Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome
Pediatric Surgery International, 08/01/2012
Mc Laughlin D et al. – This meta–analysis reveals that a large number of patients with Total colonic aganglionosis (TCA) have long–term problems with bowel control.Methods
- MEDLINE and EMBASE databases were searched for relevant articles that reported the outcomes of patients with TCA published between 1980 and 2011.
- The search terms were “Hirschsprung’s disease”, “Total colonic aganglionosis” AND “Outcome”.
- All published studies containing adequate clinical data for a mean follow-up period of not less than 4 years were included.
- Reference lists of retrieved articles were reviewed for additional cases. Detailed records of morbidity and mortality were extracted and analysed.
- This search yielded 225 articles reporting on outcomes in TCA.
- Of these, 189 were excluded for having too short a follow-up period, small or single case series, inadequate clinical data and duplicated patient groups.
- Ultimately, 36 articles from 37 centres containing useful clinical information on the outcomes of TCA in 969 patients were identified. There were 152 early deaths prior to PT (15.7 %). Of 817 survivors, 739 underwent PT.
- The mortality rate for TCA post-PT was 5.7 %. The most frequently reported post-operative complication was enterocolitis in 42 % of the cases.
- 17.5 % of patients underwent subsequent major surgery including redo PT, stoma reformation or other laparotomy. Long-term follow-up data were available in 396 patients.
- Satisfactory or normal bowel control was reported in 60 % of the patients. Soiling, faecal incontinence or other poor outcome was reported in 33.5 % of the cases and 6.5 % of the patients had undergone conversion to a permanent ileostomy for post-operative complications.