an inttaparenchymal fibe r-optic monitor or .u bararnnoid bolt ahou ld be lQIed) 1. normallC P: unlikely that a l urgical lH ion uilts. Manage ICP medi . cally and. ita rvc wa. iOMrted. wi t h CSY d r. in lce 2. elevated ICP (a 20 mm Hg ): inject 3-4 ceofai. into ~.ntriclfa through rvc, then ~btai n portable intraoperati ... AP I kull x·ray hntra-opera· tive pneumoer>C pa,.
24. Held tl"lluma
NEUROSURGERY
•.
ma ... effect with" 5 mm ofrnidLine shift ia explored" with bUrT' hoh!CI) on the lide oppGllite the direction of shil\ b. ifM maN effect. intrscrsnial hypertension is managed medically .nd with CSF drainage B. routine use of exploratory bUrT hole. for chiLdren .... ith GCS .. 3 haa been found not to be justified" INOIReCT OPTIC NERVE INJURY
• ~'II> of head trauma pIItienti manifest an associated injury to ..orne portion of the vitual . yUem. Approximately 0.5- 1.59\1 of head trsuma pSlient& will lustai n u,dirrcl in· jury ( M oppoaed to penetrating trauma) to theoptk nerve. mOllt ofl.en from an ipsilateral blow to the head (usuilly frontal. occasionally temporal. rarely occipital )"'. The optic nerve mDY be wyided inw 4 aepentt: Intraocular (1 mm In length), intraorbital C25-30 mm). intraana lieu llrOO mm), and Intr.crllnisl flO mm). The inl.racanalicul.r seament i. the molt cornmon one damaged with tlOHd head il\iuriea. Funduao;opic . bnormalit," vi sible on initial exam ind ic.tu anterior iTUuri.. Cil\iury to the ;nl.raocular aegment (op· tic di sclorthe lO· I ~ mm of the intrsorbit.l aegrnent immediat 1'l-ea t ment'": No pl'Olipettive I tudy hu been c.rried out. Opt icnervadecomp","ion hat been advocated for indirect optic nerve injury, however. the rel ult. aN! notdelJ'ly betur than upectant management with tbe exception that documented dlland villlal loM .p. pe.rI to be s strong indication for surgery. 1Tansethmoidal ie the accepted rouw. and i. u9uaJlydone within 1.3 weeks from the tnum .... The UM of"lIIegldoae l tel'Oid.- ru.y be appropriaUl a9 an adju""t to diagnatil and treatment. POST-TRAUMATIC HYPOPITUITARISM
Trauma i, a rare Ca Use ofhypopituitaril m. it may follow cloeed head injury (w;th or without basilar &kuJl fracture)or penetrating leaum . ... ln 20 ca_ in th.lit 24.2.4.
Exploratory burr holes
In a trauma patient. the dinicsl triad ofaltered men u l status. unilatenol pupill.ry dilatation with 10811 of lilfht ren"", and contralateral hem,ipareaia il lJ'oOllt often due W Up' per brainst INC1ICA T/ONS I. clinical criteri.: based on deteriorating neurologic eUm. Indication s in &'It (rare): patient dying of rapid tran, tentoria] herniation (sa below ) or bnoin8t Cboice of aide for initial b urr bole Start with a temporal hUrT hole (s« below) on the side: L ipsibteral to a hlown pupil. Thi s will bean the correct side in' 85% of epidurals'" and other extra-axial maM les;ons~' 2. ifboth pupils are dilated, use the side of the fimt dilating pupil (i( known) 3. ifpupil. are equal. or it i. not known which . ide dilated lint, place on side ofobvious external trawna 4. ifno locali2ing clues, place hole on kfiside (toevaluate and decompress the dam· inant hemisphere)
zygomati9%). When burr holes were positive, the first bUIT hole wIIS on the correct side86%ofthe time when placed as above. Six pstients had significantextl"aaxial hematoma. missed with exploratory burr holes (mostly due to ;ntomj"llete bUIT h~ le exploration ). Only 3 pa· tients had the above neurologic findings as a res ult (If intraparenchymalilematomas. Ou t come Mea n roll~w-up; 11 mus (unge: 1-31). 70 of the 100 patients died . No morbidity or mortality wa s dir~tly attributable to the burr holes. Fou r patients with good outcome and 4 with moderate d isability hsd positive burr holes.
24.3.
Intracranial pressure
24.3.1.
General information about ICP
CEREBRAL PERFUSION PRESSURE (CPP) AND CEREBAAL AUTOREGULAT10N
Seconda ry brain injury (i.e. following the initial trauma ) is attributable in part to cerebral ischemia {$ee&cDndaryinjury. page 635).1"h.e critical parameter for brai n function and survival is not 9ctually ICP, rather it is adequate cefebral blood now (e BF) to meet CMRO. demands (for II diRcussion ofCBF & CMR0 2, 763). CBF is difficult to quantitate, and can only be measured cootinuously at the bedaide willi specialized eq uipment a nd difficultY'"'. However, CBF depends on cerebral perfusion pressure (CPP), which is relste:! to [CP (which is more easily measured ) "' shown in Eq 24·1 .
_pag.
NEUROSURGERY
24. Head trauma
."
cerebral
pef(~sion
mean llJ1~ri.I
in lracranial
pressun:'
Eq 24·1
0' CPP ,. MAP ' - ICP
.....: lhe o " 1(1.15
,...
1'Taumatic IC·H'l'N may 00 due any of the following yoongcl' i!d18ll >7 (alone or in variolls combi na tions): 1. cerebral edema lerm itlanlsl 2. hyperemia: the normal response to head injury"". !he &\10 01 kAMillOO I,.... Possibly due to vasomotor paralysis (lou of cerebral "young' 10 ",Uder" enid Is autoregulation). May be mare significsnt than ede· no! peeisely delined ma in raising ICI'"' (su page 655) mav ~ o.uIlallll !J C
CT scan a nd e levated ICP
brtdyc:lriN
~aIoIy WrtguII~
Whereu CT fi nding .. may be (Offtlau-d with ...i.1t ofIC·HTN. no combination orCT find inp T.ble 24·', Ria. 'aeton lor IC· h8$ been shown to aUnw a«unlte eat imate. ofac, HTN with a norm.1 CT tuallCP. 60% nfpatientl with closed head injury a nd a n abnonnal CT" will have IC·H'I'N". S8PdlOmmHg Only 13% of patient.s with a IllltIIl.Il! CT scan decelebcalt or dtoonir::alt po:I&IUIJn;J on will have IC·HTN". If"""',""er, patienu with a mob".-.am (~aI or bila1fl~ normlll cr AND 2 or more riak fllctors identified in Tobl~ 24·18 will have ~ 6O'lo ri$k ofIC· HTN . If only 1 or none are present. ICP will be incnaaed in only 4~.
'11'>'0,.,.
24.3.2.
ICP monitoring
INDICATION S FOR ICP MONITORING
Guidelines"· ": brai n C1'" Or '" 2
*
,",uory 'CS."
••ry~.";'.
lIeurologic criteria"":: ~,.". h ... ."" , o,,';o,~"mo taloon l and eilher: A. an "bnorra al .dmitting helld C1'" OR B. II narmaI. CT. but wi th .. 2 of the risk rllCt.on in Tobie 24·18
som~ centenr monitor patient_ who don't follow commandl . Rationale: pa t ients who follow rooJImands (GCS '" 9 ) are at low ri$k for IC·HTN, and one can foil""", sequentillllf!urolofric h.ml in these patients and irus:tilute further eYllua t ion or t,.ettment but CoHTRAINOICATIQHS (RELATIVE)
1. • ..... ke· Pl'lIent; monitor ulu.Uy not necallry , can roUow II .... ", e~"m 2. coagulopllthy (incl ud ing DlC): fTeq:uenl ly seen In aevere head injury. Ir .n ICP monitor i, _ n tial, lake I taPi to correct OOIIgulopath) (ITP, platelall .. '> .Rd "- -..bo0..1
...
consider $ubaracbnqid OOlt Or is'~{81 monitor (an rvc or iMroparenehymal mOfliwr is contraindicat.e DURAnO N OF MO NITORING DIC monitor when ICP nonnal 11: 48·72 hre after withdrawal of !CP therapy, Ca u·
t ion: IC·HTN may have delayecal evacuation ilI - 0.5%" ·" 3. malfunction or obstructlon: with nuid coupled devices, hig her rates ofobstruct:on oc· Cu r at ICPs > 50 nun Hg 4. malposition: 3% ofiVCs require operative repositioning
Table 24-t9 Compllcationa ratel with va rious types ollCP mon itors
,,
INF€CTION wrTH ICP MOi'IITOiiS
aom~ SlWie$ repon this as iofOC'".ion, tIu! do 001 d IStin_ There is no consensus regarding guish tie_en clinically sognifieal\l inled .... on clinically signifies nt infection (venLriculitis or meningitis). See TabU 24-19 forcolanization ratn . Fever, leukocytosis and CSF pleocytosis have low predictive value (CSF cultu~~ are more helpful). Dura t ion: One itudy" found periprocedural antibiotics (cefuroxime 1.5 gm IV q 8 hrs for ~ 3 doses) were 8.lI effective as continuing antibiotics for the entire duration of EVD, and Were less expensive ,
Risk (actors identified"' ..; I . intrace~bral hemorrbage with intraventricular extension 2. ICP> 20 mm Hg 3. duration of monitoring: one study fOWld an increased risk with moniwr durtltion > 5 days (infection risk reaches 42% by day 111)77." and the ~commendation ....'as ",,,de to propbyl"ctic"lly ch"nge catheters .. , 5 d"y interv"l,.. A re<ent "n" ly"ia" found a non· linear increase of ris k during the first H).12 days after which the ra te diminished rapidly. with M significant reduction in infection ratA! in patientl; lU\derguing prophylactic change ofmonito,.,. at" 5 days 4. neurosurgical operation: including operatioJIJI for depressed skull fracture 5. irrigation of system 6. other infections: septicemia, pneumonia Factors Jl2I. associated ..... ith inaeased incidence of infection: I . insertion of rve in neurO intensive care unit (ill8tead ofO.R. ) 2. previous rvc 3. drainage ofCS F 4. use of steroids fuatment of infection: Removal of device if at all possible fi f conti nued lCP m"nitoring ill required consid· eration may be given to inserting a monitor at another site) and appropriate antibiotic.. . 24. Head uauma
NEUROSURGERY
TYPES OF MONITOR S
I.
Intrave ntrh,ul a r ca th e te r (tvC): AKA utemal velliricular d rainage (EVO), conneded toan utemal preuure tnned....,er vie fluid·filled tubinl. Theltanda..-d by which others are judged (a llO _ ltlINl~"lrie"lorc .. IMI~r (lVC) belowr A. advl ntage,: 1. relativt ly lowe r CO$l 2. in addi t ion to meuuring pr~..ure, allow, therapeutic CS F drainall 3. Play be recaUbr.ted to minimiu meaaurement drin B. dia.dvlUltll'l I . may be dimcult to inMrt into compreued or displ.« • . l olution: allow CSP to drain and expelai. b. caution: do not a llow exce..ive IImou:l1 ofCSF to dra in (may al· low o~tnJction of catheter. lubdu.al (ormat ion .. .). Do ~ inject fluid to fluah air into brain rollowin~ decomp~ ..ive craniecto my: due 10 the f.ct t hat the monitor i. no loo~r in a dosed space. this i. a normal rind in, in this Mltin,
4.
A DJUNCTS TO ICP MONITORING
There are a nurnter ()f(l~perialenta l monitorillf teCluatioM"'. Although normal val,," (SJ o. '" 50'101 may 1Omelirn« be 8$sociated wilh poor oute()mes, multiple vef\(lU$ deSitusauOllS « 50'11», or . uSUlined Or profouod desaturation episodes are ulually associat.ed with poor GlI~ ", Suatained desaturations .should prompt In evaluation for correctable etiologies: kinking of jugulsr veio, poor catheter position _CPP < 60, vasospasm, s urgical lesion , PaCo, < 28 mm Hg. AIIo tometime. utilized i. the ute ria l-j ugular ~enou . oxyge n (lOn tent d iffe r e nce (AVdOt)ft. AVdo, '" 9 mWI (vol'" probably indicatelgl:lbal oerebra l iKhemi . ..... , while v.lue. mUd l indicat.eoerebra l hyperem ia-("IWlury perfusion" in HCHSoflhe brain', metabolic requirement" ),
ed IS rngl1 70 mm HK'" (i.e. e"(lld hypo~l\lIion).1'hco primary p i is t.\) conlnJl lCP, .imultlneaull,., CPP should supported by meinu ini", adequate MAP". SURGICAl.. TREATMENT
I. any lubduralorepid ... rnl hematomala~rthen ~ I em maxilOal lhicknsllho ... kI be removed su~l,. to elimin.te the con lribut ion oflhi. \0 IC·HTN 2. patienUl with hemorrhllCic contusions ("'pulped brain") showing progrlll,va deterianlion m.,. oeed to have IheCQntuHd brain lillue I UTe' Uy removed (-IX'P "9)
'"
24. Bead lraum.
NEUROSURCERY
3.
dlK'ompressive cranie 20. May "alternat.e" with: [" rwmjde (i.asix®) (alsosu below): adult 10·20 mg IV q 6 hrs PRN ICP > 20. Peda: 1 mglkg.6 1118 rnax IV q 6 hrs PRN ICP > 2(l B. keep patient euvolemic to slightly hypervolemic C. if IC·HTN persisu. and serum osmola rity is < 920 mOsmIL, increase man· nitol up to 1 gmilt:g, and shorten the dosi ng interval D. if ICP remains refi"actory to mannitol, consider hypertonic saline, either continuous 3% saline infusion or as bolus of 10-20 mI of23. 4% oaline (ilIC after ~ 72 hours to avoid rebound edema) E. hold osmotic therapy ifserum osmolarity is " 320 mOsm /L (higher tonicity may have no advantage and risks renal dysfunction (see below )) or SBP dOO 4. hyperventilation (llPY) to pCO•• 80.86 mm H g (for details, s.-e bf.lowl A. 11 do not use prophylactically B. 11 avoid aggressive HPY (pCO." 25 mm Hg) at a ll tim(S C. use only for 1. ~hort periods for acute neuT(tlogic d(teriorat ion 2. Or chronically for documented IC·HTN un responsive to sedation. par· al}'tics, CS F drpinage and OSmQtic the rapy D. avoid HPY during the fif$t 24 hrs aner illju ry if possible 5. 11 steroid. : the routineuseofglucocorticoids;s not recommended for t re/ltment nf patients with head injuries (su bf.low) ~Second
ti er" the rapy for pe.... is tent IC. UTN
IfIC·HTN remains refractory to the above measures, and espec:ally if there is lOS$ of previously controlled ICP, strong considera tion should begiven to repeating a head CT to rule out a surgical condition before proceeding with "second ti er'" therapies which are either effective but with significant risks <e.g. high-dose barbiturate~), or are unproven in terms of benefit On out: patients must be monitored for a drop in cardi~c index. throm· 24. Head trauma
NEUROSURGERY
boc:y1.Openill , elevated cr~atin ine clearance, and panena ti t i• . Avoid shivering whieh rai_ ICP'" 4 . derompre..iv. Q"ani~tomy: A. removal ofpo"ion of calvaria'" andlor large ann of contused hemorrhagic brain (ma kes room immediately; removu regicIR ofdis nlvted BBBJ. Contr ... v.ni,,1 ( ~ enhllncecerebral edam. fonnation "·). F1apmuat be at letl.1t 12 cm in dill mete r, a nd duropluty i3 mandatory B. if contun d. cOlUlidcr t.emporaJ tip lobect.omy (no mOra than 4-5 em on dom· inant , ide, 6·7 cm on non-dominant) (total temporallobect.omy"· ia proba· bly 100 awe..iv.) or frontal lobectomy . Has not ahown great therapeutic promi •. AlIO, lee Hfmit;;rQIU'«tomy for' moliBrnltu M e,", /t rrilor;y ;"rorcliOTl. page 772 ~. lumbar drainage: show ing .ome promite. Wa tch for "eereb,..I ..g" 6. hypenenlive ther apy A DJUNCTIVE MEASURES 1. l.idaw.in.c: 1.5 mgfkg fVP (watch forhypoten' ion, reduce dOli ifneceuary)atlealt
one minute befe>r., ende>tracheaJ intubation or auctioning. Blunu the ri te in ICP well 81 tachyeardia lind .y'temie HTN (bated on patient.e w;th brain tUffi\ll1l undergoing intublltion under light b.. bitura~nitrou. o~ida anHthHia; extrapolation to trauma patienU is unproven)'" 2. high ~uency (jet) " .. ntilation : coNlider if high level. of PEEP are required' " INS: patieot.e with reduced lung compliance, e.l . pulmonl ry edeml. traNlmit more of PEEP through lungll to thontcie VMlet. and may raile ICP). LeveJ.. of PEE P up to 10 em H,Q do not eauae dinieally .i,"ifiant incn ...... in ICP" · . Hilher le"els of PEEP:> 15·20 em 1120 Ire not recommended. AiIO. rapid elimination of P EEP may aUIe" ..>dden inena ... in circulating blood "oIume which may exacerbate cerebral edema and atao ele"at.e ICP lUI
DETAILS OF SOME MEASURES OUTUNED ABOVE
ELEVAnNG HEAO OF BEO (HOB)
Early data indicated that kHping the HOB at 30-45" optim iuci the trade-off between the following two facton III the HOB is elevated: reduciTl8ICP (by enha ... cinl venOUlJ outflow and by promoting dispJ.a.eement ofCSF from the intracranial compartment to the _pinal oompartment) and reducing MAP (aDd thus CPP) It the level of the carotid IU1.eriea . Recent data'· indieate that although mea n carotid presaure (MCP) is reduced. the ICP il al.o reduced and theCBF i. uoalTected by elevating the HOB to 30". The o" ... t of action otraiainl the IIOB i, immedilte. HrPERVENTII..A TION
Hype ..... entilation (f{PV) lowen ICP by reducing pea., which cau_ cerebral v:;t.SOoonlt.rict.ion. thus reducing the oerebral blood YOiuroe (C BV) '>l. Ofeoacem, vasoconstric· tion allO iowerl CBF which could produce foca l ischemia in a reas with prese ..... ed cerebral autonlUlation a. a ~ultof .huntinl'n. ' ... Howevu. i$Chern,a rll"!e'l not. _ rily fol· low u the 0 , extraction fract ion (OEF) may alao mcrene, up to a point"·. PRACTICE PARAMETER 24·10 '''''fRvnHlL ArlO" '011 ICP IJAI 320 mOunft..
Sl a ntl 4l rtl s - u ,: lb. 11M of ,Iucocottiroida t.teroidal is no~ r~omrnllOded ror i"'proving nutoorne or redun in nonnal areaa (&hun ting blood to ischelllic brain ti&S ue), decreased mel800lic demand ror O. {CM RO~ with accompaDying reduction o.fCBF, free radical scavenging, reduced intracellular calcium. and lysosomal slabiliUltion'>I. There is Uttle quelti(>n that barbitu· ra~ l(>wer lCP, even when other treatO"lenl.S bave failed"', but regarding (>uu(>me. studie~ have shown both bene fi t.s,n. ,.. and lac k ofsan'e""·'to. Patienl.s that d(> respond have a lower O"Iortality (33%) than those in WhDm ICP control could not be accomplished (75%)'''. The limiting factor fDrtherapy is usually hYl!9!!m1i.wJ due to barbiturate induced reduction of sympathetic tone' ·".~"" (ca using peripheral vaSildilatation ) and direct mild myocardial depres~ion. Hypotension OOCUI"ll in. 50% ofpat;enw in sp ite ofad~uate blood volume and use of dopa n,ine'''. NB: the ability to follow the neurologic exam is lost. and one must foHow ICP , "B a rbitura te C()m a~ vI. hig h.do- th er a py: l fbarbiturates are given until there ;$ burst suppression on EEG, thi a is considered true "ba rbiturate coma", This resull.3 in near m.... imal rerluctioDs in CMRO il and CBF". However. most regimens should techni. cally be caHed "high dose intravenou, therapy" si nce they simply try to establish target aerwn barbiturate level s (e.g, 3·4 mg$ for pentobarbital), even though the re is poo r correlation between serum level. therapeutic benefit. and systemic complications" . Adjunctive measures to administration of high -dose barbituratet: 1. consider a Swan·Canz (PA) catheter placed during the fin;t hour oflooding dose 2. high·dose barbiturates cause paralytic ileus: therefore NG tube to .uction to IV hyperalimentation are usually needed
INDICATIONS
•
The ule (>r barbiturates should be reserved fDr situations where the ICP cannot be
controlled by the previously outlined measures'''', as there is evidel>ce that iUl!l!~.ti.I:: barbitu rllte$ do. not fav(>rably alter outcome, and are 9$.!1ociatEOI_leCI _ 81 " ' _ as discontinuing pentobarbital due to tIig~1O$ tOOvg/l"'l! inefTeetivene5S if ICP still " 24 with adequate drug levels .. 24 hrs 8. if ICP < 20 mm Hg, continue treatment It 48 hrs, then taper dose. Backtrack if ICP rises Neuro function takes ~ 2 day. ofTpentobarbital to retu rn (He TobIe 24·23). Leve l should be _ ~ 10 I'gfml before bnin death exam;s valid . 4.
,--I
thiopental (Pentothal®)
\
I
ORUGINFO
\
I
May be useful when a rapidly acting ba rbiturat.e is needed (e.g. intra-op) Or when large dOlle$ of pentoba rbital are not available. One of many pr·, tocots foUows (note; thiopental has not ~n as well studied for this indication, but is theoretically similar to pentobarbital'''· '''): 1. loading dose: thiopental 5 mglkg (range: 3·5) TV Over 10 minutes 2. follow with continuous infusion of5 mglkglhr (range: 3·5) for 24 hou~ 3. may need to rebolul with 2.5 mglkg as needed for lep control ~. afl..,r 24 huuflI, r.. , ~'\lr~~ loe 8-10 mm depression (or,. thickness of.kllnt 2. deficit re la ted 1.0 underlying brain 3. CSF lea k (i.e. duraJ laen.lion) 4. :0 open (compound) depresiied fnct" .... S. • mOrt conservll.tive treatment is recommended for fractures overlying a ml\ior dural VeJlOUB linus
There il no evidence that elevating a dep~ skull fracture will reduce the sub. Hquent development of posttraumatic seizures'·, which lire problbl)' mo..., related to the ini t ial bl1lin injury. PEDIA TRIO'"' Mostcommon in fr CHle..,land parietal bona. Ono:' third aa closed. and these tnger childno as I relu.lt of th e thinner. more defonnable .. kull . Open fracturel teooed to occur with MVAs, dGlled fractu...,s tended to follow accidents lit home. Durai lioerationa Ire more oommon in oompouod ftlOCtU .......
Simple d e pressed skvJ l fractures No different. in outcome (MOiu..... neu.rologic d)'f,function or eosmetic appearance) in surgica l VI. nonsu r,;cal treatment. In the younger child, remodelling of the .ku1l1l511 ...... ul t ofbnin rrowth tc.nds to amooth out the defonn.ity . Indication. for ' UI"rery for pediatric . imple depr-.i . kull fracture: 1. delinite "vicW:A« of dural penetration 2. peni.!lttn t COOImetic defect in the older child alter the ,welling has subs ided 3. 1 fOC11 neurolocicdelicit related to th. fractur, (thiagroup h .. a higher incidence of du ral laceration, although it i, usually trivial) "Ping_pong ball ~ frac turetl'· A green.. tick t)'pe offractura - cavinll in ofa focal area oflh I kull ... in a cnuhed lrel of l ping·pong ball. Ulu.oy seen onl)' in the newborn due to the pla'licity of tha .kull . Indication. for .urgery No treatment i. ne<eUlry when theseOCCl.>r in the tcmporoperietal region in the ab,.. 1.<eptioD: "'p~
f,_ ....... _tty;..,.nod d"",""",nlOM of tho d"'lt tin",," "'Y too d.n~"",,"
2. OpeD fracture Ov ' r conv.xity (u8ually with durellaceration ) C, congenital l kul) d. fectl: induding defKt in tegmen tympani '" D, n.oplum (Ollteoma''', epide rmoid' '', pituitary tumor): u~uaJly caused by tumor . rOllion through aku ll 2, infection with gu· producing or, anilma 8. POl t invuive procedure: A. lumbar puncture 8. veOl rieul Olltomy C. I pinal an" th"i.'" 4. barotraum a" ' : e ." . with . cuba diving (podibly th rou"" a defect in the tegmen tym .. ni) 5. may be pote ntiated by a csr dr.inag. dev ice in the preH n« of a CSF l.u'"
Tensioll pne um oeep ha lU8 intracrani"lgu can develop el~vated prIMuttl in the followi n" H ltinp: L. when nitroul oll.ide ane.U,esia il not discontinued prior to d a.ure of t he dun '· llee ~;Irou. OJ:ilh (N20J. page I ) 2. "ball.valve· e lfectd~ 1.C an opening to the int racranial compartment ... ilh t.Of\ ti. aue (•. g. brain) that may perm it air to enter but P"OVf'nt exit of air or CSF 3. when t rapped room temperature ,irexpandl with ..... ming to body temperature: a modest increase of on ~y - 4'1. .... ull$ from thil efTKt' " 4. in the presence of cont inued prod uction b)' gn· produclng artani.nu Di agnOJli s Po.u macephalus is most IPsily diagnosed on CT'" ... hich can detect quantit~ of a ir
as low a8 0.5 mL Air appea,.. dark black (darker than CSF) and hu a Hounl/leld ooeffi. cient of ~I()OO. One characteristic finding i. the Mt , Fuji l ip in which t he two frontal poles are su rrounded by and separated by air'''''. lntr.crania l gal ",ay a lao be evident on plain skull x· raya. N; pneumocephalus usual lydOO$ not ~uire treatment, it i, crit ical todifTerentiate it from tension pneumoo:ephalUl, which may need to be evacuated if . ymptomatk It .....)' be diffi cul t to distini):llish the two; brain that hu been compressed e.g. by a chronic , ubdu ral may not e~pand and the "gas gap" may mimic the appearanoe of g811 ulloMr p.... l Ur e.
TreatmeDt When d~ to glS· producing organisms, treatment of the primary infection i. init iat· ed and t he pneumacephalus i. followed_ Treat ment or non·infect'Ol>5 . imple prM!umooephalus depends on the whether CIT not the presence of. CSF l"llk i. IUSpegTQwinglb NEUROSURGeRY
...
dura frOID the inner tahle. Anodle. poa.sibility is tbat dissection of the dura from the inner tIIble may occu r first, then bleeding O 12) infrequently' ''. Presene. ofa I kull fncture hal been identified 85 a comraon leature ofOE CH'''. Key to dia~i a: high indu of I U'picion . Avoid a false sense of$lC'urity impaned by an initial "nonsurgical" CT. 6 of1 p8tienll in ODe series improve and d.,. creued functionD l l u!'\'i .. ,,1 (Glasgaw Ou tcome Scale., 4• ...., pGgf 9(){)) from 26'lf> 10 16%. These dilkrenres s ugge$ted a trend bu~ were not statistically eignil'ieall\ 2 . til. following .. ari8bfe& wel"l! iden tir",d liS ~trongly ionueJIdng outcome; A. methanism Qfinjury: the wOnlt·(juu,ome WIiS with molO",yde IIccident.a. w,th 100% mortaUty In unhelmetro pIIti~ts. 33% In lIelmeud B. a~; correlated with oukQmeonly '" 65 yn; 9ge. with 82% morUl[;ty and 6" fUllclionel ! u!'\'ival in th;8 group lother series hlld ! imi\llr result.a"") C. ne urofcgk t~ndi tion 0 .. odmisl;ott, !he r lltio ofmorulity t.o functional su ..... lVal rIIt" I"I!la t. Table 24·26 Outcome as ed to the adtniuion Gla~gow Coma Stale related 10 a drn lu lon GCS (GCS) i. 6h~w .. in Toblf 2.·26 GCS Mortality Functional D. pwltopenltive ICP, pat lent.a with peak ICP~ SUrliVil .: 2(J mm Hi had 40% mortality. lind no PII· ~ 5% he'lI with rep,. ~5 had a funttionalsurvival 3
I
Of all the above r.. ctOT!!, only the time to 5ul'geryand post.operati.. e ICP I:II n be directly influencNl hy the trealing neurosurgeon
,
,ar
'"
...,''''....
I)olfERH£MISPHERIC SUBDURAL HEMATOMA
Subdural hematom a olong the falx bel.ween the. two ceN!br!ll tJ.emisphe ..... Ilnl£r . hemUlpheri"-II"-'..-ure) MIIY OC'cur in .hildre.n...., po~siblY llS patlente with a . mall DASOH . nd medlcslly C(mtroU .. bl e ICP au manllged e.~tantly. INFANTILE ACUTE SUBDU RAL HE MATOMA
Infantile acutA! s ubdural hemaklma (WOH) is nl\en ronside red.s I s~i al (8" of SOH. Roughly defined I I an BcuteS OH In an Infant due tominor he& SUROIC(l. CONSIOERATONS
S .... rgical opl.;O" 8
~re if not unifo rm agreement on the ben method to !ru t CSDH •. For d~a;l . of technique. (bu rr hoIu, whether Or not to u&e .... bdural drain .• ,) I « ~Iow . 1. placing two"" lT holfa, and irriga t inl th roulh a nd throulh with tepid BOline un t i! the fl!>id run. clea r 2. ti nCle -lara." burr hole with irritation and aspira t ion: I « ~Iow 3. tinll. b!>1T hole dl1linage with pla~ment of a l !>bd!>1111 drain , main tained for 24· 48 hi'll (rtmoYed when outpUt becomes negligible) 4. tw ilt dri ll craniQtlOmy:", ~Iow (note that ImaU "twilt drill" drainage withou t . ubdural d rain hal hil!her I"eCUTYeOC. rate than e.l. bu rr holel] 6. fonn.1 craniotalllY with e:rcision of $ubdura l membrana (may be r>e~ry in c8O· M which persistentty r«Ur aner above proctdures. poII6ibly due l.Oaeepage from the subdural membrane). Still a sare and valid tesed, until 24-48 hours after it ill ",moved ). May promote expansion of ehe brain and upu)· lion of rt$idua lsubc!ural flu id 4. some advocate «>ntinuOtlslumhar subarachooid infusion ....hto the brain fails to e)(pand. however there are poslIiblewmplicaeions'"
TWIST DRILL CRANlOSmMY FOR CHROMe SUBDURALS
This method is tbught to dec:ompreu the braio more alowly and Ivoid l Ihe presumed rapid press!>", 8bifla thatoecUI'll following other methods. which may be asaociated with com plicalioll.lluch lUI inlNl ptlrtnchymal (int racerebral) hemorrhale. May ."en be performed at the bedside WKler local ane-.thleliia. A ventricular catheter is inserted into th'lubdura l .~. and is drained to a .tan· dard ventri~mlly be placed o\li! r the opening to h.lp prevent rrelil blood fram _ing into the opening. SU80UAAL DRAIN
U.eor a subdu ral drain i....« iated with I dec",IH in need for repelt lurgeryfrom 19'*'1O 10....... If a s ubdural drain i. uied , a closed drainagea)'ltem il recommended. Dif· ficul t ies may occur with \li!n tri cl,llottomy ca theters becaute the holes ar. tmaUlnd are
NEUROSURGERY
24 , Head trauma
restricted to the tip region (so-designed to keep choroid plexus from plugging the ttlthelAlr when in...erted into the ventricles when used RI intended as It CSF shunt). especially with thick "(lily" fluid «In the JXlIIitivuide. 8l(lw drainage may be desirable). The drainage bag i8 maintained _ 50·80 em below the level of the head ..•· '''. P(I$t-op. the patient is kept flat ($N o~). Prophylactic antibiotics may be given until_ 24-48 hnl following removal of Uie drain. at whicb time the HOB is gradually elevated. CTsean prior to removal ofthe drain (or shortly after rem(lval) may be helpful to establish 8 baseline for lalAlr comparison in the event of deterioration. There is a case rep<Jrt of administration of urokinll5e t hrough II subdural drain to treat reaccumulation of dot f(lllowing evacuation· ...
