{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T11:16:11Z","timestamp":1784718971962,"version":"3.55.0"},"reference-count":33,"publisher":"Georg Thieme Verlag KG","issue":"05","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr Surg"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Introduction\u2003Management of patients with total colonic aganglionosis (TCA) is challenging for pediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an ileorectal Duhamel pull-through procedure.<\/jats:p><jats:p>\n          Materials and Methods\u2003Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data were collected on demographics, clinical presentation, complications, need for additional surgery, and long-term effects on bowel function.<\/jats:p><jats:p>\n          Results\u2003Of a total of 202 patients with Hirschsprung's disease, 13 were diagnosed with TCA (6.4%). Clinical presentation was variable. Eleven presented in neonatal period with distal bowel obstruction and two presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow-up was 13 years. Eleven are toilet trained, of whom five are fully continent. Six continue to have problems with bowel continence or constipation. One developed recurrent episode of Hirschsprung's associated enterocolitis. Two patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, pediatric psychologists, and clinical nurse specialists.<\/jats:p><jats:p>\n          Conclusion\u2003TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.<\/jats:p>","DOI":"10.1055\/a-2181-2065","type":"journal-article","created":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T23:09:35Z","timestamp":1695683375000},"page":"423-429","source":"Crossref","is-referenced-by-count":5,"title":["Long-Term Functional Outcomes and Multidisciplinary Management after Ileorectal Duhamel Pull-Through for Total Colonic Aganglionosis\u201420-Year Experience in a Tertiary Surgical Center"],"prefix":"10.1055","volume":"34","author":[{"given":"Kailas","family":"Bhandarkar","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"De Coppi","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"Cross","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Blackburn","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joe","family":"Curry","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,12,12]]},"reference":[{"issue":"08","key":"ref1","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1016\/j.jpedsurg.2016.11.033","article-title":"Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis","volume":"52","author":"P Stenstr\u00f6m","year":"2017","journal-title":"J Pediatr Surg"},{"issue":"12","key":"ref2","doi-asserted-by":"crossref","first-page":"2226","DOI":"10.1016\/j.jpedsurg.2008.08.049","article-title":"Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan","volume":"43","author":"S Ieiri","year":"2008","journal-title":"J Pediatr Surg"},{"issue":"10","key":"ref3","doi-asserted-by":"crossref","first-page":"1899","DOI":"10.1016\/j.jpedsurg.2009.04.026","article-title":"Clinical and genetic differences in total colonic aganglionosis in Hirschsprung's disease","volume":"44","author":"S W Moore","year":"2009","journal-title":"J Pediatr Surg"},{"key":"ref4","first-page":"68","article-title":"Total colonic aganglionosis: 30 years' experience","volume":"2","author":"D T Cass","year":"1987","journal-title":"Pediatr Surg Int"},{"issue":"08","key":"ref5","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1007\/s00383-012-3117-3","article-title":"Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome","volume":"28","author":"D M Laughlin","year":"2012","journal-title":"Pediatr Surg Int"},{"issue":"11","key":"ref6","doi-asserted-by":"crossref","first-page":"1859","DOI":"10.1016\/j.jpedsurg.2015.05.017","article-title":"Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis","volume":"50","author":"M Hukkinen","year":"2015","journal-title":"J Pediatr Surg"},{"issue":"09","key":"ref7","doi-asserted-by":"crossref","first-page":"1696","DOI":"10.1016\/j.jpedsurg.2008.01.072","article-title":"Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review","volume":"43","author":"M Menezes","year":"2008","journal-title":"J Pediatr Surg"},{"issue":"10","key":"ref8","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1007\/s00383-011-2960-y","article-title":"Total colonic aganglionosis: a surgical challenge. How to avoid complications?","volume":"27","author":"A Bischoff","year":"2011","journal-title":"Pediatr Surg Int"},{"issue":"05","key":"ref9","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1002\/bjs5.50317","article-title":"Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood","volume":"4","author":"P Stenstr\u00f6m","year":"2020","journal-title":"BJS Open"},{"issue":"07","key":"ref10","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1016\/S0022-3468(98)90515-2","article-title":"Long-term morbidity in total colonic aganglionosis","volume":"33","author":"J C Hoehner","year":"1998","journal-title":"J Pediatr Surg"},{"issue":"03","key":"ref11","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1097\/00000658-197209000-00010","article-title":"Surgical management of total colonic aganglionosis","volume":"176","author":"L W Martin","year":"1972","journal-title":"Ann