Abstract TP171: Acute Magnetic Resonance Imaging Findings In Patients With Sturge Weber Syndrome

Abstract

Sturge-Weber syndrome (SWS) is a neurovascular disorder characterized by facial Port-wine stain and intracranial capillary malformations. Patients can have multiple neurological complications including seizures, headaches, and stroke-like episodes, often leading to multiple hospital presentations. Published reports have shown conflicting results on whether patients with SWS with acute neurological complaints have positive imaging findings. Small case reports have described acute ischemic or hemorrhagic changes in SWS patients, whereas larger case series have not identified such imaging findings. Our aim was to report imaging findings in a cohort of SWS patients who presented with acute neurological complaints. We performed a retrospective analysis on all MRI studies performed on SWS patients presenting to our institution with acute neurological symptoms between August 1999 and July 2018. Imaging studies were initially evaluated by the radiologists on-call, and subsequently reviewed by a separate neuroradiologist to confirm the diagnoses. Only studies that were interpreted as having acute findings by both radiologists were counted as positive. During the study period, 23 patients with SWS presented to our institution with acute neurological complaints, of which 13 (56.5%) were male, median age 3 years, with 50 MRI obtained. Seizures were the most common indication for acute imaging (58%), followed by stroke-like episodes (32%), headache (6%), lethargy (2%) and vomiting (2%). Acute changes on MRI were found in 8 studies (16%), with restricted diffusion in 5 patients (of which 4 were presumed to be due to stroke and 1 due to ongoing seizure activity), brain edema in 2 patients and a subdural fluid collection in 1 patient. Of the patients with acute imaging findings, 4 presented with seizures, 3 with stroke-like episodes, and 1 with lethargy. In conclusion, SWS patients with acute neurological complaints can have acute findings on MRI consistent with stroke or other acute injuries. Imaging appears to be an important tool in the management of acute episodes in SWS patients. Restricted diffusion in SWS patients may represent venous congestion or other hemodynamic changes leading to acute injury.