Pregnancy and refractory epilepsy in Sturge-Weber syndrome: Challenges and outcomes

Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder caused by focal vascular dysplasia. It is associated with facial angiomas, ocular abnormalities, epilepsy, intellectual disability, and leptomeningeal angiomas. There is a paucity of literature regarding the management of epilepsy and pregnancy in women with SWS. The effects of pregnancy on this syndrome therefore remain largely undefined. Consensus on management is needed; as pregnancy has widespread effects on the vascular system and therefore may pose risk in women with SWS. This is a comprehensive review of the existing literature and case report of a woman with SWS, refractory epilepsy, and a successful pregnancy and delivery. Fifteen cases were found and, in addition to our case, 14 cases were reviewed, 11 of which discuss labor and delivery. Neurological complications were described in four cases and successful delivery without complication occurred in seven patient cases.