• June 19, 2019

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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Endoscopic approach to hypothalamic hamartoma HHs. This syndrome disappeared spontaneously within 10 days 8. Regis cB.

Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Le syndrome hamartome hypothalamique: The mass should not be under the optic tract.

Translation of “hypothalamus hamartome” in English

Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. Other types of seizure, cognitive hypothaoamique, and behavioral problems follow and frequently develop late in the first decade of life 4 The fiberoptic laser or monopolar coagulator is used through the working channel of the sheath. Transendoscopic intraoperative recording of gelastic seizures from a hypothalamic hamartoma.

It has been well known that epileptic discharge originates from the hamartoma itself and propagates to subcortical and cortical areas. Neuronavigation and the depth electrode will guide the depth of disconnection. Neurol Med Chir Tokyo ; Access to the PDF text. Advances and Technical Standards in Neurosurgery.

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Endoscopic Treatment of Hypothalamic Hamartomas

CT, MR, and pathologic findings. Epilepsy, behavioral, and hormonal problems originate from the sessile type and precocious puberty are mostly derived from the pedunculated type 13437 Fig. Therefore, concomitant scalp EEG and depth electrode recording inside HHs may be very useful in disconnecting this lesion from intractable epilepsy.


Contact Help Who are we? Depth electrode insertion into the HH with EEG monitoring would provide the margin and depth of disconnection. These classifications have been accomplished according to the symptomatology and the feasibility of treatment modalities Fig. The rate of resection was not correlated to the seizure-free condition You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.

When the telescope has been advanced through the foramen of Monro, the HH protruding from the floor and the lateral wall of the third ventricle is usually seen Fig.

Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications. Based on their shape and relationship to the hypothalamus, there are several classifications of HHs and the symptoms and severity depend on their dimensions e.

Because most of the patients have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure. Role of the hypothalamic hamartoma in the genesis of gelastic fits a video-stereo-EEG study Electroencephalogr Clin Neurophysiol.

Repeat endoscopic disconnection can effectively rescue the recurrence of seizure. Recent electroencephalography-functional magnetic resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated.

As the nearby vicinity of HHs consists of very critical structures, the utmost effort should be paid to reduce collateral damage. If you want to subscribe to this journal, hypothalamiwue our rates You can purchase this item in Pay Per View: High frequency stimulation of the mamillothalamic tract for the treatment of resistant hypothalamiqye associated with hypothalamic hamartoma.

Careful planning and a stepwise approach with the advanced surgical technique will make it possible 24 Hypothalamic hamartoma and hypothalmaique Fast-firing neurons are mostly in synchronous pairs with synchronous firing However, disconnection from the brain for controlling intractable epilepsy hypothalaique be always feasible regardless of HH size. Hypothalamic hamartoma HH is a benign indolent lesion despite the presentation of refractory epilepsy.


Memory, endocrine function, behavior, and vision are potentially at risk after surgery 782327 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. Surgical outcomes Valdueza et al.

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. 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. Discrete appearance of HHs to show the interface between HHs and the hypothalamus. This rare non-neoplastic abnormal mixture of neuronal and glial tissue of the inferior hypothalamus has been postulated to be derived from the mammillo-thalamo-cingulate tract from which HH is networking other brain areas associated with GS or the pathway from the HH to the brainstem and cerebellum 16 As the authors describe 31even giant HHs larger than 25 mm can be efficiently disconnected under guidance of a depth electrode and frameless neuronavigation system.

Endoscopic disconnection for hypothalamic hamartoma with intractable seizure. Because small HHs are often attached unilaterally, unilateral disconnection will be enough. The depth electrode will guide the disconnection surgery as well as confirm the successful disconnection 28 ,