OUTCOME Th~ is clinical improvement when the subdura l pressure UI reduced to dose to uroo which usually occunl after _ 20% of the coll ectioD is reO'l oved .... P atienU who have high subdu ral fluid prefigure tend to have 0'10..., ra pid brain expansion and clinical improvement than patients with low pressures"'. Residual subdural fluid colledions ana r treatment are comID(ln. ~ut clinical improvementdoes not requi re complete resolution dthe fluid coUection on CT. CT. showed per-sistent fluid in 78% of cases Dn post -op day 10. and in 16% ane r 40 days"'. and may take up 1(16 m(lnths for complete resolution. Recommendation: do rutt treat peraistent fluid collections evident on CT (espedally before _ 20 days post.-op) unleas it increases in sile on CT (lr if the patient sho..s nO recovery Dr deteri(lutes. 76% of 114 patients were s\l~cessfully treated with a single drainage procedure using a twist d rill craniostomy with subdural ventricular cathe~r. and 9O'!lo with one or two procedures"". These statistics are sligh tly better than twist drill ~raniostomy with aspi · mtion alone (i.e. no drain).
Compli catioDs of s urgical t reatment Although these collections onen appear innocuous. severe compl ications may occur. aod include: l. se i ~ures (including intractable status epilepticU$) 2. intracerebral helIl(lrrhll.ge (lCH ): occurs in 0.7·6%"". Very devastatiog in thia setting: One third or these patients die Dnd ona third are se~re ly di8llbled 3. failure Oflha brain to re-expand and/or r eaccumulation or the subdural nu id 4 . tension pneumocephalut 6. subdural empyema: may also occu r with unt~ated subdura ls>'" In 60'l>ofpatienta ~ age 75 yrs (and in no patients < 75 yrs), rapid ::Iecompression is aaoocialOO with hype~mia in t he cortex immedilltely beneath the hematoma. which may be related to UieC(lmplications orlCH Dr seizures..•. All complications are mo...,com· 0'100 in elderly or debilitated palienUl. Overall mortality with s urgical trestment rorCSOH i. 0..8%'. In II aeries (If 1()4 pa. t;en\.s treated m{)5t1y with craniostomy.... m(lrtality was _ 4%. all of which occurred in patients> 60 yra old snd were Coue to accompanying disease. Another large personalae· ries reportfld 0.[,% mortality"'. W(lriCni ng of neurologic , tatus following drainage occurs in _ 4 Pre&en lat j<Jn: Mean afe Ofp rto&l! nLlltion il _ 4 mon ths .... MIIY show: iignS 0 elevated intracranial pr~ ure (tel'Se or large fontan&llt, a 24.9.3.
Late complications from head injury
Long term complication8 inClude: ]. posttraumatic sci zur~: (ue P Table 2.heimer's di!ll!ase) Ill! shown in Table 24·31 8. profes8ianal boxers (mo,e risk than amateurs) Neu ...... imaging: The mOIl! cOrnmGn finding is ~rebral atrphy_ A cavum septum pellucidum (C SP) i,observed in 13~ of boxers"'. CSP in thil setting probably represent.. an ac· quire -
-
•
"'", , " 2
Ne uropa t hology includes: 1. cerebral and cerebellar atrophy 2. neurofibrillary degenerstiGn of cortical and auboorti. cal a reas 3. deposition of B'amyloid protein A. fanning diffuse amyloid plaques B. in a subset ofCTE patients this involves the vesael walla giving rise to ce· rebral amyloid angiopathy
• •
24.10.
-
•
Gunshot wounds to the head
GUJ\ljhot wounds to the head (G SWH) alX'ount for the majority ofpenetratinlr brain injurie!l. and comprise 35% ofdeaths from brain injury in persons < 45 yrs old. CSWH are the m PRIMAR Y INJURY
Primary injury from CSWH results from a number offactllrs including: I . injury til sol\ tiS.'iue A. direct scalp and/or facial injuries B. .wft tLuue and bacteria may be dragged intracranially, the devitalized tis. sue may also then support growth of the bacteria C. pressure waves of gas combustion may cause il\iury ifth" weapon is close 2. comminuted frac ture of bone: may lnjure~ubja~n t v8JICula r an dlorcorti",,( tissue (depressed skull fracture). May act as se 100 mt.: caUSe!l explosive intracranial injury that is uniformly fatal (NB, impact velocity i8 less than munle velocity) low mJl..u!e velocity bulletll (N < 250 mlsJ: u with most handguM. TIssue injury is caused primarily by lacera tion and materation along a path ~lightly wider than miuile diamete r hitt. muule velodty bullets IN7:;0 mls): from mili tary weaPOnt and huntinlr ri· nes. Causes additiooal damage by shock waveS and temporary cavitation (tissue pushed away fron. the missile causes a conical cavity of injury t hat may exceed bullet diamete r many·fold. s nd causes low-pressure region wh ieh may draw surfa E. U!tan ... s toxoid administration ANGIOGRAPHY IN GSWH
Rarely performed emergently. Wbell dOlle, us ..... lly performed on ~ d..y 2·3. lodic .. tions"' : I. un~xpeo:ted del"yed hemorrhage 2. a trpjertent to the spilla! cord ) exduding gulll!hot wounds to the head . lncludl'$ trauma irom : knives, lawo darts, arroWS .. 10. risk oI"infO ...
24. Head trauma
hieh in ciYilian GSWH,
L1I . ~ .
NRUROSURGERY
Casel wit h roreilCll body 5ti ll fl rnbedd e d In penetl'1lting tnI '~mll, it i. ".ulllly not 'ppr1lpriot.a to femo" .. an)' prolrodmg part ofllM! foreign body until the pooli.nt i. in the ope. aUng room, unlen it CIIIlJIOl bt ~yoideO. Ifpouible, it il helpruJ lo hllye .""llle. iden t icul obj.o:ct for comparison in plllnninK ulri· cltion orthe I!m~ object.... To mlnhniu "'tendinl the t "'ump I. reabl;orb, Ulullily within 2·3 v.-eeQ. Occasionally may calcify
eon.
om
Infanll maydeveiopjaun:lica (hyptrbHi rubinemia l u blood is "",orbed, occasionalIYlllal.4!a. IOdl~aAer ollMt.
Treatme nt Treatment beyond an1lauica "al mOli t never required, and mO$~ usually resolve within 2-4 weeki. Avoid th tempta tion ofpercutanl'Oully aspiratina the$e 81 the risk of
...
24. Head trauma
Nf:UROSlJRGf:RY
infection exc~ds the risk o(following them npectanUy. and in the newborn removal or the blood may make them anemic. Follow serial hemoglobin and hematocrit in large Ie· sions. If a s ubperiosteal hematoma persisl$" 6 weeks, obtaio a skull film. If the lesion is calcified. surgical removal may be indicated for cosmetic reasons (although with mnst of these the skull will returo to nonnal contour in 3·6 months"".31",
24.13.2.
Child abuse
A! 1 £InlpI1oc .'"." '" PO"'"" "'~h 1e,..·rl.I minor _ ;".,.... J T ....... J3: 38$· ~.1991 . S,..i. 5 C. R.... S E: MoIdlooad ,njury: A pi rot '-"'" .... Iy cr""Mina. J T ' ....... JJ: ' 1·3.
"Ill"
0< M.N< .. tr.,. ...
,-
V""", H A.GIo ... I R IV.S""",;dok II H. .. oJ.: O W M. Ri ... i R W.I, rion D W. C...li" P M, ProIlltm . .. ,tit in,,"1 GI" IO'" """" ",.10 OJ"'""""'" ........, b) tn~· 11,,,1 ""'mo.' of ,..i' ~ _ "'ju,i". ~ ..,I" of . no'l....1 J T rauma 16. 19·9S. I~ . B.\Ioc~ R. ChtOnl>l R M. CI,n"" G. ,,~' G uld.· 1i.",'0I" ,be tnaJt.~ ...... , or ...... h,od 1nj.". n.. S .." r ........ ""..... (N, .. Votlt). Tho "'m«i I A.C..... I.'."..r_c.._.
8.llot:t~ . CIoC.S ... C' Thc ..... of ....-. .... opc .... ,.. ;"" .... "' ... 1ft . . . l.
"""ric,Ile"'...." 1 '10r>9IUn A J. I>". M. Kul>oO• ., .1" """ro· ......, of i.".m... ' .... """"'" ,th an· ,,,,,",,I..,, ..... ropy. s..,' 101..,'""' .... 4J8"'3. ' 99S. M.nro D. Mcm" II II: 5."",,, .... 1Io1o. y of 8 . 00~" K. y",",!OOi6. 5"'1: N•• · rot 47, ' 18-1l, 1m 147. T,III1. A. M,K .. ""k w. "b(]Unl ""-"'.p: An ".","01 form 01 « ..bro . ..• "" .. ,""' ... "' " ...." """'2 1: 19·2:i. 1971. ,~ . 1I .... IIIO)lJo«.""n< of . ulri ,"". , r1;. 100 ",,,.". , in I>im on Nc."""um' .nd C,;".., Cotc. N... roIOV • .\, 5.1.1·1.1991 . 1.0:< M S. M.. ne l O.C.boll",·llou,"",. A, Ojo""";' 1> Oui«>' "
pOO'' ".'""'''' ..
NE UROSURGERY
"".
"'~'Y 22. 8601·1 . I,," H-lOO. 19119. m BiJll< P E. H o~ ' m M.(;OIO 'RJ: J, Cori,i,...nd toe. ..... """1 >40. '969. l il . O,di Tabla 25-2 Evaluation 01 WAD
lhey.", lObe used. "'e na,,,,", pan should be p~" Ironl
25. Spine inj uries
'99
Ou tcome In a Ilud,. of I 17 paloenUl < 56 ye a ~ of ,ge h ll~in, WAD Table 25-4 Recovery d ... e to ... tomoblle .eciden~ le~d\ldlng t h(,uwI U, cerviCIII frao· 01 plI.Uenla wllh WAD turea, d illoceti01lI, 0' irti ... riu elsewhere in tha bodyl cond ... ct..d in SW;lu,tan(\' fwhere . 11 !ned;.:.1 cot\.S we,.. peid by the .... te and Ihere Will no 0 l,port ... ni ty fOT litiglltion and no compen.ption fO T pein . nd s ... ffering, "l l ho ... &:h thtre ..... the paUlbility of permanent dlHbiHty), the recovery rata WIIB as ahown in Tobie 25·4. Of t he 2lllatienu with continued .yrolltonlS.t 2y~. only 5 were I"fttrictfd wll h respect to work (3 I'l!!du~ to part. time work, 20n dinbj)lty). Palients w,th persi$t..nl 1)'Inl>toma w... older. had Ino,~ varied complaint..- on 'nitinl exam. had a more rotated or In~lined hend llOll itton at th. tlmeor impect, had a IUgheriDcidellOl! ofllrelraumaUc had.chU.lI.nd had II hllilhe, incidenoe of certain pre-existin, findinp (a... ch $I radiolOlJie evidence of cervical or;tlloa rth riti.l. The Imountof damaga to the .... tomobile a " d lIIe .peed of thecers hItS liltle rel"'tionlhip to the d~ of irtiwy. and out-rome w.. not IrUluenced hy gende r, vooation. or ~ychoLoriColo I facton
• ,."
Pediatric spine injuries
25.2.
Spinal cord injury i, fai ril' u"common III children ..... ith Ihe r.Liool"Mad Injurin to spina l cord injuries being . 30: 1 in pediatrics. Only _ S'lr Q{~pinal eon! injuri.. OCCUr in children. Out- to ligamenLQU$ l.) chlldren < 8 yn ap' illlmobi liq with 1II0racic: llevation or an octipltal nceII (.I~a rna,.. "eut",1 aHlIlmenl d .... to the ..!lativlly IlIrge head) d1iJdren < 7 yn I ,\': I wi th illiunu oflhe C2 dlntoeentral'ytll:hondro,il 1_ pop '42): dosed reduction and halo immobilizatian amaidlr: pri~aryope ..tiv. t,... t~nt forilOlaled C-spi na lip,J'MntoUI InjUJias with auoeia~ de&:>l"DIi ty
700
25, Spine illiuri ..
NeUROSURGERY
PEOIATRIC CERVICAL SPINE INJURI ES
For pediatric C-aplne anatomy su pogt 142. In the age group ~ 9 yrs, 67% ofcervical spi ne injuri es occur il) the up!""r 3 segmenb; of the cervical $pine (occipuI.-C2 )" . $Y/liCHONDROSES(su pog~ 142)
Normal synchondJ"Q6es may be mistaken for fradures (esped al1y the dentocentral synchondroais of the a~la9 (.u page 142) which may be mistaken (or an odontoid fracturel. Conver~eJy, adual fractures may occu. through synchondroses"' '', Recommended treRtmen t (or fractures througll synchondroses: the tend~ncy for sy nchondrOS('s to fuse suggesw that emergency reduction followed by external lmmooiJitation be attempted. In· temal immobilitationlfusion should be reserved for persistenl instability". PSEtJDOSPREAO OF THE AnAS" Pseudospread of the atlas (defined aa > 2 mOl total overlap ofehe two Cl lateral mas8 25.3.
Initial management of spinal cord injury
The major cau~es of death in spinal cord injury (SC I) are aspiration and shock' . lni. tial . urv"y under ATLS protocl: a .." ... m"nt of airway taku p_o>plement 2. mai ntain blood pressun} (8"(' below un· der Hypolet1$ion ) A. pressors: tr.!ata the underlying problem (essen t ially a traumatic sympathectomy). Dopamine is ~he agent of choice. and is preferred Over fluids (except as necessary to rep lace losses) (see Cardiouarc"lor agenls (or shock, page 6 for pr.!$' ""ra), • Avoid phc'I)'lcphril>() (• .,., below) 6. fluids PI necesnry to replace loss· A.
~
3.
iOlCication) 2. no road! pain (with no listracting pain) 3. no I'IeIJrtlIogie delicits
-.-
Table 25·6 NATA ~elmel removal guldel1nuo
·
· ·• ·
mosI i.... ries ean be visoariled wi1IIlhe
I>e~ enm ca~ be
00rwJ wi1!11tle heliMl in place !he palien! may be immobif>l8d on aspne board wilh !he heh>eI in place !he Iao:emask can be r~ wilh special IoOIs to access lhe airNllY tr,peroXiension roost be avoided Iobring removal oIlIIe helme] and shr:Uder pads • NB: do not remove !he t~ In Ihe lie!1:1. In a oontrolled seH'ng (us.uaIy altM ~·ra)'S) !he he!mtl and shoukIaf-pads are removed t0gether as a tri1 to iI'/Oid road! IeQon Of extension P.,.,lbIe I~ lor removal 0( helmet lace mask camot be re<noYed in I reaS(nable 8rTIOUI11 01 time airNllY camel be esllitllished awn with
· · · ·
· ·
lace mask remc1Jed iii" lhreateOOg herno!;hage under Ihe ha~ rnellhal can be ~ 11ed only tr,r rerno'lal I\elme1 & strap do not hold head securely !O U\aI immobiizing !he hKneI does noI adequalelyimrnolliiile lllespin& (~.g. poor filWlg Of ~ hel'n8l) Ile/mel pre'IeIU fmmcbiization lor transportation in an apprtllrille po$iliOn
tena .. oiI~"""",'" pe6en! ia ....· stable (M.D. dedsion)
lot
mo,. _
..... Mp1lWwW,Nlta,org
C. military anti·shock trousen (MAST): immobilizes lower s pine, compensaw8 for loet mU9Cle tone in cord injuries (prevents ,enous pOOling) maintain o~ygenation (adeq uate F I0 1 and adequate ventilation) A. if no indication for intubation : use NC or face mask 6 . intubation 1. indica t ions: mpy be requi red for a . airway C'Ompromise b. hypopnea: i . fr(am par8lyzed intercostal muscles
------'-
A. 011-." "'porto orbony or lip"",ntouo . bo-orm . 1lti.. lui"" bo d_ribttl .. _ ibLy """,rrlng In "'_ po.;'n ... "'"""' hu boo" no <eportor . pt.ti. n .... ho had nourologio iQ,iu')' ' ' . _ultol'lh ...
,,,
ob"""""Hti..
25. Spine iojuries
NEUROSURGERY
'i. from paraly~ed diaphragm (phrenk nerve '" C3. 4 & 6) iii . Or from depressed LaC 2. cau tion with ;ntublltion with uncleared C-s;»ne ~. use ch.illlift (not jow Lhl"l)$t) without neek extension b. na!lOtrncheal intubation may avoid mDv"ment"rC'apine but pDttent must have Spon t.an~UI rupiratioru; c. avoided trach eoslOmy Or crioothyroidolOmy irpos.ible (may compromise loter an terior ~rvical5plne surgical appro9chea) brief lll2$&![exam t.o identify possible def",;!iI (also to document d..Jayed deterioMl· t lon); Mil p~tlent 1.0; A. movenron , B. move hand~ C. mOVe lega' D. move toe$
4.
MANAGEMENT IN THE HOSPITAL
Basic phu~a of management with respect 10 the spin.e: I . I t./Ibilizotion (med ical &. spinal ), prelimin"'}" evaluntio" &. treatn ..",t 2. evaluation or spinal st.ability 3. lubwqu"nt (deflOitive) tf@"tment
nlcal Aallesar8en1. Oplions" : the AS IA inu.-mauonol SUlndards for neurological and functionol assessment of spin "l cord injury (SCn Ii~ pase 711) is recommended
00
oukome asflfil8lllD!l~
liuidellnes" · \.heFunctionlllln'p"irmenl Measure'" (F1M"''') (Mf!: P''Be 901) il r~mmHnded Options": the mo:lified Bortbel inde"
CrmCal eare manq:emllnL .
I
(~lpnge 9(0) 1~
recommended
~_
Options"': manito.- ptltientB with "cute SCI (esJl"Ci~Uy those wlth &evere «ITviclillevel lnjurles) In au lCU or tiimilo~ monitored !letting Opllons ....: cllIdlBC, hemodynAmie& rl!llpirnwry monitnring aner acute SCI ;6 rec<Jmmended Opllons" : hypotension (SBP ... 90 mOl Hg) "hould be &vcided nr colTI!ct.ed
immobilization: maintain ba.:kboardlhud·s trapI6l!1' 12001H!) to raellitat.!! trar)5feT"l 10 C'r table, etc.. Once studies 0 .... eompleted , re1Tl\lve patient fronl backboord by logroUlng (early rernO\lnl from board reduC\'1i ri~ k ofdeo::ubitu! ulce",) hYpotension (spinal shCl 25. Spine injuries
""EUROSURGERY
4 . pregnancy S. narmtie addictIon 6. age < 13~ar. 7. pal~nt on m.i1lteMnCt! steroid.
Admin istration
I . cunce ntratiun : in the following protoool,.11 .olutioN.re mixed as 62.6 mgtml (e-g. by diluting 16 gm m..thylpred.nisolone with baetm08tMic water to 256 ml) 2. 00 11.11: 30 0"Ig/kc initi.IIV boIu. over IS minute., infuMd'1 . hown in Eq 25_1 with In rv conlroJler (thi. delive" 0.48 mllke oI".olution in 15 minutu):
bolus .. 3. 4.
,t (...L....r'" 1»'_ S """Ii'" (kl) X 1.92
(rOf IS mioules)
Eq 2'-'
followed by. 45 minute pau .. .... in kn.nce infu. lo n : then 5 .4 mgfkg/l"lr("()nlinuo" infu. ion .,.how n in Eq
25·2 (infulion ,. m.intained durinl,ny _ . , lur/lt!l)' if poaible) Eq 2S-2 d .. . . ';.", 0( molnl . ............. fiooion: ....... IherlPY .. ,Rid ...... A3 loti .n.. i'1iury, lhe inr""ion io Id",inille!U lOr 2l h.. Ihho ..py", ltatted btl .."n S l ad. " .. 01" ''1iury . ...... "'1 bt an ir> There i&controvel"ly regarding what conlili lule8 I minima.ll1Idiographic evaluation
NEUROSURGERY
25. Spine injuries
'"
orwllcllrvkal.plroe in multiple tnwoa patient. No imaging mOI!.li ty i. 10091> 'CCW1Ite.
•
PRACTICE PARAMETER 25 S' "HS A"[)''''''OI''',IA''O~ '''' 'ChL SCI ".:,' . ~-:.---==..t
Sl a nd a r clS- ; radiagl'lphic atud1U 1If1!.DIIl. indic ated Ul pa UenUl ....-ho h.... e: no OleoUI,tlltu, change. (IInli no .I Contra indica t ions I. atlanto·oc.:ipital dislocation ; ~ P"lle 717 2. types itA or Itt hangman'. fracture : see P"/l'l 725 3. skull defect at anticipated pin site ; may necessitato alternate pin site 4. use with caution in pediatric age group (do not use if age ~ 3 yrs)
'"
25. Spine injuries
NEUROSURGERY
PRACTICE PARAMETER 25 6 1"'T IAL CLOSEO REOUCT'ON ," fRACTUF>E10ISLOCAT ION CElIVICAL SCI
Option s" ea rly dolled reduction of C·. pine l'rPctur.tdislocation in,iurie. with cran ioct .... ical traction to ..store .natomic ali/CIlm~nt in .wa ke patientt • ~ recom~nded: dosed reduction in plItient8 .. it h an addi tional rostnl injury pa t ienta with C .. pi ne fracture·disloc:.tlon who (8 nnot be eumined duro ing Itl.ernpted doM'!! reduction, Or before open poiIl.erior reduction, .hould undergoce .... ic:.1 MRI before attempted reduction '. The pr"· enee of a ,ignificant heroiated disc in thi wMlti ng i.a relative indic.tion for Int.!nor decompr~ion b9lOre reduction (t ..... ical MRI i. also fftOmmended for patients .... ho fail att.,mpts at doted reducLionN8. p .. _
_a.
MP I ... or- IIIItuPlOO Of ......... 0l"" . NoI helplul ~ tIOIh u _ and lowe, uIJdonVnals a,. _1< 1M lI>domln..t I to T2 with the intervening levels distributed exclusively on the UEs (see Fig",." 3·7, page 751. The point of trans,· tion is in 25. Spi ne injuries
Nl!.VROSURCERY
C·s pine x·rays, may demonstrate congenital narrowing, superimposed osteophytic SpU rt, tToumatic n-actoJreldislocation. Oc (~ ... with later.1 m.... pl.tt'8ll'tth~ time of de com pres lion. or anteriorly (e.g. multi-lev~1 dj~tomy, or corpectomy with I trut graft .nd anterior cervicl'l plating) 8t the ume $it.tin, • • lhe laminectomy or at I' I.te r date.
PROONOSIS
In pa t ien'" with cord contusion without hematomyelia, _ M>'-' will recover enough I,.E 'trenl\h and &eM.lion to amb ulate indepe ndently, although typicdly with lignifi· cant speost icity. Recovery of UI'.: function i, u~ually not 88 good, and fine motor control is usually poor. Bowel and bladd~r control ollt-n recovers. l::lderly I'{Itient.l wi t h this condi . tion ~nera lly do not rare II! well uyoungi! r pat ient.l, with or without surgica l treatment (only 41 ~ over age 50 become ambu latory. versu. 97% for younger pall,.n ..... ). ANTERIOR CORO SVNOROME
AKA anterior spin.lartery sy ndrom e. Cord inf.n:tion jn the territory lupplied by the anterior spiMI artery. Some eay thit it more common tho n ~ntral cord syndrome. May result from occlusion of the anterior Ipinal.rt.ery. or from anterior cord com· p~ssion, e.g. by disloeated bon" fl1lgment , or by traumatic herniated dille .
Presentation I. paraplegio, or (if higher than _ C7) quadriplegia 2. di ssociated ae nao ry 106& below lesion: A. II)Q of pain and temperature a-enaation (~pinothalamic tract lesion ) B. preserved two·pol nt di ll/:rim inotion, poIeition tente , deep pre. u.. .entation (po9terior oolumn function)'"
Eva luation It is vital to differentiate II non.,urgiell condition (e.g • • nterior Ipina l art4!ry occlu· sion) from a s urgical one{f.g. anterior bone f.agment ). 11Iil requ;reloneor rnore of: my· elography. CT, Or MRI.
Prognosis The WOrBt prognQII;s of the incomplete injuries. Only - 10-20'10 ~er functional motor cont rol. Sensation may return eDough to help p",~nt itljuriel (burnl, decubitus ul~I1I .. .). B ROWN-S~QUARD SYNDROME
Spinal cord hemisection. Usually a result of penetratin, traulIla. it .. leen in 2·4'1, of traumatic . pinal cord injuries". Also roay OCCUr with nodiation ~Iopathy, cord com· pre".ion by spinal epidural bfmaton.a. large oerviules (Bet' FiRuT?- 25.J throullh FiJI/Jrt! 25·S ): L ligamente that (:(InMel toe atlas 1.1) th e occiput: A. anterior IIItlllntooccipital membrana: ceplultKI extension of lhe anterior lontiludinal ligamenL Extends from anterior margin offoralDl:'o magnum (FM) !.O an teriOl" arch of C I S _ posteriOl" atiantooocipital membrane: tonn~ theposterior margin of !.he P M tu peltt:rio~ archarCI
C. the IlSCending band of the audate ligament 2. ligwnents th at COnnect. the u15 (viz. the odontoid ) to the De· ciput: A_ tectorio l membr a ne: some authors dislinguiAA 2 c()Jn p<Jflents 1. s uperficial eo;mpon~ntl cephalad continuation of the posterior longit udinolligBmenL "' strong band connecting r.he domal ~urfoce of the dena to the ventlOnion of th e ala, Jigan>enlll (plsthion: should intersect tangentially B. BC2$L d rawn from the ba$;on 10 a pIIinl midwllY on the C2 spinoJaminar Ilne: should interseed t arly only for immobi lization in aduill, UQI. for
reduction!. Cont.rover~i.J whe ther <Jperati"e fusion vs. prnlonged immobilizstion 14- 12: mooths) with halo brace is requi:ed. H(lWever, posterior occipito·cerviClll fUll!on is USI.l"I.
Iy recommended (see (J1I(Jnlo .occipiw./ disl{)C<Jlion injIJrif5. pap 7171.
A. ~uj ..... identlf""'uon ofUl~ opioth;"" "'hkh el l '" II hll ... been treated .... ith pr .... ithoutntert:a l in,mobi liUltion lcervim! ""npr or, lICC85ionaUy, ha lo) .... ith"ul obvious dilT.".en~. Ex:emal imnmbihuL;""1 X6.& "'·""ks i.a .. ue..1.ed fGl"Type III frae. t Ureo!l btoc), : 1_ beI.,..,1 usually Men in children aner. r,,11 or mInor l",uma 2. anterior; moreo",;rwu~ ~ brllllU)
ATLANTOAXIAL ROTATORY SliaLUXATIO N Rolntmnal d~rormity a l the iltlanlQ.UUill Junction II usua Ily or . hort. d uration aod easily torTKted . Karel)', the .llantl.uial joinl
lockt in rotation I AKA allanl.O-llCC ... r in tortiOOlli8 nf any etiology). CT IIC8.D: demOllBtrates rotation of the atl ...... MRI: may a_s the oompetence of the trlllllVerse ligament.
Treatment Treatment orGri",I', syndrome: approprilte antibiotiCi for ca\Jl8uve PlIthogen with traction <M:e ~low) snd lhen im mobilization (see Tg/>k 25·111) for the INblu.ution. Traction' The subluntion may be to!duced with gentle traction (in thildren start with 7-8lbor and srad ... al1y incn!allll up to Hi lbo: over seve. al days. in adult.a start with 15 lba and srlldu.Uy inC",IM up to 20 1. Uthe .... bhu:ation is pruent > I month. traction i.1aI lucceaafuJ . Acli"e len·right neck rotation i. encouraged in tnlclion. U~ ... cible. immobilizatioo in traction Or halo is maintained II 3 months-hange: 612weeiul). SublUllatinn that cannot be reduced or lhat recu'" following: immobilization . houk! be treated by aurgieal.rthrode.;. ann- 2-3 weeu oftmction to obtain maximal reduction. The .... ua! ful ion i. C I toC21_1X'8' (23) unlf118 other fracture. or ronditiorw a re present". F... sion may be pl!rformed even if the Matioo between CI &02 ill nteompletely tiId ... c.!d .
