Total colonic Hirschsprung's disease: a 28-year experience.
OBJECTIVE
The aim of this study was to review outcomes after surgical treatment of total colonic Hirschsprung's disease (TCH).


METHODS
Twenty-five records of patients with TCH treated between 1974 and 2002 were reviewed. Follow-up data were collected using a standardized questionnaire. Objective functional outcome was assessed using a scoring system.


RESULTS
Twenty patients had aganglionosis of the colon and distal ileum, 5 of whom had a more extensive condition. One of these 5 patients underwent an endorectal pull-through (ERPT), 1 underwent intestinal transplantation, and 3 died. Four of the remaining 20 patients underwent a primary ERPT, 16 received a stoma as neonates followed by ERPT in 12, and a Martin-Duhamel procedure or Swenson's operation in 3 (median age, 10.5 months); 1 remains with an ostomy. Postoperative complications included enterocolitis (55%), anal stricture (25%), and perineal excoriation (20%). Mean follow-up were 17.5 years (+/-11.1 years). Eighty-nine percent were free of recurrent enterocolitis. Frequency of bowel movements is 1 to 5 per day in 82% of the patients, 18% have 6 or more bowel movements per day. Occasional soiling is noted in 40% (one third of those requiring nighttime diapers). Overall functional outcome was good in 83%. Those patients with the longest follow-up periods had the best stooling scores (P = .04).


CONCLUSIONS
Surgical treatment of TCH is associated with a number of complications including recurrent enterocolitis and anal strictures. Long-term outcome is quite favorable.
Wildhaber
Barbara E
BE
Section of Pediatric Surgery, CS Mott Children's Hospital, University of Michigan Medical School, Health System, Ann Arbor, MI 48109-0245, USA.
Teitelbaum
Daniel H
DH
Coran
Arnold G
AG
D000714:Anastomosis, Surgical; D001004:Anus Diseases / Q000209:etiology; D003251:Constriction, Pathologic; D013505:Digestive System Surgical Procedures / Q000009:adverse effects*; D004760:Enterocolitis / Q000209:etiology; D005242:Fecal Incontinence / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010502:Perineum; D011183:Postoperative Complications; D020127:Recovery of Function; D012189:Retrospective Studies; D012871:Skin Diseases / Q000209:etiology; D016896:Treatment Outcome