- Vein of Galen malformations account for 1 to 2% of all intracranial malformations.
- However, causes 30% of vascular malformations in the paediatric group.
- It is the most common antenatally-diagnosed intracranial vascular malformation [1].
A neonate born at 37 weeks of gestation presented with respiratory distress 3 hours after birth. Physical examination showed a systolic murmur, coarse breath sounds in both lungs and subcostal retractions. For further evaluation, a chest radiograph was performed which showed cardiomegaly and bilateral pleural effusion. Echocardiogram showed right-to-left shunting at the patent ductus arteriosus, tricuspid regurgitation, dilated and hypertrophied right ventricle, dilated right atrium and a patent foramen ovale.
The diagnosis of vein of galen malformation was confirmed via cranial ultrasonography and gadolinium-enhanced T1-weighted magnetic resonance imaging. The imaging showed a large vein of Galen malformation of choroidal type, atrophy of the brain stem and cerebellum in sagittal (Panel A) and axial views (Panel B) and hydrocephalus.
Vein of Galen malformation
Vein of Galen malformation, also known as median prosencephalic atriovenous fistula is a rare congenital intracranial anomaly. The anomaly tends to present dramatically during early childhood with signs including high-output cardiac failure and left-to-right shunt. The atriovenous fistulas in the brain can cause pulmonary hypertension and heart failure in neonates.
Supportive and endovascular treatments have been shown to improve survival rate in neonates. However, with varying neurodevelopmental outcomes, the prognosis remains poor.
Treatment plan for the patient included an endovascular embolisation of the malformation with use of prostaglandin E1, diuretic agents, inhaled nitric oxide and vasopressors. However, the neonates pulmonary oedema worsened with renal and liver failure. The neonate died 21 days after birth [2].
References
- Vein of Galen aneurysmal malformation https://radiopaedia.org/articles/vein-of-galen-aneurysmal-malformation-1
2. Vein of Galen Malformation https://www.nejm.org/doi/full/10.1056/NEJMicm1913659