15例脐膨出患儿死亡的病因分析  

Causes of death in 15 children with omphalocele

作　　者：王鹏[1] 吕成杰[1] 杨思思 马东 赵晓霞[1] 钭金法[1] Wang Peng;Lyu Chengjie;Yang Sisi;Ma Dong;Zhao Xiaoxia;Tou Jinfa(Department of Neonatal Surgery,Affiliated Children's Hospital,Zhejiang University School of Medicine,National Children's Health&Disease Clinical Medicine Research Center,National Children's Regional Medicine Center,Hangzhou 310003,China)

机构地区：[1]浙江大学医学院附属儿童医院新生儿外科,国家儿童健康与疾病临床医学研究中心,国家儿童区域医疗中心,杭州310003

出　　处：《中华小儿外科杂志》2023年第12期1104-1108,共5页Chinese Journal of Pediatric Surgery

基　　金：浙江省自然科学基金(LY22H040006)。

摘　　要：目的分析导致脐膨出患儿死亡的主要原因, 为脐膨出的治疗和预后提供参考和依据。方法回顾分析2015年6月至2022年6月浙江大学医学院附属儿童医院诊治的15例死亡的脐膨出患儿的资料, 其中男8例, 女7例;早产儿5例, 足月儿10例。收集并分析包括患儿孕周、性别、出生体重、脐膨出分型、合并和(或)伴发异常等资料。结果 15例患儿中, 巨型脐膨出13例, 小型脐膨出2例。合并肺动脉高压13例, 动脉导管未闭13例, 房间隔缺损15例, 室间隔缺损9例, 先天性膈疝1例, 心肌致密化不全1例, 右室双出口1例, 支气管肺发育不良4例, 美克尔憩室3例, 肠旋转不良9例。治疗期间出现新生儿坏死性小肠结肠炎1例, 呼吸道合胞病毒感染2例。因肺动脉高压死亡8例, 合并复杂畸形家属放弃治疗2例, 先天性膈疝无法修补家属放弃死亡1例, 呼吸道合胞病毒感染死亡2例, 心肌致密化不全心功能衰竭死亡1例, 右室双出口术后心功能衰竭死亡1例。结论先天性脐膨出患儿死亡病因多样, 肺动脉高压是导致患儿死亡的最多见原因。住院治疗期间须积极防治其他疾病和并发症。Objective To summarize the major causes of death for congenital omphalocele so as to optimize its treatments in children.Methods Retrospective review was performed for the clinical data of 15 children dying from omphalocele from June 2015 to June 2022.There were 8 boys and 7 girls.Term was premature(n=5)and mature(n=10).Gestational week,gender,weight,somatotype,complicated malformations and causes were analyzed.Results Size was giant(n=13)and small(n=2).The associated conditions were pulmonary hypertension(n=13),patent ductus arteriosus(n=13),atrial septal defect(n=15),ventricular septal defect(n=9),congenital diaphragmatic hernia(n=1),non-compaction of ventricular myocardium,bronchopulmonary dysplasia(n=4),Merkel's diverticulum(n=3)and congenital intestinal malrotation(n=9).Neonatal necrotizing enterocolitis(n=1),respiratory syncytial virus infection(n=2)and pneumorrhagia(n=2)occurred during treatment.Eight deaths were due to pulmonary hypertension.Two cases were abandoned due to complicated malformations,one death occurred without repairing congenital diaphragmatic hernia,two deaths were caused by respiratory syncytial virus infection,one child died from heart failure due to non-compaction of ventricular myocardium and another from heart failure after double-outlet RV surgery.Conclusions Children with congenital omphalocele may die from various causes.And pulmonary hypertension is a major cause.Other diseases and complications should be aggressively managed during hospitalization.

关 键 词：脐膨出 肺动脉高压 支气管肺发育不良 呼吸道合胞病毒 心肌致密化不全 

分 类 号：R725.4[医药卫生—儿科]