Vein of Galen malformations in the newborn: case series. J Neurosurg Case Lessons 2023 Jun 12;5(24)
Date
06/19/2023Pubmed ID
37334971Pubmed Central ID
PMC10550657DOI
10.3171/CASE23201Scopus ID
2-s2.0-85163201783 (requires institutional sign-in at Scopus site)Abstract
BACKGROUND: Vein of Galen malformations (VoGMs) in newborns often represent life-threatening emergencies. Outcome is difficult to predict. The authors review 50 VoGM cases to correlate anatomical types with treatment and outcome.
OBSERVATIONS: Four distinct types of VoGMs are identified: mural simple (type I), mural complex (type II), choroidal (type III), and choroidal with deep venous drainage (type IV). Seven patients presented with mural simple VoGMs with a "single hole" fistula supplied by only one large feeder. These patients were treated electively at >6 months; development was normal. Fifteen patients presented with complex mural VoGMs. Multiple large feeders joined a single fistulous point within the wall of the varix. Patients typically presented with congestive heart failure (CHF) and required emergent transarterial intervention. Mortality was 7.7% with less than two-thirds developing normally. Twenty-five patients presented with choroidal VoGMs. Multiple large arterial feeders joined at multiple fistulous sites. Severe CHF in most patients required emergent transarterial and sometimes transvenous intervention. Mortality was 9.5%; two-thirds of the patients had a normal development. Three babies presented with choroidal VoGMs with deep intraventricular venous drainage. This phenomenon caused fatal "melting brain syndrome" in all three patients.
LESSONS: Recognition of the specific VoGM type determines treatment options and sets outcome expectations.