ANTE RIOR ATl.AHTOAXrAL DISl..OCATIQN"
One third ofpatientrl ha ... neurologic delleit or die. May be dr.oe to: t. inoompetence oflhe odontoid pn:roceA A. rraeturfl B. congenital hypoplu ;' (e., . Morq ... io . yndrome. an fHJIe 33n 2. dilruption (rupt ...... ) of the tra,.."e,.., lattanlal ) [ipment (n ) (_At/onto-den. tol inrHW/. (1rJ)1), I»ge 140). Attachment point.a of tIM TL mly be we.kened in rheumatoid arthrilia. IIIOI.ted ITO" ... OI;': rupture of the transver.., ligarneM ill ~~
EvaluaLio n
CT" MRI are I'f!COmrnended to evalulte the TL
•Treatment
"""ion ill tteCOmmended when lhe TL i. di. rupted or with irretl ... cible . ubl ....utiona. Odontoid Met ........ with.n inttoc\ lig .....mt a", m.n.ged IS outlined in that section IH' 25. Spine injuriet
NEUROSURGERY
pop 728).
25.6.4.
Atlas (C1 ) fractures
A ,,
, en."", .. al. II., Le_
and e NEUROSURGERY
e.,.
25. Spine Injuri..
'"
FJ~Jioo ;8 the m().'!t OOI'\1IUOII me Type /fA Early a urg~1)' is r~mmended for aU type lLA rrn~ture5 '''. T~III
. 90% h,,"] with externaL innnobiliutioll 'lind analgesics) ifllde-qulltely maintained f(lr 8-1« of ~Iop.thy
Treat ment Rqardlen orwbllthr 01 odontoMleum ia congeni tal 01' an old non ·union f' lIdun!. ;mmobiliut.ion;1 unhkcb' W ,"ult in fU$;on. Thet1!fore, when In!tIuntntl. el@ClI! ger)' ia requi~ (utu.lI,. IllAnlOUill' anhrod.i •• _
~
623).
Opt Ions'"
rfCOmm"nded: lhe followi", pJ,in C .. pi... ,,·ra,.., AP, ~.nlouth od_
p'lien", without nt!\1I"oJ~ ti&nl or .ymPlQm.(~o with C]·2 i",labll· ity) may be followed with dinkal" I'1Itliosrnphic lu.rvm'bi...,.. \.hOft with neuroloj'ic or .ymptorns and Cl·2 lnstability A. mlY be managed with poe;terior Ct.2 inte.....1 (",ati..,n and fu&lon
.lj'o.
•
8 . optio,,.: I . poat.erio. wiring &. rLl,ion. P.... l-op halo immobiliu.lioa is ~commended following these pro Combination Cl-2lnjuries
25.6.6.
Combi nation Cl-2-injurieol are relatively common and !\Illy ,toply mM! significant lIM1cturaland m Option s'"
.ecommended: blUe trf!3trnnt primaril y on the type ofC2 iUJury recolDll)flnded: I!lIternaJ immoblliu tion of 0"10111 C l ·2 frattu." IIOnsidt'l" l ul"gical.tllbiliuotion° for thue , ituPtiont t; A. C l -Type II odontoid combl n9tion frDct uTd with lin AD! a 5 n'm B. C l ..... ngman·. oombi n,,-tion fuetu"" with CZ.S angulal lon a It" Ioi.t 01 inIto;ritt OIN C1 mo mit _MilII, mooIIIcll;o.. olin. W~IIec:MIq .... !I-.~5M .., _Wl'fIlnl!Kllol 1s (C2J itat:MaDII pIlge72 disruption nfblood flow from the aorta or seerneotp l branches, lraumelic disc herniation. Thel' F RACTURE CLA SSIFICATION ON THE BASIS OF MECHANISM OF INJURY A 8ystem adapted
from the Ofle of Allen at al. ,.. divides cervical . spine fractureldi.loca tiona into 8 major grollps based On the dominant loading force and neck position Qt the time of injury 08 shown in Table 25·26. Grades "fseverity with.n each group a re described. and a ny of these frac· tl,ll"('lj may also be alSO' ci Bted with rotatory loads.
r"'''''>T'"'"''';;.riiii;ii.,ruiiO"l-''-Wii;;;r.i;;';;;;oo~ I
I
I
Stability
Guidelines for de· abbrevia!lont: AlL • an_ ~"' Hgamenl: VB • """ebfal body. tennination of bwme· Ix • "Id"": .....-. in lWenthe$8S ~ page ........ce.. 101" IN1 !OI>ic coo.nil;al in~tabi!ity (ree in II-. pr.sence 01 SIenOSl ... any 01"' ••.......,., ir!u'~. ma1 p«> 1.1 ...... .. 4ang1e,.1.5°""H.Y" char>ge .. neu· t~ include: l.eardrop fractures ro exam. rw.!e$l is con~lIIindicale(1 ~ ~ ''''lability (su ~Iow), quadrangular fractures § Plvlov ..110 • 1M ratio of (dlstanre ffom It!e mkfj ....101 (I« P"8c 735). the POSI"'" VB 10 lhe ck>sesl pOinl on the SfllnoIamirw Treatment: mild cervical comline) (the .loP diameter 01 the tnicIdIe oIlht Ve) preuion fractures without neul'(llogic deficit Or retropulsion or bone into the s pinal CHnal are usually treated with a rigid orthosis u"til ,,-raYI s how Jw:oaling has OCCUlTed (usually 6-12 wu). Stabili ty i8 assessed with flexion·e.Un8ion views (I«pagt 708) befo", discontinuing the brace. More severe compress ion fractu res heal in a halo brace witb - 90% rate of anky· losing fusion. PRACTICE PARAME TER 25·17 &VllAXlI!.L
"",CTU~' O.G~"CA"O" ~ ·
Op1ions \&' initial treatment: dosed or open reduction is reconunended subsequen t treatment: rigid external immobi lization, a nterior arthrodesis with plate fixation, or po!Il.erior arthrodesia with plaU! or rod fixation
TEARDROP FRACTURES
Originally de!I.Cribed by Schneider & Kahn'''. Results from hypcrflexion Or axial loading at the vertex of the skull with the neck flexed (elir:oinaling the normal cervical lordosis)'" (often mistskenly attributed to hyperexl.ension because of the retroliathesio). There are varying degrees of &everity. Pathologically, the injury (oneilts of compleu dis· rupt.ion of all of the ligaments, the fauotjoints Bnd the inte rvertebral dis k'''. An important fea t ure is displatement ~the inferior margin of the fractured vertebral body
'"
25. S pine il\iuriu
NEUROSURGERY
pelIurio.l)' into the .pinal eanal'". U....... Uy "Instable. SHn in _ 5'iJ>ofpetienti in a Large aeries with ,,-ray evide nce ofce ..... ic. l.pine tr.um .... Pa tient.a Ire often quadriplegK, although .ome may be in tact and .orne may
have anlotriorcervical oord syndrome (m' ~ 716), P -...ocia~
if\j"riu a nd
rIO·
diogTllphie fIndings indude"""" 1. • ~mall chip ofbc>ne (the "teardrop") j" at beyond the 101.llriO' inferior ed&e artha
2. 3, ~.
5. 6. 7. s. 9.
involved vertehral body (VB) on lateral cervical.pint fil m ollen llSSOCiated with. fractunt throuJrh ~ ~tta l plane of Lhe VB wtlich ClI O almost alwa)'ll be seen On AP view. Thin cut CT _0 i. tOOre "Mit;y. .. large triang"J • • fragment of the .nterior in~rior VB other fr.durl!~ through the vertebral body may .1.., oec:ur the fractured vertebrae i, usually di .pla«d ~ on Lhe vertebra be""" (t asily appreciated on oblique 'Hay', 1ft Fi.{f",", 25·' J. pap 1371. However, _ _ without retrolislhuUl .... also described'" the fractured body ia ol\o,n wedged anteriorly (kyphMie), and InY'lto be wedged la\.erany dis ruption ofthe faceljoinu which may be appreciated ' 1 liepa-ration orthejoinu on lateral x-ray, tll\en unm as ked by cervical lract itln prevert.b ral 8af\...tiuue I welling CI« ~ 141, for mea l urementa) na .... owin g tlfthe inte ..... ert.brol di.c below the lTaclu,e (indicat;n, disruption)
Diatiliguisbing between tflardrop fracture nDd avu laiOli fractuN! Rationale' T eardrapfractu ... . n' Ullbedi,tin"",l,hed from a limpleavulliio D fracture of a I mall chip of bona which may 0110 relult in a . mall chip ofbone off the anterior in· ferior VB . u.u.lly pu lled offby t raction of the anterior langitud;nalligsment (ALL) in hyper~ . Although there may be disru ptinn or th e ALL in th elle CII~, it doea not u$ulllly caute In.tabUlty. Me thodo lolP" In a patie nt with a I m. 1I bone chip off of the inferior an\.erior VB , a "teardrop· {racture need. 10 be ruled-oul. Determine if tha following criteria are met: neuroll>Jically intact (betau .. of the need for cooperation, thill includes mental statui, and exeJud .. th, inebriated or ooncuued patient) , in of bone f"'ament i. Imall no malal ign ment of~rtebral bodi ... no evidetlCil! CI{VB fracture in uiitta l plane on AP C.spine x·rays or on CT nCl poIte";"r element fracture on x-ray Or CT no pre~rtebra llOn liuue swelling at level nffragment (_ ~ 14/) and no I"", Clf verteb ral body heigh t Or disc space height if the above criteri a are met . nbtain nlUion..,x\.ension C ..... pi ~ x. rays (see Flexion· ulln.ion CflrvimJ _pi_x·roy., page 7(8 ). If no abnormal m""emenl. discharge patient in riiid (Ollar (e.g. Philadelphia oxoll ar), lind rep.at the films in 4-7 ds)'II (i.e. sile r the pa in hat lub&ided ttl be certain thalalignmanl i. not being maintained by ce rvical n. u.ecle a pum from pain), Dt'C collar if 2nd aet offllms is normal. [fthe patient doea not meet the above crite ria, treat lM m all an unstable lTacture and obtain a CT scan through the fractured venebra ttl evaluete for as.ociated fractu ..... le.g.•agittal plane fracture that may not be apparent CIll pllin .·rIY). MRI ......... U.... inte,"ly of the disc and giVH lOme infClrma tion about the poIterior Jigamenta.
Trflatmflnt of teardrop fracture [fthe disc and l;gameauare int&cHdetennined by M RI) then an option i, ttl employ , halCl brace Witil the fraament is healed (perfClrmllexion-ex\.enliCln X·IIY. ane r remCIV· in, th~ halo ttl rule-out persistent inAtabilily). Alternatively, lureica l atabi liutiCln may be perfClrmed. especially ifligamenUlulor disc i.,jury i. Hen un MRI . When the i.,jury il primarily po$t.erinr d "" todlsruption of the pCllterior ligamenta end facetjClinu, and if lhere iI nCl anteriClr compromise ..fthe . pinal eanal, then a posterior (\III1un • Severe inj uries with canal compromise oft~ require a combined anl.erior deoompren ion and fus ion l perfcormed first) followed by poIlerior f... i"" ul ingeithe r I mcodifoed Bohlman triple·wire technique or lateral ma.. plates.
..un,,".
QUADRANGULAR FRACTURES''''
Four reaturu: 1. oblique vertebral body (VB) fractu .. palling from anterior" uperiClr oortical ma rgin to inferior end plote 2. JX>S\.erinr subluxation of l u perior VB on the inferior ''''8
NEUROSURGERY
2.5 . Spine i.,juri ...
3. angular kyphOllis 4. di4ruplioll or disc and BIlLeri or lInd pl~ioo 3. IInterillr narrowing and poeUlrior widening oIthe dl.., s patll 4. intt..aMd distance bo!t.... ~~n the po8te rioroortu of i.hlllaubluxoo vertllbral body and th~ anlflrior cm1.t!x of U1~ articular tIlllUes Q(thll subj""~nl vertebra 6, anterior and superior diRpls F OLlOW-UP SCHEDULE
After initial manageOlent (surgical Or nonsurgicsl) of cervical spine problems (atable or unstable) the follow.up schedule shown in Ta.ble 25·28 is sugg"ted to permit recognition of problems in time for treatment'. The $Chedule is initiated following removal of brace, c88t, completion ofbed rest and initial physical therapy.
Table 25-28 Fol· fow·upsclledule I. 3weel<s
> ,-
3. 3monlhs 4, 6monlhs
5. 1 year
25.6.9.
Sports related cervical spine injuries
Any of the previously described injuri1!3 can be sports related . Thi. section conaiders some injuries peculiar to sporta. AJso sa ~ 633 for sports'rms. Patients should be c,,",fully eva luated, and return to> competi· tion should not be allowed in the presence of neurologic deficit, radiograph ically demon·
NEUROSURGERY
ZS. Spine inju ries
".
· tnlttd injury. «ortain eonsenital C·,plne abnormalities. and poMibly for "repeat ofl'ende •• " (HI Rtlu.rn /0 ploy GM prr·pr:>rIicipo.IWn ,u.id('IiM'. P'SI 143). Type III inj .... riu are the most eommGn. Unatable inj ... riu I ho ... ld be t reated apPl'OJIriately hrft p~ 739).
Tabl a 25-30 C•• plne ..... l. led COnl •• lndlesIl Ol"l' 10'
•
~DCIIQcI _ _
~"lclpatlon
In cont.CI
,potI,'
"':--.g._... hocIo;.,.. _.~'''''.-;"g 25. S pinlinju ri",
NEUROSURGERY
1
t i 6
,
.'so ."" ~ 633 "" ~.,.,"aleg leSi (i.e. '0 os,mptorMlOc PI""'" 'atio < 0.8 il nola cont,aindcatOon 10 partiOpalion)
\hef,'''''
FOOTBALL-RELATED CERVICAL SPINE IlllJURIES
Football players with suspected C-spine injury should not have their helmet removed in the field (s .... page 702 ). The following termi probably originated as locl<e .... roolll jargon for various cervical apine-related injuries usually au!t.ained in playing football . Medical defmitiollll have suboequently been retro-fitted to them. Ao a result. the preci$e defin.itiotl-' may not be uni formly agreed upon . Although the 8em9J'ltia may differ. it ia more important from a diagnOlltic and therapeutic st.andpoint to di stinguish nerve root inju ries. braChial plexUll injuries. and spinal cord injuries. I . "emeal cor d ne uropr .... ia ' .. (CCN ); sensory changes which rnay involve numbness. tingling or burning. Mayor may not be associated with motor symptom. ofwealrnes" Or complete paralysis. Typically Imts < 15 min. (although may persist up to \tion, or vert.ebr.IBubllUl/iticm are lNquently identified in plI.tien~ with bll.ll1t VAl'.,·.... M echani s m a o r injury I . motor vehicle lcadent: the mOfiI common etiology 'JTeatmeDt Strukuwert more frequent ill pIItieDttowith VA l Who _reOOI t.rut.o!d initially .nth IV he~rin deapit.e an .symptom.tie VAllw.
25.7.
Thoracolumbar spine fractures
SoI~ IIf.pine frarturea occur at the IhOI'1lCIIlumher (1'L) junc:1.l0ll, usually 'fl2.Ll. 70'11 oftheu occur wilhout immtd.at.e neurologic inJul')'.
attempt. to identify CT mteria oIlnsllbilily o(IllQl'lcolu",ba r .pine frac\u ....,... nu, model has ,eneraily rood predicc.ve v.lue. how .... er. any atlflnlpt to create "ru IH" of in-
..
,
25. Spifla if\iuriu
NEUROSURGERY
stability will hay .. IIOrne inherent Inaccuracy . I. ~rolumn; compOSed ofanu:orior half of disc and vertebral body (VB) (indudes anterio~ a nulus fibroauR (AFl j plus the antl!rio~ longitudinnlligBl1Jent (ALl.) 2. roi4dD column: posterior hn)fofdiac: lind yet-tehral body (include&" pDIIlerior wall Ory"rV:hral body lind posterior An aDd po!It.erior longitudinlliligounent (PLL) 3. ~ column: po8t1!rio. boDY complex (p08terior arch) with interpo!led poIiterio r IiI:· amenWl\II COfllplu (aupTiUlPinOutl and intars· pinOllSliglUllent, facetJoints and Cllpllule. and ligamentum naVWD (LF) . Injury to this col· umn alon .. does Il!ll cau"" instahility
CLASSIACATlON INTO MAJOR & MINOR INJURIES MINOR INJURies
Involv e only .. part of" column and do not lead toacute in,wbHlty {whltn not accompanied hy rnl\lot ]njuR.}. lndude~:
I.
2.
3.
fract,,", oftransve .... e p~"": u!IUaU, neW"Ologically intecte uept in two ilreall: A. lA·5 - lumbosIl(:ra] ple!<Us injuries (there ma1 be associated renHI iojuri8ll. check UtA for blood ) B. TI · 2 - hre.chial plelt\l.llll\iuriea rractW"e of arti CUlar PI"(lCe6S 0. piUS interorlit'lll .. ri" isolal.ed fracturu oftbe spinous process, in Ag",.. 25-12 ThreeOOlUrnnrnoo&lDt th e TL . pine; t hese III" MAJOR INJURIES
The McAfee dallllification deacribes 6 maio tJlP"ll or!'raduretl''', A lIimpl ified ayltem WIth tou.r C9.tegoriu (oUow. (allO te~ TobIe 25·31).'
1. eo mpN!a$ioll tract ...... : compresaion failu", of IInte.rWr oolumn. Middle column iru..tKl. (unlike the 3 other UlIljur injuries below) acting n II fulcrum, A. 2 aubtype.ll ; I. lllten.ll ( rar e) 2. anlerior: mll"tc.nrnmon helween TS-'l'IIlI.nd T12·t..3 a. lateral ~·ray : wedging oflhe VB fllIteriorly. 110 10M of height of po!!terior VB , nu lIubll1llation b. CT: ' pinal canal wtact.. DlI"'ption of agterior end·plate B.. ~I j "io;o.l , no """"'"
2.
logic deficit b \l.t1lt fracture. pure axill.lll>8d -coDl p~ion or ... erUlhral body - com· prf"Mion failur~ of ante· rior a nd middlellOiuDlIlI. Occur mai nly at TLjuJ\ct ion , .... ually below""n TlOandL2 A. 5 ~Ubl~! (LS bUl"!lt rractures may constitute a
ta"' J ubt)1le.1ICI! ptJl.'e
I.
1-18) rra~IUre
of both Md ·
NEUROSURGER Y
Table 25·31 Column faHure In the lour major t'tPU o llho tacolumoar spine InJu ries'
" 25. Spine i o.J"ri~B
'"
plates: leen in lower lumbar region (where axiall08d ~ increased utension, unli ke T_.pine where axial load ~ flexion) 2. fractu re of.uperior end·plate: the moetcommon bUnlt fudu~. Seen at TLjunction. Med!anism ~ axial load + flexion 3. fracture of inferior end·plate: rare 4. burst rotation: usually midlumbar. Mechanism .. axiallcmd + rotation 5. bunt lateral ne"';on; mechanism", axiallood ~ lateral ne"';on B. radiog-raphic evaluation I. latertll x.ray: cortical fracture of posterior VB wall , lOlls or posterior VB height. retropul.ion or bone fragment from end pl ate{.) into canal 2. AP x-ray: ,ncrease ofinterpediculate distance (lPD), vertical fracture oflamin._ ~pl ilying offllcetj(>intl;: 1 IPO indicates flllluN! ofmill..d.J.l:column . 3. CT: demonst rates break in poateriorwlIll of VB with retropulsed bone in spi nal cll1181 (ave rtlge: 50% obstroction of caoal areal. ,ncreue in IPD with s playing of posterior arch (induding facets) 4. myelogram: large central defect C. clinical: depends on level (thortlcic cord more senait;ve snd les~ room in eanal than conus region), the impact ot the time of di"roption , aod the extent of canal ob~t!"\lctiQrl ~ 50% intact at initial ru.:amination (halfoflhese recalled leg numbness, tingling, sndlor weak.ness initially after trauma that .ubsided ) of patients with deficits , only 5% had ~ paraplegia 3. /lea t-belt frllc t"",,,: flexion - compressionofanterior column & distTaction failure of both middle and posterior columns A. 4 subtypes I. Cban ce fracture: one level , through bone 2. one level. through ligsments 3. two level, through bone in middle column. through ligament in an~ rior and posterior columns 4. two level, through ]jgameot in all 3 columns B. radiographic evaluation 1. pJain x-ray: t interspinous distance, pars interarticuJari. fracturea, Dnd horizonwl split of pedicles Dnd transvenle PI"OCe68. No .ubh",. at;oo 2. CT: poor for thi s type (most of fracture is in pJane ofaxial CT cuts). May detect pars fracture C. clinical: nO neurologic deficit 4. fraet ure-d is lo-ca tio l); fsilure of all 3 columns due to compreuion, tension, rotation or shear - subluxa;ion Or dislocation A. x·ray: ox:casionllUy, may be reduced wheo imaged. Look for other marke rs ofsignificant trauma (multiple rib Fractures. unilateral articular process fracturea, apinous process fractures, horizolltallaminar fractures ) B. 3 subtypes 1. flex.ion rotation: posterior and n::aiddle columna totally roptured. anterior compreased - anterior wedging a . late ral x-ray: sub!uxationordisJox:ation. Preserved posterior VB wall . Incre8!Joo intenipinous distance h. CT- rot-otion "nd off... t of VB. with j c.. n .. 1 di~mete' JumpP(! Facets c. dinical: 'l!i% neurologically intact. 50'/11 of those with defidts were complete paraplegics 2. shear. all 3 oolumns di8ropted (including ALL) a. when tTaums force directed posteriorly to anteriorly (more common) '''-B above shealll forward fracturing the posterior ard! (free floating lamina) and the 8Uperior [scet of the inferior verte-b.. 3.
b. clinica l: a1l7 ca8115 were complete paraplegic. flexion distraction a. radiographieaUy resemble ~at-belt type with addition of sub· luxoticn, or with compres.sion of anterior column> 10-20% b. clinical: neurologic deficit (incomplete in 3 cues, complete in 1)
----
A. 00"", a U \hi •• n •• ion..dia .... " ;., fl"ll&.
STABILITY ...NO TREATMENT OF TH ORACOlUMB ... R SPINE FRACTURE S
Inatabil1ty may be ul~gorited at: lit dtpla' me ~ lou of height with IIngullllion B. or. kyphotlt IJllpJation:> 40' (or:> 26'10) C. nt, neurologicdelicH D. IIr. desiC1l1.O Ihomn tenrth "fu.... of bedre.t 2. prolonged bedrest..n opt.ion if mille oflJM, a~ are prewnt
BUrlt fr Ac tures NOl all burt*- r""ctul'1!t are .like. Some burn (tlnur" IUlY e"nlu.lLy ,.u.. neuro1000cd... ficit (~n ifAOdelicit Iruti.II,r). Middlt .;oluRUI f""gmnli in t.n.l ... ndanlr'!r th ... NUra eleme.nu. en""ria ha~e been ptopoaed to differentIate mild lnu-st r•• clu~ from Nyue one.. Reroro",end.uon.lWl': l W'lPul treatment for.1l ~tien .. with partial neurolosie delio;it, or th_ with .nf\lla, defo,.",ity. 20', a .... idu.1 QIInlil! diameter" 6O'J.of normlll . or li n .nterior bod)' hei,ht ,,~01'~ pclAterior ..... i,hL
NEUROSURGERY
25. Spine i!'\iuri..
'"
L5 bu.r~t fractures: These fractures are e~tremely rare, and it is difficult for instrumentation to maintain alignment at thia le~el' ·. Therero~, if neurologicdeficitia absent or .mild, conservati~e treatment should be considered'. ·'·. Regardless oft~atment, patients will probably lose _ IS' oflordOlliB between LA and the sacrum. Permanent neurologic loss may occur'''. ElLI"ly reports ofcoll$ervati~e r.lan&gement utilized _ 6-\0 weeksofbed re9t followed by mobilization;n a brace. A mo~ contemporary approach utili~es 10·14 daYBorbed rest. The patient should be fitted with a TLSO with a unilat.eral non ·movable thigh euIfin 10' ofnexion (on either side, to reduce motion at the fractW"fl !legment). Mobilization should bedone very gradually all the pain allows. The brace should be worn _ 4·6 months, and serial x.rays should be perfon:ned 10 rule-out progressive deform ity. If surgical treatment is indialed, a posterior app roach with fusion and l1;standard d eviatic>na (SD) below the mean for healthy young adultsB. Z-lICOre: rompared to mepn value ofconnp) subject. ofu.me pge and se>: I. SO < -I lowest 25% 2. SO < _2 lowest 2.S~ 3. patienta with low·trauma fraclures or fragi lity fractures are considered 0steoporotic even if their BMO are greater than thef;t! cutoffs DEXA scan (dual energy ~.ray absorptiometry}: the prefe rred way t-o measure BMD I . proximsl femur: BMD in thia locatioo i, the best predi~to r for future fractures 2. LS spine: besl location to aqeu re.ponf;t! t-o treptment (need AP JUUI)ateral views, .inee AP oRen overestimates BMO) Post.frllc t....., conaider litionlJ 1. other cauSoe. of pathologic frsctute, especially neoplastic (e.g. multiple myeloma, rnet.astetic brean cancer), , ho",ld be ruled out 2. younger patients with OOIteoporosis require evaluation for a remediable csuse of the osteoporosi s (hyperthyroidism, steroid abuse, hyperparatbyroidism, OIIteomsIDcis, Cushi ng's syndrome)
TREATMENT"""'"
PREVENTION OF OSTEOPOROSIS High calcium intake during ch ildhood may increase peak bone mus. Weight-bear· ing eKercise al", helJlll. AlA efTeo;tive: ""tcogen(....~ ""low), bipbO are poor for internal bation. Management consists pri:narily of analgesics and bed rest folla.oed by progre!lSive mobilization , often in an extern"l brace (often oot tolerated well). Su rgery i8 rarely employed. In cases where pain control is difficult to obtain or where neural compression ca~s deficit, limited bony decompreuion may be cotlllidered. Percutaneoua vertebropla8ty (..... beiw ) is a newer option. Typical time course of conservative treatment; 1. initially, severe pain may req uire hospital or subacute care facility odmiuion for adequate pain control utilizing A. sufficient pain medication B. bed rest for about 7.10 dnya ( DVT prophylaxis recommended ) 2. begin physical therapy CPT) after ~ 7·IOda)'ll811 patient tolerates{prolonged bed rest can promote "disuse OIIteoporosiBj A. pain control as patient is mobilized may be enha.nced by a lllJllhar brace whid> may work by reducing movement .... hich causes repetitive "microfractures" B. discharge from the hOlpital wi!.h lumbar brace for outpatient PT 3. pain 8uboidea on the a"erage afte r 4·6 weeks (rllnge 2-12 weeks) PERCUTANEOUS VERTEBROFLASTY ( PVP)
Transpedicular injection of polymethy)methacrylau.A (PMMA) into the comprell.ored bone with the following goala: I. to try and stabilize the bone: may prevent progression ofkypt.osis 2. to . horUiti the duration of pain (sometimea providing pain reJiefwi!.hin minute~ to houn). Mechanism of pain relief may be due to stabiliution or bone, or due to heat released in exothEnnic curing of cement Indications 1. painful osteoporotic compression fractures: A. u5uaUy do not treat fr"ctures producing < 5-10% lou of height /L NB, •• of the 'i",e orttu. ""ritin&. PMMA i. no~ 1'0.0. . pprovtd r.. 're.lmeB' of.pm.) .."'p ..... "'n ftoctUtH, and tho, • • '" no ,...~d .... intra;ndications: A.. rrac~ures > 8Q% loss of VB heigh~ (technically challenging) B. acute burst fractures C. signi ficant canal compromise from tumor or retropulsed bone D. partial or total destruction of t he posterior VB wall : nQJ; an absolute Con~l'IIindica~ion
Complications Complication '" rate: 1·9%. Lowest when used to treotOllteoporotic compression fractu",s. higher with vertebral hemangiomu, highes~ with patt.ological fractuJ'l,!!l 1. methacrylate lwkage: A.. into son t issue&: usually oflittle consequem:e B. into spin.l canal: symptomatic spinal cord compreNion is very r are C. into neul'IIl foramen D. into disc space E . venoul: Cllon get into vena cava, case report of pUlmonary embolism 2. I'IIdirulupathy: 5·7% inddeo"" . Sumecasea may bed.,e to heat released duringceIllent curi ng. onen treated conservatively: Bteroids, palo meds, nerve block ... 3. pedicle fracture 4 . rib fr3(:ture 5. transverse process fracture 6. anterior penetrstion with needle: puncture of great WlIsels, pneumothorax 7. increased incidence of future VB compression fractures at adjacent levels Management of some a&$OlTelatell with s trongly with goad oukome from PVP)
Procedure I.
pain medication A. remember, tl\is procedure is being done with tl\e patient lyingon their stomach aad is usually being done in frai l. elderly fem ale.. who smoke.
NEUROSURGERY
25. Spine il\iuriel
Therefore use caution to avoid ovenledation and respiratory compromise
B. sedation and pam wedicatioll C. WI8 Dflocallilleathelicdul'ing needle pl.""mellt O. additiolla l pain medicatiOIl just prillr to injection use bi·plalle fluoro (IIr alternate AP and lateral views) to get needle to enter me· dial aspect of pedicle and place tip _ 112 to 213 oflhe way lhrough the VB 3. test inject with coDtraat (e.g. iohexol (Omnipaque 300) SH page 127) (do digital $ubtrac:tion study if equipment is available ) A. a tittle venous e!lhancement is aroeptable B. if you visualize vena cava 1. do not pull neoole back (the fistula bas already been created) 2. push lleedle in a little further. or 3. puoh $Ome gelfnam (soaked in contraat) throu gh the Deedle, or 4. injelA injected and pain relief''' B. PMMA approaches posterior VB wall Or elltenl dill 25.8.
Sacral fractures
Uncommon . Usuallycau.eOO by .hearfOreell. Identified in 11%ofpatient.ll wilh pelvic fracture!l'''(:. keep in mind that neurologicdeficit.ll in patienl8 with pelvic fractUN!& Clay be due 1.0 auocisted .acral fractures). The aacrum below 52 ia notessentia! toambul alion oraupportofthe spina! column, but may slill be unstable sinw presaUN! to the area may OCcur whell supine or sitting. Neumlogic ~
i.njurie~
O«.ur in 22-60%"·, Three chsracteristic cliniul presentatioIU 1hbl~ 25·33.
on zone of invoJven>ent.... .,' "" aho ..... n in
Treatment In One l\«I"ie.'IO, all3S fractu re" were treated witbout surgery, and or.
