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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Hypothalamic hamartoma HH is a benign indolent lesion despite the presentation of refractory epilepsy. Behavioral disturbances and endocrine problems are additional critical symptoms that arise along with HHs. Due to its nature of generating epileptiform discharge and spreading to cortical region, various management strategies have been proposed and combined.

Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery hzmartome gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. Endoscopic disconnection has been one of the most favorable options performed in treating HHs.

Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

Compulsive bursts of laughter are a typical and classical feature of GS 13 Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages. Other types of seizure, cognitive deterioration, and behavioral problems follow and frequently develop late in the first decade of life 4 It has been well known that epileptic discharge originates from the hamartoma itself and propagates to subcortical and cortical areas.

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 Recent electroencephalography-functional magnetic resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated.

In contrast, the cunei, bilateral thalami, caudate nuclei, hippocampi, paracentral gyri, and the default mode network precunei and inferior lateral parietal lobules are deactivated This activating network results in epileptic discharges through the premotor and frontal opercular areas voluntary systemwhich are located on the same side of the broader attachment This system conveys discharges through the motor cortex and pyramidal tract to the ventral brainstem 11 Intralesional inside HHs recordings have revealed that the internal epileptiform discharges mix with low-voltage activities 152231 Most epileptiform discharges arise from the broad attachment side 31 The presence of these neurons constitute relevant evidence for ictogenesis of HH 3918 Platinum microwire intraoperative recording of single neuron activity captured hamartomd spikes per second in over HH neurons from 14 patients.

Fast-firing neurons are mostly in synchronous pairs with synchronous firing Electric recording through depth electrode and transendoscopic intraoperative recording are also capable of recording the epileptic activities of HHs 21 Therefore, concomitant scalp EEG and depth electrode recording inside HHs may be very useful in disconnecting this lesion from intractable epilepsy.

The depth electrode will guide the disconnection surgery as well as confirm the successful disconnection 28 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.

Epilepsy, behavioral, and hormonal problems originate from the sessile type hyppothalamique precocious puberty are mostly derived from the pedunculated type 13437 Fig. Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. The HHs are divided into four categories based on MR imaging findings, demonstrating the relationship between the hamartoma and hypothalamus or the third hypothalamiqke.

Translation of “hypothalamus hamartome” in English

In earlier studies, the seizure outcomes were found to be variable with considerable complications 72026 However, surgical resection for total removal of Hypothalamiqe have led to good seizure outcomes and behavioral improvement 10 More favorable outcomes were guaranteed mostly from near total or total resection Alternatively, endoscopic surgery, gamma knife surgery, and stereotactic radiofrequency thermo-coagulation have been introduced and presented good outcomes with fewer complications.


Recently stereotactic laser ablation has been introduced and good seizure control has been achieved. Modern imaging techniques and subsequent state of the art technology in surgical image guidance systems are critical to performing this delicate job Since the hypothalamic area is critical itself, even though we must achieve a good seizure outcome, we should avoid critical complications.

An optimal surgical approach should be selected based on the individual status of the lesion and the patient Fig. Presurgical evaluation is very much the same as a phase 1 study for a medically intractable epilepsy patient. Routine scalp EEG monitoring and long-term video-EEG monitoring, interictal and ictal 99 m-Tc hexamethylprophyleneamine oxime single photon emission computed tomography SPECTmagnetic resonance imaging MRIneuropsychological evaluation, ophthalmological assessments with perimetry, and endocrinological investigations are included.

Endocrine assessment would include sex hormone and growth hormone levels, thyroid function, prolactin level, and cortisol reserve with glucagon stimulation. Depth electrode insertion into the HH with EEG monitoring would provide the margin and depth of disconnection. Surgical indications are intractable GS, neurobehavioral deterioration, and the absence of a cortical lesion on MRI. Memory, endocrine function, behavior, and vision are potentially at risk after surgery 782327 Yamartome rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2.

A monopolar-coagulating hamartoome or an Nd-Yag laser system, a fiberoptic light guide, an Xe light source, and an endovision system are other supporting instruments. An endoscopic procedure is performed under the general endotracheal anesthesia. Because most of the hamartkme have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure. A peel-away catheter is then replaced via the tract. The hypothqlamique with the sheath is then advanced into the lateral ventricle through the peel-away catheter with irrigation of isotonic solution.

