{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T19:52:43Z","timestamp":1790452363781,"version":"4.1.0"},"reference-count":45,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2018,8,2]],"date-time":"2018-08-02T00:00:00Z","timestamp":1533168000000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Colorectal Disease"],"published-print":{"date-parts":[[2018,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Total colonic aganglionosis (<jats:styled-content style=\"fixed-case\">TCA<\/jats:styled-content>) is a severe form of Hirschsprung's disease (<jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>) associated with a high morbidity. This study assessed long\u2010term functional outcome and quality of life (QoL) of patients with <jats:styled-content style=\"fixed-case\">TCA<\/jats:styled-content> in a national consecutive cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Surgical and demographic characteristics in the medical records of all patients (<jats:italic>n<\/jats:italic>\u00a0=\u00a053) diagnosed with <jats:styled-content style=\"fixed-case\">TCA<\/jats:styled-content> between 1995 and 2015 were reviewed. Functional outcome of all nonsyndromal patients, aged \u2265\u00a04\u00a0years (<jats:italic>n<\/jats:italic>\u00a0=\u00a035), was assessed using a questionnaire and in medical records. Generic and disease\u2010specific QoL were assessed using standardized validated questionnaires.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 35 patients eligible for follow\u2010up, 18 (51%) responded to the questionnaires. They were aged 4\u201319\u00a0years. A Duhamel procedure was performed in 67% of these patients and a Rehbein procedure was performed in 33%. In the questionnaire, 65% of the patients reported constipation, 47% faecal incontinence and 53% soiling. Moreover, 18% of patients used bowel management (flushing or laxatives) and 29% had an adapted diet only. Children and adolescents with <jats:styled-content style=\"fixed-case\">TCA<\/jats:styled-content> had worse perception of their general health and were more limited by bodily pain and discomfort compared with healthy peers. Their quality of life is influenced most by frequent complaints of diarrhoea and other physical symptoms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Children and adolescents with <jats:styled-content style=\"fixed-case\">TCA<\/jats:styled-content> report lower health\u2010related QoL compared with healthy peers, especially in the physical domain. We suggest standardized follow\u2010up and prospective longitudinal future research on functionality and QoL of these patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/codi.14095","type":"journal-article","created":{"date-parts":[[2018,3,15]],"date-time":"2018-03-15T15:05:27Z","timestamp":1521126327000},"page":"719-726","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Long\u2010term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands"],"prefix":"10.1111","volume":"20","author":[{"given":"D.","family":"Roorda","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery \u2013 Pediatric Surgical Center of Amsterdam Emma Children's Hospital Academic Medical Center and VU University Medical Center Amsterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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V.","family":"Schulten","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Prinses Maxima Center Utrecht Netherlands"},{"name":"Department of Pediatric Surgery Uniklinic K\u00f6ln Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. E. J.","family":"Sloots","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Erasmus MC Sophia Children's Hospital Rotterdam Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"de Blaauw","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Amalia Children's Hospital Radboudumc Nijmegen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. M. 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