title: The mid-term outcomes of TRM-PIAS, proctocolectomy and ileoanal anastomosis for total colonic aganglionosis.
abstract: OBJECTIVE
  The present study aimed 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 2012 to 2014, 12 patients (7 boys; 58.3 %) diagnosed with TCA and treated with the TRM-PIAS, proctocolectomy and ileoanal anastomosis. Seven TCA patients who underwent laparotomy-assisted endorectal pull-through (LEPT) between 2010 and 2012 were used as control group. Demographic features and complication of the two groups were evaluated. The functional outcomes were assessed by using a score system.
  
  
  RESULTS
  The procedure was successfully performed in all patients. The incidence of postoperative HAEC in the TRM-PIAS group was significantly lower (25.0 vs 85.7 %; p < 0.05) than control group within the second postoperative year. The number of bowel movement after 3, 12 and 24 months postoperatively, was 8.5 ± 3.5, 5.3 ± 2.9 and 3.1 ± 1.4 (p < 0.05) per day, respectively, in the TRM-PIAS group. The soiling was noted in 50.0 % (n = 6) of the patients in the 6th postoperative month, and 25.0 % (n = 3) in the 24th postoperative month in the TRM-PIAS group. There was no significant difference in overall functional outcome between two groups, but the TRM-PIAS group was better in terms of bowel movement and soiling.
  
  
  CONCLUSIONS
  TRM-PIAS, proctocolectomy and ileoanal anastomosis might be an effective treatment for TCA. More prospective studies evaluating the TRM-PIAS technique over longer period and with greater sample size are needed to confirm the findings in this study.
authors.affiliation: Department of General Surgery, Capital Institute of Pediatrics, No. 2 Yabao Rd, 100020, Beijing, China. l817@sina.com.
authors.forename: Qi
authors.identifier: http://orcid.org/0000-0002-1921-4979
authors.initials: Q
authors.lastname: Li
authors.affiliation: Department of General Surgery, Capital Institute of Pediatrics, No. 2 Yabao Rd, 100020, Beijing, China.
authors.forename: Long
authors.initials: L
authors.lastname: Li
authors.affiliation: Department of Medical Genetics, Capital Institute of Pediatrics, No. 2 Yabao Rd, 100020, Beijing, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Jiang
authors.affiliation: Department of General Surgery, Capital Institute of Pediatrics, No. 2 Yabao Rd, 100020, Beijing, China.
authors.forename: Zhen
authors.initials: Z
authors.lastname: Zhang
authors.affiliation: Department of Pathology, Capital Institute of Pediatrics, No. 2 Yabao Rd, 100020, Beijing, China.
authors.forename: Ping
authors.initials: P
authors.lastname: Xiao
mesh_terms: D001003:Anal Canal / Q000601:surgery*; D000714:Anastomosis, Surgical; D003082:Colectomy; D005260:Female; D006627:Hirschsprung Disease / Q000601:surgery*; D006801:Humans; D007082:Ileum / Q000601:surgery*; D007223:Infant; D007413:Intestinal Mucosa / Q000601:surgery*; D008297:Male; D012007:Rectum / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Partial internal anal sphincterectomy; Total colonic aganglionosis
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