Abstract

Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.

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