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

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...

Ruptured Omphalocele with prolapsed-intestinal volvulus with incarcerated necrosis and ileal atresia:a case report and literature review

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 stageⅠrepair,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.