Vein of Galen malformation

Vein of Galen malformation (VOGM), also known as vein of Galen aneurysmal malformation or vein of Galen aneurysmal dilatation, is a rare congenital vascular malformation of the brain. It involves abnormal arteriovenous connections (fistulas) between cerebral arteries and the median prosencephalic vein of Markowski, a fetal precursor structure that normally regresses during development. These abnormal connections result in high-flow arteriovenous shunting, causing dilatation of the vein of Galen and the surrounding venous structures. VOGM accounts for approximately 1% of all intracranial vascular malformations but represents about 30% of vascular malformations presenting in the pediatric age group. The clinical presentation varies depending on the age at diagnosis. In neonates, VOGM commonly presents with high-output cardiac failure due to the large volume of blood shunted through the malformation, which places enormous strain on the heart. Affected neonates may also develop pulmonary hypertension, hepatic dysfunction, and renal failure. In infants, the malformation may present with macrocephaly (enlarged head) and hydrocephalus caused by obstruction of cerebrospinal fluid drainage, as well as developmental delay and seizures. Older children may present with headaches, seizures, learning difficulties, or focal neurological deficits. Cranial bruits (abnormal sounds heard over the skull) may be detected on physical examination. The primary treatment for VOGM is endovascular embolization, a minimally invasive procedure in which catheters are used to occlude the abnormal arteriovenous connections, typically performed by specialized interventional neuroradiologists. The timing and approach to treatment depend on the severity of symptoms and the patient's clinical stability. In critically ill neonates, medical stabilization of cardiac failure is essential before intervention. Transarterial embolization is the most commonly used technique, often performed in staged procedures. Outcomes have improved significantly with advances in endovascular techniques, though the condition still carries significant morbidity and mortality, particularly in neonates presenting with severe cardiac failure. Untreated, the prognosis is generally poor. Long-term neurodevelopmental follow-up is essential for all affected children.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Vein of Galen malformation