The Right Colon Patch Pull-through for Total Colonic Aganglionosis: A Systematic Review

Abstract

Background: Total colonic aganglionosis (TCA) is a rare form of Hirschsprung Disease with many surgical techniques. There is limited good quality research to recommend one technique over another. The right colon patch (RCP) pull-through may have physiological benefits over other techniques but it is infrequently reported. This systematic review aims to describe this technique and report its outcomes.

Methods: A systematic review was conducted as per PRISMA guidelines using EMBASE and PubMed databases. Papers were included if patients underwent a right colon patch pull-through and had outcomes reported. Outcomes included: mortality, continence, stool frequency and complications. Papers from the same authors that included duplicated patients were excluded.

Results: Fifteen papers were included totalling 95 patients. Enterocolitis or difficulty emptying was the commonest complication and was experienced by 43 % of patients. Where reported, 33 % had incontinence or soiling but by the end of the follow-up period 78 % were stooling 3 or fewer times a day. The only mortalities reported were in patients in low-income healthcare settings.

Conclusions: Outcomes for the RCP are in line with data for other procedures for TCA. However, the wide variability in surgical techniques, particularly in the length of the patch and type of pull-through, and the small numbers make it difficult to know if outcomes might be improved by RCP. Given the rarity of TCA and multitude of techniques, a national registry would be beneficial to aid our understanding of outcomes.

Level of evidence: II.

Keywords: Boley; Hirschsprung disease; Kimura; Right colon patch; Total colonic aganglionosis.