Melikyan A G, Kushel' Yu V, Vorob'ev A N, Arkhipova N A, Sorokin V S, Lemeneva N V, Savin I A, Pronin I N, Kozlova A B, Grinenko O A, Buklina S B, Nagorskaya I A
Burdenko Neurosurgical Institute, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2016;80(3):13-24. doi: 10.17116/neiro201680313-24.
Hemispherectomy is a recognized option in the treatment of symptomatic forms of intractable focal epilepsy in patients with developmental brain malformations and some acquired lesions of one the hemispheres. The prognosis for an outcome of the technique is important in terms of the indications for surgical treatment.
We described the hemispherectomy technique and its variants and analyzed our own experience of surgery in 40 children. The most common (27 cases) brain pathology was extended unilateral cortical dysplasia with polymicro- or pachygyria and consequences of perinatal stroke. Six children had Rasmussen encephalitis; 6 patients had hemimegalencephaly; 1 child with Sturge-Weber syndrome had angiomatosis of the soft meninges. The patients' mean age was 3 years. Functional hemispherectomy (hemispherotomy) was used in most cases (37); 3 patients underwent anatomical hemispherectomy.
At the time of discharge, seizures resolved in all patients; later, no seizure recurrence was observed in 25 out of 29 cases with known follow-up (the follow-up median was 2.5 years), which corresponded to class 1 outcomes on the ILAE scale (86%). Serious complications developed in 2 cases; 1 patient died; hydrocephalus and the need for bypass surgery occurred in other 2 children. These results are discussed along with the literature data, and the indications for hemispherectomy are provided.
Hemispherectomy is a reliable and effective technique for treatment of symptomatic hemispheric forms of epilepsy in children. More than in 80% of patients with congenital or acquired pathology of one of the cerebral hemispheres, its deafferentation or resection leads to persistent elimination of seizures. Children with severe forms of intractable epilepsy should be promptly referred to dedicated centers to address the issue of advisability of surgical treatment.
对于患有发育性脑畸形以及一侧半球某些后天性病变的难治性局灶性癫痫症状性患者,大脑半球切除术是一种公认的治疗选择。就手术治疗的适应症而言,该技术的预后至关重要。
我们描述了大脑半球切除术技术及其变体,并分析了我们自己对40例儿童进行手术的经验。最常见的(27例)脑部病变是广泛性单侧皮质发育异常伴多小脑回或巨脑回以及围产期卒中的后果。6例儿童患有拉斯穆森脑炎;6例患者患有半侧巨脑症;1例患有斯特奇-韦伯综合征的儿童有软脑膜血管瘤病。患者的平均年龄为3岁。大多数病例(37例)采用功能性大脑半球切除术(大脑半球切开术);3例患者接受了解剖性大脑半球切除术。
出院时,所有患者的癫痫发作均得到缓解;后来,在已知随访情况的29例病例中,有25例未观察到癫痫复发(随访中位数为2.5年),这对应于国际抗癫痫联盟(ILAE)量表上的1级结果(86%)。2例出现严重并发症;1例患者死亡;另外2例儿童出现脑积水并需要进行分流手术。结合文献数据对这些结果进行了讨论,并提供了大脑半球切除术的适应症。
大脑半球切除术是治疗儿童症状性半球型癫痫的可靠且有效的技术。对于超过80%的一侧大脑半球先天性或后天性病变患者,其去传入或切除可导致癫痫发作持续消除。患有严重难治性癫痫的儿童应及时转诊至专门中心,以解决手术治疗的可行性问题。