9. YIIfC'Ut.r iqiu ......
10. • urgery for IlIA! compliutioM: A..
",,,"1;'\1 buUet
O. tlllld t.Wdt,m (p!UfObi'm): abt«ption ollHd fro .. a bullet O«Utl only when it lodgu in joinUl , bu r...., IH"dill: IPMI1I, ~·ind inp in "flh~
AUTONOMIC HYPE RREFLEXIA
t Key features
0 exagge rat.ed autonomic response to normally ;nnneuo"" sti",uli o occurs only in patients with spinal cord le5io05 ~ _ 1'6 o patiellts complain of pounding headache. nushing and diaphoresis llbove 1'19ion o call be life threatening. requires rapid control of hypertension a nd a ua reh for an elimination of offending sti muli \ AKA autonomic dysrenuia. Autonomic hypeTTene~a"" to, (AM ) is an e>elow T6 (only patients with le9iOfUl above the origin of the sp!anchnit outnow are prone to develop AH, aod the origin i9 usually '1'6 or below). It is rare io.
NEUROSURGERY
2ft. Spine injuries
755
firllt 12·16 W\!eM poat·injury. During 8tUleluo, norepineph.rine (NE) (but not epinephrine) i, released , Hyperllen • • itivity to NE may be partially due to subnormal resting levels of catecholamines, Ho_tatie response" indude vasodilatation (Bbow! the level oflhe ir\iur)') and bradycardia (however, sympathetic s timulation may also atuse Ulch~a rdia ), Stimulus SOUTcea causing episodes of autonomic hyperroflexia: I. bladder: 76% (distens;on 13%, UTI 3%, bladder stonel .• . ) 2. coloreclal: 19% (fecal impodion 12%, administering enema Or a upp06itory 4.%) 3. decubitus uleerslskin infection: 4% 4. . DVT 5. mi"""lIaneous: tight clothing or leg bog strllpll, procedures such"" cystoscopy or debriding decubitus ulcers, case report of suprapubic tube PRESENTATION
paroxysmal HTN: 90% anxiety diaphoresUi piloerection !>OlUIding HlA ocular finding:a: • mydrillsi~ • blurring of vision • lid retraction Or lid lag erythema of faee, neck and t.ruJtk: 25% pallor ofak.in below the luion (due to v/L!lOCOnslriction ) pulse rate: tachycardia (38%) or mild elevation over baselin e, bradyca rdia (lQ%l "splotl:hes" OVer face and neck: 3% muscle f~iculatiOn8 increased spasticity penile erection Horner's syndrome triad SOOn in 85%: cephliliPa (HlA), hyperhidr06ia, cutaneous vasod ilatation EVALUATION
In the appropriate setting (e.g . a quadriplegic patient with an acutely distended bladder), the symptoms are fairly diagtlOlltic. Many features a .... alRo COmmOn to pheochromocytoma. Studies of catecholamine levels have been inoonlliatent, hqwever they can be mildly elevated in AH . The distin· guishing feature of AM is the pncse nce of hyperhidrosis and flushing of the face in the pruence of pallor and vasoconstriction elsewhere On the body (which would be lUIusual for a pheochroruol>l M M: 1!.t " ' _ o/otk. MOy1 injuopiO" 1....... _j.«4d.ioooden: R«I"11 ,n;'"" J AMA 177- IS9'1604.1991 . H...,~." M B. Membtrol 1prd9:~,~.r.. , .."'" ",,",icol ",,,,,I _ in .............., 0/ 1...d ""ombocmbolj .... in jIIIietou "itll «,...",.I.pi'" """ ".iorio •. N.... ~ SLIppi< .... , (J): CoiclloI",, 1S... · 1.... , Ch_~ : R",;oV ' pO:'''_.'Qf, ho =--ioilloplorio.y .. p'''''''' ......... p........ , 1Phio ev,I .. , ... 0/ >pir>ol _ . m• . OnbopC\ilo Nonh Am !1: 7S-M.19M . Toln • .-!II. _ T . .......,.; A•.,,,," Eff,,,"ti . .. i\~ ..... "'Mk <arp!tf 21 . M.... "'tnCcipou."'>IO<Mion.C ... ..,.,... J 1'1< ........ "165: 86l·1O.1986. Hiffi. J H.J, .• C...."...G C. W,,"" L 1( . . . . " R.· 400J0cie di.o"""" 01 ,rou.",' c o«ip''',,,,,,,,,,,,, du.«''';on, 1.C","",*""," of pilo of""";"" oubpu. AJ II "'''' J Ro M M: r.. .."""" 0(111< .Ior liJOll>piaol«nI inju.." J N.. ..-ora II : l4&.60. 1961 . S.... M:Spi.-.Jftl>l:'''''' • ..,di,""""'",,,. I. CII.'mj>1o:, "'nJ' mo," fnO\IIl: loi: 6)0-49. 198.1 . Vo.m.... J R.(lj,;u io _ .', ' y _ . _ lA" N..re&w'1 1'n ~ 2-I·)) , lm .
"" '". S«,;"" "'~,
(>;lOfdo .. 01 "'" Spi"" IIId P' ..... •• 7 R," ~j" A m«"""i"", arI!i •• ' 101 G. Cooper P R. &ri«J T J: """""'" pla, .... !hr ........ F"""'..... ",.. i. D,h". 0 O.so.i A A . _ L C: Thocffocl of UroII JI, "" · J.I9I~ SI_GW.W..... ' .. WW· A _ " ' ......
S-01. El III...,.W5._,' W. Hip_"'" ..,~ ;. .... . . - . . - ......... spr..t ;,w"...., I ellrM<J'Ieu. ' ........... I.,d .. '. .....,_ ....... I " _ A.. S.' .,9)- Jru- 11.
'"
..... _
_...,._~,
....... .,....r
;')orr porioon. $tho! C.., oJ (I ); 19f.l!O).
~,
NEUROSURGERY
Cel'tbrovascul •• a«;r ",...bI'O-IIP#cula, diMau. pqe 869. b ll..ac.l'tbral MIM"Mllt an peg. 849, and SAlf alld all~u')'."'" an pag~
781.
26.1.
Strokes in general
CEREBRAL BLOOD FLOW (C B F) AHD OXVOEN UTIlIlATION TableJ!6· J I hawl typical CSPyaluu and theCOmolpondhl1 nlurophya;a!agic nate. COP < 20 if ~a.alty .aocilted with "d'U~mil lind irprol onaed will produet'O!!lI death'_ Howlyer , lhJ. a.uumel oonnal ll)f!taboli.c rate Ind may bot ma'l! applicable toa1obttl ceNbral hypoper(usionl.".. notion that t.Mre i. a higher CBFthruhold for kMaof elect rical ucltabill ty Ihan that et'JI death lead to the CCITlet'pt of the ischemic pen ...... b r a cell . that were nonfunction in, but lIiII via ble'. CB F;. ra.latad to blood prHII1I11 U lhown in Eq 26- 1.
ro.
CBF .o CPP • MAP ICP CVR CVII.
Eq 26-1
",halll CPP '" cereb,.. t perflliion pressw-e EHeclS of varllllon$ln ceF (_ PGIlt 647). CVR .o Cl'raWovISl:ular tell;" tance (_ btWwl. and W.:AP" mea n arterial Pf'8Uura. c"reb royatl(!ular r eoriata nce (CVJU is arrected by !he PaCO, lllch that the ra ir • lin· enT incre ...", in CBF w,th increasing Pa~ within the I'1In~ or20-80 rom HI . CVR il also nlTec-wd by cbanges in CPP wh ich produce chansu i n blood Yeuel tane via a ,nyog..ni c mechanilm.ln the f'linI"! 01 CPP '" 60- H,Omm Hg the CVR or normal brain liU " e v~ril!!llinearly 1.0 mainta.in an ahnD8! tollllUl M CSF. Tbi. phenOInI!1lOl1 j. caUed {ceMbrfll) a utorelfUlI.Uon , which i. altered in petholOKk l utU . The cerebni nletabo ii c rat e o r oxygeD ool\llUl>ption ( C~mo.) IIveralH 3.03.8 mlll 00 gm t ;l1"e/mln. 'Ib4! rat ;" ar CBF to CMRQ, (the couplin" ratio) tn the qUIescent brlli n is 14.18 , ....i th r 4 mm thick are a rI~k r.~ tor for recl,ll'Tflnl ev~ and other vascular evcnu (Ml , peripheral embolil rn, and deatb from y..tular caU&eII)' 11'l1o teTie artery eerebroY8ICular I.-ion II'" tandem arterial pathology Ta bl'2e-2 Outcom,I,.. eVAa 15·3O'lIo hemol'Thagic:": ICH, S AH orSDH
OUTCOME
The 1980 ltatiuia (01" di8poeition at time o( dilcbarge for _ 1,800 CVA!< i•• hown in Tabl# 26.2.
26.1.1.
Modifiable risk factors for stroke
I . hypel'\eolion: the moelpowerful &: treatabl.,.uk factor. Both . ystolic &: di u tolic BP are independently a:>rrelated with rialr. ofCVAa 2. cigarett41 , moki",: .... lame rilir. .. al!HOe of 1.5·2.2'-" S. bload lipide: low.,ri", lipid. may redllCe the rilk of lOme type1l ofcerf!brovascular d1_ 4. alcohol : heavy eonlwnption i, UIOcia ted with increased riJlr. cfCVA. whereaa modenwo ..... may have no.,ffaetor may bf!alightly protecti"e. Theeffects may be different for ischemic vi. hemorThegi.c eVA" 5. aotiplatelet therapy: redureo the riak ofCVA and other "lllICUlllr evente in high· rialr. patien ta. The optimal doee il not kIIown, the aeceptable ranee is SO-I300 mgfd, with a recomlOended initill d_ of 32S mgfd"
26.1.2.
Evaluation
CAT SCAN (EMERGENT) A oonoontl'lllil brain CT saul should be done ASAP to rule-ol,lt hemorrhage (inLra· parenehymlll or S AH), hematoma , early signs of isc:hemia, old infartlll Or iqjuries, end oth er lesionl (e.g. tWllOf'). Emergent CT is more strollgly indicated in the followingaituationa: 1. iranticoagul~tion (e.g. hepaJin)or thrombolytic therapy ;sooollider«l (to rulf>.out hemol'Thage, mal/d"e infarction ... ) 2. iflCH ~usperted (e.g. if level or o:oruciolllneM unw;u~11y depreaaed) 3. ir.urgicalleaion possible (He E"""'lfllCYcol'Olid ~nd4TUr«UNrly. pa(tl879)
CAT IIC8.D fiDdings wilb isc hemic e VA. ("pale" infarcla) NB: 11lese principlee do W!l apply to lmelliacunar inrarcta. nOl" to hernon"hagic CVM.
Fi n t 12·24 Iu-a: CT is nonnal in S-69'l1.0/' MCA eVA!tstion mrrectiy. or calltlOt anSwer becauleor: ET l\.Ibe, orotr8cheal trauma, se .... 1'11 dysar. thria, lllnguage barrier. or Iny oth· er problem not leoondary to a phui a, answers neitherquel t ion oorredly. or i,: aphasic. stUpot'OlIS, Or dOH Dot compl'llhend the quet.tions
Ie. Level of con sciousneu co mm aud s Patient ia uked toopen and dOH theeyes, and then to grip Ir>d release the non ·paret· ie hind. Substitute 8nother I ·uep oom· mand if both ha ndsclnnot be uled . Credit i, given for In uDe 2. Belt ga ze Tell only horizontal eye movement. Use motion to attract attention of aphuic jIlI_ tien ta, o normal 1 plrtial gue jIlIl.y (gale .bnormal in one o r both e~ •• b\.lt (0"* devi . ation or total gaze paresi. are not present) or .... tient hllan isolated cra nia l nerve JU , rv Or VI .... ruia 2 forced deviation Or total gau pal'll_ .iI not overcome by D 185 mm Hg, Or DBP > 110 mm Hg 4. rapidly improving or minor symptoms 5. history ofGJ or urinary tract hemorrhage within past 2 1 days 6. arterial puncture lit non·compressible site within past 7 days 7. patieots On anticoagulants, or those receiving heparin within the past 48 hrs 8. PI'" 15 seconds or platelet O)unt < lOO.OOOImm· 9. seizure at onset ofCVA 10. symptoms suggestive ofSAH Table 25-3 Manageme nt 01 HTN Biter 11. major surgery within last 14 days adm Inistration of r1PA lor Kute eVA" 12. anothe r eVA within past 3 months 13. blood glu",,'e" 400 mg% or < 50 ml, are atill reasonable candidates for rtF ...... TNatment protocol ""so, s.ee E;cclu,iCJnol')' criluio, page 768. R.r: alteplase I"'ctivase®): initiated < 3 h ... from onset of d~ficit (N INOS protocol): 0.09 mglkg IV bolus ov~r 1·2 mins, followed by 0.81 mg{kg ••.lflsLant infusion ovEr 60 min. ute l. The NINDS protocol" requi red that no anl;ooagulants I:Ior antiplatelet drugs be giVEn for 24 h ... after treatment, and OP wa~ mai ntained as illustrated in Tobie 26·3. Some eli ni eians prefer slatting heparin acutely afte r rtP..., howev~r the NINDS investi· gators highly recommend getting a non·contr.ut CT first si nce there was a significant in· cidence of subclinica l intracerebral hemorThag~s.
M ANAGEMENT OF PATIENTS NOT UNDERGOING THROMBOLYTIC THERAPY
These guidelines are forTIA, RIND, orCV"', but not 8M (for this .... ~ page 786) nOr intracerebral hemorrhage (ICU ) ($t~ pagt 856)". The following guidelines for initial monagement should b!' maintained 48 hT$ after last neum deterioration. 1. frequent VS with crani checks (q 1 hr x 12 hl'$, then q 2 hrs) 2. activity: bed rest 3. labs: .... routine: CDC .. platelet count, electrolyte" PTIP"IT, U,... , EKC, CXR,ABC B. -special" (when appropriate): RPR (to rule>(lut neurosyphjlis), ESR (to rule. out giant cell arteritis), hepatic profile, cardiae profile C. al 24 h",: CBC, platelet count, cardiac profile, lipid profile. EKC 4, 0" at 2 L per NC: repeat ABC on 2 L 0. 6. monitor card;a~ rhythm x 24 h", (literature qUOle1! 5·10% prevalence ofE KC changes, and 2·3% acute Mis in patients with CVAs) 6. diet: NPO 7, nutsingcare A. indwelling Foley (urinary ) cathet.erifconsciousness impaired or ,funable to use urinal or bedpan: intennilten! catheteri>.ation q 4-6 h", PRN no void if Foley not used B. accurate 1'5 &0 0'8; notify M.D. for urine output < 20 cdh r. 2 hTII by Foley, or < 160 cc in 8 hra if no Foley 8. TV fluids: NSor 112 NS at 75·1'25 cdhr for m08t patients(to eliminatedehy.One (penumbra)"'. Al· though hyperglycemia may be a atress re~pon8e and may not be neurotoXl~I, I'E \40 (malignant hy. pe rtension): ~ 20·30% reduc· Tllble 26·4 Guidelines lor lower limits of tion is desir"ble. Sodium trelltment endpoints lor HTN In alrokes nitroprusside (Nipride®1 IV No prior hlslory 01 Prior history of drip Or rv Ip betalol are .ge nts of choice; art.erial·line m~nitor recommended: sym patholytics dOnolk:Jwef SBP 16Q-- I70mrnHg lao· ISSmmHg (e .g. trirnethBpha n) conU"indi· cattd (they reduce CSF) do not ;;..,e, lOS-tIC rmI Hg 95· ' 05 rmI Hg 2. SBP>2300rOBP120-hOx20 DBPbeiow mins: ~ (unless contraindiceted. ste poge 4): lta rt at 10 mg slow rvP over2 mini , then doubleq 10 min (20, 40. 80, t hen 160mgslow IvP) untit controlled or total of 300 mg given. Main te nauce: effective dose (from "00,,,,) q 6·8 hrs PRN SSP> 180 Or DSP > 1 iO 3. SSP 180.230 or OBP 10S-12O: defer emergency treatment unle$l! there is evi. dence of LV fa ilure or if readings persist x 60 min s A. unlell$ contraindic ate ""'
"'"
OR oaptopril (Capoten®) 6.25 mg, 12.5 mgor 25 me: PO q 8 hrs
4.
sap"" 180 OT DBP"" 105: lIutihypertensive ther",py usually not indicated
ANTICOAGULANTS
H e pll rin: 1'0 date. there has been only one prospe.ctive tri~l" utili:ting heparin as it is administered in the U.S:", lind there w~ s no significant differe nce in outcome". Recent meg~trials" have shown that th e reeurrent s troke r"te in the 7 d"ys following a CVA was only 0.6·2.2% per week"'. £lfe-..r the compl ica tion rllt e h .. not been assessed prospe.c· l ively (sma U, nonrllndomi2ed studies have found sym ptomatic ICH in 1·8%. and other bleeding com plica tions in 3· 12%>1). Conversion rate of pale - hemorrhagic CVA i$ 2-5% (dog studies suggest t he risk is increased only when H'TN not well con",olled). Conclu· l ion: t he risk of heparin therapy for acute foca l cerebra l ischemia ex .... ed" IIny proven bene(it"', lind is nnl.ju.tified in mMt cases (" pe.cielly when use 1\. In 7' "",;"'11 with
duotd thr9a~ni'"
NEUROSURCERY
26. C.""brov8sculs .accidents
Stern a>mp"'Mion (particularly poIIterio. ponl). Clinical rUldinga generally increase be· tween 12·96 lin following ons.et. 8O'l. of patients d 50% of the MCA distrib ... tion 00 acan", midline shitt > 8-10 1JUll. early s ... lci effac:.!me nt and hyperdente MCA .ign". Ne ... rOllurgeon 5 O'Iay be involved in caring for th ..... pa tienl.s because lOme reporta have adv;atet! aggressive the rapiea in these pauenu in a n attempt to reduee morbidity a,nd mortality. Options ind ude: I . conventional measurn. to oontrollCP(witb or without IGP mo:LilOr): mortality i. still high . and ele vated ICP i. not. oom mon c....... ofin;tial deuriol"ltion 2. hemicra niedomy: Ir0 pi... ror •• r'; 35)'l'!l wit h :0. 1 ri.k facwrs (Sff bdow), mOllt had TlAs ea rlier) 2. e mbolism with rooognized source: 20% A. cardiac origin i8 the most COmmOn (see CardiOlJtllic brai1l embolism above), most have previously known cardiac disea se: I . rheumatic ~ ea rt disease 2. prosthetic valve 3. endocarditis 4. mitral valv~ prolapse (MVP): pre se nt in 5-1O%ofyoung adulta,in 2040%0fyoungadulta with CVA(although oneseri"" found MVP in only 2% ofCVA in young adults")
5.
3.
n.
A·r,b
6. left-atrial myxoma B. fat embolism syndrome: neurologic manifestation it ""ually gloo..l neu....,. loj,";c dysfunction C. paradoxical embolism: ASD, pulmonary AVM including Osler-Weber-Rendu syndrome, pIIl....'nt foramen ovale {s""CordiDtI~nic brain ~mboli~m aoove) D. amniotic fluid emoolism: may OCCur typically in (be post·partum period valSCu]OI,athy: lQ% A. inflammatory L Takaya$u', 2. infective: 18, syphilis, ophthalmic 'lJe 61): when COn· fIned to CNS is usually multi focal and progressive, but may mimic CVA early c. multiple .clerosis (MS) 26. Cerebrovascula r accidents
NEUROSURGERY
d.
cancer
e.
rheumatoid arthritis B. non· jnnammat.ory L fibromuscul$r dysplasia: S~ pase 63 2. carotid or vertebrlll artery dissections (i ncluding postt rllumlltic) 3. moyamoya disease : stl page 892 4.
4.
h om o cystinuria: a genetic defect in methionine mel.abolism that produces intimal thickeni ng and fibrosis in almost all ve&aels with associ.ted thromboembolic event/l (arterial and venou&, i.ndudingdural venous sinuses). Estimated risk of stroke is 1()"16%. Plltients have II Ma.fall's syndrome-like physical appearance, malar blotches, mental retardation. and elevated levels "furinary homocystine 5. pseoo"xanthoma elasticum coagu lopathy: 10%. The follnwing are associated with hypercoll.gulabJe stat.-s A. SLE: lupU5 anticoagulant - prolonged PrJ' incompletely corrects with 50150 mix. Collagen vascular disease only rarely presenUl in itially with
eVA
8. polycythemia or thrombGcyt.osill C. sickle cell disease D. TIP (thrombotic thrombocytopenic purpura) E. anti thrombin III deficiency (c:ontroveraial · not seen in large series of young adulta with CVA) F. protein C or protein 5 deficiency (familial ): protein C attenuates hemO$t.stic reactiOnl, homcnygous deficiency is fatal in the neonatal per;oo. Helerozy· gWlI deficte""y is aS$OCiated with thrombotic st rokes . A rare complication during initial therapy with warfarin is a drop in prolein C before other c0agulation factors resulting in a hypercoagulable state G. a ntipho8pbolip id·a n tibd y sy ndrome (APLAS)..·..: c8uses venous and/or arterial thrombosis. The two best known antiphospholipid-antibod· ies are anticardiolipin antibod ies (ACU ), and lupus antiCOllgulant (U C) . Once they become symptomatic, t reatment is high_intensity warfarin ther· apy to an lNR:t. 3". There a dramatic increase in thrombotic events after diso;:ontin~jng warfarin. Aspirin is useless H . following use of the drug 3,4·methylenedioxymethamphetamine (MDMA, known on th e street as ecstasy)", possibly independent of the hypercoagulable state that ocrura with hyperthermia when insufficient fluids are consutoed in conjunction with use of the drug peripa l'1.u m: 5'lt (usually within 2 wks of parturition) miscellaneous C!lU$eS: 35% A. uncertain etiology B. oral contraceptives (BCP ): BlI&O NEUROSURGERY
26. Cerebrovascular aco:idents
EVALUATION
1. 2. 3.
4. 5.
history & physical exam directed at uncovl!ring system ic disease (sa abo"" l and modifiable risk factOI"1l (t«< ltoo.... ) cardiology work up including EKC and echDCardiogram blood work (i ndude as appropriate), electrolytes, COC, pia.teletcount and/or function, F.SR (elevation mll.)l suggest SLi::. prteritis. atrial myxoma ... ). PTIPIT. VORL (should be obtained in all young adults witll stroke ), fasting li pid profile, AN A, pntitllrombin Ill . .,rotein C. PPO, sickle-cell >!Creen, toxi""logy SCrf!en (blood and urine, to RIO drugs sucll as cocaine). SPF.P.lupus anticoalf\llant. se ru m ami · no acid. tissue plasmingen.activator and -i nllibitor miscellaneous tests: UlA. CXR. CSF exam when indicated cere bral angiograptly: not always necessary for pa ti ents with obvious systemic disease or strong evideoce for cardiac embolism: may occasionally diagnose cerebra l embolism if perfornled within 48 hts of ict us
26.3.
Lacunar strokes
Small infarcta in deep nonC(lrt iC31cerf!bru m or brainTabl e 28·5 Typlcallocastem (.su Tobie 26·5) result;ng From ocdusion ofpenetrlltlions lor lacunar SIrokas ing branches of cerebral a rteries. Si ~e of infarcts ranges (in descending 'requency) from 3·20 mm (CT detects larger ones: better sensitivi ty putame, in white ruMter). caudate Small (3-7 mm) laCllnes may be due to tipohyalino_ tnaianlls sis (vasculopathy due to H1'N I of arteries ,,200 microns (may also be cause of many lCHs); this vasculopathy i~ inintemi!l CapsiJle (IC) dicative of sma ll vessel disease, unlikeJy to be prevented convoIWonal while mailer by carotid endarterectomy. Clinically, diagnO.'lis virt ually ~ by: aphasia, apr~et.agnosia. sensorimotor CVA. monoplegia, homonymous lIemianopsia (KH ). severf! isolated memory impai rmen~, stupor, roma , LOC, or seizures. L'etallacunaire: multiple lacunea - chronic progressive neutO decline with one or more episodes of hemiparesis: resulUl in invalidism. dysarthria, small-step gait (ms",he ~ petits pas), imbalance, incontinente. pIIeudobulbar signs. deme ntia. Many signs and symptoms a rf! possibly due to NPH (unrerogn ized originally).
.,.
LACUNAR SVNDROMES
M-.ior syndromes (see reference'" for others), l. pure ~ensory CVA or TlA, (the most common lacunar manifestation) usually isolated unilateral num bness offace, arm. and leg. Only 1000000fTtA go On to CVA. Lacune in sensory (posternventra)) lhlamus ..... cr detection is poor. Dejerine. Roussy . rare thalamic pain sy ndrome that may develop late 2. pure motor he miparesis (P1Iofill: (2 nd most common) pure unilateral motor deficit offace, arm atld leg with no sensory deficit, HH . ek .. Lacune in po!Jlerior limb of IC. Or in lower basis pontis where corticospinal (e S) tracta coalesce. or rarely in mid-cerebral peduncle 3. DISJ:ic hemi paresis: contralateral PMH + cerebellar ataxia oFalfected limbs (if tlley Can move). Lacune in balIi. pontis at junction of upper third and lower two thirds ..... dysarthria. nystagmus and unidi rectional toppling possible. Differential seve r ity in face, arm and legpoS.'lible because CS fibc ... dispersed by nudei pontis (unlike compact pyramids pnd peduncle) A. vanant' dysortt.ria-clumsy hand syndrome, lesion in lame locati(m Or genu of IC. May be mimicked by a cortical in farct. but lalter will lIave numb lips 4. PMH s pari ng tb e race , lse une in medullary pyramid: al onset. therf! may be vertigo and nystagmus (approaching lateral medullary syndrome) A. variant: thalllmk dementia: centr~\ rf!gion ofone thalamua + adjacent $ubthalamus - abulia. memory impairmen t + partial Horner's (miosis + an llydr05i$ ) 5. me..ence phnJolh"l amic syndrome: "top 0' the basilar e mboli s m". III palsy. Parinaud's syndrome & abulia , may have am nesia. Infarct typiclilly butterfly shaped & bilateral
,,,
26. Cerebrovascular accidents
NEUROSURGERY
6. Weber's iyndrome: Cr. N . Jl l pals)' with cGntralsteral PMH \ nG sensory IG"I. UauaH), due to oocluBlon of ' nl.tirpeduncular bra nch"" o(b"o,lar ar1.(>ry - central midbrai n infllT .. ,""'1_""_ ... ,,,,,",001OIi or ,, ~ _, ........ -.r d'" ",... ",,1I"o'. Am J ~l td7" SOIl·O. 1983. Tta« 1 F. er.,..fOfd V L S. \..owIon I T. , most to the aide of the Bneurylm SAM il ODmplicated by intrace~bral hemorrhage jn 20·40'!10, by ;nu.ventricula r hemolThage in 13·28'10, and by lubdu ral blood in 2·6'!11 1 0ft. evidence IU"el ls that nlPtu~ incidence is higher in . prins: and lIutumn pat~nls a 70)TII a,. havI a higher proportion ""ith II sevue neurologic i"lId~
Ri sk (acton (or SAW hypertenllon oral ""ntra Ocular hemorrhage Three type. of oc ular hemorrhage (Om may occur in uloci&tion with SAH. They OCCU r a lone Or in vlUioue combinaUonl in 20- 4~ ofplltienl* with SAW'. I . . ubhyaloid (preretinal) hemorrhage: l etn fundullCOpicaUy in 11·33'1f> of cues u brigbt red blooe; near the optic di .ec: th at obacu ru th e ~ nd erlying retinal v_la. May be auociated with a higher mortality rate" 2. {i ntra)ret inB.l hemorrbag.: may . urround the fovea 3. hemorrha,e with in the vitreoul humor (TerlIOn', .yndrome or j ... t TtI"""D ~ drome). OocW"l in 4·27'1f> OfUM' of.neurysmal SAM· ..... uluilly bilat.e Nl I. May OC 3 mm diameter (wmpared to intra-arterial digiUll l ubtraction anglo)"·· although rates as high as 95% have been quoted" . ~ false positive rate il ~ 16%"'. There are a number ofcompHt'8te.t" (.... m the International Cooperat;ve Aneurysm Stud~ revealed that with nonnal ron.ciou.ness. Hunt and He•• (HAR) grades 1 ami 2 had identical out«ome. and that hemiparuis and/or apha,ia had no effect on mornlity.
origInaI_'
..
Mortality: Admission Huntand Hess Grade 1 or 2: 20%. PatienU Ulken III O.R. (rDr any procedure) at H&.H Grade 1 or 2: 14%. Major cause or death in Grade 1 or 2 is rl!bleed. Sign' of meningeal irritation increases surgical ri sk.
NEUROSURGERY
27. SAH and aneurysms
'"
WORLD FEOERAnON OF NEUROLOG IC SURGEONS (WFNS) GRADING OF SAH
D .... to lack of data On the significance offea t W"\!s
such as headache, nuchal rigidi ty, and major focal neurolo¢c deficit, the WFNS Committee On a Universal SA}{ Grading Scale gTading system was developed a nd is shown in Toblf 27·4. It uses the Glasgow Coma Scale (GCS) (see Toblr 8.1, page 1541to eva luate level of conaa. Ol hemi· pleglO (+ . present. __ 80NnI)
hetI'Iipa"'' '
inlaCl ano"ry$IIl
27.3.
Initial management of SAH
Ini tia l management c once rns 1. 2.
rebleeding; the major concern during the initial stabilization"'. hydrooophalu.s; acute hydrocephalus i.s usually obstructive (due to blockage of
CSF flow by blood clot). In later stages, hydrooophalus is usually communicating 3. 4.
5. 6. 7.
