title: Focal resection of leptomeningeal angioma in a rare case of Sturge-Weber syndrome without facial nevus.
abstract: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder comprised typically of a facial nevus, leptomeningeal angioma with calcifications, and seizures. SWS without a port-wine stain is a rare variant with only 30 cases reported in the literature. Here, a case of an 8-year-old girl with no cutaneous abnormalities presenting with medically intractable epilepsy and MRI and CT findings consistent with SWS is described. The patient underwent multistage surgery with subdural electrode monitoring before and after resection of the epileptogenic focus, with complete excision of the lesion and postoperative resolution of her seizures. This is the first reported case of three-stage surgery for localized resection of the seizure focus for SWS.
authors.affiliation: Division of Pediatric Neurosurgery, Department of Neurosurgery, New York University, New York, N.Y., USA.
authors.forename: Yevgenia
authors.initials: Y
authors.lastname: Shekhtman
authors.forename: Irene
authors.initials: I
authors.lastname: Kim
authors.forename: James J
authors.initials: JJ
authors.lastname: Riviello
authors.forename: Sarah S
authors.initials: SS
authors.lastname: Milla
authors.forename: Howard L
authors.initials: HL
authors.lastname: Weiner
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D019339:Port-Wine Stain; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000601:surgery*