儿童颅内错构瘤与癫痫的外科治疗并文献复习——附3例报道

Surgical treatment for hamartoma and intractable epilepsy in children

目的探讨儿童颅内错构瘤与癫痫的关系以及外科治疗方法。方法回顾性总结3例儿童颅内错构瘤与癫痫病例。其中男2例,女1例。年龄5～5.5岁。初发病年龄自出生后1月余～5个月。首发症状均为癫痫,其中2例为痴笑性发作伴其他症状,1例为全身性发作。3例均无性征发育异常。3例均行外科手术:翼点入路1例、经胼胝体-穹隆间入路1例、经额-颞开颅1例。术中均进行脑皮质和错构瘤部位的脑电图监测。结果颞叶内侧错构瘤全切1例,下丘脑错构瘤大部切除2例,术后均无并发症。经过10～15个月的随访,2例癫痫发作明显减少(Engel-IIa级),1例癫痫发作程度减轻(Engel-III级)。结论儿童错构瘤所致的癫痫是一种药物难治性癫痫。外科手术可减轻、甚至能完全控制癫痫发作。

Objective To discuss the relationship between hamartoma and intractable epilepsy in children and try to find a better way to cure them with surgical method. Methods We carefully reviewed 3 cases of hamartoma and intractable epilepsy children in this study with 2 boys and 1 girl. Their ages ranged from 5 to half past 5 years old, and the age of the onset of epilepsy was 1 to 5 months old. Among of them, 2 cases were mainly manifested with gelastic seizures companied by other types of epilepsy （tumor located in the subthalamic areas）, 1 cases mainly with atypical absence and transient tonic attack （ tumor located in the mesial temporal lobe area）. All patients had no any sings of precocious puberty. Results After comprehensive evaluations, microsurgical treatment were performed with 1 cases of totally resection of the tumor and 2 cases of partly resection under intraoperative electroencephalography monitoring, 2 cases had significantly abnormal discharges in the subthalamic hamartoma areas. No complications were found in all cases. During 10 to 15 months follow--up, 2 cases had a significantly relief of seizure （Engel level --Ⅱ class） and the other Engel level --Ⅲ class . Conclusion Epilepsy induced by hamartoma was medically refractory and surgery can reduce or control the seizure.