The Eye That Revealed the Hidden Storm: A Neonatal Vein of Galen Aneurysmal Malformation—A Case Report and Comprehensive Literature Review
Mohamed Sadik, MD1; Ahmed Elmelhat, MD1*
1Department of Pediatrics/Neonatology, HMS Mirdif Hospital, Dubai, UAE.
*Corresponding Author: Ahmed Elmelhat, Department of Pediatrics/Neonatology, HMS Mirdif Hospital, Dubai, UAE.
https://doi.org/10.58624/SVOAPD.2026.05.026
Received: August 08, 2026
Published: September 08, 2026
Citation: Sadik M, Elmelhat A. The Eye That Revealed the Hidden Storm: A Neonatal Vein of Galen Aneurysmal Malformation—A Case Report and Comprehensive Literature Review. SVOA Paediatrics 2026, 5:5, 164-172. doi: 10.58624/SVOAPD.2026.05.026
Abstract
Background: Vein of Galen aneurysmal malformation (VGAM) is a rare congenital cerebrovascular anomaly that predominantly presents in the neonatal period with cardiovascular compromise rather than primary neurological deficits. Diagnostic delays are common because initial neurological examinations are typically reassuring, while high-output cardiac failure and persistent pulmonary hypertension of the newborn (PPHN) mimic common cardiopulmonary disorders.
Case Presentation: A preterm female neonate born at 36 weeks' gestation presented with escalating respiratory distress, severe PPHN (estimated pulmonary artery systolic pressure [PASP] of 90 mmHg), high output cardiac failure, and prominent right unilateral proptosis. Fetal ultrasound had previously demonstrated a middle cranial fossa "probable arachnoid cyst". Non-contrast cranial CT and subsequent contrast enhanced MRI, MRA, and MRV revealed a 4.5 × 4.2 × 5.0 cm right temporo-frontal extra-axial aneurysmal venous sac with arterial supply from external carotid branches and direct drainage into the distal right sigmoid sinus. Repeated neuroimaging abroad confirmed the diagnosis of VGAM.
Management & Outcome: Medical stabilization with nasal continuous mandatory ventilation, sildenafil, and aggressive furosemide diuresis relieved pulmonary venous congestion and enabled successful weaning to room air by day 11 of life. Regional neurointerventional services were unavailable; the infant was discharged on day 12 for planned elective embolization abroad. Staged transarterial embolization (3 sessions) was subsequently performed internationally.
Conclusions: Unilateral proptosis in a neonate with high-output heart failure or refractory PPHN is a critical red flag for dural sinus involvement. Postnatal vascular neuroimaging (MRI/MRA/MRV) should be performed urgently for any antenatally reported intracranial cyst. Geographic gaps in pediatric neurointerventional services remain a major determinant of clinical outcome.
Keywords: Vein of Galen aneurysmal malformation; Persistent pulmonary hypertension of the newborn; High-output cardiac failure; Neonatal proptosis; Transarterial embolization.