title: Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  This long-term investigation of TCA patients treated according to Sauer's technique documented a good QoL despite a reduced BFS.
authors.affiliation: Department of Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria, ee.amerstorfer@gmail.com.
authors.forename: Eva E
authors.initials: EE
authors.lastname: Amerstorfer
authors.forename: Günter
authors.initials: G
authors.lastname: Fasching
authors.forename: Holger
authors.initials: H
authors.lastname: Till
authors.forename: Andrea
authors.initials: A
authors.lastname: Huber-Zeyringer
authors.forename: Michael E
authors.initials: ME
authors.lastname: Höllwarth
mesh_terms: D002675:Child, Preschool; D003106:Colon; D013505:Digestive System Surgical Procedures / Q000379:methods; D005260:Female; D006627:Hirschsprung Disease / Q000601:surgery*; D006801:Humans; D007080:Ileocecal Valve; D007223:Infant; D008297:Male; D059351:Organ Sparing Treatments; D011788:Quality of Life; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome