ArticleBMC surgery2018
Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome.
Article in BMC surgery, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.
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Who cites it
9 citing papers in PubMed, 1 synthesis or guideline pooled it, 26 citations in OpenAlex.
- Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.Journal of pediatric surgery · 2021Pooled it
- Automatic ganglion cell detection for improving the efficiency and accuracy of hirschprung disease diagnosis.Scientific reports · 2021Trial
- Growth and dietary effects on gastrointestinal symptoms in children with Hirschsprung's disease.Pediatric surgery international · 2026Observational
- Tight Junction Defects in Aganglionic and Ganglionic Colon in Children With Hirschsprung Disease.Laboratory investigation; a journal of technical methods and pathology · 2026Article
- Total colonic aganglionosis: management and long-term outcomes at a referral centre.Pediatric surgery international · 2024Article
- Review
- Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood.BJS open · 2020Article
- Intestinal microbiome and fitness in kidney disease.Nature reviews. Nephrology · 2019Review
- Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management.Pediatric gastroenterology, hepatology & nutrition · 2019Review
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundTotal 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.
methodsA 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.
resultsEleven 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.
conclusionThe 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.
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