An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through

Long‐term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands

“…In our population, 65% of the patients reported constipation, 47% reported faecal incontinence and 53% reported soiling. This is comparable with, or somewhat higher than, reports in other studies .The use of bowel management was reported by 18% of our patients and an adapted diet by 29%. Functional problems were reported more often in questionnaires than in medical records.…”

“…In the TEPT procedure, an ileoanal anastomosis is created, aiming to reduce the risk of faecal stasis and development of enterocolitis. There is currently no consensus on which technique may be superior in patients with TCA [6][7][8][9][10]12,19,29,30,34,45]. In this cohort we could not test the difference between the different techniques because of the limited number of patients.…”

Is complete resection of the aganglionic bowel in extensive total aganglionosis up to the middle ileum always necessary?

“…Marquez et al [1], in a comprehensive review, showed a mortality rate of 1.9% after the definitive operation. Cheung et al [9] published a review of 10 cases in 18 years in which 4 patients were submitted to 1-stage procedure at a mean age of 27 days. According to this author and Wildhaber et al [5], the concerns in performing 1-stage pull through in total colonic aganglionosis include the possibility of incorrect leveling of the aganglionic segment and the inability to adapt to the more rapid transit time resulting in perianal excoriation.…”

“…Perianal dermatitis is one of the most common complications of pull-through operations [4,5,9] and tends to lessen in severity in time. In our series, we had one exceptional case in which the problem was not manageable and the patient was given an ileostomy 4 years after the pull through.…”

Barium enema findings in total colonic aganglionosis: a single-center, retrospective study

“…With advancements in surgical techniques and meticulous management, the mortality rate and bowel function of patients with TCA have been improved [6]. The important problem in TCA is not surgical management but rather its prompt diagnosis which decreases the rate of misdiagnosis and allows patients to receive treatment timely to adequately handle of neonatal symptoms by conservative or surgical treatment, which decreases preoperative mortality [2,4,[7][8][9]. In patients with "segmental" HD, rectal biopsy is the standard approach for preoperative diagnosis [10].…”