Andersons pediatric cardiology 1323
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FIG.50.4 MagneticresonanceimagingofaneonatewithaveinofGalen
aneurysmalmalformationshowingmultiplearterialfeedersintothedilated
venouschannel(arrow).
ThenaturalhistoryofVGAMsiscloudedbythefactthatmanyhistorical
reportsincludemalformationsthatresultindilationofboththemedian
prosencephalicveinofMarkowskiandtheveinofGalen.Duetotherarityofthe
lesion,meta-analyseshaveprovenhelpfulinunderstandingoutcomesinpatients
withVGAMs.Inonemeta-analysis,42nearly77%ofpatientswhodidnot
undergotreatmentwerefoundtohavedied,whereasinanothermeta-analysis,
theriskofpreoperative(includingendovasculartechniques)suddendeathwas
foundtobe6%.40SpontaneousthrombosisofVGAMshasalsobeenreported
andisnotuncommon.40,43Medicaltreatmentincludessupportivetherapy,
antiepileptictherapyforseizures,managementofhydrocephalus/raised
intracranialpressure,managementofheartfailuresymptoms,andrespiratory
supportwithmechanicalventilationifneeded.Ultimately,directtreatmentofthe
lesionisrequired.Surgerywashistoricallyperformedbutwasassociatedwith
highmortality.However,surgeryisneededinsomecasestomanageintracranial
hemorrhagesandhydrocephalus.
Thetreatmentofchoiceinthecurrenteraisendovascularembolization,which
ismostcommonlyperformedviathearterialapproach(carotidartery
cannulationviathefemoralarterialroute)andlesscommonlyfroma
transvenousortranstorcular(directlythroughtheoccipitalbone)approach.43,44
Thegoalofendovasculartherapyisnottoobliteratetheshuntcompletelyatthe
firstsetting(Fig.50.5)butrathertocontrolsymptoms.Often,morethanone
procedureisneededtoresultincompleteocclusionorcontroloftheshuntor
shunts.44Smaller-boresheaths,catheters,andmicrocathetersenablethe
proceduretobesafelyperformedinevensmallneonates.Mostoftenthearterial
feedingvesselsareoccludedwithembolicliquidagents(e.g.,N-butyl
cyanoacrylate)toensurethattheverydistalsmallfeedingvesselsareoccluded.
Coilsaregenerallyusedasanadjuncttoliquidembolizationagents.Recently,
useofamicrovascularplughasbeenreported.45Inalargesingle-center
experiencefromFrance,themortalityfromendovasculartreatmentwasnearly
11%,similartothe12%foundinalargemeta-analysis.44Theseprocedurescan
betechnicallydemanding,andcomplicationsofcerebralhemorrhage,cerebral
ischemia,legischemiaandcerebralvesselperforationcanoccur.44Allpatients
withVGAMsshouldbefollowedforlifetomonitortheirneurodevelopmentand
cognitivefunction.
FIG.50.5 SelectivecarotidangiographyinaneonatewithaveinofGalen
aneurysmalmalformationafterthefirstendovasculartreatmentwithN-butyl
cyanoacrylateandcoilsshowingresidualflowintothedilatedvenous
channel.
