Gamma knife surgery for epilepsy related to hypothalamic hamartomas

Abstract

Numerous neurosurgical approaches are available for children presenting with hypothalamic hamartomas (HHs) associated with severe epilepsy. A concern regarding the impairment of short-term memory after resective surgery is promoting the exploration of less invasive alternatives like radiosurgery. Gamma knife radiosurgery (GKS) can lead to a real reversal of the epileptic encephalopathy. Three years after radiosurgery, 60% of the children have an excellent result with complete seizure cessation in 40% and rare nondisabling seizures in 20%, often in association with dramatic behavioral and cognitive improvement. No permanent neurologic complications have thus far been reported. Rare transient cases of poikilothermia have been observed. GKS is clearly the safer approach for these difficult patients. Young patients with severe epilepsy and neurocognitive comorbidity must be treated by using a curative approach as early as possible. Topological type (according to our original classification) is the major feature for selection of the best treatment strategy. Type I HH deeply embedded in the hypothalamus is treated safely and efficiently by GKS. Type II HH can be resected by either endoscopic or transcallosal approaches or treated by GKS depending on the parent's choice and severity of epilepsy. In small type III HH, GKS is the safer procedure because of the very close relationship to the fornix and mammillary bodies. Types V (rarely epileptic) and IV are frequently operable by disconnection. Very large type VI (or mixed type) with a large component above the floor of the third ventricle must be disconnected, and then the upper remnant is best treated by GKS using a staged technique. Overall, when the lesion is sufficiently small, GKS offers a rate of seizure control comparable to microsurgery but with much lower risk. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a reliable evaluation of the role of GKS.