ContentslistsavailableatScienceDirect
Clinical
Neurology
and
Neurosurgery
j ou rn a l h o m e pa g e :w w w . e l s e v i e r . c o m / l oc a t e / c l i n e u r o
Endoscopic
third
ventriculostomy
in
the
management
of
hydrocephalus:
Outcome
analysis
of
168
consecutive
procedures
Sérgio
F.
Salvador
a,b,c,d,∗,
Joana
Oliveira
a,c,
Josué
Pereira
a,b,c,
Henrique
Barros
c,e,
Rui
Vaz
a,b,caDepartmentofNeurosurgery,CentroHospitalarSãoJoão,Oporto,Portugal bNeurosciencesUnity,CUFPortoHospital,Oporto,Portugal
cFacultyofMedicine,UniversityofPorto,Oporto,Portugal
dFacultyofHealthSciencs,UniversityofLúrio,Nampula,Mozambique eInstituteofPublicHealth,UniversityofPorto,Oporto,Portugal
a
r
t
i
c
l
e
i
n
f
o
Articlehistory:
Received30May2014
Receivedinrevisedform25August2014 Accepted31August2014
Availableonline15September2014
Keywords:
Hydrocephalus
Endoscopicthirdventriculostomy Ventriculocisternostomy
a
b
s
t
r
a
c
t
Background:Endoscopicthirdventriculostomy(ETV)isthetreatmentofchoiceforobstructive hydro-cephalus,buttheoutcomeisstillcontroversialintermsofageandaetiology.
Methods:Between1998and2011,168consecutiveprocedureswereperformedin164patients,primarily children(56%<18yearsofageand35%<2yearsofage).Thecausesofobstructivehydrocephalusincluded tumouralpathology,Chiarimalformation,congenitalobstructionoftheaqueduct,post-infectiousand post-haemorrhagicmembranes,andventriculo-peritonealshunt(VPS)malfunctions.SuccessfulETVwas definedbytheresolutionofsymptomsandtheavoidanceofashunt.
Results:ETVwassuccessfulin75.6%ofpatients,but19%ofthepatientsrequiredVPSinthefirstmonthafter ETV,and5.4%requiredaVPSmorethanonemonthafterETV.Fourpatientswereultimatelysubmitted forsecondETVs.Inthisseries,nomajorpermanentmorbidityormortalitywasobserved.
Conclusions:ETVisasafe procedureandaneffectivetreatmentforobstructivehydrocephaluseven followingthedysfunctionofpreviousVPSsandinchildrenyoungerthantwoyears.
©2014ElsevierB.V.Allrightsreserved.
1. Background
Theincidenceofcongenitalhydrocephalusisestimatedtobe 0.7casesper1000livebirthsindevelopedcountries[1],andthe incidenceof neonatalhydrocephalusisestimatedtobe3–5per 1000livebirthsandpredominantlyoccursinmales[2,3].
Hydrocephalusisoneofthemostcommondevelopmental dis-ordersinchildren;itismorecommonthanDownsyndromeand congenital deafness [4] and is the leading indication for brain surgeryinchildren[5].
Abbreviations:CSF,cerebrospinalfluid;CT,computedtomography;ETV, endo-scopicthirdventriculostomy;MRI,magnetic resonanceimage;VPS,ventriculo peritonealshunt.
∗ Correspondingauthorat:DepartmentofNeurosurgery,HospitaldeSãoJoão, AlamedaProf.HernâniMonteiro,4200-319Porto,Portugal.Tel.:+351913186726; fax:+351225025766.
E-mailaddresses:sfsalvador.neurocirurgia@gmail.com(S.F.Salvador), jago79@gmail.com(J.Oliveira),josuepereira@sapo.pt(J.Pereira), henriquepbarros@gmail.com(H.Barros),ruimcvaz@gmail.com(R.Vaz).
