Orphanet: Syndrome congenital d hamartome hypothalamique. Request PDF on ResearchGate | On Jul 1, , J. Mounach and others published Puberté précoce liée à un hamartome hypothalamique. Request PDF on ResearchGate | On May 1, , N. Ech-Cherif El Kettani and others published Hamartome hypothalamique révélant des crises gélastiques.

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Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per Hyopthalamique Careful planning and a stepwise approach with the advanced surgical technique will make it possible 24 Hypothalamic hamartomas associated with epilepsy: Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy.

Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas.

Chabrol abF. Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Complete disconnection between the HH and hypothalamus can be confirmed by observing air density along the disconnection site on postoperative brain CT scans or signal changes on MR images 78.

Instrumentation and procedure for endoscopic surgery The rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2. Furthermore, they did not suggest any treatment strategy for Type 2 sessile HHs. Villeneuve abV. In bilateral attachment and giant HH cases, preoperative electrophysiological evaluation mostly revealed unilateral propagation of epileptiform discharge 31 Consequently, complete disconnection is considered to be more important than resection.


A monopolar-coagulating electrode or an Nd-Yag laser system, a fiberoptic light guide, an Xe light source, and an endovision system are other supporting instruments.

Endoscopic Treatment of Hypothalamic Hamartomas

Discrete appearance of HHs to show the interface between HHs and the hypothalamus. This system conveys discharges through the motor cortex and pyramidal tract to the ventral brainstem 11 There hgpothalamique be minimal working space within the third ventricle for forceps and coagulator, as hypothalamisue as definite interface either on the neuronavigational image or direct visualization.

Le syndrome hamartome hypothalamique: Ouhabi aA. Most small HHs less than 15 mm in diameter can be effectively managed by endoscopic disconnection 27 Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. Because most of the patients have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure.

Therefore, one stage operation or single modality of treatment will lose their effectiveness in managing HHs But even though HHs attach to the bilateral hypothalamus, unilateral disconnection is usually enough. Three of four patients with postoperative disconnection-like syndrome mental dullness, verbal anomia, unilateral tactile anomia, and lack of somesthetic transfer were observed by Choi et al.

Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction.

For severely affected patients, relieving intractable epilepsy would outweigh these complications and clinically significant damage to a mammillary body or fornix would not be evident. Personal information regarding our website’s visitors, including their hamattome, is confidential.


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Orphanet: Syndrome congenital d hamartome hypothalamique

Hypothalamiquue anterior wiping motion of the endoscope could result in critical damage to the fornix, optic apparatus, or hypothalamus. The disconnection will begin at the border between the hamartomas and the mammillary body and proceed along the midline posteroinferior floor of the third ventricle Fig. Thus, the authors performed an endoscopic disconnection only for the intraventricular type of hamartoma Type III.

Open in a separate window. Zerhouni aA.

Clear visualization of a distinct cleft or indentation and the border connecting HH to the hypothalamus will be the critical point for performing staged endoscopic disconnection for giant HHs. Their report concluded that, as hemispherectomy gave way hamartomf hemispherotomy because of serious complications, the complete removal of HHs for controlling seizures was not required anymore.

Neuronavigation and the depth electrode will guide the depth of disconnection. Because these patients are mostly clinically and socially impaired, pursuing resection or disconnection would be more beneficial for these patients