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Total colonic Hirschsprung's disease: a 28-year experience.
J Pediatr Surg. 2005 Jan; 40(1):203-6; discussion 206-7.JP

Abstract

PURPOSE

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.

Authors+Show Affiliations

Section of Pediatric Surgery, CS Mott Children's Hospital, University of Michigan Medical School, Health System, Ann Arbor, MI 48109-0245, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15868586

Citation

Wildhaber, Barbara E., et al. "Total Colonic Hirschsprung's Disease: a 28-year Experience." Journal of Pediatric Surgery, vol. 40, no. 1, 2005, pp. 203-6; discussion 206-7.
Wildhaber BE, Teitelbaum DH, Coran AG. Total colonic Hirschsprung's disease: a 28-year experience. J Pediatr Surg. 2005;40(1):203-6; discussion 206-7.
Wildhaber, B. E., Teitelbaum, D. H., & Coran, A. G. (2005). Total colonic Hirschsprung's disease: a 28-year experience. Journal of Pediatric Surgery, 40(1), 203-6; discussion 206-7.
Wildhaber BE, Teitelbaum DH, Coran AG. Total Colonic Hirschsprung's Disease: a 28-year Experience. J Pediatr Surg. 2005;40(1):203-6; discussion 206-7. PubMed PMID: 15868586.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Total colonic Hirschsprung's disease: a 28-year experience. AU - Wildhaber,Barbara E, AU - Teitelbaum,Daniel H, AU - Coran,Arnold G, PY - 2005/5/4/pubmed PY - 2006/6/10/medline PY - 2005/5/4/entrez SP - 203-6; discussion 206-7 JF - Journal of pediatric surgery JO - J Pediatr Surg VL - 40 IS - 1 N2 - PURPOSE: 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. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/15868586/Total_colonic_Hirschsprung's_disease:_a_28_year_experience_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022346804006360 DB - PRIME DP - Unbound Medicine ER -