Chen Feng,Fu Zhong,Fang Tao,et al.Ruptured Omphalocele with prolapsed-intestinal volvulus with incarcerated necrosis and ileal atresia: a case report and literature review[J].Journal of Clinical Pediatric Surgery,2022,21(02):179-185.[doi:10.3760/cma.j.cn.101785-202012030-015]
囊膜破裂型脐膨出并脱出肠管扭转嵌顿伴回肠闭锁的诊治及文献复习
- Title:
- Ruptured Omphalocele with prolapsed-intestinal volvulus with incarcerated necrosis and ileal atresia: a case report and literature review
- Keywords:
- Hernia; Umbilical/SU; Intestinal Atresia/SU
- 摘要:
- 目的 总结囊膜破裂型脐膨出（ruptured omphalocele，RO）的诊治经验。方法 回顾性分析赣南医学院第一附属医院收治的1例RO并脱出肠管扭转嵌顿伴回肠闭锁患儿临床资料，同时检索PubMed、Springer Link、Google Scholar、中国生物医学文献数据库、中国知网、万方和维普数据库，对关键词为脐膨出（omphalocele）、囊膜破裂（ruptured）的文献进行系统性回顾分析。结果 共检索出13篇文献，包含1959-2020年报道的18例RO患儿，结合本例共19例纳入研究；其中11例为巨型RO，8例为小型RO，腹壁缺损面积最大为10 cm×10 cm；合并肠闭锁3例，肠扭转2例，肠旋转不良2例，同时合并肠闭锁和肠扭转1例，肺发育不良和（或）肺动脉高压5例，寄生胎、隐睾1例，Edwards综合征、双侧桡骨发育不全1例，Turner综合征1例，无合并症3例。8例小型RO均行一期修补术。11例巨型RO中，2例直接行一期修补术，1例巨型RO利用寄生胎皮肤行一期修补术；其余8例行Silo袋和（或）网状补片延期闭合。1例术后因全部中肠切除致短肠综合征而需长期营养治疗并等待肠移植，1例一期修补术后出现皮瓣坏死，3例反复腹腔感染致粘连性肠梗阻，1例术后出现难治性腹泻。19例中，除1例终止妊娠、2例手术后死亡外，其余患儿术中及术后均未出现严重呼吸循环功能障碍，均存活。结论 RO常合并严重出生结构异常，应重视产前诊断，产前完善相关染色体检查，做好围产期评估；选择阴道分娩需慎重，巨型RO建议选择剖宫产，注意保护脱出脏器。出生后应立即手术，小型RO可选择一期修补术，延期修补术是巨型RO较为安全有效的治疗方法。
- Abstract:
- Objective To explore the experience of diagnosis and treatment of Ruptured Omphalocele(RO) in order to improve the level of diagnosis and treatment of the disease by pediatric surgeons.Methods The clinical data of a 38-week-term infant with RO were retrospectively analyzed.The databases of Pubmed, Springer Link, Google Scholar, CBM, CNKI, Wanfang and CQVIP were searched for the relevant publications using such key words as omphalocele and ruptured.Also a systematic review of literatures was performed.Results The 19 cases enrolled included 11 giant RO and 8 small RO.The maximum area of abdominal wall defect is 10 cm×10cm.Concurrent conditions included intestinal atresia(n=3), intestinal volvulus(n=2), intestinal atresia with volvulus(n=1), intestinal malrotation(n=2), pulmonary dysplasia with or without pulmonary hypertension(n=5), parasitic fetus and cryptorchid(n=1), Edwards syndrome and bilateral radius dysplasia(n=1), Turner syndrome(n=1).All 8 small RO underwent phase I repair.Among the 11 cases of giant RO, 2 cases underwent phase I repair directly, and 1 case of giant RO underwent phase I repair using parasitic fetal skin.1 case of capsular suture, external drug coating and abdominal wall hernia repair, 8 routine Silo bag with or without reticular patch delayed closure.1 case of short bowel syndrome due to whole midgut resection needs long-term nutrition treatment and waiting for intestinal transplantation, 1 case of flap necrosis after stage Ⅰ repair, 3 cases of adhesive intestinal obstruction caused by repeated abdominal infection, and 1 case of refractory diarrhea after operation.With the exception of 1 patient of termination of pregnancy and 2 patients of postoperative death, the other children did not have have severe respiratory and circulatory disturbance after the operation and all survived.Conclusion RO often complicated with serious abnormalities, which directly affect the prognosis.We should attach importance to prenatal diagnosis.Relevant chromosome examination and a good job of perinatal evaluation is needed.Vaginal delivery should be choosed carefully.Giant RO is recommended to choose cesarean section.Try utmost to protect the organs outside the body.Surgery should be done immediately after birth.Phase I repair can be selected for small RO and delayed repair is a more safe and effective method for giant RO.
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备注/Memo
收稿日期:2020-12-10。
基金项目:赣州市指导性科技计划项目（2020GZ2020ZSF045）
通讯作者:刘潜,Email:liuqiangmu2017@126.com