Currently,many patientswithhydrocephalusareconsidered candidatesforendoscopicthirdventriculostomy(ETV).Theidea ofanintracranialnon-prosthetic“internalshunt”toovercomethe obstructionsitetoachieve cerebro-spinalfluid(CSF)circulation andavoidtheuseofventricular-peritonealorauricular prosthe-seshasgainedwiderincreasingacceptanceinthelast20years[6]. Theendoscopicfenestrationofthethirdventriclefloorhasbeen usedwithincreasingfrequentlysince theearly1990s primarily becauseoftechnicalimprovements(e.g.,lightingsources, magnifi-cationandimageresolution)[7,8].Thesurgicalindicationsforthis procedureinclude thefollowing:stenosisoftheaqueduct, idio-pathicstenosisoftheMagendieandLuschkaforamina,somecases ofDandy–Walkermalformations,andpost-haemorrhagic hydro-cephalus[9–17]. ETV may also beperformed in selected cases of hydrocephalusthatare caused bymass effectof tumoursof thepinealgland;tectalplateorposteriorfossa; some suprasel-lar,quadrigeminalcistern,orarachnoidalextraventricularcysts; orevenmidlineintra-ventricularcysts[9,10,18–25].
Highersuccessrateshavebeenreportedforpatientswith steno-sis of theaqueduct [11,26–30]. Lower successrates have been
reportedforpatientswithpost-infectioushydrocephalusandfor post-haemorrhagicpatientswithpriorventriculo-peritonealshunt (VPS) failures [28,29,31–33]. This procedure is considered less effectiveinpaediatricpopulations,althoughtheminimumagefor theprocedureremainscontroversial[9,26,28,29,34–36].
In this retrospectivestudy, the surgicalindications, surgical techniques,nosocomialoutcomesandresultsof168consecutive ETVsthatwereperformedin164patientsattheCentroHospitalar deSãoJoãodoPorto(CHSJ)overaperiodof13yearsbeginningwith theintroductionofthetechnique(performedbetweenDecember 1998toDecember312011)werereviewed.
2. Methods
2.1. Patientpopulation
BetweenDecember1998andDecember2011,168consecutive ETVs(77.1%oftheneuroendoscopicprocedures)wereperformed at the Centro Hospitalar de São João do Porto (CHSJ) in 164 patientswithobstructivehydrocephaluswhowerefolloweduntil December31,2012.Thepatientswerepredominantlymale(the male:femaleratiowasapproximately3:2).Theaverageagewas 22.1yearsatthetimeofsurgery(56%ofthepatientswerepaediatric and20.8%wereinfants),andtheageofthemalegroupwasslightly younger(19.6vs.25.7yearsinthemalesandfemales,respectively). Theaveragefollow-upwas77.6months(13–168months). Mag-neticresonanceimaging(MRI)diagnosticconsiderationsincluded T1withthinreconstructioninthreeplanes,T2,CISS,flairand cine-phasecontrasts.
Theproportionofpaediatricpatients(i.e.,thosebelowtheage of18years)was56%,31.6%wereyoungerthantwoyears(mean 6.7months),andtheyoungestpatientwas6daysold.Amongour patients,19.0%were2–10yearsold(mean5.8years),5.4%were between10and18years(mean13.9years)and44%wereadults (mean45.6years).
Theselectionofthe168casesofETV(ofthetotalof817surgeries forhydrocephalusthusexcluding649VPSs)wasbasedonclinical andimaging(i.e.,computedtomography(CT)andMRI)evidence forobstructivehydrocephalus.Thegroupincluded34patientswith previousVPSs.
Proceduralsuccesswasdefinedbyclinicalimprovementsand VPSindependence,andfailuresweredividedintwogroups,early failure(withinonemonthoftheprocedure)andlatefailure(after onemonth).
