A Simple Technique for Endoscopic Hypothalamic Hamartoma Disconnection in Refractory Epilepsy: 2-Dimensional...

Hypothalamic hamartomas often cause refractory epilepsy, best controlled with surgery. A transcallosal interforniceal approach provides good outcomes although it has resulted in some complications including fornix lesions with transitory and permanent memory losses.1-2 Endoscopic disconnection is less invasive, avoids interhemispheric approach, callosotomy and fornix as well as propagation of epileptic discharges.3,4 Laser disconnection does not allow objective measurement of the vaporized tissue and can cause significant hypothalamic edema. We demonstrate a simple hypothalamic disconnection endoscopy technique using the VarioGuide frameless stereotactic system (Brainlab, Germany), bipolar coagulation electrode (Karl Storz, Germany), and 3-French Fogarty catheter (Edwards Lifesciences, USA).  We present a 12-yr-old right-handed girl who developed normally until 4 yr old when gelastic seizures began. Antiepileptic drugs and gamma knife radiosurgery did not control the seizures. Magnetic resonance imaging showed a nodular 13 mm left hypothalamic mass protruding into the third ventricle. The patient consented to surgery.  We used a rigid 30° 6-mm neuroendoscope (Aesculap, Germany) with VarioGuide to reach the ventricle. After visualizing the foramen of Monro, the bipolar coagulation electrode perforated holes along the hamartoma/hypothalamus interface. The Fogarty balloon catheter was inserted into each hole and gently inflated to connect the holes and disconnect the mass from the hypothalamus.  The patient's postsurgical recovery was excellent, presenting intact short- and long-term memory without neurological deficit or endocrine complications. She continues seizure-free 2 yr after surgery. We have successfully used this technique in 4 more cases and, if results continue to be good, will consider it a useful tool for these infrequent malformations.