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Proctocolectomy with ileoanal anastomosis in treatment of total colonic aganglionosis in Hirschsprung disease

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Title: Proctocolectomy with ileoanal anastomosis in treatment of total colonic aganglionosis in Hirschsprung disease
Author(s): Timonen, Anni
Contributor: University of Helsinki, Faculty of Medicine
Discipline: Paediatric Surgery
Language: English
Acceptance year: 2016
Abstract:
Aim. We aimed to assess complications and functional outcomes of restorative proctocolectomy (RPC) with ileoanal anastomosis (IAA) performed on children with total colonic aganlionosis (TCA) in relation to patients with ulcerative colitis (UC). Methods. Medical records on complications, stool frequency, day- and nighttime continence, enterocolitis/pouchitis and fecal calprotectin levels of HD patients who underwent RPC with IAA in a single center were compared to similarly treated patients with pediatric-onset UC. Results. Median operative age of HD patients was 1.6 months and 5.6 years at follow-up. Fourteen patients received J-pouch and two straight IAA. Stool frequency for daytime was 3.5 and nighttime 0. Total continence rate was 79%. At least two enterocolitis/pouchitis episodes occurred in 81%, while histologically verified pouch inflammation was observed in 27%. An increased value of fecal calprotectin was observed in 4. Conclusion. Outcomes were encouraging. Stool frequency and fecal continence appeared better preserved and the frequency of histological pouch inflammation and fecal calprotectin levels were lower than in UC patients.
Keyword(s): Total colonic aganglionosis Ulcerative colitis proctocolectomy ileoanal anastomosis Hirschprung disease


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