Hypothalamic hamartoma: the role of surgery in a series of eight patients

Mottolèse, C.; Stan, Horaţiu; Bret, P; Berlier, P.; Lapras, C

doi:10.1007/s003810000361

Cited by 43 publications

(21 citation statements)

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“…13) The first manifestation of CPP in patients with HH occurs before age 2 years in 82% of cases 13) and occasionally before their 1st birthday. 2,14,47,69,76) Rapid progression of pubertal signs and bone age advancement are additional characteristics of CPP with HH. 13,44) IV.…”

Section: Cppmentioning

confidence: 99%

“…However, surgical results for CPP have markedly improved 2,11,42,47) and postoperative complete disappearance without complications of symptoms and signs of CPP has been reported in a total of 19 patients. 2,11,42,47) Theoretically, surgery for HH presenting with only CPP is less risky than surgery for HH with seizures, because the lesions in the former instance do not seriously involve the hypothalamus. 4,11,72) Simple transection of the stalk in peduncular HHs may cure the endocrine dysfunction without serious risks.…”

Section: K Arita Et Almentioning

confidence: 99%

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Hypothalamic Hamartoma

Arita

Kurisu

Kiura

et al. 2005

Neurol. Med. Chir.(Tokyo)

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The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have yielded important data. Categorizing HHs by the degree of hypothalamic involvement has contributed to the accurate prediction of their prognosis and to improved treatment strategies. Rather than undergoing corticectomy, HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, total eradication or disconnection of the lesion leads to cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life. This review of hypothalamic hamartomas presents current classifications, pathophysiologies, and treatment modalities.

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Section: Cppmentioning

confidence: 99%

Section: K Arita Et Almentioning

confidence: 99%

Hypothalamic Hamartoma

Arita

Kurisu

Kiura

et al. 2005

Neurol. Med. Chir.(Tokyo)

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“…14,15,20,23) Most previous studies have included single cases or series, some of precocious puberty only, others with epilepsy only or both. 10,12) We recently reported disconnection of hamartoma of the fourth ventricle which resulted in total relief of seizures in two patients. 6) In the present study, we performed disconnection of the hamartoma using either open cranial surgery or endoscopic surgery guided with a frameless stereotactic robot in a series of 17 children and adolescents referred for intractable epilepsy due to hypothalamic hamartoma, and propose a classification for hamartoma to aid in the selection of the surgical treatment.…”

Section: Introductionmentioning

confidence: 99%

Disconnecting Surgical Treatment of Hypothalamic Hamartoma in Children and Adults With Refractory Epilepsy and Proposal of a New Classification.

Delalande

Fohlen

2003

Neurol. Med. Chir.(Tokyo)

250

175

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Hypothalamic hamartoma associated with intractable epilepsy is a major problem when surgical management is discussed. Professor Olivier Delalande is Chief Neurosurgeon of the Department of Pediatric Neurosurgery, Fondation Ophtalmologique Adolf de Rothschild, in Paris, France. In recent international symposia, he has reported a series of hypothalamic hamartoma in children and adults with refractory epilepsy, managed with various surgical techniques according to his new classification of hypothalamic hamartomas. With the permission of Professor Akira Yamaura, Chief Editor of the Neurologia medico-chirurgica, this stimulating paper has appeared in this issue. The strategies of his surgery using surgical excision, surgical disconnection or endoscopic disconnection are challenging but safe. His surgical outcomes are that 8 patients are seizure free, one patient has only brief gelastic seizures and 8 patients are dramatically improved. I hope that the authors will have the opportunity to again examine the outcomes in their series several years from now.Tatsuya TANAKA, M.D., D.M.Sc., President of the 30th Annual Meeting of the Japan Society for Pediatric Neurosurgery. AbstractA series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 5...

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“…These suboptimal results created a need to develop alternate surgical strategies to improve seizure freedom rates while limiting surgery-related morbidity. 35,42 Nevertheless, a pterional approach seems best for targeting pedunculated HHs that cause CPP. Even a Complications of pterional approach.…”

mentioning

confidence: 99%

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

Mittal

Montes

et al. 2013

FOC

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Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.

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Hypothalamic hamartoma: the role of surgery in a series of eight patients

Cited by 43 publications

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Hypothalamic Hamartoma

Hypothalamic Hamartoma

Disconnecting Surgical Treatment of Hypothalamic Hamartoma in Children and Adults With Refractory Epilepsy and Proposal of a New Classification.

Hypothalamic hamartomas. Part 2. Surgical considerations and outcome

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