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Published in: Pediatric Surgery International 8/2015

01-08-2015 | Original Article

Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve

Authors: Eva E. Amerstorfer, Günter Fasching, Holger Till, Andrea Huber-Zeyringer, Michael E. Höllwarth

Published in: Pediatric Surgery International | Issue 8/2015

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Abstract

Purpose

In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique.

Methods

All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores.

Results

Of eight TCA patients (2 females, 6 males), seven participated in the follow-up survey (median follow-up time 13.5 years (range 8–31.6). Early postoperative complications involving bouts of enterocolitis in 3 and anal strictures in 5 patients ceased with age. Bowel movements ranged from median 3–4 times a day (range 1–8). Bowel-function score (BFS) was reduced in 6 patients (median 16 points, range 8–19, max 20), who also reported soiling incidences with the need for night-time protective aids in two. QoL assessment, however, documented a good outcome with a median score of 10 points (range 7–13, max 13).

Conclusion

This long-term investigation of TCA patients treated according to Sauer’s technique documented a good QoL despite a reduced BFS.
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Metadata
Title
Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve
Authors
Eva E. Amerstorfer
Günter Fasching
Holger Till
Andrea Huber-Zeyringer
Michael E. Höllwarth
Publication date
01-08-2015
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 8/2015
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-015-3743-7

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