Hypothalamic Hamartoma: Pathophysiology and Treatment(<SPECIAL ISSUE>New Therapies in Pediatric Neurosurgery)

Hypothalamic hamartomas (HHs) are classified into two types, parahypothalamic type and intrahypothalamic type, by the degree of hypothalamic involvement as seen on MRI. This categorization has contributed to the accurate prediction of their prognosis and to improved treatment strategies. HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, the total eradication or disconnection of the lesion leads to the cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Recent reports on stereotactic radiofrequency lesioning and the transcallosal interfornicial approach showed promising results for seizure control. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life.