Giant Hypothalamic Hamartoma in an Infant: A Case Report and Review of the Literature

Abstract

Giant hypothalamic hamartomas (GHH) are extremely rare lesions in infants and usually intrinsically epileptogenic. We present the case of a 10-month-old girl child presenting with drug-resistant seizures and a giant hypothalamic lesion that was confirmed as hamartoma on histopathology. Surgical decompression and disconnection from the hypothalamus was performed with the intent of controlling her seizures. Unfortunately, the patient developed right middle cerebral artery and posterior cerebral artery territory infarction, possibly due to vasospasm or thrombosis of the vessels. The patient had a stormy postoperative course but has recovered well neurologically at the 18-month follow-up. Histopathological examination revealed abnormal clusters of NeuN-positive neurons, which was confirmatory of hypothalamic hamartoma. A review of the published literature on infantile GHH, its management and the postoperative complications is undertaken in this short report.

One-Stage High-Density Focal Stereo-Array SEEG-Guided Radiofrequency Thermocoagulation for the Treatment of Pediatric Giant Hypothalamic Hamartomas

“…The estimated incidence of HH is one per 100,000–1,000,000 ( 6 ). Giant HHs are even more rare, and to the best of our knowledge, only sporadic cases have been reported in childhood ( 5 , 7 – 12 ). In contrast to the previously accepted hypothesis that epileptogenesis is strictly a cortical phenomenon, recent depth-electrode recordings suggest that intrinsic subcortical epileptogenesis may arise within the hamartoma, and then spread to the surrounding cortex, including the frontal and temporal lobes, giving rise to secondary epileptogenic foci ( 13 ).…”