Hypothalamic hamartoma associated with intractable epilepsy is a major problem when surgical management is discussed. Professor Olivier Delalande is Chief Neurosurgeon of the Department of Pediatric Neurosurgery, Fondation Ophtalmologique Adolf de Rothschild, in Paris, France. In recent international symposia, he has reported a series of hypothalamic hamartoma in children and adults with refractory epilepsy, managed with various surgical techniques according to his new classification of hypothalamic hamartomas. With the permission of Professor Akira Yamaura, Chief Editor of the Neurologia medico-chirurgica, this stimulating paper has appeared in this issue. The strategies of his surgery using surgical excision, surgical disconnection or endoscopic disconnection are challenging but safe. His surgical outcomes are that 8 patients are seizure free, one patient has only brief gelastic seizures and 8 patients are dramatically improved. I hope that the authors will have the opportunity to again examine the outcomes in their series several years from now.Tatsuya TANAKA, M.D., D.M.Sc., President of the 30th Annual Meeting of the Japan Society for Pediatric Neurosurgery.
AbstractA series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 5...