Surgical treatment of epilepsy in Sturge–Weber syndrome in children

Abstract

Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.

Surgery for infants with catastrophic epilepsy: an analysis of complications and efficacy

“…The patient in this series was chosen for operation under the age of 1 year due to the intractability of her seizure disorder to the point of having up to 20 seizures a day despite numerous anti-epileptic medications. Previous studies have shown excellent seizure control rates with hemispherectomy for patients with SturgeWeber syndrome [36,37]. High intensity of seizure activity has been correlated with deteriorating mental status [38] while early surgery has been associated with better developmental outcomes [36]; all of which was considered when deciding to bring this particular patient to surgery.…”

Focal Resection of Leptomeningeal Angioma in a Rare Case of Sturge-Weber Syndrome without Facial Nevus

“…Out of the patients who underwent complete seizure focus resection, 71% were found to be seizure free. Bourgeois et al [14] and Arzimanoglou et al [15] both advocate early surgery, with evidence of improvement in developmental outcome as compared to patients with later resection. The degree of seizure control appears to be affected by the extent of resection rather than the timing of surgery.…”

“…Bourgeois et al [14] analyzed 27 children with SWS, and found 100% seizure control with hemispherectomy or functional hemispherotomy, as opposed to 58% of seizure control with focal resection. However, 5 out of the 6 children with treatment failure in the latter group underwent an incomplete resection of the epileptogenic focus, which may account for the persistence of seizures.…”

Hemisphärotomie bei einer 17-jährigen Patientin mit Sturge-Weber-Syndrom

“…Bei 75-90% der Patienten tritt eine Epilepsie auf, die in 60% pharmakoresistent ist [4]. Studien an Kindern berichten von einer Anfallsfreiheit nach fokaler Resektion und Hemisphärotomien in bis zu 58% der Fälle [2]. In einem Kollektiv von 32 Hemisphärektomie-Patienten wird eine Anfallsfreiheitsrate von 81% berichtet [5].…”