2.2. Surgicaltechnique
Allpatientswere operatedon undergeneralanaesthesia, in thedorsaldecubitusposition,withtheirheadsstabilised.Inthe introductionoftherigidendoscope(MINOP®;Aesculap,Tuttlingen, Germany)transcorticallytowardstheMonrocavity,0–30◦optics wereused,andtheworkingchannellengthwas18cm.Wealsoused twocannulae;onehadanexternaldiameterof4.6mm(13F)and itsownchannelsfortheopticandforirrigationanddrainage,and another6mm(18F)cannulawithanotherchannel(2.2-mm diame-ter)fortheintroductionofmicro-endoscopyinstruments.Themost frequentlyusedopticwasthe0◦viewingangle.
Afteridentifyingthethalamo-striatevein,theseptalvein,and thechoroidplexusattheleveloftheMonroforamenandavoiding thefornix,theendoscopewasadvancedtothethirdventricleas identifiedbythethinmembranethatformsitsfloor.This mem-braneis bluishin colour and islocated beforethe mammillary bodiesandposteriortothetubercinereum.Forthecannulationof thisstructure,aFogarthytypeballoon-tipcatheterwithablunttip wasused(4French)toachieveanopeningdiameteronthefloorof
Table1
ETVfailureadjustedforaetiology,gender,andage.TheAetiologydistributionand logisticregressionwereusedtocomputeoddsratios(ORs)and95%confidence intervals(95%CIs).
AdjustedOR(95%CI)
Aetiology
Aqueductalstenosis 1
Chiari 3.26(0.96–11.1)
Tumour 0.46(0.14–1.48)
Othersa 0.92(0.33–2.57)
Age 0.97(0.95–0.99)
Gender
Female 2.00(0.90–4.45)
Male 1
aCysts(19),post-infectious(12),post-haemorrhage(12),Dandy–Walker malfor-mation(4),occlusionofthebasalcistern(4).
thethirdventricleofatleast5mm.Noneofthecaseshadhistories ofpriorcoagulationofthethirdventriclefloor.Theendoscopewas theninsertedthroughthecisternostomytothepre-pontinecistern toallowfortheidentificationofthebasilararteryandconfirmation oftheexistenceofaflawlesscommunication.
Afterremoving the endoscope, duraplasty and biologic glue wereused.Themeanoperativetimewas75min.
During the surgical procedure, continuous irrigation with Ringer’slactate(at37◦C)wasutilisedtopreventventricular col-lapse.Cisternostomieswereachievedinallcaseswithoutresorting tostereotactictechniques,radiologyorcomputerised neuronavi-gation(usingthehands-freemethod).
3. Results
ETVwaseffectivein75.6%ofcases,19.0%requiredtheinsertion ofaVPSsysteminthefirstmonthpost-ETV,and5.4%requireda laterintervention(VPSorre-ETV).InallineffectiveETVcases,the patencyofthestomawasverifiedbeforeplacingtheVPS.
AsshowninTable1,theincidenceofETVfailurevaried signif-icantlyacrossthedifferentagegroups(Chisquaretest–p=0.012); theETVincidencedecreasedwithage(pforthetrend=0.00)and wassignificantlygreaterwhentheaetiologywasaChiari malfor-mation(OR=3.4).
AmongtheETVfailurecases,23.2%ofthepatientswho under-went ETV required an additional procedure, and 1.2% required morethantwoprocedures(duetoclinicalandradiologicalfindings relatedtocomplexhydrocephalus).
Theprocedurehadtoberepeatedinfourpatients,includingtwo failures(10monthsand9yearsafterthefirstprocedures)andtwo obstructionsbyfibrin(twomonthsandfouryearsafterthefirst procedure).Threeofthesepatientswerechildrenolderthantwo years.
Nodeathsweredirectlyrelatedtothesurgicalprocedures.One ofthecases(withanunderlyingpathologyofaChiari malforma-tion)experienced subsequent ETV failure (13months after the procedure)anddevelopedacuteandfatalintracranial hyperten-sion.
In78patients,post-procedure,nonspecific,self-limitedfevers (38◦C)werediagnosed,and80%ofthesepatientswerebelowthe ageof18.Inthesecases,nomicrobialagentswereisolatedfrom bloodorCSFsamples.
