Surgical treatment of hypothalamic hamartomas.

Hypothalamic hamartomas are aberrant masses, composed of abnormally distributed neurons and glia. Along endocrine and cognitive symptoms, they may cause epileptic seizures, including the specific gelastic and dacrystic seizures. Surgery is the treatment of drug-resistant hamartoma epilepsy, with associated positive results on endocrine, psychiatric, and cognitive symptoms. Recently, alternatives to open microsurgical treatment have been proposed. We review these techniques and compare their efficacy and safety. Open resection or disconnection of the hamartoma, either through pterional, transcallosal, or transventricular approach, leads to good epileptological control, but its high complication rate, up to 30%, limits its indications. The purely cisternal peduncular forms remain the only indication of open, pterional approach, while other strategies have been developed to overcome the neurological, endocrine, behavioral, or cognitive complications. Laser and radiofrequency thermocoagulation-based disconnection through robot-guided stereo-endoscopy has been proposed as an alternative to open microsurgical resection and stereotactic destruction. The goal is to allow safe and complete disconnection of a possibly complex attachment zone, through a single intraparenchymal trajectory which allows multiple laser or radiofrequency probe trajectory inside the ventricle. The efficacy was high, with 78% of favorable outcome, and the overall complication rate was 8%. It was especially effective in patients with isolated gelastic seizures and pure intraventricular hamartomas. Stereotactic radiosurgery has proved as efficacious and safer than open microsurgery, with around 60% of seizure control and a very low complication rate. Multiple stereotactic thermocoagulation showed very interesting results with 71% of seizure freedom and 2% of permanent complications. Stereotactic laser interstitial thermotherapy (LiTT) seems as effective as open microsurgery (from 76 to 81% of seizure freedom) but causes up to 20% of permanent complications. This technique has however been highly improved by targeting only the epileptogenic onset zone in the hamartoma, as shown on preoperative functional MRI, leading to an improvement of epilepsy control by 45% (92% of seizure freedom) with no postoperative morbidity. All these results suggest that the impact of the surgical procedure does not depend on purely technical matters (laser vs radiofrequency thermocoagulation or stereotactic vs robot-guided stereo-endoscopy) but relies on the understanding of the epileptic network, including inside the hamartoma, the aim being to plan an effective disconnection or lesion of the epileptogenic part while sparing the adjacent functional structures.

[Epilepsy surgery during infancy and early childhood in France]. / Chirurgie de l'épilepsie de l'enfant et du nourrisson en France.

BACKGROUND AND PURPOSE: We present the epilepsy surgery activity in infants and children at the Fondation Rothschild Hospital, the main center dedicated to this activity in France. METHOD: A prospective study was conducted from 2003 to 2007 based on three populations: (1) children selected as candidates for surgery, (2) children undergoing presurgical evaluation and (3) children undergoing surgical procedures for epilepsy. RESULTS: Children selected as candidates for surgery: 304 children were referred and discussed by our multidisciplinary staff. They came from Paris and its suburbs (40%), the provinces (43%) or from other countries (14%). Sixty-one percent of them were included in our surgery program and 24% were excluded. Sixty-one percent of them were under 10 years of age. Children undergoing presurgical evaluation: 296 children were recorded: 140 EEG (47%), 46 with foramen ovale electrodes (16%) and 110 with invasive recording techniques (37%). Seventy percent of these children were under 10 years of age. Children undergoing surgical procedures: 316 children underwent surgery; 68% of them were under 10 years of age. The surgical procedures were focal resection (136 children), vertical parasagittal hemispherotomy (77 children), resection and or disconnection for hypothalamic hamartoma (69 children) and 34 had palliative surgery (callosotomy or vagal nerve stimulation). CONCLUSION: Eighty to 100 children undergo surgery each year in our department for drug-resistant partial epilepsy; 70% of them are less than 10 years of age. This activity is part of a network of pediatric neurologists who are deeply involved in treatment of severe epilepsy in children.