Surgical outcome of vagus nerve stimulation therapy for patients with Sturge-Weber syndrome
Abstract number :
1.343
Submission category :
9. Surgery / 9C. All Ages
Year :
2017
Submission ID :
344655
Source :
www.aesnet.org
Presentation date :
12/2/2017 5:02:24 PM
Published date :
Nov 20, 2017, 11:02 AM
Authors :
Takuma Higo, Juntendo University; Hidenori Sugano, Juntendo University; Madoka Nakajima, Juntendo University; Hiroharu Suzuki, Juntendo University; and Takumi Mitsuhashi, Juntendo University
Rationale: Patients with Sturge-Weber syndrome (SWS) are diagnosed by facial port-wine nivus, glaucoma, and cerebral leptomeningeal angioma. Seizure and stroke-like episode develops in infant which causes psychomotor deterioration and neurological disability. The treatment with anti-epileptic drugs (AEDs) is started in early life, though about a half of cases shows pharmaco-resistant. When the patients fail in seizure control by neither AEDs therapy nor surgical treatment, vagus nerve stimulation (VNS) is expected as an alternative therapy for them. However, the VNS effect for SWS has not been clarified yet. In this paper, we reported surgical outcome of VNS for patients with SWS. Methods: Seven patients with SWS who underwent VNS in our institute were enrolled in this study. Four patients had previously experienced surgery. Surgical outcome was evaluated by McHugh’s classification and compared them between during three months before and latest three months after VNS. We regarded class I and II as a good outcome and class III to V as a poor outcome. We also evaluated the clinical factors regarding therapeutic outcome as followings; 1. Frequency of focal impaired-awareness seizure (FIAS), 2. Frequency of tonic clonic seizure (TCS), 3. Distribution of leptomeningeal angioma and brain atrophy, 4. Number of AEDs, 5. Previous epilepsy surgery. Results: The median seizure duration was 56 months, and mean observation period was 29 months. Three patients resulted in class IA, one patient in class IIA, and three patients in class IIIA. Frequencies of FIAS and TCS were not related to surgical outcome. Patients with wider leptomeningeal angioma and brain atrophy were tended to result as poor outcome. Patients who had more than four AEDs showed poor outcome. Previous failed surgery resulted a little influence on outcome. Conclusions: VNS showed some efficacy for seizure control of SWS. However, patients with obvious brain atrophy, wider leptomeningeal angioma, and taking many AEDs tended to result in poor outcome. Funding: No funding was received.
Surgery