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Functional hemispherotomy for epilepsy in the very young
Journal of Neurosurgery: Pediatrics ( IF 2.1 ) Pub Date : 2022-08-05 , DOI: 10.3171/2022.6.peds21521
Joshua Pepper 1 , William B Lo 1 , Shakti Agrawal 2 , Rana Mohamed 2 , Jo Horton 3 , Selina Balloo 3 , Sunny Philip 2 , Ashish Basnet 1 , Welege Samantha Buddhika Wimalachandra 1 , Andrew Lawley 2 , Stefano Seri 4 , A Richard Walsh 1
Affiliation  

OBJECTIVE

Epilepsy is one of the most common neurological disorders in children. Among very young children, one-third are resistant to medical treatment, and lack of effective treatment may result in adverse outcomes. Although functional hemispherotomy is an established treatment for epilepsy, its outcome in the very young child has not been widely reported. In this study the authors investigated seizure and developmental results after hemispherotomy in children younger than 3 years.

METHODS

The authors reviewed a prospective database of all children younger than 3 years with medically intractable epilepsy who underwent functional hemispherotomy at the authors’ institution during the period between 2012 and 2020. Demographic data, epilepsy history, underlying etiology, operative and transfusion details, and seizure and developmental outcomes were analyzed.

RESULTS

Twelve patients were included in this study. The mean age (± SD) at seizure onset was 3 ± 2.6 months and at surgery was 1.3 ± 0.77 years, with a mean follow-up of 4 years. Diagnoses included hemimegalencephaly (n = 5), hemidysplasia (n = 2), hypoxic/hemorrhagic (n = 2), traumatic (n = 1), Sturge-Weber syndrome (n = 1), and mild hemispheric structural abnormality with EEG/PET correlates (n = 1). Eleven patients achieved an Engel class I outcome, and 1 patient achieved Engel class IV at last follow-up. No deaths, infections, cerebrovascular events, or unexpected long-term neurological deficits were recorded. All children progressed neurodevelopmentally following surgery, but their developmental levels remained behind their chronological age, with an overall mean composite Vineland Adaptive Behavior Scale score of 58 (normal: 86–114, low: < 70). One patient required insertion of a subdural peritoneal shunt, 1 patient required dural repair for a CSF fluid leak, and 1 patient required aspiration of a pseudomeningocele. In 2 patients, both of whom weighed less than 5.7 kg, the first operation was incomplete due to blood loss.

CONCLUSIONS

Hemispherotomy in children younger than 3 years offers excellent seizure control and an acceptable risk-to-benefit ratio in well-selected patients. Families of children weighing less than 6 kg should be counseled regarding the possibility of staged surgery. Postoperatively, children continue to make appropriate, despite delayed, developmental progress.



中文翻译:


功能性大脑半球切开术治疗幼儿癫痫


 客观的


癫痫是儿童最常见的神经系统疾病之一。在幼儿中,三分之一对医疗有抵抗力,缺乏有效的治疗可能会导致不良后果。尽管功能性半球切开术是癫痫的一种既定治疗方法,但其在幼儿中的结果尚未得到广泛报道。在这项研究中,作者调查了 3 岁以下儿童半球切除术后的癫痫发作和发育结果。

 方法


作者回顾了 2012 年至 2020 年间在作者所在机构接受功能性半球切开术的所有 3 岁以下患有难治性癫痫儿童的前瞻性数据库。人口统计数据、癫痫病史、潜在病因、手术和输血细节以及癫痫发作并分析了发育结果。

 结果


本研究纳入了 12 名患者。癫痫发作时的平均年龄 (± SD) 为 3 ± 2.6 个月,手术时为 1.3 ± 0.77 岁,平均随访时间为 4 年。诊断包括半侧巨脑畸形 (n = 5)、半侧发育不良 (n = 2)、缺氧/出血 (n = 2)、创伤性 (n = 1)、Sturge-Weber 综合征 (n = 1) 和脑电图/脑电图轻度半球结构异常PET 相关 (n = 1)。在最后一次随访中,11 名患者达到了 Engel I 级结果,1 名患者达到了 Engel IV 级。没有记录死亡、感染、脑血管事件或意外的长期神经功能缺损。所有儿童在手术后神经发育均取得进展,但其发育水平仍落后于实际年龄,瓦恩兰适应行为量表的总体平均综合评分为 58(正常:86-114,低:< 70)。 1 名患者需要插入硬膜下腹膜分流器,1 名患者需要因脑脊液漏出进行硬脑膜修复,1 名患者需要抽吸假性脑膜膨出。 2名患者体重均不足5.7公斤,第一次手术因失血未完成。

 结论


3岁以下儿童的半球切开术可以很好地控制癫痫发作,并且在精心挑选的患者中具有可接受的风险效益比。应就分期手术的可能性向体重低于 6 公斤儿童的家庭提供咨询。术后,尽管出现延迟,儿童仍能继续取得适当的发育进展。

更新日期:2022-08-05
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