Hemispherotomy for drug-resistant epilepsy in bilateral Sturge-Weber syndrome: illustrative cases

Abstract

Background: Sturge-Weber syndrome (SWS) is a congenital neurocutaneous disorder characterized by angiomas of the face, choroid, and leptomeninges. Seizures in these children often present within the first 2 years of life. SWS is typically unilateral, but bilateral SWS occurs in approximately 15% of cases. Bilateral SWS is associated with earlier seizure onset and poorer cognitive, developmental, and functional outcomes. More than half of children with SWS develop drug-resistant epilepsy requiring surgical intervention. Hemispherotomy has been established as a successful treatment for unilateral SWS, but resective surgery has traditionally not been considered a treatment option for patients with bilateral disease.

Observations: In this report, the authors present the cases of 4 children (7 months-2 years of age) with bilateral SWS and drug-resistant epilepsy with a unilateral electroencephalography predominance. After a multidisciplinary conference in each case, all children were successfully treated with unilateral hemispherotomy. These patients achieved prolonged periods of seizure freedom postoperatively, a better quality of life, and demonstrated improved developmental progress at long-term follow-up.

Lessons: This case series suggests that functional hemispherotomy may be a safe and effective therapeutic option for improving seizure burden in cases of bilateral drug-resistant SWS with asymmetric seizure burden. https://thejns.org/doi/10.3171/CASE25125.

Keywords: Sturge-Weber; drug-resistant epilepsy; epilepsy surgery; hemispherotomy; pediatric.

FIG. 1.

Case 1. Preoperative axial T1-weighted MR contrast studies demonstrating enhancement of bilateral hemispheres, left greater than right (A and B), as well as enlargement of extra-axial CSF spaces and cerebral hypoplasia bilaterally (C). Four-year postoperative follow-up image demonstrating expected postoperative changes, including left hemispheric disconnection and a widespread increase in right gray matter volume (D).

FIG. 2.

Case 2. Axial T1-weighted preoperative MR contrast studies demonstrating abnormal FLAIR signal consistent with left-dominant bilateral SWS (A) and enlarged ventricles and sulci attributable to parenchymal volume loss (B). Postoperative images demonstrating expected changes (C and D).