Treatment of Hypothalamic Hamartoma

Kim, Dong Seok; Park, Yong Gou; Choi, Joong Uhn; Chung, Sang Sup

Abstract

This study presents seven patients with hypothalamic hamartomas diagnosed on the basis of MRI. Histological confirmation was performed in one patient who underwent surgery. Four patients presented with epilepsy, including gelastic seizures. Other symptoms were behavior abnormalities in 3 patients and precocious puberty in 4 patients. We classify hypothalamic hamartomas into four subgroups according to MRI finding: Types la lesions were less than 100mm in lesions less than 10mm and mammillary body. Type lla lesions were more than 100mm in diamenter and sessilely attached to hypothalamus with slight hypothalmic displacement and Type llb lesions more than 10mm and with marked displacement of hypothalamus. We could achieve good result with surgical resection in one patient with Type llb hamartoma associated with gelastic seizure that was unresponsive to medical treatment. We performed gamma knife radiosurgery in three patients with gelastic seizue(2 patients with Type lla and 1 patient with Type llb) and three patients with precocious puberty(2 patients with Type la and 1 patient with Type lla 1 patient with Type llb) and three patients with precocious puberty(2 patients with Type la and 1 patient with type lb). The authors propose direct surgery as a treatment for this progressive syndrome and gamma knife radiourgery is alternative treatment for high risk patients.