Background: Intracranial vascular abnormalities in Sturge-Weber syndrome, including leptomeningeal angiomatosis, anomalous cortical venous structures, and transmedullary developmental venous anomalies, are well recognized. Prominent vascular flow voids on T2-weighted magnetic resonance imaging (MRI) are occasionally identified in patients with Sturge-Weber syndrome, raising concern of arteriovenous malformations, a congenital high-flow vascular malformation with a risk of bleeding.

Methods: We report four patients with prominent flow voids on conventional MRI that suggested high-flow lesions.

Results: Diagnostic evaluation was performed with cerebral angiography in one patient and with a combination of magnetic resonance angiography and magnetic resonance venography in three patients. In all four patients, the conventional MRI-identified lesions represented prominent developmental venous anomalies and not arteriovenous malformations.

Conclusions: This series highlights that developmental venous anomalies may appear in individuals with Sturge-Weber syndrome as unusually large and seemingly high-flow lesions on MRI. Noninvasive imaging with magnetic resonance angiography and magnetic resonance venography can be used in the management of such patients for further characterization of these vascular structures.