(due toxic efTect ofblood breakdown products on a rachnoid gTanulation.) (seeHy· droctpholus oft~r SAR, page 790) delayed i""hemic neurologic deficit (nINO). usually attributed to vasospasm. Begins to be of O(JJlCem 8e'~ra l days following the SA}{ hyponatremia with hYpovolemia: M'f page 788 DVT and pulmooary embolism:.re pn~ 25 seiz ures: I « JX18f 787 detennining source of bleedi ng: usually requ;re$ 4·vessel cerebral angio gTAphy . The timi ng of this takes iotoconsideration the patienta conditiQn (uostable or pre·morbid patients are not candidates) and the possibility of early surgery (an. giogTaphy i. UIIually do ne as SOOn as p H VPONATREMIA FOLLOWING
SAH
SAH
Backgro u nd Hypovolemia and hyponat remia ~uently follow SAJi as a. res~ lt of natriuresis and diuresia. Although hyponlltremia had been attributed to a rise in ADH" (thought to produce SlADH with hypervolemia). tile ADH inCnlmenl is u~ually transient, lasting only ~ 4 days and hypervolemia did not OCCu r. Another theory is based on the fact that there is often II delayed peak in atrial natriuretic ractor (.ANF) (a 28-amino add polypeptide) after an initial smaller rise" that "'as frequently followed by urinary loss of sodium le .. rebra l salt w astiDg (CSW). su pose 14) that mimics SlADH, and volume depletion . Although CSW has dearly ~n .hown to be the cau'le of h~ponatremia in the majority of these patie nts", there are still doubts that A.."F Is Jh~ operative natriunltic factor in SAH". A ri.e in ANP and brain natriuretic peptide (BNP ) al\.er SAJi i. associ · ated willi the development of a negative fluid balance'"'. Routine labs are identical in SIADH and CSWl-'. but the extracellulAr fluid volume (which is more diflicull to mew>ure) is low in CSW and is normal or el evated in SlADH (see Tobie 1.7, pll!ge 15 for a comparison of the two condition.). The neurologic effects of hyponatremia (,ee POse 12) may mimic delayed ischemic neurologic deficit from vasos· pasm, and hyponatremic patients have about 3l.imes the incidence of delayed cereb ral infarction after SAH than normonatremic patients"". factors that may increase the risk or hyponatremia alter SAH incJud~: history ordlabetes, CHF, cirrhosis, adrenal insufficiency. Or the use of any of the following drugs: NSAlDs. acetaminophen, naTC:(ltics, thiazide diunltics"'. Treatment II Caution! ~strictiog fl uid, which is the treatment for SlADH may be haurdous in the case orcsw (wh ich is mOre likely to OCCUr after SAH thnn i. S IADH) since dehydrstion increases blood viscosit y wbich uacerbatcs ischemia from vasospnsm"". treat hypovolemia with infusions ofcrySt.alloid (e.g. NS), PRBCa, or colloids (avoid repeated admin;';tration ofhetasta rch , su abo",,) treat the hyponatremia ofCSW with supplement.al PO sa lt intake, NS or hyper· tonic saline (rapid colnlCtion or over-(:OlTection ca rries the risk of central pontine myelinolysis (C PM), !u pGie 12), Or flu-drocorti80ne acetate (0.2 mg IV or PO BID, ri$k$ include pulmonary edema, hypokalemia and HTN ), ""~ page 15 • one study used UreS (U rf!apbilq) 0.5 gram!ilkg (dissolve 40 gm in 100·150 ml NS) IV Over 30-60 mm s q 8 hrs"" and used NS + 20 mEq KCVL at 2
".
27. SAH and aneurysms
NEUROSURGERY
mllltglhr .. the main IV until the hyponatnlm ia waa ,",'nl d.!;;urf HYDROCEPHALUS The frequency ofbydrouphalu$ (B CP) On the initiod CT alter SAH depends ollihe defining criteriA U5~. with a repo~ range of9·S1.,.. .... A realii!tie rang" i.s _15.20% of SAH patiellbl. with 3Q·60,-,>oftheaeshowing no impairment nfoon$CiQUsnes6"" , ''', 3%of thOH ,!ithW HCr on Initia l CT de.velop HCP wilhin 1 wef!k'''. F'""to,." relt to Mntribuw to .tut.. HCP indudll: blood interfe ring with CSF now througb tht' Sylvisn aljuedud, fourth ~.,nt~iole outlet . Gr i:ubar.chowd Sp!lef'. Ind/llr with N!.bsorption ,1 the arachno id granuilltio"", Findings usodat.ed with acute HCP includ e''': inc:reasing ege "dmilSion CT fjnd ingB: Intrave.ntrioular blood, d,(fu5e s ubarachnoid blood, and thiek focal accumulation ()fsubarachnoid \)I00d (iMrapa.renchympl blood did III!l. tOrn:!IDL6 with chmnie Hep. lind palienU with a normal CT had nlow Incidence) hyp.. rten$ion: on admiUion, pnor to admission (by history), or post-lLp by location: • post~rlor cin:ulatioll aneurysms have II higher iotidena- of HCP • MCA aneurysm5 f;OrTelBLe with low incidente of HCP ,,,illCellatleiL"": hyponatl'1!mill, patients who were not nlltrt Gn acimil1lion, U96 of preoperativlI ."Iifibrinolytic agellts. lind low Glasgow outtome &cOre TN; lItm ent
About half the potienl8 with BtU"' HCP and impaired consciousnen Improved lpont.aneously'''. PatiellL'i in pOQr gnde (fl&.H fV·V) with large venlriclu may be l ympl CHRoNICHCP Chrontt HCP I.s due 1.0 I'iJ·arachnoid adhe9ion9 Or penna nent impainnent of the atllchnoid grllnulatlons. MUIt' HCP d~ not loevitably lead to cl1ron"ic HCP, 8"",5~ (reported range in literature''') of an ruptured anturysm IIILtitllts, and · 50% ofthOEle with acute HCP foll(>Wing SAl-! need permalW'lt CSF diver!UOIl . '!'he presence ofintrDvefJtric1118r blood increases this risk"", There ill ront.oversy as to wh&t.h~. the u&e ofventricu· lostomy for acute HCr increas.esl" Gr po>!llibly even decreases'" the incidence Gfshunt depeodency.
'"
27. SAHandaneuryijma
NEUROSURGERY
27.4.
Vasospasm
Cerebral v8S08pasm;s a condition that i, most commonly seen following aneurysmal suba rachnoid hemorrhage (SAlt ), but roay also follow other intracranial hemorrhages (e.g. intraventricular hemorrhage from Avr.1"·. and SA}{ ofWlllnown etiology), head tra uma (with or without SAHI"'. brain surgery, lumbar puncture, hypothalamic injury. infect ion , and may be associated with preeclampsia (su paS( 64 ). Vasospasm has two not-necessarily r"":,,,cilable definitions (see lh(rnilulfIS below); I. clinical vasospasm; U t 1M/ow 2. radiographic va~spasm; I;t!e 1M/ow
27.4.1 .
Definitions
CLINICAL VASOSPASM
AKA de layed ische mic ne urologic deficit (DlND), AKA symptomatic vasospasm. AIIclAxfd focal ischemic neurologic deficit followingSAH. Clinically characterized by confusion or decreased level of consciousness with focal neu.rologic deficit (speech or motor)_The diagnosis is one ofexdusion, and sometimes cannot be made with cerUllinty. For dinical findings, $« pase 79/ . RADIOGRAPHIC VASOSPASM (AKA ANGIOGRAPI-/IC VASOSPASM)
Anerialnarrowing demonstrated on cerebral angiography, onen with slowing of Ctlntrast filling. The diagnosis is solidified by previous Or su~uent angingrams showing the same vessel(s) with normal caliber. Since only larger arteries may be visualized angiographically. the diagnosis is limited to narrowing of these vessels . SYMPTOMATIC VASOSPASM
When the DlNO corresponds to a region OfYa80spasm seen on angiogram, this is somelimes referred to as ' symptomatic yaS06pasm~.
27.4.2.
Characteristics of cerebral vasospasm
Clinical findings Findings u.ually develop gradually, and may progress or fluctuate. May include: J. non_locali~ing findings A. new or increasing HlA B. s lteration s in level of consciousness (lethargy ... ) C. disorientation O. meningismus 2. focal neurologica l aign$ may occur indudingcranial nerve palsies Bnd foca l JI)Otor deficits. Also, symptoms may tluster into One of the following ·syndromes" (vasospasm incidence is b.igher in the distribution of the ACA than in that of the MCA) A. . ., t-erior cOrICA not 1111 patientll with SAH de'·elop CVS. and CVS can follow other insults besides SAH (e_g head trauma ""thout SAH) the Hunt and Hess grade on admission cor..,la tes with Tllble 27-5 Conel l-the ri\lk ofeVS {S, sevCfe vasospasm >200
.-'", "'CT
*
50 cm/!;ec may luggest va sospasm, There is lell colTelation between velocities and vll$O!l' pasm in the anterior ce rebral arteries (ACAl. Di slinguishing vaSOSPIISfII from hyperemia (which incre""es blood flow velocities in both the MeA and the ICA} is facilitated by us· ing the ratio of these velocities (the so-ca lled Lindegaard ralio ) al$o s",own in Tobit 27. 9.
27.4.5.
Treatment for vasospasm
SeeJKl6e 796 for management protocol. Numeroua treatments for cerebral arterial vaS0:9P11sm have been evaluated. See the au .... ey articles by Wilkin.''', ,.. for an "'~tensi v'" list of agenta and techniqu",s studied. Va· _pum in humans dD 1Teatment options rail into the following categories: 1. direct phannacological anerial dilatation A. smooth muscle rela~ants: I. calcium channel blocke ....": did Dot succeed in counl ~Ptetlow) to increase sap in
l~
increment.l untill\f!uro-
logietltiy im proved or the follow in, endpoint.l all reached • el",vateCVP to 8· 12 em H20, Or PC WP to 18.20 mm (for undipped oneul')'lma: CYJ> 6·10 COl H:tO. PCWP 6-10 mm He) • maxlmUIIl SP in clipped anaury.lOS: SSP < 240 mIn Hg. mean SP < 160 (fo r unclipped aneurylm: S S P < ]60) • redo.u:tlon of elevated TeD rudinp hlIck toward, "-seline then 81l0w SP to fall to level required to I Ultain a~ptoble ne..rologie func\.ion ift riple · H therapy fail l. UM endovucular ted>niq ... if 8...,il.bI. (f« po.p 795)
He
a.
Method" of ind uc ing hyP erdynamic: th erapy Proceed to each step only ifnfflded to roeet above endpoinUJ or reverse neu rolO(icdefidt. I . volume e~pnoa ",.in..." thot ' - I
A. " - CVPt
'"
27 S AM and aneuryltnl
NEUROSURGERY
I . catheu!r related sepsis: 13% subclavian vein thrombosis: 1.3% 3. pneumoth<Jra~ : 1% 4. hemothorax: may be promoted by coll(ulopathy from dextran '" 2.
NEUROLOGICAL OUTCOME
The above protocol was used in 58 patient.. with vasospa sm (22 unsecured aneurysm, 2 SAH of un MOWn etiology) with the following result..: neurological improvement ooeur~ in 8 1%; temporary in 7%. Nochange was Mlt'n in 16%. l&il> deteriorated .
CALCIUM CHANNEL BLOC KERS
Tr ials wi th calc ium c h a n ne l h loekeMl Calcium channel blockers (CCB) ( AKA calcium antagonist) block the · slow..:hannel" of calcium innux which ~uces tbe contraction of smooth and cardiac muscle, but does Dot affect skeletal muscle. It is thus theorized that the abnormal contraction of vas· cular $moo th muscle that may contribute to vaS09pa.m may be mitigated by the adminiStration ofCCBs. CCBs may be more beneficial in neuroprote Sid e e{{ec ls o{ eeB s Possible side effect.. include: 1. systemic hypotension: may be mitigated by admioistra~ion ofintravenous volume expansion 2. renal failure 3. puLmonary edema
27.5.
Cerebral aneurysms
EPIDEMIOLOGY
Incidence difficult toestimate . Range ofautol'$y prevalence ofaneury8ms: 0.2·7.9% (variability depend8 on use of dissecting microscope, hospital referral and autopsy pattern. overall interes t). Reoent studies '" indicste~.kng of 15%. Ratio of ruptured ,un· ruptured (incidental) aMurysm i. 5,3 to 5:6 (rough est imate is 1,1, i.e, 50% of these aneurysm~ rupture)'''. Only 2% of aneurysms pTaent during childhood'''.
NEUROSURGERY
27. SAH and aneurysms
'"
ETIOLOGY
The e~act patbophysiologyoflbe develOpment ofaneUry$m$ i~ still CQntroverliial.ln CQntraat to ntracranial blood "essels, tbere is less elastic in th e tunica media and adventitia of unrupt ured aneurysms, and those ... ith SAH, should unde rgo angiography Dnd aubuquent surgical rep.ir of aoy aneuryl ms discovered (especial ly those> I cm diameter). A decision anal~'Bis study"' determined that !!Creening witb M.Rt. was beneficia l compared r.o treating patients once they bec8 me symptomatic. In a young patient with AOI'KD with eithe r s hi.tory of aneurysms or a kindred of ADPKDwith aneurysms. repeat !!Creening may be effectively ~peated ev· ery - 2·3 ~O ... (in s kindred of AOPKD without aneurysms. every $-20 yrs was recomme nded )2O".
27.6.
Treatment options for aneurysms
The best treatment fo r a~ aneurysn, depends on the condition of the patient, the Mstomy of the aneurysm, the ability of the s urgeon, a nd must be weIghed against the na t ural history of t he condi tion. When t reat ment is indicated , surgical "dipping" of the aneurysm at the neck to eotdude it from the cirtulation is considered 1 be the optima l treatment for most ruptured aneurysms . For unruptured aneurys ms, Iie~ page 816. The id eal gool of surgical treatment is usua lly to place a cli p acl'OU the neck of the llneurysm t.o excl ude the Meurysm from the cirt ulation (see belowl without occ luding normslvessels. ""'hen the aneurysm cannot be clipped because of the nature of the aneurysm , or poor medical condition of the pat ient, the options below may be considered. Decisions rega rding treatment optiOlU have to take into account the natural history of the aneurysm. This involves informa tion rela ted primarily 1: I. risk of bleeding into su barachnoid space A. ror ruptured aneurys m. : t his i. the ri sk o( rebluding: _ pap 189 Il for unruptu red aneurysms: ' " fXlRe 816 C. (or cavernOuS carotid artery aneurysms: this risk is low (su pose 818) 2, SpootO lleoU' tb r omb osis of lin a neurysm is a rare occurnnce>'*"'" (estimates in " .. topsy series is 9-13%"'). However they may reappear--, a nd delayed rupture may OCCur someti mes even years lllter
Thera pies t bat do Dot d ir ec tly ad d ress the ane u rys m The hope he re is that the aneurysm will not bleed and t hat it will thrombose (_ abou~l. 1. continu. m.dieal man"S.. m.. nt initigt.d on admiui on: i .... control of HTN, con(in· ue calcium·chsn nel blockers. stool softeners ... continue bed rest fo.- 1 week th en allow bedside commode 2. treotm ~nt options generally lll!l used A. antifibrinolytic therapy (e.g. ,·amil>OCsproic aci d (EACA)): _ NB: I:illl:. l!SEll. Reducet. rebleeding, but incre8Jl,," the inci der>ee!lf a rterial VaSOs· pasm and hydl'OC'lpha lus"" B. seria l LPs : an historical treatmen t"·, may increase the risk of a neurysmal rupture
E ndovasc ul a r and othe r
" no ns u rgicaJ ~ tech niq ues to t r e at t he a neurys m I . t rapping: e ffective trea t ment requires distal AND proximal art.!ria l i n ~lTuption by direct surgica l means (ligation or ocdu sion with a cl ip). by placement ofa detachable ba lloon"', or some combination. May also inco rpo rote vascula r bypass (e.g. EC-IC bypass) to ma intain flow distal to trapped segment'" 2. proximal ligation (hunteria n ligation ): useful for gia nt aneurysms""''' ' For non ·
.m
27. SAH and aneurysms
NEUROSURGERY
3.
giant nneurysm6 provides little benefIt and adds the ris k or thromboembolism (whic h mny be reduced by O. Of aneurysms no~ initi811y o«luded"': I . 46% progressively tbrombosed 2. 26% show...! st able neck remnan ts 3. 28% showed enlargement of residual neck 2. late failure A. failure of partially obliterat 75 yrs ): there appear. to be a s ignificant reduction in morbidity with ODC rompared to MS 4. aneurysm ronfiiUration-: A. dome-ta-neck ratic> (AKA fundu.·to·neck ratio) " 2 n. and an absolu\.e neck dismeter ,, :; mm Poor ~andidates for OOC: I. giant aneurysms b 2Q mm diameter)'" 2. very small aneurysms"" 3. aneury~ms with wide necks'" (including small aneurysms ) ControveT3;a! a~a8 with ODC: I. unruptured aneurysms Patients with re~ idual filling of the aneurysm should undergo surgical dipping since there is significant risk ofrebleed ing.
27.7.
Timing of aneurysm surgery
Cc>ntroveT3Y exists between so·called ' uriy lur£ 'u pq.ge 815 for timing issues related to basilar bi furcation al1eury~m • .
EMly surgery advocated for the following reaSOnS: 1. ifsucoessful. vil'l.ually eliminates the risk of rebleeding which occuI'll most fre· quently in the period immediately following SAJi (see &bludirtg. page 789) 2. facilitates treatment ofvasQ8pasm whlch peaks in incidence between days 6·8 post SAH (never seen before day 3) by allowwg inductin ofal'l.erial hypertension and volume expansion .. ithoutdanger of anewysmal rupt~ 3. allow. lavage to remove potentially v8sospasmogenic agents from contact with vessels (including use of thrombolytic agents. $H pag~ 794 ) 4. ~Ithough perative mol1.ality is higher. overall putient rtlortality is lowe ..... Arguments against early surgery. in favor orlale . urgery include: l. inllammation and brain edema are mOllt IIf!vere immediately Mlowing SAH A. thi. neteuitates more brain retracUon B. at the sa me time thissoft.ena the brain making retraction more difficult (re27. SAJi lind aneurysms
NBUROSURGERY
~r~ttors hv, more wndency to I~c• • ow Ihe more fri~bl. b.~in ) 2. Ihe presence of.olid dot that hOI not hod time to lyae imped"lurgery 3. the ri~k of ;ntra-ope.. tiv. rupture;, higher with eo.ly surgery 4 . possible inc.eaa
el in surgical oomplieationl in good lUld bad grode patiellts with anteriorartulation aneurys",,'" 3. aneurysms difficult tocl ip becauM! oflllrge si,... or difficult location ne/'rative drainage ofCSF from cisterns 3, diuretics: msnnitol and/or furosemide. Although proofis lacking, lowering lCP by this or any means msy theoretically increase the risk ofrebleeding'"
L umbar spin al drainage May be inserted with Touhy needle following induction of anesthesia (to minimize BP elevation), prior to final poiIitioning. CSF is gradually w:ithdrawD by tbe anesthesiologiat onlY aner the dura is opened (to minimize chancea ofintrsoperative aneurysmal bleeding), usually a total of30-S0 ec are removed in _ 10 ec aliquots. Risks include''': aneurysmal rebleeding(" 0.3%), back paiD (10%, may be chronic in 0.6%), catheter malfunction preventing CSF drainag C EREBRAL PROTECTIO N DURING SURGERV
PATHOPHYSIOLOGY OF CEREBRAL ISCHEMIA
The ce .... bralme t a bolic rate or o"yreD consumptio n (CMRO.) (_ poge 763) arises from neurOns utiliting Imergy for two functions: 1) maintenance of cell iotegrity (homeoslallis) which nonnally oc 27.10.
Aneurysm type by location
27.10.1.
Anterior communicating artery aneurysms
'""' aingle m.,.t eommon li to of . neurysrmo prese nting with SAH"'. May present with diabeu.. insipid .... ( 01) or other hypoth. lam ic dysfunct ion .
CT SCAN
SAH in these.""ury,,,,,, "'11 ... 118 in blood in the anterior inte rhemispheric fiss ... .., in esse ntia lly all cafeS. a nd il associated with intraoerebral hematoma in 63'10 of casestol• Int raventricular ho.matoma iSleen in 79'A> of cafeS, with the blood ente";ng the vent ricles frorD the intracerebral hematoma in .bout one third of these . Actlte hydrocephalus was present in 25'lt of patient8 (I. te hydrocepha l..... . QOmmon aequelae ofSAH. w • • not s tud. ied ). Frontal lobe infarct8 0teur in 2O'k. usual ly several days followingSAH .... One of the few ca ...... orthe ra ... findingo fbill'lteral ACA d istribution infarcla is vasospasm (ollow. ing helMlThage from nJpt ... ..,of.n ACoA .neurysm. Thi, result8 in prefron tal lobotomylike findings Of8p8thy and abuli • .
ANGIOG RAPHIC CONSIDERATIONS
Essenti.1 to evaluate oontral.teral carotid, to de termine i(both ACAli fill the ancurysm. lf the . ne ... ryam fi UI wit hooe aide on)'. it is desirable to injel:t tl-•• othe r , ide while cross eomp~uing t he lide t ha t fills the aneurysm to lee if cnllater.1 now is plUent. Also. determine if either carotid fill. both ACAa. o r if ea 27.10.6. 1.
Ophthal mic seg ment aneurysms-
O phthalmic segment oneurysR1.9 (OSAs) include (t\B: nomenclature varies among au· thon): 1. ophthalmic artery aneurysms : 2. superior hypophyseal artery aneuryiml: A. parac1inoid varia n t : usually dlle$ not produce visual symptoms 6 . suprasella. variant: when giant, may mimic pituitary tumor on C1'
P RE SENTAT 10N (EXCLUDING INCIDENTAl DISCOVERV)
QPffTHALMIC ARTERY ANEURYSMS
.
An$ In Vsm,ura', IeriH-, lK fl·traumatic VAA MOIit VAAs arise at tbe VA·PICAjunction. Other sites: VA·AlCA. VA·BA. A NG/ooRAPHIC CONSIDERA nONS
Angiography ofVAA sho uld DSSI.'SI the contralateral VA for P8tency in case of the need to trap the aneurysm. Al lcock leet (vertebral angiography with carotid compression) may be used to aKllesa patency of circle ofWi1li". Test o.xlusion with a balloon c8th· eter can determine ifpatient will tolerate ooclWlion (a double lumen balloon will even allow measurement of illstal back preS8ure).
PICA ANEURYSMS For PICA anatomy. Ilet! Figurt 3· / / . page 80. For arteriogram. see Figu re 5·5. page 183. Comprise - 3% of cerebral anel,lrysms . 3 common sites: 1. VA at the VA-PICA junction''': A. S8.c 27.1 0.7.2.
Vertebrobasilar junction aneurysms
ANGIOGRAPHIC CONSIDERAnoNS
If add itional vie ws are needed t.o beuer demons tra te aneurysm Try oblique 15' away from injection side. center beam on foramen ma.gnum. orient tube 2S' Towne. Try ,ubmentovertex view .
~· ray
'"
27. SAR and aneurysms
NEUROSURGERY
SURGICAL APPROACHES
t. suboccipital spproach: for molt; perfol"lTled in l~te r~1 oblique po3ition 2. aubtempOral.tran$te nl.Qrisl approach if the ve rtebrob8llilar Jl,Ulct ion II high: per· formed in lupine poailion
27.10.7.3.
AreA aneurysms
ANGIOGRAPHIC CONS/OERA nONS
JJ add iti o n a l v ie w . a r e n eed e d 10 be tte r d e m o n s tr ate a n e urys m Try AP or lubmenlOvertex view, center beam on n8llion, orient x·ray tube If>' caudad.
27.10.8.
Basilar bifurcation aneurysms
AKA bu ila r l ip a ne u ryama. The most common poateriOf" circulation a neurysm. CompriH. 5~ ofintracranialaneurylm•. PRESENTATION
]llOit present with SAM indiltinguishble from SAJi due 10 ante rior ci rculation an· . ... rySm.1 rupture. Enll~mentof the aneurysm prior to rupt ure may rarely compress theopticchia.m - bitempollli r.eld cu!(mimicking pituitary t umor), orotca$ionally lIlay comprtU the third ne~ al itexil.5 from the interpeduneular fOlIl8 - otulomotor ne""'e JWlI.r-. CTIMRISCAN May occllion.lIy be _0 00 C'l' Or MRI II round rna .. in region of suprasellar cis · tern. W,th SAM . tend to _ blood In inteJ"fleduncular ci stern with $Om.. ",nux into 4th (a nd to a I.....,r "'tent., third and lateral) Vf'ntride. CkcalionaUy may mimic pretruncal nonaneuryamal SAH c-. ~ 823). ANG~PHY
Dome usually pointlluperioriy. Should "".Iuate now through pO 27.11 .
Unruptured aneurysms
Unruptured intracranial aneurysms (UlA) includes in cid ental ane uryams (thOlie that do not produce any symptoms and are discovered incident ally) aDd aoeurysms that produce symptoma other than those due to hemorrhage (e.g. pupillary dilatation due to third ne ......e compression ). UlA meritwnsideration for trea t ment l ince the outcome from SAB with or withoutsurger)' is poor even under the best ofcireumstances. About 65% of patients die from the firat SAll.... and even in patienl.ll with nO neurologic deficit after
'"
27. SAH and a neurysms
NEUROSURGERY
aneurysm rupture, only 46% fully recover, and only 44 'k retum to their fonner jobs', es· timated prevalence of incidentsl aneurysma ill 5-10% oft·he populali(>n'. PRESENTATION
See items other than "rupt ure" in
Pr~..,nl{J.lion
of UntUI)'smf. page 800.
NATURAL HISTORY
Risk of bleeding from UIA differs from aneuryslTI.'I that have ruptured . Troe risk is not I<novm ..... ith tertain ty. The largest. most detailed s tudy to date is the ISUIA" '. Spontaneous thrombosis of un ruptured aneurysms may OCCUr rarely (stlt P'18t 802) . The natural history and treatment tel!ults are influe nced by'" :see Surgical oulCO~ be· low): L patient fattor" A. historyof previousSAH from a separate lIneuryam'" signifitantly increases the risk of rupture of an UIA B. patie nt age C. tonttll"l"ent medital conditions aneurysm cha racteristics"" A. aneurysm size: the most important predictor for future rupture (ue be/ow) except (for unknown rell!ons) in patie n\.!! with prior SAB from anothe r source B. lotation: p .-1
Man agement recommendations baaed o n aneurysm s i ze Numerous recommendations have been made for a critical size llbove ,.... bieh an Ul,lrup. lured aneurysm should be considered for .urgery. and bave included 3 mm'*', 5 mm .... 7 mm" ', and 9 mm' ''. And again . the patient's expected longevity must be taken into aC' COunL One proposal is to promptly treat unruptured aneurysms ," 10 m'n. to repair thOlle measu ring 7·9 mm in young Rnd middle·aged patienta, and to follow smaller aneurysms with serial allgioguphy.... S ummary or the American Heart Association Stroke Council recommendations Table 27·13 summariz-
[=:T;'~'~'~'i':7:-:'1'===S~~~~~~~~~~::J
rnent made based on a \'tI factol'll favoring treat_ review of the literature'"' (only level JV and V evi · dence was found. and therefore only grad e C recommendations Can be made (Le. an array ofpoten. tialactions. any of which could be considered ~ppro_ priate»U"'). Pat;entafor whon, expEl artl1nll l epl l la~I ' (rom r up.lure IntO ,pheno:d II ' n" llul ually with traumatiC a neuryl ms, - fKlll'
820)
"d . ~ carot, nnR>
3. emboli Indica tions for t reatment: I . un rupt ured CCAAlI: the natural history il not p~jHly kMwn A. Iymptomatic; pa t ient.s with int(ll"r.ble pa in or vi l u.1 problem,.. U. gi"n! aneu ryama: eapeda!Jy those that straddle the cli noidal ring'" C. aneUry!lm~ that enlarge on IIlri,,1 ima,in, D. controvenial: ineidenlal lneu .... in the diat ri bution 0(, I~ic(arotid ,rtery (or which carot id l!ndarte~tOlny is indieat.ed. 'l'bl!re has been no evide" CfI that doing the endarterectomy inere.." the ri. k of .... pt.u re, I "d ... indicated above, mOlt ruptur" are not life threa~nin ll, "d 10 the urotid disene should be t reated according to il'a own ".erita 2. ruptured CCAAol: A. emergent !.reatment for easel with epillaxi, Or SAH B. ul'8ent !.reatment for CC F, with severl! l!Yl! p.i" -. Analysi. of ease reo A....bwO 27.14.
Traumatic aneurysms
Traumatic aneurysms (TAB) comprise < I,*,ofintraerani.l aneut')'Sm ..... "'. MOil are actually false o;on euryama, AKA paeudOllneuryams la ""ptu .. of.1l thevellel w.lllay· erS with the "wall" ofthe aneury.m being formed by aUTTOundi n, «",bralat""eture~"). They may occur rarely in childhood. The mechanism o(injury ulually falls inloOooeohhe followin, group$''': I. those arising from penetrating trauma : uaually from gunshot ",ouMb. althourh penetration with 0 .haTP object (which il leu common) may be, mo.. prone 100 cause traumo;otie an eury sms''' 2. those arising from dose::! head injury: more common. Theories o(pathogeneeia in. elude tr.ction injury 100 the venel w.lI or entrapment within a fractUnl. Tend 100 oca>r either: A. peripherally I . distal anterior oerebr.lartery aneury. m&: ROOIIda'Y to impart against the ialeine edge 2, distal cortical artery aneu rysms: olten auociated with I n overlying akull frartu re, someti....... s growing akull fmctu", B. at the skull base. usually involving the ICA in one of the following l itH: I . petroUI portion 2. caYi!moUS carotid artery: a. aneuryam enlargemeot mayeause. pro. virtually.l· arusi"e ca~rnoul .inus ayndrome WRy$ t.MOciatb. ""ptur.. may lead to a poettraumatic ea· ed with b...... l rotid..:avernous fistula {IU {X16e 845) or akull fractures to mauive epistaxis in the pusenoe o(a sphenoid .inu. frarture'ow" 3. l upr.dinoid carotid artery 3. I. trarenlc: fol/owinr lUl'(tty in Or around the akul1 bese, the ain uaes.ororbita(in· dud, ... followin, tmn..phtnoid.l.u~ry''') P r Ue ntatio n I. del.}'fll intraemni.1 hemorrh.ge (Iubdural. l u barachnoid. intrl"entricullT. Or intr.... renehymal ): thtt mOllt 2,5 em I~ 1 inch ) diameter. Two types: saccular (probably an enlarged "berry" aneurysm / lind fusiform , Comprise 3-5% of intrscmnia l aneurysms; peak age of presentatiGn 30-60 years; female:male ratiG" 3: 1. Drake's series of \74 giant aneurysms""': 35% presented as hemorrhage, with 10% s howi ng some evidence of remote bleeding. The bl~ing"'te is unknown. but is probably less than the ~ 2%lyear for non-giant aneurysms.