Onepatientexhibitedself-limitedbleedingoftheponticartery duringthestomaballoondilatationandrequiredandexternal ven-triculardrain(EVD)andposteriorVPS.
Table2
Aetiologydistributionforthe164patients.
Aetiology n %
Congenitalmalformations 74 45.1
Idiopathicaqueductalstenosis 39 23.8
Chiarimalformation 22 13.4
Othera 13 7.9
Tumours 52 31.8
Cysts 15 9.1
Infections 12 7.3
Haemorrhage 11 6.7
aOther (congenital malformation) includes vascular malformations, Blake’s pouchcyst,Dandy–Walker,andocclusionofthebasalcistern.
malformation,particularlystenosisoftheaqueduct,andthis diag-nosiswasfollowedbycancerasshowninTables1and2.
The obstructions of the CSF predominantly occurred at the levels of the aqueduct level (63.1%) and the fourth ventricle (24.4%). Obstruction at Monro level (9.5%) and subarachnoid spaceand obstruction at morethan one level (3.0%) werealso observed.
Second procedures during the ETV were performed in 69.3% of the patients; 27.1% of the procedures were VPS removals, 27.1% were tumoural biopsies, 9.5% were intraven-tricular cyst fenestrations, 2.4% were septostomies, 1.8% were aqueductoplasties,and1.4%(onepatient)wasanOmmaya intro-duction.
Hydrocephalus secondary to tumour was foundin 52 cases (31.0%) thatwere predominantly male (2:1)and averaged27.4 yearsofage(5monthsto77years).Inthisoncologicgroup,7.7% ofthepatientswerebelowtheageof2years,32.7%werebetween twoand9years,7.7%werebetween10and17yearsand51.9% wereadults.
Amongalltumours,48.1%werelocatedintheposteriorthird portionofthethirdventricle,46.1%intheposteriorfossa,and5.8% intheanteriortwo-thirdsofthethirdventricle.Themeanfollow-up timewas63.4months(1–162).
Histologicalevaluations revealed malignant glial series (i.e., ependymoma,astrocytoma,oligodendrogliomaandglioblastoma multiform) in57.8% of thecases, embrionarycells (i.e., medul-loblastoma,pineoblastoma,andPNET)in26.9%ofthecasesand benignforms(i.e.,meningioma,haemangioblastoma,granuloma, macroadenoma,neuroma,craniopharyngioma, andglioneuronal tumourrosette-forming)in11.5%ofthecases;1.9%ofthecases correspondedtometastasis,and1.9%correspondedtolymphoma. Thesuccessrateinthisgroupwas88.5%.Thetechniquewas ineffectivein11.5%ofthecases,and7.7%requiredVPSswithinthe firstpost-ETVmonth.
Therewere39 (23.2%) patientswithstenosis of theSylvian aqueduct(51.3%due tocongenitalmalformations).Theaverage ageofthesepatientswas30.4 years atsurgery, andtheywere
Table3
Successandearlyfailureratesbyagegroupamongthepatientswithidiopathic aqueductstenosis.
Agegroup n Success(%) Earlyfailure(%)
<2years 8 3(37.5) 3(37.5)
≥2yearsand<10years 4 3(75.0) 1(25.0)
≥10yearsand<18years 3 3(100.0) 0(0.0)
≥18years 24 21(87.5) 2(8.3)
followed-upforamedianof87.1months(range:3–167months). Therewasaslightfemalepredominanceamongthis population (53.8%). The success and early failure rates of this group are describedinTable3.
In23.1%ofthesepatientsVPSswasrequired,and15.4%required VPSwithinthefirstmonthafterETV.Twoofthesepatientsrequired anadditionalETVasshowninTable4.
