Comprehensive review of procedures for total colonic aganglionosis

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Purpose: A variety of procedures have been described for the treatment of total colonic aganglionosis (TCA); however, there is no consensus as to a superior operative method. The objective of this review is to evaluate the operative methods used to treat TCA and their effectiveness with respect to morbidity, mortality, rates of enterocolitis, and functional outcomes. Methods: We searched Medline (1950-2007) and the Cochrane Library for studies reporting the operative treatment of TCA. Search terms used were "aganglionosis," "Hirschsprung's disease," "total colonic," and "long-segment." Studies evaluated were limited to those that solely discussed the operative outcomes for the treatment of TCA. A meta-analysis was then performed for morbidity outcomes in those studies describing a single operative procedure. Results: The search yielded 271 articles related to TCA. One hundred twenty-two articles discussed operative treatment of Hirschsprung's disease, and only 26 of these articles discussed operative management of TCA. Nineteen articles were isolated for statistical analysis on the basis of results for perioperative outcomes for a single procedure. These procedures included Martin (4), colonic patch modifications (4), Duhamel (2), endorectal pull-through (1), Kimura (2), Boley (2), Rehbein (2), Soave (1), and J-pouch ileoanal anastomosis (1). Overall mortality was 1.9%. Meta-analysis revealed morbidity rates ranging from 10% to 64% depending on procedure category without significant outliers. Higher morbidity rates were found for the Martin-type procedure using the descending colon but with noted higher within-group variance due to small sample sizes. Enterocolitis was noted in 7 studies with a mean of 22%. Conclusions: There is no superior operative method for the treatment of TCA with respect to perioperative morbidity, mortality, enterocolitis, and functional outcomes. The operative technique performed should be selected on center and surgeon familiarity and expertise.

Original languageEnglish (US)
Pages (from-to)257-265
Number of pages9
JournalJournal of Pediatric Surgery
Issue number1
StatePublished - Jan 2009


  • Aganglionosis
  • Congenital megacolon
  • Hirschsprung's disease
  • Long segment
  • Total colonic


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