NEUROSURGERY
27. SAH and aneurysms
'"
May also present II TlA5(by reducing flow Or by emboli) or as a rn a"". About one third hive I neck Imen,bl. toclipping. EW.WATION
Drake cont.endl that even alUlr thorough radiographic e"aluation , actual operative vilua litation ill the only way to defi nitively assess the a neu rysm and its branches. Angio,... lO: On.en undereatiruteothe lite o(the le~io" se The actual etiology has y.e t to be determined, but it may be secondary to rupture of a am all perimesencephalic ve .n or capilla ~, Presenta tion Patients may present with seve re paroxyllmal HlA, meningismus, photophobia, and nausea. 1..0115 consciousneSli is rare. These patients a re usually not cri t ically ill (all "'ere grade lor 2 ), however, (ompli.:atiGns such as hypolUltrclllip or cardiac abnormali. t ies may Qccu r. P reretinal hemorrhages and sentinel HlA have not o::.:urred. CT and/or MRI demonstrate characte rist ic findings (s« below) although it may initially be mi95ed on CT"'". and LP may yield bloody esp. All have negative angiography.
or
Epidemiology PNSAH has been reported to oomprise 20·68% of cases of angiogram·negat ive SAHw ,,,,,,, (dependi ng on th e ti ming ofCT, adequacy of angiograph y, and the definition of PNSAH). However, the true incidence is probably Illore in the range of 50.75'lr""'. The reported age range is 3·70 years (mean, 50 yrs>W, 52·59%.re male, and pre. e~isti ng HTN was present in 3·20% of patients.
Relevant a n atomy Posterior fossa cisterns, The perimellencephaJic cisterns indude: interpeduncular, cru ral , ambient a nd quadrigeminal cisterns. The prepontine cistern lies immediately anterior to the pons. LiJiequist's membrane (LM)'"": BasiCIIlly considered to separate the interpeduncular cistern frorn the chiasmatic cistern'" (fanning a oompetent bamer in only 10·30%). In fu rther detail, the superior leanet ofLM (diencephalic membrane) separates the interpeduncula r cistern from the chiasmatic cistern medially and from the carotid cisterns laterally""'>U, The inferior leaf· let (the me$encephalic membrane) !l '"
References
"', ..... F f': S"f]iI " ... ....", Qr "'kSt.""'i ~fD{ ' •. 01.1."""')'.".. Ctin :\I",n)5IOI". :n. " ~l~ , 1\1811. Gno< .. K .... "".,.,(&/>OFF.I""-" ... , 'I,. J N... '.....,.~ S)'",",.$-~l " m
nl mmninj Hi HHiH mh H~ h[ mmIi a{ Hif itt! nt n ~
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;, H i 2.I~~ ~.~~~. ii~Hh~i ~i~~, n!!~~ i j!i, r"[e! ~~~,.~Hn :? ~";;' 1 ~~~uHlih l~! H~Hj fi~H i"'~1! "'1. 3:;: ~ i .... .,.~· ~- f~"-I! -~"~f" - S 1", ('" -~l"'!lt : "" ..... 2" 0 ..
1fHiHnUitmm nH.·~t!;h!! in Ui!~!~f~itj~f~l.~!f ili~,~-!!mHm~~HmHH! It~!dm~i1,mm Hlii~n 1m m[tUft mmm ~'jf~~HnHni'H~m h H H/ ~~~ ;" J.' '' ~-'' - - ""~l~< ..
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~
l- ~"-
jt hi~ ii~, ..
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ji" ."",,'"
t . ....
~~
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... p~>
~
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C'>
".
~~;_ , i
~:i",CN to~ ", .
'[
{e-
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l.,, - ;:i" "';;::~i - ..5, i >~ · "
.!.-~kO -~- r "i !I "
if- ~ i~; ~i ;"iJ~ i-1 ! :"' ~£.~~! H~ji .H i," ~ ;:~H~ ,:~~ ' i~ H~!~1 [Ie 'Z" " -jU s:'7 ~. ~ ... t.-< ~lr .. ~F" ~~ n ~ ft ~;< ~.~ r>(Hd ~ .monl.. ,.: " '"""';"I~ ...... &«1 c< "I~P. RodioloJl' 11'. 0.11"" ",,, p",ru'ionai.l.- Ih< s"""" eo.,.",,;1of "'" "mmS ,lIould I>< ,,,.,«11 A",,,· ",,,;,y ... I)'>;O, ,' I... """", Sl; ,$06.IS. 1999. m . Au.".... I I. O;n FG. M,hk G 1.1, ,,~I,: M.., .... ""'''' ofcc«",. I . Om" FuMe, f"," '"" >iol _ ... 0) ..... 0;, N.... 5,>11). 611 ·) . 19n O.A"C'l"'" K'A , Fod~'" H. ~'ilI" t • .,..· ......1 and _'1_ :o-9. '~l ~ . lw>o A M .l.cbl."" It · F... ;Iial ... ' ....... 01."" •• !}''''' •.J """......... 66, S11-8. '9!7_ Mdr< w, It 1· lm dUM. p"'rnanc:y.ncl ,"" ~ i u,". 1"........ ""101",27. 155·66.1 990. C..... I"'" S. v ..... , M w. o."" K a ..., d/.: Cn"01 """,'ie ~ i"""" ., .. I'mpC:1! or',,"h..d veiN: {due to irM;TN.;ed YlnoUS p._ure) 2. 3.
HEMORRf«GE Peak age for hemorrh agE ill between 16-20 yn'. 10'if0 Plort al ity. S/).MI'lh Dlarbi d il)'" (ne.,rolot!icel delbill from each bleed. FClr • dj-.;U6tIOD ofbemorrh.ged unnll preplncy.'" PfYlrlllM:y.t j,unlCl'GlliQl J,_r~~ PIIP 825. I. 82" bll"e e signi flcant intraparelldl),m.1 tomponent· 2. l uw.ueblloid hemorrhap: _ p4gIt 78 1 3. inttllwon tncular hlmorrhtt", 4. lpOnlallfOlUi ,"bd"ral hern.toma: &:I!f ptJ{Ir 676
Hemorrh"e rei llte d to AVM . ;:r.e S ma n AVM. ",,011 to preM!nt mora Oott.... .. hemorrhap th.ndo lute_f·'. n ""18 poIItull~ thllt Ioreer AVMs Pl'ft(lllted e. Mi~u .., man 01Um .impl)' bel:ause their aize made them more likely to invol\li! Ihe amell. Ha"-l!YtT. smell AV?ob are DOW thlMlght tel h.v~ much higbfr PI'N&III"1 in t he ~ing arteries'. Concl ... ion: . mall AVl>U are mo ... lethllilhall l.rpr ~. ~d life tim e risk o r bem o lTbage an d r ee W'T'en t h e mo rrbal'e Th~ ."er.ge risk ofhem....m.ge from li n "'\1M is ~ 2-4'it> per)'~ (reminder: riel .1. .1""';"0, ... PII-', and may ITOw (although .Iower than h.. Dlangioblutomu). shrink. Or remain unchanged wi lh timeH.
PATHOLOGY Gro.. appea rance r_mbl" a mulberry (faeelioul Jy but :leacriplively called a "h emolThoid ofth. br.in"l. Light mitr Cembral cavemgUll malrormation~ (eM ) Wml'rise 5-13\l, ufGNS vascular malfo .... matiuM, and develop in O.02-0 . 1a~of the population (bllHd on large a ulOpay't and MRJ aerics"). Most lire I lIpml.enwtiol, but 10-23% afe locaLed In Ule ~tf!rior fosaa wIth ~ predilection for the pons ..... 'Rarely, moy OC1yri" . OlOtrnveraial If hemarThage jn _....
Sei'tu rca; The rate ofnew·seill-lre onset i112.4'i1:Jyr'·. EVIJ,WAT/ON
CT: May miss many Imln , and even some Large JelI ;o n• .
MJU; 'T'2WI MRL illl \he roost ",n9iti~ t.er.t. Findinga IIJ't! similar to AOYM in gene",1 (mi..,ed signal"""", with low lIIignal rim l (8fe abowl. 'I'be. d iagnosi . is Itrangly .uC~sto.d by finding mulliple lesiona with thesecharacterisliCli and a po5itive falllily hi$wr:f". A venoU6 malfonnfttion may boo IIOOn ~djaeenl to solitary GM 6. but not wiLh multipl e Ic~ion6". r.mlappurnnce is nearly pathognomonic, and angiographY;9 not necessary in dusaically ap~ .rin~ C85U . Angiography; Does nol demoMlrate le&ioD. TREA ThlENT/MANAGEMENT
Optian$' I. o~e,...,.e 2. $uriPcal C> '"
28. Vascular rualfnrmBti(>1l8
NEUROSURGERY
I. new ollll:t ...j~"..." there .. a 8"U""lion tha t f~II1DviDg CMs before "kindhnl(' (_ pQg~25S) OIXUfl may have R better chBn~ ofpn:~ent ins futuN. ... iauref 2, difficu lll ina and apprDl'rlal'lI~neti~ o;:OUnMllini.
PROGNOSIS Wh;!n CMs ea.n be CDDlplelAl1y remD~oo , th. rilk orfurtur JTOWth or h~rThage i. _nli811y permanf.nl iy eliminated'" (ho.... ever, rKUrTeoe.ohympl.QlJUI hili report.. o.d elkr pIImftl and even _mioaly-complete remo ....I"'·-), For CMI \ n!aWd aur';ClIliy, pBti~nl.& RIled to be aware that pDIIl.-Op neurolot1e won· all..ing is VIII')' common, ...pecially with brltnstern eMIl". WOno!!ning may be Iran.ient". bUI may tab mont.ba to r'!SOlve.
*"
28.4.
Dural AVM
AKA dU1ll1 anerio.eDOU' ru;lula. Vascularabnorma1iu. in wh..icb an l.urinvl!nOua ahWlt iawnt.lined within the l",al\eUq(th~ dun mater, axduli.. tly",pplied byb ..... eha orthe carotid or vertebl'lllll1.4!ries IMorore they p Ep/DEMJOLOOY D"~1s compriM 10-15'iL orlll intracranial AVM~ , 61-66'10 occur in femal*1, and paU",nia Ire ut .... lly in their 40', 0.50'1. They OCtur rarely in children, and when Ihe, do thly tend to be coruplu IDd bilatenl.
EnotOGY EvidfonCll .uppOrta the ract that DAVM I ofille tra,.,,· .. _i&mQid ~inuljunction aAl nQI cnnlt!Dital but art! acquired 1...1001, relultlni rrtllll coiliterli revlnC"lariulion foJlowing thromboalll of a veD(lIlS l onU'" (of\wligmllid ,i· nul ocelulion , J>C>Mibly rrom chronic infection or Irluma) TIl4I oc:cipital arl.t!ry i. th~ dominlnl r~r in DlMI
-.
Table 211-7 Cllnl(:allindlogs In 27 petJt!nll with dural AVMs"
PRESEWT'ATION COnI/lOon findinp Ire listed in Tabl. 28.1 Vioull lm_ pIIinotllt Indudedob!leurtl tion, Ind two pltienLl thatwe~ blind from th.ront~llly ,u1!Y8u.d ICP, The rlskofbl~in8 i, lfU thin with ~rtnth)"lnll Av;.I .. OA~II m.,,11O bft uymptoma tic.
NEUROSURCERY
28, Vueular malfonnationl
,.,
TREATMeNT
Indications: I . neurologicdyafun~tion 2. hemorrhage 3. refractory symptoms
Endovasc ular e mboliza ti o n May be perfonned trallSarterial or trallSver'lOUS. Glue, coils, Or a combination may be used.
Surgery Preoperative embolization by an inte ...... entional neuroradiologist will usually fscili· tate surgical treatment-. The literature is rife with warnings about rapid blood loss that frequently OC(:Utll during surgery for these lesions (intludi ngjust inciaing the /lcalp). with one reportofS units lost in 4. rninu\.e!J following elevation orthe bone nap"". Thus, the u~e of the crainiotome is discouraged. as a ai nus Or venoualBooration could produce a fatal hemorrhage. Contingencies for the rapid administration of blood products must be made (large bore central lines).
Stereotactic radiosurgery May be used pO$l-embolization rt. Pan et al" reported a complete obliteration rate of 56% of tralUiverselsigmoid fistulae treated with only radiosurgery (1 650-1900 cOy) or with radi08W"gery after s urgery/emboliution had f/liled to produce complete obliteration. 7 1% of the patients were cured of tbeir symptoms. Radiosurgery represents an important als
Natu ral hi stoo:y Untreated VOG malformations have a poor prognOllis, with neonates having nearly 100% tnCrtali ty. and 1_12 month aIds having. 60':lIo mortality, 7% major morbidity. and 21 % being normal'". Perencbymstou s AV1>ls behave similar to other AV1>h .
Treatment Ve iD of Gal eo. roaJfonuatio" s: Pediatric patients are onen;n poor medical condition, limiting the efficacy ofoperat,,'e treatment. Treatment options for the .... include emboliution of the main feeding arteries. Prognosis is poor. Tbose presenti ng with hydroceph· 28. Vascular malformations
NEUROSURGERY
.Iul from aqueductlll ob.~ructi on often do .o at the end orthe fil'lt year orli re. Neurollu ra"ical excision mBy be con8idel"td here, .nd the prognosi. i. better. Parenchym.tou. AYM with e n\ ....ged VOG, 1'he AVM i, treated by the same methodl .. olher AVM. (emboH!.tion, resection or radiOl urgery ).
28.6.
Carotid-cavernous fistula
C.rotid-c.",ernoUl u.tula (CCF) may be divided into poatAl'l uma tic and apontllneoul. I . traumaHe Onc]udini iatrorenl e): oe of lll:l!....Ilr!! CCF eponlll,ulOU.ly thrombose. the ... l9re one may obIerve u,.... u lOlll n vil ualecuity iII.tllble. Ind intra-oc:ul.: ]>l"eUure i. < _ 25. Hi,h flow lesion. Or th OR ...oci.ted with propeuiVi! v;.u.1 deterioration requi re treatment, u,ullly in the form of~lloon emboHuhon by.n interventional neuroradiol.,,;st. BAL LOON EMBOt.IZA TION
A detllc:h.ble I.tex b.ll00a ill in fl.ted in an .ttempt to reduce flow completely or lu fIkiently to .lIow thrombosi •. Routef Iv.ilable inclu.d.e: J. transarteri,1 through 'nte",,11 urotid A. ~1l00ll ill pI.oed. wiWn the r.l tula ;!.felf, Pn!-.trvina: the parent (urotid ) artery: lhe bett option'". U,u,lb pOQ;ble only if lneUry,m neck ill n'lTOW 8. Ihhill f.u. (e... wide aDeury.m neck). two ballOON m.y be pl.oed. on either .ide offinull 1.0 trIp it bac:rifJOet carotid ,FUry, therefore tel t ocdUIIlon mUlt be done fint to det.ermine if patient cen tolenl4lthill). Alto. d,nier
NEUROSURGERY
28. VIlKular m.irormalionl
."
2. 3.
of embolization ",jlh th is transanerial through .,xlema] Cllrot id, ... ",, (ul only for dura ll'.nul88 t .... ".venou.: A.. Iraverling heart to enter jugular .. ein. then th rough petrosa l , inul to ttly· ernQHI to) ",ai t for vei n \.0 be· come arteriatiU fo. e current ooD'llllUcia Uy produced " ...iona we re available (toner than Mu. t ,void I.oeratina: vein inaide orbit. and avoid di' la l ligs!;on ofvein witbout proximal ooc:lution (,hunts even mora blood Into eye) Oneill.l) .
28.7 .
"",,*,,,,,,,_ ....",,,.,_. Mllfot.
M N.
o.
""tUfJ''''' fo< C"<mOII< hem.l"l:iomao , J ri.. "," "'' ' 93 (S.ppll): 70·7,2000. Pollo wilh "",oi01...... p. Rty C." "'" ... """')'''''. or o)oe , .. u "i. of GoJt,\ . Rod OoloI; .. I''''lIOm". 1 "t>dy ofll CIS«. J N....... ~iol 5; 9 1·10"!. 1978 , VOSIrJ.l1 M G: "Viol af ,10< beM
• •
rfV'II. givl!tl IV within 4 houra ofonHt limit.l the volume orl.he lCH the u"rulnu. oI'sU'1Cery is still con t ronui .. l, hilt ~ms limited to SOme ce~bel1llr MlTIOfThllges and select supratentorial nunorrhages thlltCllm' within I cm oI'~ cortical $UlrtM:e
EPIDEMIOLOGY
_.NCE
Theaea>nd to e levation orap or inetellied CBr (iee£lio«Je~r b6owl. Iy.
Appnwlima~ly
Ri ..k fadQn The following are epidemiolO8ic mk facton, Ilso INEliOlOfl1H, pase 650 for others. I aiCe: LM ;ncid"n.,., it>Cn!1l5eI .ignificlUltly a/\e. "&1' 55 )'Uri and double. ..... illl e~ dead.. of.se untillge:> 80 yl'5 where incidence it 25 tllllft lhtIt during pr"Ywu. dead ... )Wlat.iYe risk r.... Ig" :> 10 YI'5 i"l 2. ,endu: moTe eommo in men 3 1'11010: in tho! U.S .ICII . lfeclll blatka mOre than Tlbl.29-1 Rt!lall~.rl.kof ..... hites. May be related to higher prevllentfl of IC" wlU'I ElaH cOMumplion •. II.
HTN In blllck •. Tnddentfl mly .Iso bf. higher in onentAl.· pRYioUI CVA (any typel il\C~_ .;,k to 23:1 llcoIIol roruoumption'" A. rtceot UM: moderate U' heavy ah:oho1 con· lumption both wilhin the 'U hours.nd the ..... eek P~;"I tM ICH .... e .. nek r.ct.o ... rOT ICII' ...hown 10 1'11111" 29·1 8 . chronic UlIe: one 'Iud)' SU~Ut.l Iha tcon· I Utllin, :>, dl"inb" d.,. LI)C7'eUH the risk oflC H by _ 1 times..• ... C. len In patwnl.5 wilh hi,h fthanol eon·
NEUROSURGERY
Period pIIO/IO
AnIounr
Rtt.tiy.
Ii EtOH)
rII~
I ~-
41-120
.M
211 Int.nlceJ'ebral hemorrbage
.," " ., 1-150
2.'
•m
•••
...
5umption ..... ere mere eomm(lnly lob"r' 6. cigarette smoking: does llilI, incre".e the risk of ICH' ·' 7. atreet drop: c " .i. haa been reported ..: when UIIOCiated wilh vIIIICu lopBthy the lenu pOStpartum cereb ral a nFopatby ha. bHn used C. vnculn rindinp; I. .... me ca.... auocialed with i.... la\.ed cerebral vIIIICulopathy in the ab... nce or .y.temic vaaculi!,ia" 2. delDOnltl1lte va_pIIlm 3. aome cueaahow findings (e.,. patchy enhancement inoccipitallobea) l uggeative of cerebrovaacullr d)'Mutoregubti.on CI« ~ 64 ) • . lOme casu allow no valCU laT-relaled abnormalitiea 12. p"'t-operll ivl: A. foUowi", c.rotid endarterectomy « upper brain stem - vertkal gaze palsy, relraction nystagmus. skew deviation, loss of convergence, ptosis, miosis. anisocoria, % unreactive pupils. HlA in 20·4O ome\hemoglolin. !oo . i$(>.1n1_1O brain. ! .1IypO-1nt ....... I - 1"Iypom1 __ • " - 1119111)1 CEREBRAL ANGIOGRAPHY """'" 1M ABC, lySe. 1/1" HgD Deco/I"IeS o:>xt,ac.ltular For making the diagnosis of the ICH iLgelf. angiography dlama.gnellc lnon·p;o,arnagnelc) """'" ""... 81"'-, cannot reliably differen tiate the mass e/Teet J'rom an IC H from tha t due toan ischemic infar0'" rompLieauollS (prim..;ly lrue Randomized prospective ",tudie! (RPS) In the current CT/s urgi c ai era One RPS"· found lower mortality for patients with GCS 7·10 treated surgicauyB. However, surv ivo rs in this group were all severely disabled (none were independent).
""grot.
". ,h_ numbo .. m .~ und.""";m ....... mony I>"';.~ .. d;,'Obt!nefit from surrery ror ~hemofThllges, aJsowith poor outcome. in all patients. InUorna t lon a l STICH ''': enrolled L033 patient. but hMd poIsible selection bias (thl .etponsible neUI'OSUrJeOn had to be uncel't8in of the benefita of medical VI. surgical trutment). ·urly l urtery" had a IOmewhat long median tim. to trea tmellt of30 hour.. and 2611> of medically truted patients cTOHed over and bad . urgery at a mean of SO hou .... Given thue limitations. th erondu$ion W&$ that for i UJll"otentorial lCH there wll8 no benefit of . arly ,u~ry blthou/l;h ther. may bave been lOme benefit in the subgroup th at a hematoma withm 1 em of th e corticallurfacel. TIli. trial mly be viewed as a comparieon of early vi. del ayed surrery in patlenUl lubjectively judged to need lurgery by the inv"tir'tor. A pilot Itudy to in"eltigate minimally invuiv e proc:eduru (ftereot.llctic i/lftill,tion oflPA and then .. pint;on of the clot l il pllLJlned.
Concluaion The dec i.io" to operate therefore mult be iodividulli:r.ed bued on patient', neuroJO(ic rondit;on •• ize a nd lo"'tion of hematoma. plLtient"t~. and the patient'. Ind the family. wish el Mocerning "heroic:" meas ura ;n the face ofcatutrophic lIIneN.
Guidelinea for coollideriog . urg'flry
VI.
medical m anagement
(for separate indications for sW'rery for ~ hflmo fTh • • I « below) I. NON-SURG ICAL: facton that favor medical manarement A. minimally s)'ttLptomatlc Inionl : e.g . alert patient with .u btle hflmiparail (especially palienta with GCS > 10"') R. situations with litll e chance of good outcome 1. high ICH fl (ue Tab!. 29·61. 3 pacicnu. had labor or pOI!.-panurn hemorrhallel (1ft post 852 and .If() Prf8IJ(JTIC}' &0 ;,, /eguQniDl heml1TrhtlfIf on page 825).
Tabla 29-6 .~,,~,..~,O'!P::".~ '_'" UauaUy ama ller or more f lowly develop ing hemorrhages , clinically may present a~ irritability, nI' POlisible p resenta tions Abnormally incr"". ing OFC (croning pet centile curve. f..loIr tha n body w~ght). lethargy. apnea and btl.dyeard il. vomiting. Theni' i. propeu.iv", dilatalioo oflhe ventneular system on serial UIS Or CT; if LP i, done the OP i. o~n:>- 120·140 mm CSF. D IAGNOSIS
Ultrasou nd ( UIS) Performed thrOUgh the open font.aneJles'-. Accuracy. 88to (91'lo se.aitivity. 86' speci ficity)'''. UIS is invaluabl", beeause: it demonstrateli thesiZfl of the vmtridea, the location and , ize ofth" he""'toma. and the thicl c."
SURGICAtANTEFIVENTIONItL TREATMENT
Due to poor opent;"!! retul u, . urgieal evac .... ticm of an intracerebral hemolTh.,. in the newborn;s not indicated with the possible ucepUnn of. posterior foua hemorrhage tIIu,ing brain at """
moderale ~"
Ptogrenlw hI'" drocephalus ("/o)
." ....." .,,,,, 0
15-25
Lollg-term The effect of low grade SEH on long-te rm Deurodevelopment has not been studied well . Most ir",esti gato~ feel that higher grades ofSE H aTe associated with greater degrees of handicaps than matched cont.rols. In one study of 12 infan ts with G rade II SEH tre ated with serial LPs and in the 7 with progressive ventriculomegaly with VP s hunt followed for a mean of 4 .5 years found all were ambulawry and 75% had IQ within normal range lO' .
29.4.
,.
,.
References
HlO/IgI,nO <WI "'."' • Acu""'" _iol pl S" .kc 19· 'la7_90. 1983. « ..... "' •• 011 t>< ... . ",. w.h 'fIOC" I "'(..,.""" '" ,... tbOCh&ni.m or ... ",.",.....'" i.r."" .... J rI ... ,..,.L...,1[.pN• • n>! HI: 92·]. 1951. Homi, C R. Oomdotf W. A,..,I; A L' H.mon!>a,ic o.
~
" " ". • ". ". ". o.
". ~.
'"
Aldri< ~ 1.1 S. S ...""'" S .. , Or«. be,. H S: C., "" • b~rKl) . ....;". SoC",. i...... ,,",ke. c..n,,' l 0(1' ",.1·7. )933 • K... C S. FoII!· .,.,. 31' J99-400. 1'.181 K. m• • W ,.. V,,",oIi C M. 8 ..... l M. o ..1_ .. In . ..,.."'.ry .... " •• •• "".1 J 1'1 . ... 01 1'1 ".,.... '1: ~J " •ri" " ". 00 .
"
,..pi
'flOC""'"
,~
• "'."'* " c._"
29. Int rllCBebral hernor rbllge
"'mot·
,">k. I~ II!. 19S1 (""",ct). G ... ob 2S4-9,199" Ki",,,,,,, I ll , C.."o M I. AnrIo""" S. I. ' M> Slim· ,I,..... c( ,i" .... ,"" pi ..... ""'•• "".. _ b, AI,,,"p'0d0 " ' 101""'· N.oIgl'< Mod l ' 1 ~1. 199!I , Gt«.bI I's)'c· "'nf.. <mot· ",.,• . S""". ;$(1), I·,. 19\17, Fo.Jj.i V. To","". R. To,I:..""bI S. f/ 1_"" ""i.'"""I1,-..;.. J l'Wu ..... ,.,; IO; 51 · 7. 199-1. S, S.h V.Iboy."'i S. "aI.: 8100d poaloW< .oo,ro! ....... _ lOX of .y",~.",." ton .. S"",,_ 2'/, 1806-9. 1998. Gorwl KOJ>" "'"" irHroco-ol
"'!T"oOITti>J W S: Spon_ io~ ... .i'l "'mc(Ii, ? ...... [01, • • 01 S7. 168 2.. . JOOO (.. ~o(19·".2000. W,,,,>< ... ful .... and lim ,,, of '"<Ji",,1 '='"",01. N,"· ""."u~ I': toIl·6.19$oI . K""",....,i S. So'o A. K'i1 .... ;. 001 ... """ .. ...,.. ,,",u"""'J"",.IOf he ....• ""Ie. S.... N.uool 00 [ 1 ~ 15·11: 21·1.
1\9.
120.
'11 .
'"
m.
ll'.
"""'1'
'.pt>.. ",..
1.0"".,
1""."".
tn._ m,
c1.I' ''''"
1lS . C;"b!> P A, T.... _ i. ,,,,,,,,..,,,,,,,,.1 .. he_>l:< _ ... "h i.".._,,,,,,I ... FtCombi .... -'i ..... pto''''OOS'. '''''''OIOr T«hnic.IO ... , , _ i'I", .
,.
......eo'l' 0·-..... ,_
Rohd< V.S.!I.. C. H...1et W F~ I""""'.''''ul.. _ ..... ,io> .. IUSm,no;,dolOlty 51; 4-07_". 199' . 121. Oo,,,,,,D.wle, K.B ..... LM: r.. ~" ... 1IotpO P RESENTATION
Carotid artery les ions a re collllidered .y mpUlmatic ifth~"f! i. one or mo re later.li:.inll iAChemicepisodes IIppropriBte Ul the diAtribution o(the I""ion (1I 1e$ion i. mn.ide rtd lIon·specific visual compin iMt... d iu lnesll. OT syncope not associated with TlA or I ttoke'). The majority 180%) nfcarotid atherothrombertie ltrllk..~ occur withou t wlITlling symptoms' . Symptomatic carotid di5eBSe ma y pna.ent al II '['lA. RIND Or eVA wi th any of the following find in gs: retinal insumciency nT infarction {centra l retinal .rt.ery is a b nmch "f the oph· UI/l lmit artery~ ipsilatual mnnocu.lar blimlnessA. may bet t.e:nporllry: IIm"UJV ~i~ tu g lllboOfS B A. Sorgery lor IIr. ""...,,1I0Il 01 SlroIce.
The I'!\IlItionBhip between t he degue ofl1arrowinlC based on the NASCETdefini tin ys. thlltofthe ECST has also been nl!maled by equation" as 81:10 .... n in €q JQ-2. '4l!enoslS % ~"'nnSli .. 0.6 ~ ( by I;CST) (by NASCET)
....
Eq :10-2
DuP!.EX DOPPlER U/. TRIISOIJND B-mod~ imaC"eYalulltes the ~"1A1ry in CTD$S-&""tiolllll plane, lIod 5pectrum "naIY~ls shows blood no ..... Perfurms poorly with a "stri ng " fII". Cllnoot , Olil where there is perful im failure o.r flow r.i lure . SofT\ '"
• lime and dOlO depend~M i rrev~ ... ibl. inhibition of pl~Utl~t ~KgJ'III.WOO and prolong. ' tion orbl ... dioll t.lmu. T.ku - 10 d'ya (or benefi~ 10 beronle apPII,/,\,,,t- , UN or dopl' rIogrel i, recommended o"e. ticlopidine (He iwlow). SrOE I r nCTa: Sipl fi canl neulrop"'nia (.hI(llu!.e nelltrophll COllnt CAN e! < 12001mm :l) IIr ~i.'!'anulocytoa;. lIO/Ueti01~1 wllh thromhocy!.Openia oecu.. ;n _ 2 .. ... Nilltropenli WluaUy enlllU within 9Q dayt or inllillini: thenlpy, and normIH~ ... with difCQntinuaUon orthe drug in \·3 weeks. Thenr01"1ll1 ,. 1"I!8e1'<ecI (or "Iilnla intolenont of ASA or (or wbonl ASA h.1 not been approved (VIL; previoul thrombotic e VA. TIA in women" .,,). Coo traindicated In helU~poietit cliloroenl [neuo-openi., th,ornboc)1openi • .•• ), heI"DOIltaUc dllOtde ... Or active pa~hologielll bleedh"li, or wllh K "tnl hep!tticdr-fundiDn. Ur. 250 rna PO 1310. Prior to iniliating ~ ••apy, check CBC. pl.teJ.lwunl. JJ ANC .. 12001mm'. iniliate \ielopld;ne th'flIpY, ~'o. ANC 450· \200, weigh riak VI. beRtfil.l, and jfdecl.!.ion is made to iniu.to! l.hefllPY, do 100 CII uliO\llly. fo r ANC < 4!iO therapy i. oem· t rairldicat.ed Check CDC q 2 W~tk• • a manw, lind the .... al\er only i' lign.or.ympt.om. ofin reelinn appellr,
~
c]opidogre] (PlllviX®)
\
I
ORUO INFO
\
1
Anothtr th,~nopyridlne (.ff n~), .. Ith II lower ;~idtnct o{.v~ n ... trop.-ol. (0. 04% ) t han titlopidlRt 10.8"') and;1 cloa OpUons-. unilateral CEA (or: .teno.l... ~ with B or C uIOfl" (..-r]XJ6e 870)
IICA ........ OplJon s". Ip.ilateraJ CEA for: lteno.i, .. 75'" irre.po!C.UYt! or«KItn\ateni.tenQt:i. unilateral CEA +CABC for: bilate r. i .lymf\Wmat ic c....otid UfoOli • .. 7~ + CARe required lpeilatcnl CBA fo.: llnibuer.1 C1l rotid I lellOlli... 7~" CABG reqllirft!
