儿童裂隙脑室综合征临床特点分析  

Analysis of clinical characteristics of children with slit ventricle syndrome

作　　者：武洁[1] 王荃[1] 方铁[2] 赵光远[1] 王龙[1] 霍枫[1] 钱素云[3] Wu Jie;Wang Quan;Fang Tie;Zhao Guangyuan;Wang Long;Huo Feng;Qian Suyun(Department of Emergency,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Department of Functional Neurosurgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Pediatric Intensive Care Unit,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院急诊科,100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院功能神经外科,100045 [3]国家儿童医学中心,首都医科大学附属北京儿童医院重症医学科,100045

出　　处：《中华实用儿科临床杂志》2021年第20期1543-1547,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：首都医科大学附属北京儿童医院儿童用药专项(YZQN202008)。

摘　　要：目的了解儿童裂隙脑室综合征(SVS)的临床特点,提高儿科医师对SVS的认识。方法回顾性收集2015年6月至2019年5月首都医科大学附属北京儿童医院急诊科确诊的SVS患儿病历资料,分析其临床特点。结果共纳入10例患儿,原发病分别为蛛网膜囊肿3例、先天性脑积水3例、Dandy-Walker综合征2例、特发性颅内压增高综合征1例、颅内出血后继发脑积水1例。首次分流手术年龄为1.3(0.3~12.8)岁,确诊SVS时的年龄为9.9(3.8~13.3)岁,分流术后4.4(0.5~12.0)年出现SVS相关症状:不同程度的间断头痛(10/10例)、呕吐(10/10例)、精神烦躁(4/10例)、抽搐发作(5/10例)、视力下降(2/10例)、颅内压增高(10/10例)等。SVS发生时,患儿脑室形态均呈裂隙样。3例经甘露醇治疗症状缓解后未行手术,7例内科治疗无效后急诊行腰大池-腹腔分流术。随访1~5年,9例未再出现SVS相关症状,1例内科治疗患儿的病情多次反复。结论SVS系分流术后的罕见并发症,主要临床特点为间断头痛、分流管泵充盈迟缓;影像学示脑室呈裂隙样改变。分流术后出现颅高压征象,且影像学未见脑室扩张时,需高度警惕SVS。SVS患儿如药物治疗无效,应尽早手术,以改善预后。Objective To identify clinical characteristics of slit ventricle syndrome(SVS)in children,thus improving pediatricians′understanding of pediatric SVS.Methods Clinical data of children diagnosed as SVS in the Emergency Department of Beijing Children′s Hospital,Capital Medical University from June 2015 to May 2019 were collected and retrospectively analyzed.Results A total of 10 children with SVS were included,including 3 cases of arachnoid cyst,3 cases of congenital hydrocephalus,2 cases of Dandy-Walker syndrome,1 case of idiopathic intracranial hypertension syndrome,and 1 case of hydrocephalus secondary to intracranial hemorrhage as the primary disease.The age of first shunting,and that at diagnosis of SVS were 1.3(0.3-12.8)years,and 9.9(3.8-13.3)years,respectively.SVS-associated symptoms appeared in 4.4(0.5-12.0)years after shunting,including intermittent headache(10/10 cases),vomiting(10/10 cases),irritability(4/10 cases),seizures(5/10 cases),diminution of vision(2/10 cases),and intracranial hypertension(10/10 cases)at varying severities.When SVS occurred,the cerebral ventricle presented slit-like morphology.Three cases were relieved with mannitol treatment and 7 cases were treated with emergency lumbar-peritoneal shunt after mannitol failure.During 1-5 years of follow-up,9 patients did not have SVS-associated symptoms and 1 case with medical therapy had recurrences.Conclusions SVS is a rare complication after shunt surgery,with the clinical manifestations of intermittent headache and slow valve refilling conforming;imaging showed that the cerebral ventricle was slit-like morphology.When there are signs of intracranial hypertension after shunting and there is no cerebral ventricle dilatation in imaging,SVS should be highly vigilant.Once SVS is confirmed,surgical intervention should be introduced as early as possible after treatment failure of medication,so as to improve the prognosis.

关 键 词：裂隙脑室综合征 颅高压 儿童 

分 类 号：R726.5[医药卫生—儿科]