Summary

Vein of Galen malformations (VGM) are rare intracranial vascular anomalies that constitute 1% of all intracranial vascular malformations. Untreated VGM have a very poor prognosis. A high proportion of patients who present in the neonatal period rapidly deteriorate and succumb to congestive cardiac failure. The prenatal diagnosis and possible in utero referral to highly specialised centre of postnatal care have resulted in considerable improvement in prognosis. The authors present a case of a postnatally diagnosed VGM in a full-term infant presenting with progressive cardiac failure and necrotising enterocolitis secondary to gut hypoperfusion. They emphasise the importance of prenatal diagnosis by pulsed wave Doppler and colour-velocity imaging for subsequent referral to a centre of excellence in a multidisciplinary approach involving neonatologists, paediatric cardiologists and interventional radiologists in order to optimise the management and limit the neurological sequelae in children with this type of malformation.