Chiarimalformationswerepresentin13.1%ofthesecases(28.9% wereduetocongenitalmalformations).Theaverageageof this groupwas4.8 years,andthemedianfollow-uptime was104.1 (18–164)months.Menpredominatedinthisgroup(63.6%).Most ofthepatients(81.8%)werebelowtheageoftwoyears,andthe overallsuccessratewas41.0%(50%forthoseolderthantwoyears and38.5%forthoseinyoungerthantwoyears).Theearlyfailure rateswere50%forthoseyoungerthantwoyearsand25%forthose olderthantwoyears.Thepatientsbelowtheageof2years pre-sentedwithChiariIImalformations,and theremainingpatients presentedwithtypeI.Fifty-ninepercentofthepatientsrequired VPSs,andhalfofthesepatientsrequiredVPSswithinthefirstmonth followingETV.
Therewerenostatisticallysignificantdifferencesinthe out-comes(p=0.515)betweenthepatientswithChiarimalformations whounderwentprimaryETVs(10patients)andthosewho under-wentETVforpriorVPSfailure(8patients).
ETVwasperformedin34patientswithpreviousVPSfailures (20.7%oftheseries)including21malesand13females. Twenty-threeofthesecaseshadtriventricularhydrocephalus,andeleven hadtetraventricularhydrocephalus.Mostofthesepatientswere belowtheageoftwoyears(n19),ninewereagedbetweentwo and17years,andsixwere18yearsoldorolder.
TheobstructionsinthepatientswithpreviousVPSmalfunctions wereattheaqueductlevelin21patients,thefourthventriclelevel in11patients,andtheMonrolevelintwopatients.Nineteenof thesepatientswereyoungerthantwoyearsold(withobstruction attheMonrolevel:1,aqueductlevel:8,andthefourthventricle level:10),ninewerebetweentwoand17years(withobstructions attheaqueductlevel:8andatthefourthventriclelevel:1),andsix wereadults(withobstructionsattheMonroandaqueductlevels; 1and5,respectively).
Table4
DatafromthepatientswhoweresubjectedtorepeatedETVs.
Gender Diagnosis Ageatfirst surgery
Timebetween surgeries
Reasonfor re-intervention
Currentage VPS Currentstatus
PreviousVPS AfterfirstETV AftersecondETV
F Neonatal
infection
6years 10months Failure/closure 9years Yes Yes Yes LWD
F Idiopathic
aqueductal stenosis
13years 2months Obstructionby
fibrin
13years Yes No No AWD
F Chiari 6years 9years Failure/closure 15years Yes Yes Yes LWD
F Idiopathic
aqueductal stenosis
71years 4years Obstructionby
fibrin
76years No No No AWD
ThereasonsforVPSfailureweremechanicaldysfunctionin28 (82.4%)patients,infectioninfivepatients(14.7%),andaforeign bodyreactioninonepatient.
Thesuccessrateinthisgroupwas61.8%;21ofthe34patients becameVPSindependent(meanfollow-uptimeof96.8months; range:22–167months).TheVPSswerereplacedin38.2%ofthe patients(13of34),andtheaveragetimeelapsedbetweentheETV andthenewVPSplacementwas8.6days(2–18days).
4. Discussion
Endoscopyiscurrentlywidelyappliedinneurosurgeryeither aloneorincombinationwithotherprocedures.ETViswell estab-lishedasatreatmentoptionforobstructivehydrocephalus[37,38]
andmightalsobeusefulinothercircumstances,suchastheVPS failure[39–41],asperourexperience.
ThesuccessofETVisgenerallydefinedbytheclinical improve-mentandVPSindependence,andsuccessratesvaryfrom50to 90%[42–44].BeemsandGrotenhuis[9]reportedoneofthelargest seriesofpatients(339)and achieveda successrateof76%.The overallsuccessrateinourserieswas61.8%,whichisslightlylower thanthat described inthereviewed literature.Wedefined ETV failureasthereappearanceofsymptomsofintracranial hyperten-sionfollowedbyCTorMRIconfirmationwheneverpossible.After asuccessfulETV,thesizeoftheventricularsystemcanrequirea fewmonthstostabilise[45–47],andthepresenceofaflowvoid appearstocorrelatewithclinicalsuccess,astheabsenceofflow voidcorrelateswithclinicalfailure[48].However,asBuxtonetal.
