Yield of Emergent Neuroimaging in Patients With Sturge-Weber Syndrome Presenting With Acute Neurologic Symptoms

Background/aims: Seizures, strokelike episodes, and headaches are common complications in patients with Sturge-Weber syndrome. Based on our experience, we hypothesized that patients with Sturge-Weber syndrome have frequent urgent neuroimaging studies when presenting acutely to the emergency department. In this study, we aimed to determine the incidence of acute imaging studies in this patient population and to evaluate the prevalence of findings such as acute intracranial hemorrhagic or ischemic strokes.

Methods: To determine the frequency and yield of brain imaging, we conducted a retrospective chart analysis in patients with Sturge-Weber syndrome who presented to Boston Children's Hospital with acute neurologic symptoms between 1996 and 2016.

Results: We reviewed 136 encounters of patients with Sturge-Weber syndrome. In 73 of 136 encounters (53.7%), patients underwent a total of 89 imaging studies, consisting of 47 head computed tomographies (CTs) and 42 brain magnetic resonance images (MRIs). Twenty-two percent of patients imaged underwent both CT and MRI scanning of the brain. Patients with strokelike episodes or headaches were more likely to be imaged compared to patients presenting with seizures (89.7% and 100% vs 34.4%, respectively). None of the neuroimaging studies showed acute hemorrhagic or ischemic strokes.

Conclusions: Acute neurologic manifestations of Sturge-Weber syndrome frequently lead to urgent neuroimaging. In our cohort, there was no imaging evidence of acute hemorrhagic or ischemic strokes. In addition, emergent imaging in patients presenting with breakthrough seizures did not result in meaningful changes in management.