Sturge Weber Syndrome with Concomitant Infantile Vein of Galen Aneurysmal Malformation: Role of Multi-modality Imaging in Diagnosis

Abstract

IntroductionSturge Weber syndrome is a neurocutaneous disorder, characterised by vascular malformation with capillary venous angiomas. Though it presents with vascular anomalies, association with vein of Galen aneurysmal malformation is rare. ReportA 2-year-old girl presented with delayed developmental milestones, head enlargement and convulsions. Examination revealed an ill-looking child with head enlargement, hypotonia and bilateral blindness. Computed tomographic angiography revealed gyriform cerebral calcifications with vein of Galen aneurysmal dilatation, showing multiple feeding arteries. Findings were also corroborated by ultrasound. DiscussionThis experience underscores the value of imaging in revealing this complex angio-architecture, which is necessary in the diagnosis and management.