30. Occlltlivt! a!n!br"'''llICular di.eaae
N£UROSURGt'RY
PRACTICE PARAMETER 30 I tl,S'''PfOMAlIC CA"OIH, ',I 7/j" imllpMlJy~of qo,, 'ral~U!r. 1
• '1A!1'>Il"'".so'' clI~tid
NIl: IlION mooiIoc81_ 10 .... _
.......y . - 10 N
rNa. "' bgI>I ~ (unI>t< c~u. 110m ~l"
NATUllAl fI'STOO'r' SI~k. ralll whh a" U1.IlUIl!UlIM!le rnroll d bruit il Z'lI-Iyr on ci1hf.l.ide. 1m. ;pailaleral CVA r.II is O. I -Q .ot'l.'yr, wh.erelU i\ II O.7·6'1r1yr when It.~ bruit;I a~mPl'nied by ipsi\D tua\,I.en~i." Hi1ltof Pl'tl~"Ulwith It.e.rIOIIi,.1I()'I, on d upl"il aclln deve lopf:blbl~ c.rdillo ~robolL Surron.a were fltqLlil'td to have II perioper.tiwe morbidi ty and mor'llliity rllte of < &'II. Net fi v....y .... r rid< ior .U ilroke or perioperalive stroke or death: in the CEA group, .... I L8$ in the rnedicalgroup (p, maoagemenlwith CEA (wheo perfonned by a ~UT' geen wilh a low complication rate , a8 described) reduces hia annual nak of all strokes from 0.5% to O.17'll>(the reduction of risk for severe stroke is less ). The benefit from CEA is realized within less than one yellr after the CEA. This is in contrast to the ACST trial (xe Clbo~) and is most likely due to the lower perioperative event l'Ilte , The risk from mortality from other CIIu..,S (indu ding Mil i$ _ 3.9% per yea r . Combined eVA and duth rates in community hOllpitals>' while improved over the last 20 yn, remain s higher at _ 6.3% thao at tenters used in this study.
Ve teTao's Adminis trat ion Coope r a ti ve Study (VACS)" eEA reduces ipsilsteral neurologic events, but did not red...:e the rllte ofipsilateTliI eVA3 1I0r dea th (most deaths were eecondllry to MI ). This trial did not indude women.
CASANOVA st udy" No difference in outcome between CEA V8. aspirin (new eVA or death ), but an un· usual protoco] lessened it's statistica l validity'".
30.1.1.2.
Carotid endarterectomy
INDICATIONS
Table 30·3 shows the status of cur· N!nt studies for thesurgiclll treatment of carotid stenoais (NB: some of the results may be contradictory). The North American Symptomatic Carotid Endartere 1801110). eOPD, age > 70 , &evere obesity neurologic risk faclQrs; progTeuing neUm deficit, resolved defiCit < 24 hrs pl'f!-op, generalized cerebral illChernia. recent eVA < 7 days pre-op. deficits from multiple CVAs, frequent TIAs not controlled by ant,-coagulants (cre$Cendo 'T1Ao) angiographic risk factors; contnlaterallCA occlusion. siphoD stenosis, plaque ex· tending> 3 em distally in the ICA or > 5 cm proximally in CCA, carotid bifurca· tion at level ofC2 with sbor t/thick neck, sol\ thrornbw. propagating from ulcerated lesion Sased on above risk factors in 1.935 endatte.rectornies, 4 grades defined wi t h the risks of morbidity and mortality (excludes minor complications not cousing permanent morbidity) shown in T(!ble 30·4 ; from
..... t... t lor dollnition 01 medicoll• • ngiOg"'p/llc. _nd neurolOgic nol< laclO's
Original paper (342 operation s) included in non·perman .. nt morbidity 5 patienta who developed seizure$ associated with periodic lateralizing epileptiform di8cl>arges (PLEDs) pGst-op that ""ere initially difficult to control. butdid not require long·term anticonvulsants (AEDs). All 5 had high.grade stenosis and a . mall pre.op CVA. and reac· tive hyperemia post·endarterecWII\Y. Sundt recommends pl'f!-op /\ED. in these patients. SUDdtcautions that although the risk ofCV A is highest in Grade 4, some ofthe moatdramatic results ocma: dysphagia, air hunger Dr woneninc hoarseness, difficulty ..... a llowing
B.
d~ngers:
1. uphyxiation: most immedillte d anger 2. stroke 3. exnnguination (unlikely. Unle8lllkin d08ure ia also disrupted) C. J.t.e (often delayed weeks til monthl ): false .neuryam". Risk", 0.33.... Pn.en l.l as ne 50%". Rcsrenasis within 2 y1'$ is usually due to fibroua hyperplasia, after 2 y11I it ia typically due to atherosclerosis" cerebral h yperperfus ion syndr o me (AKA normal pressure byperperfusion breakthrough ): classically thought to result from return of blood flow to an srea that hulOllt autoregulation due to chronic cerebral i""hernia typically from high. grade 9tenosi s. Controversial". Usually pres: Dlay develop5-7 days post-op, Longstanding HTN may O«l>r as a result of the los! of the carotid ainus baroreceptor re!lex
COMPLICATION MANAGEMENT I. post-op TlAs
A. ifTtA occurs in recovery room, emergency CT (to RIO hemorrhage) and then angiogram recorumended 10 assess for ICA or CCA occlu sion (... s. emboli) B. ifTIA OCCUrs later, oonsider emergent OPG; if abnormal - eme~tlt .ur· gery n fneu ralogiCIIlly intacl, pre-op angiogram is appropriate )" 2. fIXed POSl.-op defIcit in distribution of endartere.:tomi~ carotid A. if delicit occurs immediately post-op (i.e. in PACU ), recommend immediate re-exploration without delay ror CT Or angiogram" (case reports of no defi· cit when now re-established in" 45 min s) . For later onset, workup i$ indio cated. Technical ooMideration& for emergency r&-(lperation· " • ....., I. isolatethe3amri~(CCA, ECA,&JCA) 2. occlode CCA 1st, then ECA, and ICA last (to minimite emboli) 3. open arteriotomy , check backflow; jf none, paS/! a No.4 Fogarty catheter into ICA, gently innate and withdraw ~avoid intimal tears) 4. if good backflow establishd, dose with ... ein patch graft Ii. remo"'e tonuou9 v\lS$Olll ibly indudina barbitW'lu.u (thi'lpental balUIe. of 125· 250 mg until 15-30 second burn l uppreu ion on EEG, followed by I mall boI UI in · jN:tion. or c:on.tant infuoi'ln to maintllin bu .. t , uppreu;on") A. EEG I'GOllitoring B. SSEP JtI(Inito ri na C. musu.rement of di l tal , tu mp preuure efter CCA occlulio:l (unreliable), •. , . using a shunt if l t ump preu ure < 25 mOl H,
30.1_1.3 ,
CarotId angioplasty
Initi al c:ooefit . nd Ito. hi,1I ""' .... lity. E"'orxoncy",rsoll' ....y be of bonefit in .... ... deficit ir",,".nt ;. 01.1\ ond CT 110""01
NEUROSURGERY
30, Occlusive
~rebro·vascular
disease
."
4. c r escendo TIA5: TIAs that abruptly increa&e in frequency to "- &everal pe r day (C 30. Ow). May be Been in O;!; odontoideum (8ft pai~ 730). SYMPTOM COMPLEXES
Predicting site oflesion bllsed only On clinical e"lIluat;c>n is vel')' unreliable. Atheromatous ond stenotic lesions occur mO;!;t frequently at VA origin . VBI symptoms may be due to: 1. hemodynamic insufficiency (may be the !M$I oommon etiololfY), including: subcla vian &tew : re\'erned now in VA due 10 proximal stenQ5i~ af subcla. vian artery 8teno~i~ ofboth VAsor of c>ne VA where the otheris hypofunctional (e .g. hy· poplastic. occludNl. Or tenninates in PICA)causillg reduced distal flow in fa< .. ofina dequ"le cooJlaloonl. (, .... 80w h .. n/@r·. ~Ircir~ bqlo .... ) 2. embolis m from ulcerations 3. atherosclerotic oco:lu5ion afbra ins tem perforators NATURAL HISTORY
No clinical study accu rately defines the nstural h'story . The e!ti m&ted stroke rate ;s 22.35'11& QVer 5 years. or 4.5·7'1> per year" (one study estim&ling 35% stroke rate in r. yesrs did not use angiography). Risk ofCVA af\.er first VBI·TIA has been estimated as 22% far finn year".
EVALUATION
Adequate invel!ltigation requjres selective
NEUROSURGERY
four.ve~el
angiography.
30. Oc
NEUROSURGERY
InK f,bro .... b.nd", remova l ofolteophytic spurs ..... ) ra:ommendillion.: Forcompreuion at C I-2, it i, 9UltK"ested that VA decompreN ion be pe rforraed DlI the initi al treatment. Thi. should be followed by DCA to verify maintenan~ of patency with head turning. PatienUi who fail dinically Or on DCA , hould undergo C I·2 fu , ;on". PatienUl need to know prOI and conI of each option. M.tlagemetl~
30.2.
Cerebral arterial dissections
t Key featur"
• hemorrhage into the l1Iediolloye r of an arU!t)' • lIlay be .pontaneou l or t.tra ... m. t ie, rnl be intracranial or IeXtracrsnial • may preae nt with pain ru:ually iptilate ral Horner'•• yndrome (in ""rotid din edionl) 1'WCVA.or SAH , • elCtracranial diued.ionl are uluaUy t reated medically Cantic:oarulationJ. intr._ cranial dia&eetionl with SAH a .. t.Te.ted .... rgiClllly
kilo).
NOMENCLATURE
Some confuaion hu ari l4l n bfcauq of mconaisteot tllml inology In the lite.rstw-e. AI· th~ugh bl- no means !~-,-,da~, V.mau .... ha, '",g'"ted the f~llow i",c'. ."'. . . ."'''' dissectOr! ell1!a\1a~ DC blood bel_ me RIIIIII nI 1TIfdia. ~ IImnIl N rJOWinOOf lit'-
....,
~oibk.od be'rWeeij riiiI ~~. 01 81 WIll fIISM, causinsI-..ysmal dla\ition, MIktr may fI4lIUID no the lUbArac:trnoid ,_ ,",""",.~"""""c;;;~c. DC ~fIery wnh S\bseQuenI.~.bOn 01 the em..HCi.i., hetnltoma, may Of may rot produce lminalnarlOlll'lllg
diSOO(ting ariel>"
I)'SITI
...
"*"
P ATHOPHYSIOLOGY
'rhe leaion common to all di.uerilll dissections as a sOurce ofSAH (a nd venebral di ssections most o!\e n present as SAH). Multiple dissections occur in ~ lO% (Ihe m~1 common: bilateral vert(>bmoosilar l"'lions). CUNICAL
Cerebral ane rial dissections may cause symptoms by: embolization secondary to; • pilltelet IIggr~ation stimulated by the exposed surfaces • dislodged thrombus (formation of which i9 enhanced by reduced flow) reduced distal flow """,;mdary to; • thrombosis duoo to reduced flow • oedusion of the tme lumen by the expansion or the mural hematoma subarachnoid hemorrhage (Itypkal presentation, may be more common with pos. terior circulation diSll~tion than With anterior ci~ulatioll)'t The moslcommoll presen tation ill patients < 30 yrs of age was due to carotid dissee· t ion without SAH. In patients> 30 yn, vertebrobss,lar artery (VBA) dissection with SAH was the most common"'. Headache, usually severe , often predates ne"rologic deficit by days or weeks. See following S ...
30. Occlusive cerebro·vascular disease
NEUROSURGERY
MRl ~riogram
is the gold standa rd
~st.
bul MRI (more than MRAJ is catching up .
Crescent sign: brigM signal in wall of ca rotid on T2W[ axial images(hematom& in
~essel
wall) .
May visuali~e intimal nap and distinguish a Role of MRA ;8 yet to be determined .
diss~tion
from a fusiform ane urysm .
CT MDT. Unreoognized or forgotten trauma or sudden head motion may haveoccWTed in aome case, reported as spont.aneous. Ctceptfon bei~g a patient with direct trauma causing pr(lxim81 VA involvement. This predilection is possibly expla ined by the fact that the first lind third portions of the VA are movable. whereas the &e<Xlnd and fourth are relatively immobilized by bone.
TREATMENT
Except for cases presenting with hemorrhage or large ischemic ~troke, medical thera py should be started amergently, and eonsi9ts ofantieoagulation, with heparin acutely, followed by oral IIgen~'e.g.Coumadin) probably for II total of6 months. Indications for surgery: Surgical therapy i. required for diasectfons presenting with SAH (due to their propensity to rebleed ) and is recommended for most intradural dissections . For extNldural lesions it is indicated far dissections that prolfT"'ls (angiographically) Or for persistent symptoms in spite of adequate medical therapy . At the time of $u'llery, tbe site ofdiElSeCtion may be real (}fnow DC""'" th., oit ofdjs~tion8hould push the intima back against the wall B. ifthe dissection is pro>ie e fl ll i"g deroctl.
Findirtg'l include: 1. non ·filling or segments o(si nuses, Or filling defects 2. prolonged circulation ti me: in 50% of cases (may need delayed films to see veins) 3. stumps and abnormal collateral pathways
LP OP us ually increase.:!. CSP bloody or
~8nthochromic
BLOODWORK
To detect predisposing conditions when the etiology is unknown . Some tests that may be uselul indude evaluation for thrombophilia (protein C and S lo vels, anti phospholipid antibodies) liS well a8 testa for specific predisposing conditions (Cac. Factor 11level, serum homocystine level, pa rO%ysnlsl noct urnal hemoglobinuria (PNH ) panel. leukocyte alklliine phosphata~). 30. Qeclusive cerebro·vaseulllr disnse
NEUROSURGERY
UL TRASOUND FOR DST May be used in diagnosi s
ofsu~rH!r
sagitta l sinuH thrombosis in the neonale'''.
TREATMENT
Should be aggreuive beca use reeoverability of brain is prob~bly greater than with aruna l occlusive stroke. Management is complicated because meaSureS that counteract th romb05is (e.g. anticoagulation) tend to inc~ase the risk of hemorrhagic infarct (the risk ofwhicb is alrelOdy increllsed), and measures that lower ICP tend to increase blood vi""""ity - increased coagulability.
Specific I.
2.
3. 4. 5. 6.
7.
8.
m e o !lu.NlS
correct underlyin,lt abnormality when possib le (e.g. antibioti"ll for infection) h epa rin (systemic): (..,~ pose 22 for dOlsing) especially ifpatient ill in DIe. Sever· slstudies show ~ lower mortality rate with beparin than without'''''''. It remains the best treatment even when there isevidenceo firotracerebral hemolThage with the attendant risk of increasing the size oflhe hemorrhage'''. There is no consen· sus on dUl"8tion of treatment or if warfarin shou ld be "sed afterwards. S uco:eu rate may be higher ifadministered before patient becomes moribund avoid steroids (red uces f,brinolysis, increases coagulation) UROSURGF:RY
30. Ocdosive cerebm-vs.sculs r disease
'"
Moyamoya disease
30.5.
Progressive spontaneous ooclus ion of OM Or usually both leAl; (at the level of the
siphon) and the ir major
bra nc~a.
with serondary formation of anutamolic coll ateral
capillary network at the base of the brain which fancifully "'~rnble8 "moyamoya", the J apanese word for "puff of smoke"'''. With progression, involvement incl udes the proxi· mal MeAs and ACAs and on rare 0«88;on the vertebrobasilar ,ynero. Aa50Ciated aneu')'lIIIlS f.s« ~Iow) a nd rarely AVMs'''' ,.. may be obse rved . Eventually the d ilated capillary (moyamoya ) vessels dinppear with the devel op· ment of roJlateralH from th .. ECA (meningeal colla lerals are call1'd "r ete mirabile").
Pathophysiology Etio logy: The etiology is not known, altho ugh the imroW'le system mll, be involved. Pathology: The ma in tnmlui of involved intracranial skull base arteries are narrowed due t() thickened intima (lipid deposits ooc.u r without eviden« of in (hunmMion). The in· t.ernal elastic lamina may be thinned or duplicated . There a re area! off()Ca l fibrin deposi tion and thinning of the vessel wall. particu larly the media and adventitia . Similar vMt: ub r changes may also OCCU r in the heart, kidney and other organs, suggesting it may be a syst.emic vascular di .... ru;.e.
Associated a n eurysms Intra craniallll\llltt&lIUi are frequently as.sciated wi th moyamoya disease (MMD) . This may be a result of the increased now through dilated a)lIat.e rals, or it may be that patients with moyamoya may also have a congenital defect in the aneriaJ wall that predisposes them t.o aneuryslJl.!l. 3 types : 1) usual sites of aneurysm a in the Circle of Willi s, 2) in peripheral portions of «rebral arteries. e.g. posterior/ante rior choroidal. Heubner's, and 3) within moyamoya vessels. 'Ibe frequency of aneurysm I in the ve..-tebrobasilar sys · tem is ~ 62% which is much higher than ;n the general population'''. Aneurysmal SAH may be the actual Cause of SOme hemorr hages that were erroneously attributed to moyamoya vessels.
E PIDEMIOLOGY
Risk factors: A history of inn am mati on in the head & neck region has been impl icated. Demographics: Incidence in Japan is higher l< UlOO,OOOIyr) than in North America. 1'wo peakll (may not be same diseaBe): juvenile, age P ROGNOSIS
T he mortality tate in adul ts (~ 10%) is higher tha n for juveniles {~4 .3%)'''. The ca use or death was blee-di ng in 56% of9 chi ldren and 63% of30 adult... Wit h treatment the prognosis is good in 58%"'.
30.6.
,. ,.
..
References
We,""hn L. Md..- "': n" cOUll< 0( Iron,ie .. ;•• Chemie Ntu,""""" l8: 611·80. 1'111. Le.y () E: How ,,.n,..,,, ... ,,.n~c" i"')wmie.,· Lo •
.. " ".
". " ,.
". " ". ".
'J1l>P.""""" po"'."
En"
.11< 0'" hi,h-O orOl< MRC f"j 'opu"''''';d _ref)' ,,,.!. I.an). 16'1--11 . 200) K.. buR< K. Ri«>o .. J I . Mo,"y IlT: S,.tIJr.. fol· Iow;"t i, o(,o. irl<m"c"""id .... or. eo .. teport . ,' 1,.",".,",,,y 28: 1 ~8 · S1. ' 991 Smith II. II. . """"T S. lI."""n W F' T..."Iuro ...1 OIIl oopt"",or''''c.",tor>l """,1_ Clio Nou"," ,." ,]L 111·"'. 19\11 Ibo.,W S . _ . W D' C.... ", .. g.iopW,y. C.,,· I...,p N"' ....... 119(n~ 1-6 , 1991. U B. 0);L A "''''''t'«'l.. ~wy ~ •• 'o...,.I""1 U 44)·)(j. 1988, I. R A." ~I .. Sur· ." ,,1 P""'mI' vm,tlnl IN,.,,",u'll 61,8)'''81,198--\, Fe. 101 W. Plops,,, D G. a."....", J 0 : A."roII!· . ,,1 "'com~o. 19'H . Prou·Thom&i H 11.. 8o
' ..... or .... I.,... "''''''''''"' ... Rod~ 16: 11945.1911. LomAli Oon>Iou« itI.~u· ""'" """ io ••""....11«"'...... Cldhb B ~io II, 1'»-111. ''ISO, KuIu .. "" S. K... S .... ",,,,,,,, S• .,tII ~ u.. of .,hl 010 .. 1", "' •• ".""..... """ ~ ho," •• pIoo:~ .. ....,......"..d....., J N•• --... 8S laU-J . ~".,.
".IIom"
,-
K,... .... I .I(",j. ..... II. K, ... "",,. 1• ., .,. l",cob ~i"= Karnolally performance ,"Iplus pcale (modified" I)
I
HEAD INJURY
The Rane hos Lo!J Amig09 /ioe Ble (TllblI!3 1·2) i, often us-ed in ra ting j.IOtientdi,· ability renewing head il\inf}', The Glasgow o ut com e Mcal e (Tobbl 31·3) is frequently employed ill outfXIme g!l!lessmenL Tab;e 31 ·2 Ranchos Los
I ,
NE:UROSURGE;R'I
lL Oul.Come
a~rnllnl
'"
CEREBROVASCUU.R EVENTS Severa) outcome grading leale. have Cle 31·$ appearll tohave great· er seositivity". The total ranges from 0 to 100 (a score ofl00 implies functionsl indepen· dence. not necessarily normali ty). Of all the facto .... independence in bathing w88 tbe m""l difficult. Abili ties On the Barthel index tend to return in a fairly consistent orde r. lind W mOSt patients with the same SCOre will have similar patterns ofdisability. SPINAL CORD INJURY
Functionalllldepelldellce Mea _ developed to provide uni_ Tsble 31 -6 The Funcllonal Independenee Massure'" (FlU) form e~ aluat;on of disability for spinal cord il\iuries. RateB 18 itelD$ shown in Tal>le 3 1·6 (13 mot<Jr. eo cogniti~e) on the 7 le~el scal e show n in TabU 31·7. The F IM'M baa high internal consistency and iJo s goad ind icator of burden ofeate'" OJ. IU",' - " (F[MTH):
,
Tsble31 -7 The7 FlU'" raflng levels of dlsabiUty
31.1.
..
J( ,,",""ok, D .... B""I>0:"," H: In E'.oI ......... of o:n.1 J H. AmI;",,"'" G C. ""I.: Trill 1. 1$>: Sll-li. 199) . Un"' .. JM.tl 40 yrs. suspect extradurally",phoma (pri.o1ary or secondary) Or leu ke",ic deposits (chloroma), especially with pre·existing diagnosis ofhe"'stopoietic or lymphatic disorder c. epidural metastases become increasingly common after age 50 yTS. Occurs in up to 10% of cancer patients. 5·1 0%o(ma]jgnancies prel;ent initially with cord oomprell$ion. Also Set poge 516. 2. intradural-extramedul lary (40%): meningiomas, neurofibromas 3. intradural.int ramedullary: primary cord tu mo", (ependymoma, astrocytOma) and rarely intra",eduJlary mets (.t e poge 508) B. carcinO",.tou8 meningitiS: neurologic delicit usually cannot be localized to a single level Csee page 491) C. paraneopiastic syndrome: including effect;; on spinal cord Or On peripheral nerves 4. vascular A. hematomalhemorrhage 1. ~pinal epidural hematomat: usually anociilted with anticoagulation thrrapy" (su page 353 and page 23) a. traumatic: following LP Or epidunlanesthesia Csee pos' 23) b. spontan eous': tare. Includes hemorrhage from spinal cord AVM (a.ee page 347) or from vertebtal hemangioma (seepage 512) 2. spinal subaraelvooid h8lnOrrbage: as in api nal epidu ral hematoma (w, abo" ). this may also be IlO'St·traumatic (e.g. following LP'·· ") or second ary to spinal cord AVM 3. spinal subdu ral hematoma 4. hematom~tia B. spi nal cord infarction' uncommon with the elimination of syphili tic endarterit is. MOEit often in the t.erritory of the anterior Bpinal artery. SPllring 1lO'Sterior col\.m08. MO$t commonly _ T4 level (wat.er.hed zone) I. atherosclerosisofradio:ular artery in elderly patien t with hypotension is now the major cause orthia rare conditicn 2. damping aorta during surgery (e.g. fOr abdominal 8or1.k aneurysm) 3. hypotension (relative or absolute) during s'n gery in the sitting position in the presence of spin al stenosis". May be improved by avoiding absolute hypotension. using awake fiber_optic intubation and positioning. inttaopera t ive SSEP monitoring a~d inducing hypertension if
NEUROSURGERY
32. Differential diagnc-sis
."
chang" (lttur wilh poe:i tion ing. Ivoidan.:e of sitting po, itioo. and ayoidin, hypoerntxion. hyperexteneion and tracUoa 4. aortic d,ssection 5. emboliUl t lon of,pinal art.l!riu C. .pinal cord Avr.1 (I« pap 341)t: 10·20% p~.ent .. ludelen on ... t of myelopathy u.ually in patienta < 30 YT*". myelopathy may be .econdllr'Y to: I . rna .. err«t from AVM: , pinal AIfMlaccount fo r < 11% ofl"iolll p~ • • ntine a, oord "tumorl" 2. nlptore - SAM. lI,matomyalta. or epidura l hematonUI 3. watershed in fa~tion due to ·,teal" 4 . 'pOlluneoUI tllroDlboti. (n« rot;,in, myalopothy of Foix. All\ioua · nin '" diwaMl"): prfUnta a, lpa,tic - nlllXld paraplegi • • with a-.:endin, HnIOry ,.....1 D. nldiation myelopathy: due to microvatc:uJa r ocel ulion (I« pagt 536) E. HCOndary to iodina~ ron treat mate rial uaed for m«ellteric or /lort ie an· aiOl'nljlhy . Elpec:.lly wlltn angiogt'/lmmed in prelen.:e or hypoten8ion. wile .. cardiac output i, ,hun~ away from yifl«rI and i nto ,pinal radicular aruri". Treal ment: pla.:e patient litting. remoye" 100 ml of CSF via Lr and repl~ with equal amount of .. lin", over 30 miM" 5. autoimmune A. demyelinati ng: acute (idiopathic: ) trlon lvu.... m y",li ti, (ATM) (o« pagr 5$). reak incidence during fitsc 2 d«adel orlife. Abrupt olllle l ofl.£ weakne ... ttnlOry 10K. back pain . Ind Iphincter di.turbance indil tinguisllable rrom I pinal cord oompl'HBion. Thoracic region mostoommon. CT. MRI and myeLo,... m alillorrna i. CSF - pleocytOlit and hyperproteinemia B. multiple .del'Olir (I\o1S): II diagnosed in only 1'10 of potienlll p~$tllting III acute tranlyetse myelopathy. Although more common in young adulls, MS aln occw alany time in life. My~lopllthy of MS is usually insidiOWI. and is usually incomple:.e (i.e. some sparing). AfI'«ta 1J\~!in. thuB 8paring gray matter. Abdominal cutaneo... renue:r, are almOilt always ab5ent in MS C. Devic . )'Ddn> .... e l neuronly~litit optica): a varia nt of MS characterized by acute bilate , al OJ:tic neuritis and transverse Dly~litis. In some cases Ipi nal cord edema may becomf! so $f!ver~ as to eause complete block on myelogTaphy. More eomml)n in Asia a.od India than U.s. or Europe O. post·yiral (or post.valXination): may be etiology of auto· immune prcun l i.e. tran""e""" myelitil). Vi",1 prodrome pn'Sf'nt in _ 31910 of ca,a of ATM. Viral in fed ion is u!ualJy most damaging to gray mat~' (e.g. pOliomyeliti~) 6. metabolicltoxic: A. bubacute) combined ~yalem di ~aee (eS!)) lAKA lubacuteoombined &1 methytmalonic ~ (altocb~k homocyat.eine to RIO fol.tedeIlciency)
NEUROSURGERY
7.
T2WI MR I may demonstrate Inerea&ed lignal within the white malter oftbe 'pinal cord, predominantly;n the p(lflerior oolumn. but ml,y all(> be leen;n Ipinothalamic traCIa B. toxin.: l""alanO:'lth,tiea ull of patienl!! will continue to have persistent symptom.8. A structural diagnosis is possible in only _ SO% of these patienl$. Th""e patients account for 85% of the 0081 in 100t work and compensation". Differential diagnoais includes caus·
..
32. Differential diagnosis
NEUROSURGERY
y ofll.",te IlDd .u""",,'"" LBP Ii.t.-d abo""",... w~n ...' L degenerative .:ondit iolll' A. deCC'ntl"lth'e lpondyloli.tbesill_1K'«I1241 8 . Iptnalltenoei. {a lfl'din, the .pinal c:.IIn.1l C. lateral fKess .yndrome 2. .pondyloarth~thiN A. .nIty .... i..,.pondyliti.: look fOf erwivlchll\lI. . .«nt W $ I jointllnd 110" iti .... I.eIt ior H]..\·827 IlDtipn B. Pliler. dlHa.M of the llpine: "",rtebul involv.mft'lt il vlfY coma\(ltl ,n II pII. tienta with fllp"l1 di.llse 3. Pl'y, NB; Ul, petonu.1 d ivl.loll ortha ",jalk nllrve "'no.lo til be m~ vulnerabla til inJury Ulan thl tibial divi.ion .
...... 11._ 1001."'" 1"'.......1...",1 in,1,,";.. ~ ..... "'" Wee '.8from hip dl. 1....I>.. n .... fnI .... mo, otf.b ,wo"nd. , h\ied s while the patieot ru ista. Tn t i. poeitive ifit rellroducu pain) 2. . houlde r pain in very I'Ommon in pOlymyalgia rheumatic. tin ~ 61). typi ce. lly wo ..enl wi01 IT.DYemeat 3. inu . .apular pIIi n: . comnlOn Incation for ,."ferred pa In with cervic~1 rpd icu lopathy. may aLeo oa-ur with cholecyat it i. Or lOme .houlder pa01ologies 4. MI: lOme u _ af ce."ie"l radieulopa01y {eapeci.lly lel\ (;$) may pruent with
NEUROSURGERY
3'2.
Oirre~nti. 1
diagneli.
