Hemispherectomy in the treatment of pediatric symptomatic epilepsy of children

Melikyan, Armen; Kushel, Yu V; Воробьев, А. Н.; Arkhipova, N. A.; Sorokin, V S; Lemeneva, N.; Савин, И. А.; Pronin, Igor; Козлова, А Б; Grinenko, Olesya; Буклина, С Б; Nagorskaya, I.A.

doi:10.17116/neiro201680313-24

Cited by 11 publications

(3 citation statements)

References 26 publications

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“…On these grounds, hemispherectomy and hemispherotomy are still considered the gold standard in the surgical management of SWS-related epilepsy [4,9,38,50,52,72,73,88]. Data from the literature support this observation, a seizure-free outcome being achieved in 80% of cases in several large series [6,46,55].…”

Section: Surgical Optionsmentioning

confidence: 99%

“…From a review of the pertinent literature, SWS children with refractory epilepsy show a good epilepsy outcome [2,4,9,14,16,17,19,33,35,37,38,41,49,50,62,64,72,73,79,81,83,88,109,112,118,121,124,129,133,137,140], especially if compared with other hemispheric diseases [73,109,124]. Actually, a seizure-free condition is achieved in 79-82% of the cases, independently from the used surgical technique.…”

Section: Late Post-operative Outcomementioning

confidence: 99%

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Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons

et al. 2020

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Purpose Sturge-Weber syndrome (SWS) is a neurocutaneous facomatosis characterized by facial and leptomeningeal angioma, glaucoma, seizures, and neurological disability. Therefore, a challenging multidisciplinary interaction is required for its management. The goal of this paper is to review the main aspects of SWS and to present an illustrative pediatric series. Methods The pertinent literature has been analyzed, focused mainly on etiopathogenesis, pathology, clinical features, diagnostic tools, management, and outcome of the disease. Moreover, a series of 11 children operated on for refractory epilepsy between 2005 and 2015 (minimum follow-up 5 years, mean follow-up 9.6 years) is reported. The series consists of six boys and five girls with 6.5-month and 16.2-month mean age at seizure onset and at surgery, respectively. Seizures affected all children, followed by hemiparesis and psychomotor delay (81%), glaucoma (54%), and other neurological deficits (45%). Results All children underwent hemispherectomy (anatomical in three cases, functional in two cases, hemispherotomy in six cases); one patient needed a redo hemispherotomy. Mortality was nil; disseminated intravascular coagulation and interstitial pneumonia occurred in one patient each; three children had subdural fluid collection. Eight patients (72%) are in the ILAE Class 1 (completely seizure and aura free), two in Class 2 (only auras, no seizure), and one in Class 3 (1-3 seizure days per year). AEDs discontinuation was possible in 73% of cases. The most important news from the literature concerned the pathogenesis (role of the mutation of the GNAQ gene in the abnormal SWS vasculogenesis), the clinical findings (the features and pathogenesis of the stroke-like episodes are being understood), the diagnostic tools (quantitative MRI and EEG), and both the medical (migraine, seizures) and surgical management (epilepsy). The epileptic outcome of SWS patients is very good (80% are seizure-free), if compared with other hemispheric syndromes. The quality of life is affected by the neurological and cognitive deficits. Conclusions SWS still is an etiological and clinical challenge. However, the improvements over the time are consistent. In particular, the neurosurgical treatment of refractory epilepsy provides very good results as long as the indication to treatment is correct.

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Section: Surgical Optionsmentioning

confidence: 99%

Section: Late Post-operative Outcomementioning

confidence: 99%

Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons

et al. 2020

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“…Cirurgias de ressecção (lobectomias, hemisferectomias) ou de desconexão (calosotomias, hemisferotomia) têm sido descritas como opção de tratamento para pacientes com a síndrome de Sturge-Weber que cursam com epilepsia refratária e, especialmente, àqueles com déficits neurológicos estabelecidos (hemiparesia, hemianopsia e atraso no desenvolvimento neuropsicomotor) (67) . Um resultado cirúrgico bem-sucedido estará relacionado à ressecção ou desconexão eficiente da região cortical comprometida e, quando indicada, a intervenção precoce terá maior probabilidade de melhorar o resultado do desenvolvimento (68) .…”

Section: Aspectos Etiológicosunclassified