Surgical outcomes of total colonic aganglionosis in children: A 26-year experience in a single institute

Abstract

Infants with TCA had satisfactory outcomes after the modified Duhamel pull-through operation. Based on our experience, we suggest that the pull-through operation could be performed earlier, even when there are loose stools from the enterostomy.

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.…”

“…Eighteen (51%) of the 35 questionnaires were returned after a median (range) follow-up period of 11 (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) years. Of these 18 patients, 10 (56%) were aged 4-11 years, four (22%) were aged 12-17 years and four (22%) were aged 18 years or older.…”

“…The primary treatment of HD is surgical resection of the aganglionic segment and reconstruction of the continuity of the intestinal tract [6][7][8][9][10]. Over the past decades, treatment has been improved and mortality decreased, but patients with TCA often experience functional complaints, such as soiling, faecal incontinence, severe motility problems and enterocolitis [3,[11][12][13][14][15][16][17][18][19]. It is unclear if these functional complaints result in decreased quality of life (QoL) [20].…”

Nutritional management in a total colonic aganglionosis with ileostomy: case report

“…Uzun segment HH veya TKA' da TEPT yöntemi de uygulanabilmektedir. Öncesinde transizyonel zonun seviyesinin belirlenmesi ve/ veya intestinal mezonun serbestleştirilmesi için mini umblikal insizyon ile laparatomi veya laparaskopi yapılması önerilmektedir [2].…”