'"
5.
symptoms that lire ~ uggestiv~ of an acute myocsrdial infarction reflex sy mpathetic dystrophy: may be difficult to distinguish &om cervical radiculopathy . Ste llate ganglion blockl! mllY help"
32.1.7.
Neck pain (cervical pain)
Thi s &e 32.1.9.
Burning handslfeet
I . s pinal cord syndrnmell: A. central cord synd rome (CCS): SI! 32.1.10.
Muscle painltenderness
I . fibromyalp: I « abow 2. myopathy
3. ".t.atin" myopllthy
32.1 .11 .
Acute paraplegia or quadriplegia
.i. «(If
Entitiet e&u.ingli pin a.l cord comp MMi"n us ... ally pml;enlas: paraplegia or ·parequadrip~ri"'pare.is), urinary re~nc.ion (m.y require checlting poIt·""id residua l todet«t), and impaired sensation below l.,v.,1of com pression. Maydevelop ClVer houra or day,. ~fl.".ta mlY be hyper· or hyptHlctive. Tht,e mayor may not be a Babinaki .ign. E.duding trlu m•. the most common C8UU i. compression by tumor or boo • .
E tio lo gi es Some overl.p with myelopathy. For itfms with astuisk, see M:ftiopallly, page 902: 1. in infancy (m,y produce "noppy infant syndrome") A. We rdn ig-Hotfmllfln disea~, AKA spinal m ... so:ular atrophy: congenital d~neration of . nterior hom alb. Only rarely evident at birth (where it pre5('nts as a paucity of rom·ement), prodUCe!! weaknul, areO ...x;"'. and tongue fascicul.tions with norm.1 sengtion. Progre.eea over the fi rstYN T or two toquadriplqil B. spinal cord injury duriog parturition: . . .re sequela ofbl"ftCh delivfry C. ton&"nit.l myopathi",,: e.g. u.f3nt;~ acid rnaltue deficiency (Pompe dia-
'~umor'rIA": a tr8nsi~nt d~r.ci~ in a pati~nt indi8~iogui8hable froro aD ischeroic'rIA
with 0 tumor, may be clinica lly
B. 'rIA-like symptoms may OCCu r as a prodrome to a loba r iotraC )'mOully. tome e(lntl)nd that the tl) rm m ,u:roflow with nonnal or mildly enlarged ventrides A. "externa l hydrocephalu .": promintnt aubanlchnoid spate, and t... 1 terns (see Er'UfUll hydro«pho/o,. (AKA NII's1l uurnol hydrot:epholtAJ. pnge 181) B. l ubdunll nuid I. hema toma 2. hygroma 3. efru.ion (benign and aym ptomat ic, _ /KIi~ 6781 C. eereb ral edema: fO!I).e conaider thi, to be a rorm or peeuclotumor ~rebrl'" I , tox.ie: e.g. It1I d encephalopathy (from chronic lead poilOning) 2. endocrine: hypopanlthyroidi lm, plac:toMm,a, hypOphIMphala.ia, hy. pervitami l>O&ia A, ndrenal inlufficiency ... D. ramili,l (hereditary) mac:n)Cranil E, idiopathic F , megaleneephaly (AKA macrencephaly ): an enlal"if!d brain (IOU ~ 113) C. neurocut,Lneou s Iyndromel: Ul uallydue to increased volu meorbraln tillue (megalenceph aly. ue obot..!)O>. Seen especiall, in neurofihroma~il an.d con~n itel hypennelllI>08il (l1emiJUlI tract (large compre.-i"" le.io"" ruay cause bilateral alteration of facial sensation) thal chieny mpnifeats in dim. inution of pain and tem;r.rature llenae wilh little effect on touch $en$e"'. The tract usually axten II as far down a8 ~ C2 (although il may occasionaJly extend down to C4 )
32.1.26.
Language disturbance
1.
aphaaia: A. i!l.iury Iare detaila. s"'" pagt! 506. Some fa~j.Qrs pertinent to this location": A primary bone 1. chondroma 2. chondrosarcoma: rare io the traniQvertebrsl junction. Lobulated tumOTl! with calcified area~ 3. chordoma 4. OIItelJChondroma (chondroma) 5. osteobhQtoma:ue~511 6. o9teoidO!lteoma:su page511 1. giant-cell tumor! or bone: typically arille in adolescence. Lytic with bony collapse" B. meta~tatic: including 1. breast cancer 2. prostate cancer 3. molignant melanoma 4. paraganglioma C. miscellaneous
'"
32. Differential diagnol is
NE:UROSURGE:RY
plasmacytoma multiple myeloma
1.
2. 3.
4.
~illOphilic ,,-anuloma; OIItcOlytlc dtfp.ct wilh prog"ellive vf. rlebrel ~l1apsoe. O a.
C. O. E. F. G.
a-yptoco«us
mycoplasma coccidiom~~
""hinOC'OCCWI schistoosomiasi. H. para/lOllimiasi. I. all pergilloi, i, J . candidiui. K. herpes simplex enCf!pbaliti/l (HSE): usually terr.poral lobe (1ft ~ 225) 3. inflammatory A. demyelinating diHue 1. M5; u. ually in .. hite m atter , periventricu lar , with littl" mas. effect 2. progreuive multifoccal leukoencephalop.U.y (P!\o...): u. .... lly white matter B. gummas C. granuloma. O. amy loidool;, E. un:oidosi . F. v,"ullti. or .rUri t;' G. c:oll s~n Ya/lCu l.r di,,,,... indudinr: 1. periart.eritil nodosa (PAN) (_ pogt 61 ) 2. .y.t.t!mic lupul erythe m.~u. (SLE) 3. granulomat.ou.arteritil 4. vaicula r A. multiple aneu ry.m. (congenital or atheTOl(lerolic) S . multi ple henl(llThai C. venous inra"'tion5 (especially in dural8inu5 thrombo&is,!Me page 888) D. moyamoya disease (.we page 892) E. 8ubacutc hypertension (as in ma\jgnant HTN, eclampsia ... ) - symmetric connuent lesions with mild mass elTect and patchy enhlll">Cement UlIually in occipital l ubcorcical white matter (su page 64) F. multiple strokes 1. lacuna r st.rokes (I'etat lacunaire) 2. mUltiple emboli (e., . in atrial fibrillation, mit.ral valve prolapse. SBE, air ~mboli) 3. sickl e cell disease S. hematQmas and contusions A. traumatic (multiple hemorrhagic contusions, multiple SOH) B. mUltiple "hypertf!nsive" hemorrhages (amyloid angiopathy, etc.) 6. intracranial calcifications (see page 933) 7. miscellaneous A. radiation necrosis B. foreign bodies (e.lI . post gunshot wound) C. periventricular low densities 1. Binswanger's disease 2. transependymal abBorpticm ofCSF (e.g. in active hydrO in pedl B. meningioma (parasella r, tuberculum sellae. or diaphragm sella): to differ· entiate tuberculum IJ000&. <W.t..t.W..r.W.). AI..." the M ilo ia yQ"Qllv not "plQrg 32. Differential diagnosis
NEUROSURGERY
32.2.12.
Orbital lesions
4 rornpartmen\.!l or the orbit: ""ula. (AKA globe, AKA bulbar), optic nerve sheath , intrarona!. and extraeon"L CT remainl a strong imaging modality within the orbjt{lell8 susceptible to motion artifact than MRI. images bony "t""eluTes to good advantagl!J. I. neoplastic
A. cavernous hemangioma: the most rommon hfIlii:D primary intra-orbital neoplas m. Choroidal hemanginroa ;s seen in Sturge-Weber syndrome B. fihrohisti(ICytoma
discrete l uman that may occur a! a lipid exudate causing retinal delilchmenL Mlly mimic retinoblast 0.5 em dia anl possibly asso ciation with cogn itive impai rment and a high pnlvalence of psychiatric symptoms" unCOmmOn A. Fahr's disease: progressive idiopathic calcification of medial portions of bat· al ganglia , sulcal depths of cenlbral cortex. and dentate nuclei' oo B. hemangioma. AVM. Sturge·Weber syndrome, von Hippel·Lindau disease C. basal cell nevus syndrome (falx, tentoriu.m) o Godin's syndrome. Associsted findings: mandibular cysts. riband vertebral deformitie., short metacarpal •. Medulloblastoma seen in several patient\! E. cytomegalic inclusion disease F. encephalitis (e,g. measles. chickenpo~. neonstal herpes aimplex) G. hematomas (SOH or EDH, cbronic) H. neurofibromatosis (choroid plexi) L toxoplasmosis J . tuberculomas; tuberculous meningitis (t reated) K. tuberous scleTO$is L. hypopsrathyroidism (including post-thyroidectomy calleS''') and pseudohy· poparatbyroifuro M. multipl e tumors (e.g. meningiomas. gliomas, metastasu) N. cysticercosis cyst: may be single or multiple (see NeuTTJC:I$li«rt;O!;<s, page 2S6)
32.2.17.
Intraventricular lesions
IntTaventricular tumors nlpre~ent only - 10% ofCNS neoplasms . Adue to differen" tiating a (uroor located within the ventricle from an intrllparenchymal tumor invaginat.ing into the ventrieie is a · cap." ofCSF surrounding an intraventricular tumor On CT Or MRI. The following is from a 'lOeries of73 patients with an intraventricular lesion on CT seen at UCSF'''. 1. aBtrocytom a: 15 patients (20%). The most commOn lesion. Hydrocephalus (UC P ) present in 73%~ hyperdense On non-eontTast CT (NCCT) in 77%. Locations in de$cendingorder of frequency, frontal hom (7) third ventricle (4) atrium (AKA triione) (S) fourth ventricle (1) 2. colloid cyst : 10 patients(14%). Only seen in third ventricle at foramen ofMoDrO. 50% byperdense on NCCT. 9 of 10 enhance (sef J>Ol1f 457). DOx includes nn· thogranuloma 3. menin ~oma : 9 patie nts (12%). 8 in atrium. 1 in frontal hOrtl. All hyperdense with dense uniform enhancemenl 4 calcified. 5 or6 hsd dense tumor blush on angiogram, ID06t supplied from anterior choroidal artery, posterior choroidallesa common. Tbought to arise from aradtnoidal cell. within the choroid pinus 4, e-pendYlJlo m a: 7 patients ( 10%). 4 in 4th ventricle. 3 in body of lateral ventricle 5. cr aniop baryngioma: 5 patients(7%), All in Srd ventricle. Al l with punctall! calcification. Squamous epithelial rests in region of lamina terminalis are felt lOgive rise to this uncommon variety of craniopharyngioma 6 medullo bl astom a; 4 patients (5%). All filled 4th ventricle. All hyperdense with homogeneou l enhancement 7. cYMticercos is : 4 patients (5%). 2 in 4th ventricle. anterior Srd in 1. panventricular in 1 (N S: incidence related to geographic locatioo). s« pafJe 236 8. choroid pleIU s papillo ma : 4 patients (5%). 2 in late ral ventricle (1 bilateral), 1 in 4th , 1 in Srd. Non·obstructive HCP in 3 (possible CSF overproduetion). l ntense blush on angiogram 9. e pid ermoid: 3 patients. All In 4th ventride. All hypodense with nO enhancemenl The most COmmon 4tb ventricular low density leSion in the U.S.
".
S2. Differential diagnO$IS
N8UROSURGERY
10. d e rmoid: 2 patients. 1 in 4th ventricL.. 1 in (TOnUlI hom. Both had free f1o-ting fat in ventriclea 'uegeslive ofcylt rupture. Tendency to form in mklll ... 11. c bo r oid pl~ . carc ino ma: 2 palienu. Both in atri um ofl.terBI ventricle. 80th e:thyma with edem/l lind . hin. lnt.enM blu$h on angio. NS: very rare lesion 12. Hubependymoma: 2 patientll. I in 4th ventricle. I in fl"Qntal horn. Both itodente with minim.l enhan~ment. Moet ~mmonly in floor of 4th ventricle nellT obex 13. ependym al "yot: 2patientll. Both in lateral ventricle. Absenceofcommunication deroonstuted by metriumide ci, t.emo,..aphy 14 . • raehnoid e)'at. I patient. Lat 1 pao ..... nod c:ysbrClilM oSIIuI;oIy 1IY"i!~ < .. .. ,.n. \Ita. J i".u ...... 'l! Sot: l-I]. '1 , 1996. 11_ C, MolOO' K.Sl'OI>o R."QI.: Mlnd,bl< oIId
""" .... pI~'io'\I_h r", 1"" « 11.. _ of "'" ,1 '.'pm""". I. The ......1fI E.Pemceu y A. k_ W T "of: """' ......... 'I"« of ..............., ...... N«oro... f"I:-'6. l!I(I)
11 7.
s..w_:
""'''''''I)'
III.
bnj.,
"pt'
119.
'" 121.
Pot",
!'leu,., ..,
c....,...
...,
In
In.
IF.-.nchJ
32. Differenti,,1 di"gnosis
_.riot
.pt""''''''''''
""'"1"0-
"'ot,,, __ .pond, ","
'". 0.,,,,,,, ,.. '"
)(1m,
Fo/;:vI M B. Wilh..". R l, M""'IOII4> $: CT""" MR iOl>, I.. r........ of 1':">I: 1'roptoo"ItId """OJ• ......" . ,.,..u .... ...~ ~ 32: 7(l6.I!. 1991 HI...., P 1. He""", S 1... """'" A 1: Eoo .... b~ , "" __ .. in diOJ"".".lrId ,... ,_ nl. Ca.tI ... p ,.,.... """,.. 1O(:lI ~ I· , . 1998 D. N. ..... 8.B""''' T. "01, Dntrup"-"l: C ... 2.-'!I8~. "" Ellfl J M«I llll; 1610- 8. 1939. C . ~ I.,. 101 D. QoIm T M. K.II, ,,," I H. #oI" teoion> of bodia i" onI. yioo'"1 ~yl~I •. AM Rht"",DIo Jl : )'ol·l. 1972. R"""",n ~ H.Si""""" F .... (odI ,) : Thupi ... lo-d cd .• W.8 . Sou_ •. Phil.... lpOi •• 1m.
Iop""'"
"",,,iIm
NEUROSURGERY
NEUROSURGERY
32, Differential di sfI'Ilosis
'"
_ I j(- MUfOfI'la ( ('fIIII 'd)
A 11'.1l1li8'.161 .bl:Nev..t_ !Iocalo!d • ~.,""i"l booto ) lIIMbminal c..... _ ",flu 106 , 11! ill "",h.pIo: IoCIetalts iobducelll ",,"} ~ 11 faL~b:I,l;tl". foil" ~ due IObr..-... rompmt_ TIl door 10 diul ,,.,,.... 665 M 10 ~lit~#IeCI1CP (IOn! IUIIIOI' .a()4 due 10 illc:reuecl inInoc,.".' ~'" sa6 rulln .. L~. kimbl.t p.mcl~r( 616
differenlia l diagl>OSis 924 lu,""", of 517 lumors diff'renlial diagnosis 924 .~onll sbearing 632 1X0II0tme.;.560 mc:IlIS .. IK:
AZT® (IU
~idovud; n.)
B Babinski "gn 88 with cerebellar infarction 772 with cel'\lic:a11l""'dylOlic myelop,!lthy 333 back plin 289 chronic )OJ wnsel'\lll!ive unUnent 29S differenlill diagllOsis 907 f.;loel back lynd~ 314 in cancer PI,i.ntl 516 in spinal Paget' s disca.. 341 physicol euminll!ion 292 plycholo,ic.1 compo""''' 29 1 p;sychosoc:i.1 factors 296 "red nagl" 292 ",ith SAH 782 back school 297 hadofcn fo. elhancl witildrl"'al150 for Spall;'; i.y 368 intrathecal :J.69 fortrigeminal ncu,algi.330 in ALS 53 bacleriallncury,m 821 bll/;lerial endocarditis and cK Iht''''py 662 bo$.lL, illeml in l..... ma 68 L t.l1'/ICtlynpeo>diN.., 68(1 basal pil,Li. cak,r", .. ion 934 (0 .....
'n P,lttiMO .nd .pinal
aNI ~rtd:nl O$I ....... yc1ili. 244 Banle', .i,n 6)1. 66S BCNU (sec CII mUSliroe) bCllenooppe, cranium t84 dt$Cl'iphO" 101 wil" erl1>iolynoslosi. 99 wil" lambdoid synoslosis tO I be .. e .. . itver cranium (_ ~." coppe, trani_
familial inLrocnni.l lneu..,.,1II syr>drome 801 f.mili.1 syndromes wi.h o-' S Mool'S 406 Flm.ir$(see ramcickwir) fannlnl or cc",ial .pil\eS 142
ras( ic In S60 rlll lJlin tcho(.... MRI) I lS fa .. " ;",,, 1 1
,,,
ooMRIl)S f.1 tmboll$m .yfldromr 174 rat.uptHHSion MRI 13S febrllr ttl.uteS 259. !~ and mesi" tempoi'll ttkro.;l 251 dcf'in ;llon 264 Pr«(dinl I ialu, tpolepl;'; ul 264 fKOI ino;onlinenoc in uuda equina .yndrome lOS fdlNo..- 276
INDE.X
f«"nllln8 pil 41 fClal SIhe ,,,
F· w... ""pansc (on EMG) 143,293
G pbapentin 278 fOf neuf Gill proo:edu ••
)lj
"abcIl169,69 GI"'IO'" com_scale IS &.-.di", KlleS (..", ' ·d/
m;~«1424
concuSSIOn 6))
D... mu,Oupon (awt"O:)'tomo.) ,,1
multiple 413 multiple primary 413 n.sa1919
op1lc 420 oO lcome for mali'" .... a~rocyLOm.14IQ pH«y' ic Nltoo;ylOma 417 ,"U...,III 416
lCCUOI4n U~"""':N41'
d..:mothcrapy 4 I ~ for hi,h snde tiU'OC'ylornu ...... vasosptolm
m
WOIId Fcdt ..... ioo> of ~"r:>SU...-..
"",,,hnoid hemorrhlge) 7M
grand.mal KiIII"" ($CO: ~i."
...)
, ........ iud
an
(...1>-
lon;(o(!onic
granuloma.ous Inglll l, S7 GRASS image (on MRI ) 135 e"hlllCfmtnt in A '1M, 84 1 Grave.' d ilt"lsc 44$ , 929
now
Go, delini,ion 5~
vea' (leftbul vein of allen 82 grea' er «eipoLlI ntf\'e J78. S6J ..,ptTf~ .., ptlJ"Olal ncrve 594
K"'''.'
Gnsel', ~yDdrome 112 GOOII n INDEX
growth honno ... 44 1 acmmcply 44 1. 447
held nUlio" in .urgery 600 head injury (s«lnn.ma - head)
bMxbt-mi~,1 ~(ure" 456 lre.tllleni for 449 and Creutzfeld,·hkob d,suse 228 gua ... thcdi ... block 397 GUIl'ielmi de'ach.ble coils &03 guideline (praclice guideline)· denni.ion iii Guiden"". for .he M""agemen' of Severe H..d Injury 635 Guilford brace 741 Guill.in·Bam syndrome 5.1, 905 guns~ woond. 10 peripheral """,es 563 '0 me tnchial ple~us 562 10.he head 684 In,ibioliu fo.685 en ..... ncele~i. wou".;l6~5 lndiel. ions for an giography 686 10 lhe .$pi"" 733 Guyon's (&n11571 gyral enhance men. dUreren,;.1 diallnosis 765 onCT 765 on MRl165 gyri of Hesch l 61!
bt-lldache44
H HACE (sec higJIal1i.ude cerebfal emma) h.ir",n...,nd skull 27, 932 Hakim shun' 192 Hakim·Adams syndrome (see I>Ormal pressure hydl'OCt:phalus) hal..,."pam 35 Hald impaired coagul .. ion 181 .798 for C(ln!rasl media reaclion 128 horero!Opia 112 Heubnc,'s artery 77, UJ. III occlusion 778 Heyer·Schul!e shun! Ion H-sra fI624 hiccups f,om slcroids II UU lmen! chlorpromatin~ r rlloTl.zine$) I I ... ith la!( ral moduliIII)' syndrome 111 high Ihiutd< urt"bral edema 637 high . hiuJde e.",bra! edema (HACE) 687 hippus ~88 hiltam,nc rt lease wilh morphine 2 release .... ith paralyliClt 38. 40 hislamine., (H,) ... caS""i". fDfhr:ad IIlIurna 651 hi scaminiC mignine 43 hisliocylosis X 482 diffen:nria! dia~nosis 931 Hivid!> (see ulcitabinc) HNPP {.ee heredilary ncuropa thy wilh liabilily 10 "..lSurc pals,es) Hoffmann's sign 89 Holt..nhorsl plaques 810 hollow skull phonomenon (sig n)o-n CRAG 167 Holmes-Adie 's popil38J holop..,..,ncephat y 112 Hoher valve 193 Homan,' .ign 26 homocy .. inuri. _00 arur,a l di.s«!ions 883 and urebrovO$Cul.r ".nOllS .hrombosi. 888 a!>d strokes 173 homonymous hemiaOpsia (:see hemianopsia. homonymous)
INDEX
honeymoon paralysis ~9 honeymoon seizures 285 hQuk effee! (on prulaclin levell 446 OOri>.on!.I,venkallumNr vil>lve 189,193 horiwntilivuion of 1M fact'I joim 138 H""""r' , syndro"... 583 due II) pilui!ary apoplexy 439 follo .... ing cervical discec10my 321 from cervkal cordolo-my 392 from r.lellale gaflJ,lion block 627 in brachial plexus injut)' ~61 in carotid di.section 885 in fibromuscular dysplas..a 63 in Raoder's pantlrigem.inll ... u.algil 588 wilb ce\lpil. ISS for .."'bntl pl"Olcclion during anourysm surgery 807 wilh closed head injury 6S8 h ypot h~roi dism
100 pituitary lumors445 from aminoglulclhimidc 451 hypoxia foltowinll head ,,·lIuma prognos,k us neu.op .. hy 554 idiopathic cm ni. 1 polYlIOurop.lhy 918 idlof>3thic int,"cmni.l hyp · ,p;... ) lnoohej4> (see linzaparin) inoov.a>284 i~gu;n.1
In~(=amrinone)
INR
(>CC inlemlliOfll1 Norm.hed Ralio) insen$ilive 'pace 29~ inSlabilily ('pinal) (see spinol $IIbiliry) insulin loler:>nce l.sl444 insulin·like growlh faclor·1 (see >omalornodin-C) inle..:mlll nerve block 628 inlerrosral ""uralgia jll •• reo".1 nerve bloot for 628 oplnl l cord slimtlhlion for 39S in~rhemisp herima 62. 938 ~uk(mia
"nd inlface.eb.al hcmormlie 861 and PML 231 leukoaf&iO$i~ 936 leukoc: ""c ph"lopaLlly diffe.en~j.1 diagnosis 926 from intra·.n.ri.1 BCNU 416 f......" RTX ~ meLllo\J'e..~e 535 level C"pine Sunace I~ndmarks 11 in spinal cord injuric. 698 \evctiracelam 217 l.ev()-Dromoca~( ... e levor,>/lMol) levophe1Iin) low densily "",as (in moyamoya disease) 893 low grade glioma 408 low ICP syndrome 197 low molt.LA" ,n S.... H 711>' for r.. 1ed bKt l)'I'dtOme' ) 16 p3do.nt «1>0 831 ;" head IBU..... '" 1 in .... ~tpk KInoItIS 51 in P"tpMCY III in .......1metl ~20 k> ........ !.p",e ~ ptrfUl ......... e,V!oed
m'crocyslie rncnl in co.m.a 156 in head ".ulTllt 637 in myclomeningrx.le 115 in spina) cord injury 710 innmke 766 neurololY 44 neurolysis of the mOU..,.".
acoustic (.... Koustic neuroma) facial ""....e 594, 922 {rigeminal922 neuroma in continuity 563 r>euromu""ul ... bloc king agc n" 38 neuromyelitis optic. 9()4 neulOn spcc:ific enol>&e 500 Ncu","~"'" (St:(; gabapenlin) neuroparalY!1sid/:) nitrofurantoi n and peripheral neuropathy :;51 ni. rogen ba la nce 6SO nitroglycerin IV for hYpt'nen.ion 4 nitropru"idc 4 for au lOOOmlC hyper«:fk:x ia 756 in neurogenk pulmonary edema S niuosourcu 407 nitrous oxide ancst~si. ."d p""umot:eph.lus j NizOf1llIomy 612 -,pltal ner>'C cntnp<mn, .563 ocdpilll no""lIg;8.563 lOil/l I'rIe1U~ w1!h odoowid frJIClurts 728 occlpilll ""un::ctomy,l.64 occqm.ll p~gi"""pI\fIly 100
",,,,man',
ns
v, frona'(i..'umfnc:otot hll 184
.."
OC(,piIa'
frIc,u~619
ocdl"l&I"IVlI",1 dti~n
Itl call\e (Of iUliiDCOnl,S33 oculomo!or palsy 51} from p-romm a~'Ul)'lm 80S is 818,918 in Guinlin·Bam' S4 multiple (WIIOCUIat mijt 69.69 OPLL(seeO$,iflCaliof! of the posteriol krngitudi. nallillmc:nI) opsodonus S88 optic glioma 420 m neurofobrorMiosis SO) oplic nerve delI,in lherapy 22 In Cu,blng·' .yndlomc: 44() in mUltiple myeloma SIS ste,oid induced 11.748 ""alment 749 ... i,h prol..,til\O,nn 44(l wilh ,ene. sympalhetic dy>uophy 391 oOleoporu.is clrcumscnptl (SO., Poget's di$«$f; of the skull) osleosclerosis 91 g 0111,;.378 in g.niculate 11¢uralgi. 386 in glossopharyngulneuntl.gil )86 OI il;. h)'e spine 341 pain 376 bad r..,., blCl pain) cluuISi.396
JIO$lerio< loop of ICA 79 poslerior lumbar in,.rbody fusion 300 JIO$'eoled reduclion in ff1lCIUfeJdislocalioR w'Vical SCI 709 Injoel i"" Iherapy for Iow·bKk pain 298 lumbar (u,ion bo~ lI1"'''''lendersJsubslilules 301 com:lalion wilh outcome 301 for disc hemialion 300 for low back pain 299 fusJOn wi.h s::enosi. arid spoRdylo",,'.si.329 Illsion wilh s::eRosi. w'lhoul spoRdylol;.thi:,i,329 pedicl~ screw nuti"" 300 ,adiographic:assessment 301 use of po>1'f()la.~ral fusion and/or inlCrbody fusion 299 management ofSCt in lhe hospiw 703 melhylprednisolone in SCI 104 MFtI & discography .sindication fo, lumb., fusion 299 occipill l coodyl. fraclll"'s 72 J odonloid f'''''HlIts 728 o.od""'oi!kum7JI peQveoous C>bs/fUClion 496 orbiLlll587 differenti.1 di.glK>Si. 9'29 piluitary 445,927 p"udo~lnthoma el"lieum 801 "nd moyamoya disease 192 .nd ~ro ke in young adu lls TIS PSNP (see progres.ive supra."",,1ear palsy) psoa. abscess in PoI,'s disu:w: 245 in spinal epidural absce ... 241 PSR (..., percuLllneous "';geminll rhizolOmy) psyeoollCnic sei.ures I>QJI '"
rebuild"p in lTIO)'artlO)la disea~ 893 rcc()mbinant ICtivlled C()Igulalion facIO' VII (see fK lorV[[) recombinan, tissue p[asminog saccular Il\tu!')'sms 800
STtR ;"'"In VoIRlj for _Mebl1lJ fX1W.es l S I SlDku .... daln5I11tod19IS 5!r1i'''III_.8 follo,",inglumba, diKleCtom),)07 for Addison •• " cri"s II fOf Btll', 1"'\'), S9S
fOr bral" l~mor406 (or bl'OflC~osplSm foli(y,o..,n,IV ~lr.ISI in_ JCCi ion 129 rorCllpIIll ll"ncl syndmmo S61 for 'e~bnllOSU:Ss 221 ror«~tonl mt" JU)e,U8, 4go for t)'Sliccroo..os 23& fordl",,;. zjO for &lon, cell 1",,11115 60 fo, ... ruhoI .. ounds 10 lhe htad 6U 10 the >P'III' 7();l for ~ "Uul)' 6(j I fat ' nl11lCC:ffbnJ hcmontu..., IS1 ror low bock pII'" 291 lat "'ythl" 56 for IIe1&mcyltoc..lined :01........1o;Itl"~ion \lOoI subaaM Klorosl'" po.lICIIC .1Id EKO ~h.n~ 719
famllill inlfllCtMiall""...,..mlllO l felal hydantoin 272, 281 I10ppy infllll 9 1~ fonrnen ",",milD c:ompteuioft l OS I'o)rb(s· A 1brill'll (.rnenorrhu·..laolon-l>ea)
syndrome
~t·Kcnncd~&S, 427
diIY.renllal diaJ_i,91 4 w illi IIosMlpllaryn&ul ~u ..l&ia 186 syncytial meni n&1oma 427 lynOesmophylU in am-ylot'''' spondyl~is 343
Villaret', lyn6romc 86 viral tn~pha lilil 22.S VislarillB> (..,.. hydl'OJly.ine) visuII CO World Hulin Org.. izs.;on das~ifk"ion of amoc:y,omas 411 cl ..sirocl tion of Min tumor. 40 I
wour> x
V.leb"",e741
z Zan~(see
nlniridine)
Zarontio® (0« ethcsuximi De&;ripUo~
Class
posterior LE, to heel
5 6
normal mild dysfunction modem Ie dysfunclion mooerate-severe dysfu nction severe dyslunctioll total paralysis
Descriplklo nO.rmal function in all areas slight weakness 011 dose inspecllon obvious but nol disflQuring c·bviou s weakness and/or dislig uring asymmetl)l oorely percep1ible motion ro movement
MUSCLE STRENGTH (PAGE 548) Grade
0 1
2 3 4
5
Sl1engtJ1 no conlraclioo flick;)r or lIace contraction mO'l!!mEmt With gravity eliminated movement agalnsl graviy {4- slight resistance movement againsl resistance 4 m~erate resistance normal strength suong resistance
"t
GLASGOW COMA SCALE (PAGE 154)
HUNT-HESS SAH CLASSIFICATION (PAGE 785)
Best vemal
DescriptlO/1
umuptured aneUlysm asymptomatiC. Of mild HIA and slighl nuchal rigidity 110 acute meningeallbrai~ re
