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.
Highlights
Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung’s disease occurring in 3–10% of the cases
Total colonic aganglionosis (TCA), defined as aganglionosis extending from the anus up to 30–50 cm proximal to the ileocecal valve [1, 2] is a rare variant of Hirschsprung’s disease (HD), occurring in approximately 3–10% of all cases [3]
As quite modest ileal resection in addition to colectomy may lead to rapid bowel transit and marked malabsorption of nutrients after surgery, all patients were regularly seen by our interdisciplinary team consisting of pediatric surgeons, gastroenterologists, and nutritional specialists
Summary
Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung’s disease occurring in 3–10% of the cases. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA. Total colonic aganglionosis (TCA), defined as aganglionosis extending from the anus up to 30–50 cm proximal to the ileocecal valve [1, 2] is a rare variant of Hirschsprung’s disease (HD), occurring in approximately 3–10% of all cases [3]. It represents a major challenge for both surgeons and pediatricians because it is difficult. Many patients are at risk for complications, including failure to thrive, anemia, electrolyte imbalances, dehydration, incontinence, and perianal dermatitis
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