Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy

Abstract

Introduction

There is increasing evidence that removal or disconnection of hypothalamic hamartomas can lead to seizure control and improvement in behavior. The purpose of this study is to report our experiences with endoscopic removal of these hamartomas.

Methods

This is a prospective review of the early outcome of 44 patients undergoing endoscopic resection utilizing frameless stereotaxis and a micromanipulator.

Results

All patients had normal-sized ventricles. Complete removal was possible in 14 patients, 13 of whom were seizure free at follow-up. Four patients suffered complications that have persisted for more than 3 months: One had significant hemiparesis and three had persistent difficulties with short-term memory. Postoperatively, no patient suffered a permanent hormonal deficiency. In one patient who had undergone a previous attempt at transcallosal resection, the procedure was abandoned because of the lack of internal anatomic landmarks. In a second patient, insufficient mass could be removed due to the size of the lesion and the distorted anatomy. Consequently, 1 week later, the patient underwent a transcallosal approach.

Conclusion

In appropriately selected patients, sessile hypothalamic hamartomas can be removed completely or they can be completely disconnected from the hypothalamus with an acceptable risk. At least a year after surgery is needed to evaluate the efficacy of the procedure in terms of control of seizures and improvements in behavior and emotional ability.