Vein of Galen aneurysm malformation (VGAM): closing the management loop

Abstract

We report a case where an antenatal scan showed a cerebral vessel dilatation consistent with a vein of Galen malformation (VGAM). After delivery the diagnosis was confirmed on MRI scan. The infant's condition was stable in relation to the two important clinical indicators, mainly not in congestive cardiac failure and no hydrocephalus. The vein of Galen assessment score as used in Hopital Bicetre was 20/21. The scoring system is based on a multiorgan assessment including the five parameters cardiac, cerebral, respiratory, hepatic and renal (Table 1). A score of < 8 indicates no treatment, 8-12 means immediate intervention while a score > 12 delays intervention to circa six months of age. At four months the head circumference accelerated with hydroceaphalus on scans and the infant became clinically symptomatic. Urgent embolisation was performed. The procedure uses N-butylcyanoacrylate (NBCA) glue as the embolisation agent, which in this case was inserted through the left posteriomedial choroidal artery. Complete obliteration was achieved. The infant subsequently required insertion of a ventriculoperitoneal shunt for the hydrocephalus component. The infant has had no recurrence. He remains well on objective examination he has mild developmental delay. In summary this case highlights the value of antenatal diagnosis,the VGAM assessment score to determine the optimal time of intervention and the effectiveness of embolisation.