Vein of Galen Malformation

A neonate born at 37 weeks of gestation experienced respiratory distress 3 hours after birth, presenting with subcostal retractions, coarse breath sounds, and a systolic murmur. Diagnostic imaging, including a chest radiograph and echocardiogram, revealed cardiomegaly, bilateral pleural effusions, a patent foramen ovale, dilated right atrium, dilated and hypertrophied right ventricle, tricuspid regurgitation, and right-to-left shunting at the patent ductus arteriosus. Cranial ultrasonography and gadolinium-enhanced T1-weighted magnetic resonance imaging confirmed a large choroidal type of vein of Galen malformation, along with hydrocephalus and atrophy of the brain stem and cerebellum. Vein of Galen malformations are rare congenital arteriovenous fistulas in the brain that can lead to heart failure and pulmonary hypertension in neonates. Although supportive and endovascular treatments can prolong survival, the prognosis remains poor with variable neurodevelopmental outcomes. Despite staged endovascular embolization and other medical interventions such as mechanical ventilation, vasopressors, inhaled nitric oxide, diuretic agents, and prostaglandin E1, the patient's condition worsened with pulmonary edema, liver failure, and renal failure, leading to death 21 days after birth.

A neonate born at 37 weeks of gestation experienced respiratory distress 3 hours after birth, presenting with subcostal retractions, coarse breath sounds, and a systolic murmur. Diagnostic imaging, including a chest radiograph and echocardiogram, revealed cardiomegaly, bilateral pleural effusions, a patent foramen ovale, dilated right atrium, dilated and hypertrophied right ventricle, tricuspid regurgitation, and right-to-left shunting at the patent ductus arteriosus. Cranial ultrasonography and gadolinium-enhanced T1-weighted magnetic resonance imaging confirmed a large choroidal type of vein of Galen malformation, along with hydrocephalus and atrophy of the brain stem and cerebellum. Vein of Galen malformations are rare congenital arteriovenous fistulas in the brain that can lead to heart failure and pulmonary hypertension in neonates. Although supportive and endovascular treatments can prolong survival, the prognosis remains poor with variable neurodevelopmental outcomes. Despite staged endovascular embolization and other medical interventions such as mechanical ventilation, vasopressors, inhaled nitric oxide, diuretic agents, and prostaglandin E1, the patient's condition worsened with pulmonary edema, liver failure, and renal failure, leading to death 21 days after birth.