[49]noted,weconsideredthat“clinicaloutcomeisthemost impor-tantguidetosuccessorfailureasreductioninventricularsizeis bynomeansguaranteed,andradiologicaloutcomesalonemaybe misleading,and therelianceonthemshouldbeavoided”. How-ever,itisimportanttoconsiderthatourpatientpopulationwas a heterogeneousgroupinterms ofage,gender,and underlying pathologies.Allofthefailures(i.e.,thosewithinthefirstmonth aftertheprocedure)requiredtheplacementofaVPSasdescribed intheliterature.
AgewasapredictorofETVfailureinourseriesasevidenced bythestatisticallysignificantdifferenceinprocedurefailurerate accordingtoage.However,theunderlyingpathology shouldbe takenintoaccount.
Regardingmorbidityandmortality,areview[50]2884patients whohadundergoneETVhasbeenpublished.Inthisreview,the permanentmorbiditywas2.38%,andtherateofpermanent neu-rological complications (e.g., hemiparesis, gaze palsy, memory disorders,andalteredsensorium)was1.44%.Theoverall complica-tionratewas8.5%.TheothercomplicationsrelatedtoETVincluded intraoperativehaemorrhagefromtheependymalveins,choroid plexusorbasilararteryanditsbranches(3.7%),permanentdiabetes insipidus,weightgain,andprecociouspuberty.Theearly postop-erativemortalityrateduetosepsisandhaemorrhagewas0.21%. WithinthefirstmonthfollowingtheETV,thereported complica-tionsincludedCSFleakage,ventriculitis,subduralfluidcollection, andre-stenosisofthestoma.
Itiswidelyacceptedthatthecomplicationrateisrelatedtothe experiencesof eachcentreand eachindividualsurgeon[51].In thepresentstudy,asingleneurosurgeon(JosuéPereira,oneofthe authors)performedallneuroendoscopies,waspresentforall neu-roendoscopicprocedures(morethan250insameperiodandmore than200forhypophysealpathologies)Onlyrecentlyhasanother neurosurgeonin our clinicalreachedautonomyregarding neu-roendoscopicproceduresandhasremainedunderthesupervision oftheheadneuroendoscopyneurosurgeon.BourasandSgourus
[51]recentlyperformedameta-analysis,andtheoverall compli-cationratewasfoundtobe8.5%.Warf[52–54]reportedagreater successratefor endoscopic third ventriculostomywithchoroid
plexuscauterisation(ETV-CPC)thanforETValoneevenininfants. RecentlyKulkarnietal.[55]concludedthat“earlyNorth Ameri-canmulticentreexperiencewithETV-CPCininfantsdemonstrates that theprocedurehasreasonablesafety in selectedcases.The degreeofCPCachievedmightbeassociatedwithasurgeon’s learn-ingcurveandappearstoaffectsuccess,suggestingthatsurgeon training mightimprove results”.Wedidnot performthis tech-nique as most European (literatureevidence is increasing, but still scarce– e.g.,approximately30articlesrelated totheissue onPubmed).Inourseries,weobservednomortalityandavery lowrateofmajormorbidity.Mostofourmorbiditywasrelated to post-procedure unspecificand self-limited fevers(peaking a 38◦C)in 78patients(46.4%); mostofthesepatientswere chil-dren,noagentswereisolatedfromthebloodorCSFsamples,and noantibiotictreatments wererequired.Weobservednosepsis. One patient(0.6%) exhibitedself-limited pontic arterybleeding during the procedure. We observed no hypothalamic dysfunc-tion.
