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先天性巨结肠症拖出术后残留无神经节细胞症:一项系统评价和荟萃分析

Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis.

作者信息

Friedmacher Florian, Puri Prem

机构信息

National Childrens's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.

出版信息

Pediatr Surg Int. 2011 Oct;27(10):1053-7. doi: 10.1007/s00383-011-2958-5.

DOI:10.1007/s00383-011-2958-5
PMID:21789665
Abstract

PURPOSE

Most patients with Hirschsprung's disease (HD) have a satisfactory outcome after pull-through (PT) operation. However, some children continue to have persistent bowel symptoms after the initial operation and may require redo PT. Redo PT operation in HD is usually indicated for anastomotic strictures or residual aganglionosis (RA). We designed this meta-analysis to determine the incidence and outcome of RA among patients with HD following PT operation.

METHODS

A meta-analysis of redo PT operations for HD reported in the literature between 1985 and 2011 was performed. Detailed information was recorded in patients with RA and transition-zone bowel (TZB), including recurrent bowel problems, histological findings on repeat rectal biopsy, type of redo PT operation and outcome.

RESULTS

Twenty-nine articles reported 555 patients with redo PT operations. 193 (34.8%) patients demonstrated abnormal histological findings on rectal biopsy with 144 patients showing RA and 49 patients showing TZB. These 193 patients presented with persistent constipation (n = 135), recurrent enterocolitis (n = 45) and abnormal histology of the pulled-through bowel (n = 13). Mean age at redo PT was 4.4 years (range 4 months-17 years). Redo procedures were Duhamel (n = 57), transanal endorectal PT (n = 40), Soave (n = 35), Swenson (n = 10), posterior sagittal approach (n = 1) and not reported (n = 50). Follow-up information after redo PT was available in 134 (69.4%) patients and not available in 59 patients. Of the 134 patients, 99 (73.9%) patients had normal bowel habits, 19 patients had persistent constipation/soiling and 16 patients had recurrent enterocolitis.

CONCLUSION

This meta-analysis reveals that RA and TZB are the underlying causes of persistent bowel symptoms in one-third of all patients with HD requiring redo PT operation. Most patients have a satisfactory outcome after redo operation. Rectal biopsy should be performed in all patients with recurrent bowel problems after PT operation.

摘要

目的

大多数先天性巨结肠(HD)患者在拖出式(PT)手术后预后良好。然而,一些儿童在初次手术后仍持续存在肠道症状,可能需要再次进行PT手术。HD患者再次进行PT手术通常是由于吻合口狭窄或残留无神经节细胞症(RA)。我们进行这项荟萃分析以确定PT手术后HD患者中RA的发生率和预后。

方法

对1985年至2011年间文献报道的HD再次PT手术进行荟萃分析。记录RA和移行段肠管(TZB)患者的详细信息,包括复发性肠道问题、重复直肠活检的组织学结果、再次PT手术的类型和预后。

结果

29篇文章报道了555例再次PT手术的患者。193例(34.8%)患者直肠活检组织学结果异常,其中144例显示RA,49例显示TZB。这193例患者表现为持续性便秘(n = 135)、复发性小肠结肠炎(n = 45)和拖出肠管组织学异常(n = 13)。再次PT手术时的平均年龄为4.4岁(范围4个月至17岁)。再次手术方式为杜哈梅尔手术(n = 57)、经肛门直肠内拖出术(n = 40)、索阿韦手术(n = 35)、斯文森手术(n = 10)、后矢状入路手术(n = 1),50例未报道。134例(69.4%)患者有再次PT手术后的随访信息,59例无随访信息。在134例患者中,99例(73.9%)患者肠道习惯正常,19例患者持续便秘/便污,16例患者复发性小肠结肠炎。

结论

这项荟萃分析表明,RA和TZB是所有需要再次PT手术的HD患者中三分之一持续性肠道症状的潜在原因。大多数患者再次手术后预后良好。PT手术后所有复发性肠道问题的患者均应进行直肠活检。

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