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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Orphanet: Syndrome congenital d hamartome hypothalamique

However, most intraventricular HHs are excellent candidates for endoscopic resection or disconnection 823 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.

Because breaking down the tract for spreading epileptiform discharge from HHs to the frontotemporal cortical region will be the optimal solution for this lesion, further aggressive maneuvers may not be necessary 19 The telescope with the sheath is then advanced into the lateral ventricle through the peel-away catheter with irrigation of isotonic solution.

Support Center Support Center. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, hamaetome, updated or deleted. This syndrome disappeared spontaneously within 10 days 8.

Endoscopic disconnection for hypothalamic hamartoma with intractable seizure. Therefore, one stage operation or single modality of treatment will lose their effectiveness in managing HHs The HHs are divided into four categories based on MR imaging findings, demonstrating the relationship between the hamartoma and hypothalamus or the third ventricle.

Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction. However, there are known factors to achieving good outcomes with minimal complications 10 Neurol Med Chir Tokyo ; Laguitton aM. Access to the text HTML. Advances and Technical Standards in Neurosurgery.


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For severely affected patients, relieving intractable epilepsy would outweigh these complications and clinically significant damage to a mammillary hamartomr or fornix would not be evident. National Center for Biotechnology InformationU. Furthermore, they did not suggest any treatment strategy for Type 2 sessile HHs.

Le syndrome hamartome hypothalamique: Top of the page – Article Outline. The fiberoptic laser or monopolar coagulator is used through the working channel of hamartomme sheath.

An optimal surgical approach should be selected based on the individual status of the lesion and the patient Fig.

Surgical indications are intractable GS, neurobehavioral deterioration, and the absence of a cortical lesion on MRI. Transendoscopic intraoperative recording of gelastic seizures from a hypothalamic hamartoma. 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 hajartome investigations are included.

A proper trajectory directly to HHs and keeping the disconnection margin straight are essential instructions for minimizing complications. Role of the hypothalamic hamartoma in the genesis of gelastic fits a video-stereo-EEG study Electroencephalogr Clin Neurophysiol.

Endoscopic Treatment of Hypothalamic Hamartomas

Hypothalamic hamartomas associated with hypothalzmique Ouhabi aA. EEG and video-EEG seizure monitoring has limited utility in patients with hypothalamic hamartoma and epilepsy. 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.

Abilkassem bA. In earlier studies, the seizure outcomes were found to be variable with considerable complications 72026 Neuronavigation and the depth electrode will guide hamarrtome depth of disconnection.

Epileptic network of hypothalamic hamartoma: Alternatively, endoscopic surgery, gamma knife surgery, and stereotactic radiofrequency thermo-coagulation have been introduced and presented good outcomes with fewer complications. As per the Law relating to information storage and personal integrity, you have the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data.


High frequency stimulation of the mamillothalamic tract for the treatment of resistant seizures associated with hypothalamic hamartoma. Slit-like lateral ventricle and giant HHs within a 6-mm space of the third ventricle are no longer limitations in endoscopic disconnection Intralesional inside HHs recordings have revealed that the internal epileptiform discharges mix with low-voltage activities 152231 Repeat surgery for hypothalamic hamartoma in refractory epilepsy.

Consequently, complete disconnection is considered to be more important than resection. Access to the full text of this article requires a subscription. Modern imaging techniques and subsequent state of the art technology in surgical image guidance systems are critical to performing this delicate job After confirmation of the extent of disconnection and seizure outcome, a second disconnection can be performed with fewer complications.

Firing behavior and network activity of single neurons in human epileptic hypothalamic hamartoma.

Hamartomas of the tuber cinereum: Complications Transient short-term memory loss, weight gain, thalamic infarction with memory loss, and hemiparesis have been reported as early complications.

From hypothalamic hamartoma to cortex: The rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2.

Personal information regarding our website’s visitors, including their identity, is confidential. CT, MR, and pathologic findings. The histopathology of hypothalamic hamartomas: