摘要目的 探讨儿童时期巨型脐膨出的诊疗经验,以提高小儿外科医师对该病的诊疗水平.方法 回顾性分析1例9岁的巨型脐膨出患儿的临床资料,同时通过检索PubMed、Springer Link、Google Scholar、中国生物医学文献数据库(CBM)、中国知网(CNKI)、万方和维普数据库,对关键词为巨型脐膨出(Giant Omphalocele)、儿童(Children)、延期(Delayed)的文献进行系统性复习.结果 共9例息儿符合条件纳入本研究,年龄17月龄至15岁;腹壁缺损面积最大者为35.0cm×25.0 cma,最小者为9.4 cm×7.7 cm;合并法洛四联症和双侧腹股沟斜疝1例,合并动脉导管未闭1例;合并盆腔异位肾和胸骨分裂1例;合并右乳缺如及右侧胸肌发育不良1例.7例行脐膨出一期修补术,2例行分期手术(其中1例腹腔置入组织扩张器,1例使用生物材料补片覆盖),1例出现暂时性血压增高,1例术后行机械通气10 d.结论 巨型脐膨出的治疗策略多样,但在不具备手术条件的时间和地方,应做好囊膜局部护理,待囊膜完全上皮化后再行手术,是个可选方案.儿童时期巨型脐膨出的手术治疗,重点在于自身材料合理运用,重建腹壁达到理想的功能和外观.

abstractsObjective To explore the experiences of diagnosing and treating giant omphalocele in children so as to improve its cognition among pediatric surgeons.Methods The clinical data were collected from one 9-year-old child with giant ompbalocde.The databases of Pubmed,SpringerLink,Google Scholar,CBM,CNKI,Wanfang and CQVIP were searched for the relevant publications using such key words as giant omphalocele,children and delayed.Also a systematic review of literatures was performed.Results For 9 eligible children,the age range was 17 to 180 months.The largest area of abdominal wall defect was 35.0 cm × 25.0 cm and the minimum 9.4 cm × 7.7 cm.Concurrent conditions included tetralogy of the Fallot & bilateral inguinal hernia (n =1),patent ductus arteriosus (n =1),pelvic ectopic kidney & sternal division (n =1),right breast deficiency & dysplasia of right thoracic muscle (n =1).Repairing was one-stage (n =7) and staged (n =2).A tissue expander was placed in intra-abdominal compartment in one case while another case covered with biomaterial patch.One case experienced transient hypertension while another required postoperative mechanical ventilation for 10 days.Conclusions The treatment options for giant omphalocele are diverse.When one-stage procedure is not feasible or carries a high risk,topical care of omphalocele sac is more reasonable.Surgery should be performed immediately upon a complete epithelialization of omphalocele sac.The goal is to utilize proper materials for reconstructing abdominal wall to optimize function and appearance.