Complete and curative excision of hypothalamic hamartomas via the orbito-zygomatic approach

摘要

Sir, Hypothalamic hamartomas (HH) are congenital non-neoplastic lesions arising from the tuber cinereum, rnamrnillary bodies or the floor of the third ventricle with reported incidence of 1-2/million. They may present with intractable seizures (gelastic), mental retardation or precocious puberty.[1] We report two patients of HH managed by excision through a skull base approach.Case 1: This was a first case of a 3-year-old girl brought with the complaints of episodes of uncontrollable laughter since early childhood. She experienced multiple seizures daily despite being on two anti-epileptic drugs. Developmental milestones and neuropsychological assessment were age-appropriate. Magnetic resonance imaging (MRI) revealed a 1.75 cm x 1.5 cm sessile non-enhancing hamartoma attached to the floor of the third ventricle, just ventral to the left mammillary body [Figure la-d]. Her hormonal profile was in the normal range. Electroencephalography showed frequent random runs and bursts of theta and delta activity, predominantly in the central leads with intermittent generalization of the slowing. She underwent left frontotemporal craniotomy with an orbito-zygomatic osteotomy (FTOZ) and a transsylvian approach using the carotid-oculomotor triangle as the approach corridor. The hamartoma appeared nearly like normal brain tissue and was shaved off parallel to the floor of the III ventricle. Post-operatively she developed transient occulomotor nerve paresis, right hemiparesis and transient diabetes insipidus, all of which resolved within a month. She is seizure free 1 year after surgery and is off all medications. Post-operative MRI brain revealed total excision of the hamartoma with no residue [Figure 2a and b].