Hypothalamic Hamartomas: A Comprehensive Review of Literature – Part 2: Medical and Surgical Management Update

Abstract Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated incidence ranges from 1 in 50,000 to 1 in 1,000,000. Hypothalamic hamartomas are associated with different clinical presentations including various types of seizures, most characteristically; the gelastic seizures, precocious puberty, cognitive impairment and behavioral changes. In this review, the authors discuss the recent advancements in the medical and surgical management of hypothalamic hamartoma that have been achieved over the past few decades. This review also discusses the advantages and disadvantages of each surgical line of management and factors determining the best individualized approach.