TheroleofageinpredictingthesuccessofETVremains con-troversialintheliterature.Severalauthorshavereportedsimilar resultsinchildrenandadultsanddidnotconsiderageasalimiting factorfortheindicationofETVevenforinnew-bornsasdescribed by Spennatoet al.[56] and severalother authors[9,11,57–59]. Otherworks,suchasthoseofBuxtonetal.[60],Kadrianetal.[61]
andKoch-Wiewrodtetal.[62]asreferredtoinSpennatoetal.[56], reportedtheoppositeextremeofsuccessratesbelow30%ininfants andconsideredanageunderoneyeartobeacontraindicationfor ETV.
The majorityof authorshave described intermediate results withsuccessratesofapproximately50%[52,56,58,63,64]and con-tinue to advocate ETV as the first-line treatment for children andinfantswithobstructivehydrocephalus.Weconcurwiththis opinion.Obstructivehydrocephalusistypicallydefinedbasedon themeanratiobetweenthesizes(asdefinedbythemaximum cross-sectionalsurface)ofthethirdandfourthventricles,which is typically approximately0.5 [65].Our radiological criteria for thedefinitionofobstructivehydrocephalusincludedanincrease inthisvalueinadditiontothepresenceofenlargedoftemporal horns,transependymaloedemainthelateralventricles,outward bowingoflateralthewalls,inferiorbowingofthefloorofthird ventricle [66] andtheabsenceof flowvoids at thelevelof the aqueduct [67]. Post-infectious(12) and post-haemorrhagic (11) caseswereincludedinthisseriesandcategorisedasobstructive hydrocephalusbecausethesecasesfulfilledourclassification crite-ria.Suchcasesrepresentedlessthan5%ofthepost-infectiousand post-haemorrhagiccasesinourcentre.Oursuccessratewas50% ininfants,whichoverlapswiththatreportedintheliterature.The reducedsuccessrateamonginfants(particularlythosebelowthe ageofsixmonths)islikelydueto(1)thefactthatthesepatients exhibitagreatertendencytoformnewarachnoidmembranesthat leadtotheobstructionofthestoma[68]and(2)todeficitsinthe reabsorptionofCSFduetotheimmaturityofthearachnoidvilli
[59,69].
Inbothcongenitalandacquiredstenosisoftheaqueduct,Jones et al.[13,29] and,more recently,Mugamba and Stagno [13,29]
reportedhighsuccessratesthatwererelatedtotheacquiredtype, theageatsymptomonsetandthesurgicalprocedure[13,70,71]. Similarly,inourseries,wefoundasuccessrateamongadultsof 87.5%, which issimilarto thatreportedin more recent studies
[13,56].
hydrocephalusassociated with myelomeningocele is due to an obstructionoftheexitareaofthefourthventricleresultingfrom anassociatedChiarimalformation[74].
Severalauthors[37,75–77]havereportedthathydrocephalusin ChiaripatientscanbetreatedwithETVandthatthesuccessrate ofthistreatmentcanbeashighas50%.Thesuccessrateinour serieswas40%,whichiswithintherangeofratesthathavebeen describedininternationalseries.Inouropinion,thesuccessofETV inthesepatientsfurthersupportstheutilisationofthisprocedure forselectedpatientswithChiari,althoughthenumbersofpatients withthisconditioninourserieswaslow.
TheroleofETViswellestablished,anditssuccessinthe treat-mentofobstructivehydrocephalusduetointracranialtumoursis recognisedbothinpaediatricpatients(asdescribedinrecent arti-clesbyWonget al.[78],DiRoccoetal.[79],andotherauthors
[21,80–82])andadults[83,84].
Intheabovereferencedseries,thesuccessratesforthe restora-tionof CSFflowwerehigh,and weobservedasimilarresultin ourseries(88.5%).OnlyfourpatientsrequiredVPSsintheearly stage,andtworequiredVPSsatlatertimes.Thelesionsof19cases weresubmittedtoendoscopicbiopsiesthatallowedforhistological diagnosesforthesepatients.Thisprocedureisindicatedprimarily forlesionsinthequadrigeminalplateandtheposteriorthirdof thethirdventricleasdescribedbyWongetal.[83],Morgenstern andSouweidane[84],Popleetal.[21]andDepreitereetal.[85]. Histologicaldiagnoseswereachievedinallbiopsiedcases.
