Mid-term outcomes of transanal rectal mucosectomy & partial internal anal sphincterectomy, proctocolectomy and ileoanal anastomosis for total colonic aganglionosis

Abstract

Objective To evaluate the mid-term outcomes of total colonic aganglionosis (TCA) after transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS), proctocolectomy and ileoanal anastomosis. Methods From January 2012 to November 2016, 25 boys and 15 girls underwent TRM-PIAS, proctocolectomy and ileoanal anastomosis. Their postoperative complications were evaluated. And mid-term functional outcomes were assessed by a scoring system. Seven of them underwent laparotomy-assisted endorectal pull-through (LEPT) between 2010 and 2012. Results Thirty-four patients were followed up for 1 year. The incidence of postoperative Hirschsprung’s-associated enterocolitis (HAEC) was 73.5% (25/34) and 92% (23/25) of them were occasional. The postoperative incidence of HAEC in TRM-PIAS group was significantly lower than control group (25.0% vs 85.7%, P<0.05) within the second postoperative year. The daily number of defecation was 3.7±2.3 (1-10). And 22 (64.7%) patients stayed free from soiling and 25 (73.5%) showed no perianal excoriation. According to the 2006 WHO Child Growth Standards, 30 (88.2%) patients grew up normally. Objective bowel function was assessed by a published score system. All of them showed good overall functional outcomes (14.5±1.3 points). TRM-PIAS group fared better with regards to overall outcome, defecation and soiling. Conclusions TRM-PIAS, proctocolectomy and ileoanal anastomosis are efficacious for TCA. And there is a high postoperative incidence of HAEC. Key words: Hirschsprung disease; Enterocolitis; Quality of life