脊髓脊膜膨出术后症状性CHIARI畸形的诊治  

Treatment of symptomatic Arnold- Chiari malformation after closure of myelomeningocele in children

作　　者：李太平[1] 李红超[2] 娄平阳[1] 

机构地区：[1]河南省人民医院神经外科,郑州450003 [2]郑州大学第二附属医院神经外科

出　　处：《医药论坛杂志》2015年第10期21-24,共4页Journal of Medical Forum

摘　　要：目的总结脊髓脊膜膨出术后症状性Chiari畸形的临床表现,发生发展形式,探讨术前状态与预后的关系。方法分析2013年1月—2014年10月援非（Eritrea）期间收治脊髓脊膜膨出187例,合并CHIARI畸形149例,13例术后发生了延髓功能障碍和后组颅神经症状,经证实与分流障碍无关。临床表现为神经性吞咽困难,吸气性喘息或喘鸣,发作性紫绀,呼吸暂停,后仰型斜颈或角弓反张,运动障碍和声带麻痹。年龄小于3月患儿病情明显进展较快,2~5d即出现长时间呼吸暂停和/或心率减慢。所有病例均接受限制性后颅窝和上颈髓减压术,若存在延髓或上颈髓空洞,行空洞-蛛网膜下腔分流,未脑积水分流患儿同时行Torkldsen分流术。结果8例（61.5%）症状完全或近全缓解,2例残留轻度饮水呛咳,1例残留刺激状态下喘鸣,2例（15.3%）术前表现双侧声带麻痹的患儿症状无改善并死于呼吸衰竭。结论神经性吞咽困难,吸气性喘息或喘鸣,发作性紫绀,后仰型斜颈或角弓反张,呼吸暂停,运动障碍是症状性Chiari畸形的常见症状,长时间呼吸暂停和/或心率减慢,双侧声带麻痹是脑干严重受损的临床表现。年龄小于3月的患儿病情进展明显较快,需要早期诊断和早期手术干预。Objective To summarize clinical manifestation and its pattern of presentation of symptomatic Chiari malformation,and to investigate the relationship between outcome and preoperative status. Methods Totally 13 cases symptomatic Chiari malformation among 187 cases myelomeningocele treated by Chinese Medical Team in Orotta Hospital Asmara Eritrea from Jan 2013 to Oct 2014 were retrospectively analyzed. Clinical manifestation： neurogenic dysphagia,stridor,cyanosis,apnea,opisthotonos with torticollis,motor dysfunction,and vocal cord paralysis. Neurological impairment deteriorated more rapidly in cases less than 3 months old,commonly it took only 2 ~ 5 days to progress from onset of symotoms to profound apnea and / or bradycardia. All cases underwent limited hindbrain decompression and cervical laminectomy,1case also with syringosubarachnoid shunt,cases without ventrculoperitoneal shunt accepted Torkldsen CSF diversion at the same time. Results 8 cases（ 61. 5%） had complete or nearly complete resolution of all signs of brainstem compression,3 cases（ 23%） had mild to moderate residual deficits,2 cases had mild dysphagia for liquids,1case had stridor when agitated. 2 cases（ 15. 3%） who showed bilateral vocal cord paralysis before surgical intervention died of respiratory failure. Conclusion Neurogenic dysphagia,stridor,cyanosis,apnea,opisthotonos with torticollis,motor dysfunction,and vocal cord paralysis are common among symptomatic Chiari malformation patients,long- term apnea and / or bradycardia,bilateral vocal cord paralysis indicate profound brain- stem dysfunction. Patients less than 3months old deteriorated rapidly that necessitate early diagnostic evaluation and surgical intervention.

关 键 词：CHIARI畸形 后颅窝和上颈髓减压术 脊髓脊膜膨出 

分 类 号：R744[医药卫生—神经病学与精神病学]