下丘脑错构瘤所致癫痫的手术治疗

摘要

Objective To investigate the surgical treatment and its effect in children with hypothalamic hamartoma with epilepsy as the main manifestation in order to provide basis for the treatment of the disease.Methods The clinical manifestations,operation methods and effects in 20 children with hypothalamic hamartoma with epilepsy as the main manifestation admitted to the Department of Pediatric Surgery,the First Hospital of Peking University from March 2012 to December 2015 were analyzed retrospectively.Seventeen children underwent right frontal craniotomy in stage Ⅰ.The hypothalamic hamartomas were resected via the transcallosal transseptal interforniceal approach.Two were treated with staging operations combined with interhemispheric translamina terminalis approach and 1 hamartoma posterior to the pituitary stalk was resected via the pterional approach.Results The hypothalamic hamartomas of 11 children were resected totally,6 were resected near totally,and 3 were resected subtotally.Postoperative seizures disappeared in all children.All Engel classification reached grade Ⅰ.Twelve children experienced transient high fever after procedure,1 experienced temporary memory deficiency,3 experienced polyuria,1 had hypernatremia,10 had hyponatremia (including 3 hyponatremia convulsions),and 2 had hyperphagia.Conclusion For children with hypothalamic hamartoma with epilepsy as the main clinical manifestation,early surgical treatment is safe and effective.%目的 探讨以癫痫为主要表现的下丘脑错构瘤患儿的手术治疗方法及其效果,从而为临床上对该病的治疗提供依据.方法 回顾性分析2012年3月至2015年12月北京大学第一医院小儿外科收治的20例以癫痫为主要表现的下丘脑错构瘤患儿的临床表现、手术方法以及手术效果.17例患儿Ⅰ期行右额开颅,经胼胝体-透明隔间腔-穹窿间入路切除下丘脑错构瘤.2例联合经纵裂终板入路行分期手术,1例经翼点入路切除垂体柄后方错构瘤.结果 全切除11例,近全切除6例,大部切除3例.所有患儿术后癫痫发作均消失,Engel分级均达到Ⅰ级.术后出现一过性高热12例,短暂性记忆缺陷1例,多尿3例,高钠血症1例,10例出现低钠血症(其中3例出现低钠抽搐),2例患儿出现多食.结论 对于以癫痫为主要表现的下丘脑错构瘤患儿,早期采用手术治疗安全、有效.