Li Sen,Pan Weihua,Tan Xiang,et al.Anorectal malformation associated with Hirschsprung’s disease: a report of three cases and literature review[J].Journal of Clinical Pediatric Surgery,,20():161-167.[doi:10.12260/lcxewkzz.2021.02.010]
Anorectal malformation associated with Hirschsprung’s disease: a report of three cases and literature review
- Keywords:
- Anus; Rectum/AB; Hirschsprung Disease
- CLC:
- R657.1 R574
- Abstract:
- Objective The association of anorectal malformation (ARM) with Hirschsprung’s disease (HD) is rare so that a misdiagnosis is frequent in clinical practices.The objective was to improve understanding through its clinical diagnosis and treatment.Methods From September 2003 to October 2019,a total of 3 boys with perineal fistula ARM were associated with HD.More clinical cases were retrieved from the databases of Wanfang,Weipu,CNKI,PubMed and Medline up until October 2019.Results Three patients underwent Soave operations (17-36 month).The postoperative follow-up period was from 2 to 11 years.The postoperative incontinence symptoms persisted for several years.After such conservative measures as dietary adjustments and defecation training,defecation condition gradually improved and fecal control was decent.There were 42 boys and 32 girls.Associated syndromes were found in 41 children.Diverting enterostomy at birth was performed in 42 cases and 21(21/42,50%) were definitely diagnosed as ARM coexisting with HD before ARM correction.In 53(53/74,71.6%) cases,abdominal distension and constipation persisted after anal angioplasty.The average diagnostic time of HD was 8 months after conservative measures.All of them underwent radical megacolon operation (2 day-28 year).And 40 cases underwent Soave’s pull-through (40/74,54.1%).Other procedures included Duhamel procedure (18/74,24.3%),Swenson’s pull-through (8/74,10.8%) and Rehbein’s pull-through (8/74,10.8%).Conclusion For postoperative children with ARM,the possibility of concomitant HD should be considered when constipation persists and worsens and conservative treatment is ineffective.Its pathological diagnosis is based upon rectal mucosal biopsy.Soave’s operation is ideal for anorectal malformation associated with Hirschsprung’s disease.
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Memo
收稿日期:2019-09-05。
基金项目:2019年上海市"科技创新行动计划"（编号：9441905500）
通讯作者:王俊,Email:wangjun@xinhuamed.com.cn