Hypothalamic hamartoma

Abstract

Hypothalamic hamartomas (HH) are developmental malformations growing within the hypothalamus and the third ventricle with patterns similar to benign tumors. HH are often associated with mild to severe drug-refractory epilepsy. The spectrum of the epileptic disorders induced by HH is very wide, but the natural history of disease is often characterized by severe seizures leading to mental retardation and possibly death in severely affected infants. Stereotactic Radiosurgery (SRS) provides excellent seizure outcomes without the risks of devastating neurological complications inherently associated with microsurgical resection. Early radiosurgical intervention leading to seizure freedom and excellent long-term psychosocial outcomes has been recently described. We summarize evidence regarding the role of SRS for HH and the appropriate timing for treatment delivery even though further studies are expected.