Focal resection of leptomeningeal angioma in a rare case of Sturge-Weber syndrome without facial nevus. Pediatr Neurosurg 2013;49(2):99-104
Date
01/18/2014Pubmed ID
24434861DOI
10.1159/000357357Scopus ID
2-s2.0-84896490749 (requires institutional sign-in at Scopus site) 5 CitationsAbstract
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.
Author List
Shekhtman Y, Kim I, Riviello JJ Jr, Milla SS, Weiner HLAuthor
Irene Kim MD Assistant Professor in the Neurosurgery department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
ChildFemale
Humans
Meningeal Neoplasms
Port-Wine Stain
Sturge-Weber Syndrome
