TREATMENT OPTIONS FOR HYPOTHALAMIC HAMARTOMAS—NO LAUGHING MATTER


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Abstract

Surgical Management of Hypothalamic Hamartomas with Epilepsy: The Stereoendoscopic Approach.Procaccini E, Dorfmuller G, Fohlen M, Bulteau C, Delalande O. Neurosurgery 2006 Oct;59(4 Suppl 2):ONS336–ONS346.OBJECTIVEHypothalamic hamartomas (HHs) require surgical treatment in patients presenting with refractory epilepsy.METHODSThe authors report on a single-center series of 33 patients (24 males, 9 females) who underwent surgery between January 1997 and April 2004. They experienced several types of seizure (gelastic, tonic, partial, atonic, generalized tonic-clonic, dacrystic, infantile spasm, mental retardation, and behavioral and endocrinological abnormalities). Forty-nine interventions were carried out. Every patient, with the exception of the first, underwent hamartoma disconnection (pterional approach, six patients; endoscopy, 15 patients; both, 11 patients). The endoscopic approach was carried out with a frameless stereotactic system to enhance feasibility and efficacy of the disconnecting procedure.RESULTSSurgery-related neurological complications occurred in two patients, both after a pterional microsurgical approach. Furthermore, two patients experienced panhypopituitarism and one patient experienced transitory central insipid diabetes. All patients but one showed recovery or considerable improvement of their epilepsy (Engel Class 1, 48.5%; Engel Class 2, 3%; Engel Class 3, 45.5%; mean follow-up duration, 1 year 7 months).CONCLUSIONSAccording to the proposed classification of sessile HH into four types, the best candidates for endoscopic disconnection are Type 2 and Type 3 HHs. In the present series, 90% of patients affected by Type 2 HH became seizure-free and the remaining 10% improved; of those with Type 3 HH at presentation, 35.3% recovered and 60% improved. Neuropsychological and endocrinological test results showed improvement in many patients. Data from our series demonstrate that frameless stereotactic endoscopic disconnection should be considered as the treatment of choice in the presence of favorable anatomic conditions.

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