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. The confines of the protruding hamartoma and the normal hypothalamus can be easily determined by direct visualization; however, for difficult cases, the depth electrode is inserted prior to endoscopic surgery to guide the depth and the margin of disconnection.

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. The direction will be posterior to anterior and lateral to inferior midline Fig. Neuronavigation and the depth electrode will guide the depth of disconnection. The fiberoptic laser or monopolar coagulator is used through the working channel of the sheath.

Under direct visualization, the hamartoma can be coagulated by a monopolar electric coagulator or removed by microforceps Fig. When the thin pial membrane or arachnoid membrane is reached, dissection is complete. 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 7 hypothalamiqje, 8. Endoscopic approach to hypothalamic hamartoma HHs.

The direction of dissection around the lateral border of HHs.

Relatively small ventricle and foramen of Monro. Discrete appearance of HHs to show the interface between HHs and the hypothalamus. The first step of disconnection with monopolar coagulator along the lateral border of HHs. Disconnection of HHs hjpothalamique the hypothalamus with a monopolar coagulator. Their classification was based on either pedunculated or sessile sagittal orientation and the treatments included open surgical removal or medicinal intervention.

Furthermore, they did not suggest any treatment strategy for Type 2 sessile HHs. However, it did reveal a very important feature that sessile-type HHs usually cause GSs 1 Their report concluded that, as hemispherectomy gave way to hemispherotomy because of serious complications, the complete removal of HHs for controlling seizures was not required anymore.


As the nearby vicinity of HHs consists of very critical structures, the utmost effort should be paid to reduce collateral damage. Thus, the authors performed an endoscopic disconnection only for the intraventricular type of hamartoma Type III.

Since Choi et al. These classifications have been accomplished according to the symptomatology and the feasibility of treatment modalities Fig. There should be minimal working space within the third ventricle for forceps and coagulator, as well as definite interface ahmartome on the neuronavigational image or direct visualization. The gamartome should not be under the optic tract. However, most intraventricular HHs are excellent candidates for endoscopic resection or disconnection 823 An immediate seizure-free condition was the single most reliable factor for long-term seizure outcome.

The rate of resection was not correlated to the seizure-free condition Consequently, complete disconnection is considered to be more important than resection.

Most small HHs less than 15 mm in diameter can be effectively managed by endoscopic disconnection 27 Because small HHs are often attached unilaterally, unilateral disconnection will be enough. But even though HHs attach to the bilateral hypothalamus, unilateral disconnection is usually enough.

Endoscopic Treatment of Hypothalamic Hamartomas

In bilateral attachment and giant HH cases, preoperative electrophysiological evaluation mostly revealed unilateral propagation of epileptiform discharge 31 As the authors describe hypothalmaiqueeven giant HHs larger than 25 mm can be hjpothalamique disconnected under guidance of a depth electrode and frameless neuronavigation system. After confirmation of the extent of disconnection and seizure outcome, a second disconnection can be performed with fewer complications.

Repeat endoscopic disconnection can effectively rescue the recurrence of seizure. Patients with most unilateral attachment, intraventricular type, and small HHs less than 1. Slit-like lateral ventricle and giant HHs within a 6-mm space of the third ventricle are no longer limitations in endoscopic disconnection 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.

Neuronavigational neuroendoscope with a high definition visualization system will never miss the perfect entry point, trajectory, and entry for the small ventricle Complete removal of larger or hypothaalmique HHs is very difficult or nearly impossible with acceptable morbidity. However, disconnection from the brain for controlling intractable epilepsy would be always feasible regardless of HH size. Transient short-term memory loss, weight gain, thalamic infarction with memory loss, and hemiparesis have been reported as early complications.

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. This syndrome disappeared spontaneously within 10 days 8.

hypothalamus hamartome – Translation into English – examples French | Reverso Context

Acceptable morbidity has made multiple-staged operations possible 2431 Hypothxlamique proper trajectory directly to HHs and keeping the disconnection margin straight are essential instructions for minimizing complications. An anterior wiping motion of the endoscope could result in critical damage to the fornix, optic apparatus, or hypothalamus. 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.

Because these patients are mostly clinically and socially impaired, pursuing resection or disconnection would be more beneficial for these patients The endoscopic disconnection may not always be possible for every HH.

However, there are known factors to achieving good outcomes with minimal complications 10 ,