Pepper Joshua, Lo William B, Agrawal Shakti, Mohamed Rana, Horton Jo, Balloo Selina, Philip Sunny, Basnet Ashish, Wimalachandra Welege Samantha Buddhika, Lawley Andrew, Seri Stefano, Walsh A Richard
1Department of Neurosurgery.
2Department of Neurology.
J Neurosurg Pediatr. 2022 Aug 5;30(4):400-409. doi: 10.3171/2022.6.PEDS21521. Print 2022 Oct 1.
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.
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.
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.
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.
癫痫是儿童最常见的神经系统疾病之一。在非常年幼的儿童中，三分之一对药物治疗耐药，缺乏有效治疗可能导致不良后果。尽管功能性大脑半球切除术是一种既定的癫痫治疗方法，但其在非常年幼儿童中的疗效尚未得到广泛报道。在本研究中，作者调查了3岁以下儿童大脑半球切除术后的癫痫发作情况和发育结果。
作者回顾了2012年至2020年期间在作者所在机构接受功能性大脑半球切除术的所有3岁以下药物难治性癫痫儿童的前瞻性数据库。分析了人口统计学数据、癫痫病史、潜在病因、手术和输血细节以及癫痫发作和发育结果。
本研究纳入12例患者。癫痫发作开始时的平均年龄（±标准差）为3±2.6个月，手术时为1.3±0.77岁，平均随访4年。诊断包括半侧巨脑症（n = 5）、半侧发育异常（n = 2）、缺氧/出血性（n = 2）、创伤性（n = 1）、斯-韦综合征（n = 1）以及伴有脑电图/正电子发射断层扫描相关性的轻度半球结构异常（n = 1）。在最后一次随访时，11例患者达到恩格尔I级结局，1例患者达到恩格尔IV级。未记录到死亡、感染、脑血管事件或意外的长期神经功能缺损。所有儿童术后神经发育均有进展，但其发育水平仍落后于实际年龄，韦氏儿童智力量表综合评分总体平均为58分（正常：86 - 114分，低：< 70分）。1例患者需要插入硬膜下腹腔分流管，1例患者因脑脊液漏需要进行硬脑膜修复，1例患者需要抽吸假性脑脊膜膨出。在2例体重均小于5.7 kg的患者中，首次手术因失血而未完成。
对于精心挑选的患者，3岁以下儿童大脑半球切除术能实现良好的癫痫控制，且风险效益比可接受。对于体重小于6 kg儿童的家庭，应就分期手术的可能性提供咨询。术后，儿童尽管发育进展延迟，但仍持续取得适当进展。
