Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: experience in Mexico.

OBJECT: Hypothalamic hamartoma is a nonneoplastic malformative mass of neurons and glia in the region of the hypothalamus. Because of its location, open surgery is associated with high morbidity and mortality rates. Gamma knife surgery (GKS) may be an efficient and safe treatment approach, which produces little morbidity. The authors describe the results of GKS in three patients with hypothalamic hamartomas. METHODS: All patients were male, aged 3, 12, and 15 years. The lesions were classified according to the Valdueza scale: one was Type IIb and two were Type IIa. The patients presented with gelastic seizures (15-20 per day), generalized epilepsy, behavioral abnormalities, and alterations of the sleep cycle. Precocious puberty was present in one patient. The Type IIb tumor had a volume of 1.8 cm3, and the Type IIa tumors were 597 mm3 and 530.1 mm3. The lesions received 12.5 Gy, 14 Gy, and 15 Gy, respectively, to the 50% isodose line. The patients were followed for 30 to 50 months. After 3 months, all patients showed improvement of their sleep, behavior, and epilepsy. At the present time, these patients are receiving low-dose antiepileptic agents and have achieved adequate social development and school integration. CONCLUSIONS: Gamma knife surgery appears to be a good, safe, and effective option for the treatment of selected hypothalamic hamartomas. No morbidity or mortality was associated with these three cases.

Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery.

OBJECT: The goal of this study was to examine the role of gamma knife radiosurgery (GKS) and adjuvant neuroendoscopic procedures by assessing the outcome in 10 patients with craniopharyngiomas who were treated between 1998 and 2001. METHODS: In 10 consecutive patients (six males and four females, mean age 12 years [range 7-32 years]), there were nine mixed and one solid craniopharyngioma. In the last three cases, neuroendoscopic procedures were conducted. Two cases required insertion of an Ommaya reservoir. In another case partial removal of the cyst wall was performed using an endoscope to create a communication with the cerebrospinal pathways and thereby preclude the need for shunt insertion. CONCLUSIONS: Multimodal management of craniopharyngiomas seems to provide a better rate of survival and greater long-term disease control. It is suggested that GKS combined with adjuvant neuroendoscopy should be used as an alternative in treating recurrent or residual craniopharyngiomas if additional microsurgical removal cannot guarantee a cure.