VPSfailuresarerelativelycommonandhavebeendefinedby MugambaandStagnointermsofindicationsforendoscopicthird ventriculostomy[13]as“anyrevisionperformedontheprosthesis afterbeingimplemented,aphenomenonbeingdirectlyrelatedto timewhichseemstobemorefrequentinchildren”.Thisdefinition issupportedbyBilgineretal.[86]andMartonetal.[87].Inour serieswealsoobservedthat28ofthe34patientswithprevious VPSswerechildren.ETVisasafeandeffectiveprocedureforthe treatmentofappropriatelyselectedpatientswithVPshuntfailure, andMRIevidenceregardingflowobstructionisessentialforthese cases.AsdescribedbyBaldaufetal.[70],thesuccessratesforsuch casesareapproximately70%[88–90].TheoccurrenceofVPS mal-functionsdoesnotinfluencetheincidenceofETVfailure[13,91,92]
becausethemajorityoffailurestendtooccuratanearlierstage. TheclosureoftheETVstomahasalwaysbeenrecognisedasa causeoffailureoftheprocedure,andanewETVprocedurehas beendescribedasanalternativetoVPSbyseveralauthors, includ-ingPerettaetal.[93],Siominetal.[94],WagnerandKoch[68]. Theseauthorshavereportedsuccessratesbetween65and 75% buthavealsoreportedworseoutcomesforchildrenparticularly thosebelowtheageoftwoyears.Intheirseries,Mahapatraetal.
[95]reportedastomaclosurerateof9%andare-ETVsuccessrate of93.2%;themajorityofthelatterpatientsexperiencedlate fail-ures.AccordingtoFukuharaetal.[31,96]andCinallietal.[11],the performanceofanadditionalETVduetothefailureoftheprimary procedureshouldbeconsideredbasedonintracranialhypertension andtheabsenceofflowinMRIcinephase-contrastimages.
We had four cases of stoma closure; these cases exhibited signsof intracranial hypertensionand flow obstruction onMRI cine-phase-contrastimages.Allofthesecaseswerefemale,two exhibitedclosureofthestoma,andtheothertwoexhibited occlu-sionbyfibrin.ClosureofnewETVoccurredintwopatientswithin oneyearoftheprocedure,andthesetwocasesrequiredVPSs.The datafor thepatientswho weresubjected torepeatedETVsare presentedinTable4.
Basedontheabovediscussion,ETVrepresentsafeasible alter-nativetoVPSinthetreatmentofhydrocephalus.Alargenumber of children might benefit from ETV, which is a simpler treat-mentoption thatdoes notrequire theplacement of prosthetic material(i.e.,aVPS),hasalowerrateofassociatedcomplications
[97–100]and,webelieve,requireslessfrequentfollow-upsand thusreducesthesocio-economiceffectsonthefamiliesandthe patientsthemselves[101,102].EvenincasesofVPSdysfunction, ETVisanalternativeforthetreatmentofappropriatelyselected casesduetoitsgoodsuccessrateandtheabsenceofother associ-atedcomplications.
5. Conclusions
Endoscopicsurgeryisexperiencingincreasinglywidespreaduse inthetreatmentofobstructivehydrocephalus.Thistechniqueis associatedwithlowermedium-to-long-termcoststhantheuseof CSFshuntdevicesandavoidsthecomplicationsassociated with shuntdependency. Thistechnique is considered tobe thefirst choicetreatmenteven forlatentcases becauseit ispossibleto avoidtheuseofVPSsandofthecomplicationsrelatedtoVPSuse.In ourseries,theuseofaVPSwaspermanentlyavoidedin134cases, including31ofthe53totalcaseswhowerebelowtheageoftwo years.
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