脑室-腹腔分流术治疗甲基丙二酸尿症并脑积水  

Methylmalonic aciduria combined with hydrocephalus treated with ventriculoperitoneal shunt

作　　者：李辉[1] 高阳旭[1] 张宏武[1] 刘宝富[1] 沈笠雪[1] 姚红新[1] 

机构地区：[1]北京大学第一医院小儿外科,100034

出　　处：《中华实用儿科临床杂志》2017年第10期788-790,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的总结脑室-腹腔分流术治疗甲基丙二酸尿症合并脑积水临床经验，探讨其临床应用价值。方法选取2012年9月至2016年5月在北京大学第一医院诊断为甲基丙二酸尿症合并脑积水患儿12例。其中男7例，女5例。12例患儿均行脑室-腹腔分流术治疗。术后继续行药物治疗，以临床表现及影像学复查结果作为调节分流阀压力的依据，适当调整分流阀压力。回顾性分析脑室-腹腔分流术治疗甲基丙二酸尿症合并脑积水患儿的临床资料。术后随访预后情况，对患儿的临床症状、体征、影像学资料、手术并发症及预后等进行研究。结果随访3～36个月，本组12例患儿无手术死亡，无脑积水术后严重并发症发生。患儿颅内高压临床症状均明显缓解或消失，头围进行性增大停止，前囟张力明显降低，双眼＂落日征＂消失，4例惊厥、癫痫发作患儿术后症状缓解1例。7例视力低下、视力减退患儿术后视力较术前均有好转，但均未恢复正常。1例听力减退患儿术后听力恢复正常。患儿随访期间头颅CT检查均显示脑室较术前明显缩小，间质性脑水肿明显改善。结论脑室-腹腔分流术是甲基丙二酸尿症合并脑积水患儿有效治疗方式，可使此类疾病患儿临床获益。ObjectiveTo summarize the experience of the ventriculoperitoneal shunt in treating children with methylmalonic aciduria combined with hydrocephalus, and to assess the clinical value.MethodsFrom September 2012 to May 2016, a total of 12 patients with methylmalonic aciduria combined with hydrocephalus in Peking University First Hospital were enrolled, including 7 boys and 5 girls.All the 12 patients underwent ventriculoperitoneal shunt.Drug the-rapy was performed after surgery.The clinical manifestations and imaging findings were used as the basis for adjusting the pressure of the diverter valve appropriately.The clinical condition of patients were evaluated retrospectively.The patients′ clinical symptoms, signs, imaging materials, surgical complications and postoperative prognosis were analyzed.ResultsAll the cases were followed up for 3 to 36 months, no death case and no serious postoperative complications of hydrocephalus occurred.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement stopped.The anterior fontanelle tension decreased significantly.＂ Setting-sun＂ sign of eyes disappeared.One out of 4 cases with convulsion and epilepsy was relieved after the operation.Seven cases of poor vision or vision loss, postoperative visual acuity were improved though not recover to normal eyesight.One case of the children with hearing loss, postoperative hearing recovered.During the follow-up period, the head CT showed that the ventricle was narrowed significantly, interstitial brain edema improved obviously.ConclusionVentriculoperitoneal shunt is a effective method for treating children with methylmalonic aciduria combined with hydrocephalus, which is beneficial for patients with these diseases.

关 键 词：甲基丙二酸尿症 脑积水 脑室-腹腔分流术 

分 类 号：R726.5[医药卫生—儿科]