Journal Article

Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome

Fuchs, Jörg, Urla, Cristian, Kirschner, Hans-Joachim, Lieber, Justus, Warmann, Steven W., Obermayr, Florian, Schweizer, Roland, Busch, Andreas

2018

Overview

Background Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung’s disease occurring in 3–10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA. Methods A 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire. Results Eleven patients were included in the study. The median age at surgery was 6 months (range: 3–72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis ( n  = 6), ileorectal anastomosis ( n  = 1), J-pouch ( n  = 1) and Duhamel procedure ( n  = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27–199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life. Conclusion The majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Age / Anastomosis / Anastomosis, Surgical - methods / Anemia / Care and treatment / Child / Child, Preschool / Children / Colectomy - methods / Colorectal surgery / Cyanocobalamin / Feces / Female / Folic acid / Follow-Up Studies / Functional outcome / Gastroenterology / Hemoglobin / Hirschsprung Disease - diagnosis / Hirschsprung's disease / Humans / Ileostomy / Ileostomy - methods / Infant / Interdisciplinary aspects / Internal Medicine / Male / Medical personnel / Medicine / Medicine & Public Health / Metabolic outcome / Metabolism / Organic chemistry / Ostomy / Parents / Patient outcomes / Patients / Pediatric research / Pediatrics / Postoperative Period / Quality of Life / Rectum - surgery / Research Article / Retrospective Studies / Serum levels / Studies / Surgeons / Surgery / Surgical outcomes / Surgical techniques / Surgical treatment / Surveys and Questionnaires / Systematic review / Total colonic aganglionosis / Transferrin / Transferrins / Treatment Outcome / Visceral and general surgery / Vitamin B12 / Weight