Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series.

AIM: To describe presentation, clinical course, and outcome in postneonatal presentations of vein of Galen malformation (VGM).

METHOD: Children older than 28 days presenting with VGM (from 2006-2016) were included. Notes/scans were reviewed. Outcome was dichotomized into 'good' or 'poor' using the Recovery and Recurrence Questionnaire. Logistic regression was performed to explore relationships between clinico-radiological features and outcome.

RESULTS: Thirty-one children (18 males, 13 females) were included, presenting at a median age of 9.6 months (range 1.2mo-11y 7mo), most commonly with macrocrania (n=24) and prominent facial veins (n=9). Seven had evidence of cardiac failure. VGM morphology was choroidal in 19. Hydrocephalus (n=24) and loss of white matter volume (n=15) were the most common imaging abnormalities. Twenty-nine patients underwent glue embolization (median two per child). Angiographic shunt closure was achieved in 21 out of 28 survivors. Three children died of intracranial haemorrhage (1y, 6y, and 30d after embolization). Ten patients underwent neurosurgical procedures; to treat haemorrhage in four, and hydrocephalus in the rest. Outcome was categorized as good in 20 out of 28 survivors, but this was not predictable on the basis of the variables listed above.

INTERPRETATION: Postneonatally presenting VGM has distinctive clinico-radiological features, attributable to venous hypertension. Endovascular treatment is associated with good outcomes, but more specific prognostic prediction was not possible within this cohort.

WHAT THIS PAPER ADDS: Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage.