Pediatric Surgery International

, Volume 31, Issue 8, pp 773–780 | Cite as

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

  • Eva E. AmerstorferEmail author
  • Günter Fasching
  • Holger Till
  • Andrea Huber-Zeyringer
  • Michael E. Höllwarth
Original Article



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.


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.


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).


This long-term investigation of TCA patients treated according to Sauer’s technique documented a good QoL despite a reduced BFS.


Total colonic aganglionosis Sauer’s procedure Long-term study Ileo-cecal valve Bowel function Quality of life 


Conflict of interest

This study has no conflict of interest and has received no funding.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Eva E. Amerstorfer
    • 1
    Email author
  • Günter Fasching
    • 2
  • Holger Till
    • 1
  • Andrea Huber-Zeyringer
    • 1
  • Michael E. Höllwarth
    • 1
  1. 1.Department of Pediatric and Adolescent SurgeryMedical University of GrazGrazAustria
  2. 2.Department of Pediatric and Adolescent SurgeryClinical Center KlagenfurtKlagenfurtAustria

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