Surg"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1007\/BF02616871","article-title":"Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease","volume":"7","author":"B Duhamel","year":"1964","journal-title":"Dis Colon Rectum"},{"issue":"06","key":"ref13","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1016\/S0022-3468(81)80830-5","article-title":"A new surgical approach to extensive aganglionosis","volume":"16","author":"K Kimura","year":"1981","journal-title":"J Pediatr Surg"},{"issue":"03","key":"ref14","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1097\/00000658-199009000-00002","article-title":"A personal experience with 100 consecutive total colectomies and straight ileoanal endorectal pull-throughs for benign disease of the colon and rectum in children and adults","volume":"212","author":"A G Coran","year":"1990","journal-title":"Ann Surg"},{"issue":"01","key":"ref15","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1053\/jpsu.2002.29429","article-title":"Proctocolectomy and J-pouch ileo-anal anastomosis in children","volume":"37","author":"R J Rintala","year":"2002","journal-title":"J Pediatr Surg"},{"issue":"01","key":"ref16","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1053\/jpsu.2003.50004","article-title":"New perspective for the management of near-total or total intestinal aganglionosis in infants","volume":"38","author":"K Sharif","year":"2003","journal-title":"J Pediatr Surg"},{"issue":"06","key":"ref17","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1055\/s-2008-1038895","article-title":"Long-term results of the treatment of total colonic aganglionosis with two different techniques","volume":"18","author":"S Barrena","year":"2008","journal-title":"Eur J Pediatr Surg"},{"issue":"06","key":"ref18","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/j.jpedsurg.2005.03.043","article-title":"Long-term outcomes in total colonic aganglionosis: a 32-year experience","volume":"40","author":"M A Escobar","year":"2005","journal-title":"J Pediatr Surg"},{"issue":"03","key":"ref19","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1016\/0022-3468(95)90064-0","article-title":"Is normal bowel function possible after repair of intermediate and high anorectal malformations?","volume":"30","author":"R J Rintala","year":"1995","journal-title":"J Pediatr Surg"},{"issue":"05","key":"ref20","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00383-003-1009-2","article-title":"Histopathological differences between recto-sigmoid Hirschsprung's disease and total colonic aganglionosis","volume":"19","author":"V Solari","year":"2003","journal-title":"Pediatr Surg Int"},{"issue":"04","key":"ref21","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/S0022-3468(86)80193-2","article-title":"Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients","volume":"21","author":"K Ikeda","year":"1986","journal-title":"J Pediatr Surg"},{"issue":"04","key":"ref22","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1053\/j.sempedsurg.2012.07.004","article-title":"Total colonic aganglionosis in Hirschsprung disease","volume":"21","author":"S W Moore","year":"2012","journal-title":"Semin Pediatr Surg"},{"issue":"01","key":"ref23","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.jpedsurg.2008.10.055","article-title":"Comprehensive review of procedures for total colonic aganglionosis","volume":"44","author":"T T Marquez","year":"2009","journal-title":"J Pediatr Surg"},{"issue":"11","key":"ref24","doi-asserted-by":"crossref","first-page":"1301","DOI":"10.1007\/s004640090092","article-title":"Definitive treatment of extended Hirschsprung's disease or total colonic form","volume":"15","author":"A Bonnard","year":"2001","journal-title":"Surg Endosc"},{"issue":"12","key":"ref25","doi-asserted-by":"crossref","first-page":"2352","DOI":"10.1016\/j.jpedsurg.2009.07.057","article-title":"An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through","volume":"44","author":"S T Cheung","year":"2009","journal-title":"J Pediatr Surg"},{"issue":"01","key":"ref26","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/j.jpedsurg.2004.09.033","article-title":"Total colonic Hirschsprung's disease: a 28-year experience","volume":"40","author":"B E Wildhaber","year":"2005","journal-title":"J Pediatr Surg"},{"issue":"05","key":"ref27","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1136\/adc.86.5.348","article-title":"Hirschsprung's disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis","volume":"86","author":"L Ludman","year":"2002","journal-title":"Arch Dis Child"},{"issue":"03","key":"ref28","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1016\/j.jpedsurg.2018.04.019","article-title":"A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations","volume":"54","author":"A Vilanova-Sanchez","year":"2019","journal-title":"J Pediatr Surg"},{"issue":"04","key":"ref29","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1053\/j.sempedsurg.2012.07.008","article-title":"Long-term outcomes of Hirschsprung's disease","volume":"21","author":"R J Rintala","year":"2012","journal-title":"Semin Pediatr Surg"},{"issue":"09","key":"ref30","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1016\/j.jpedsurg.2021.01.043","article-title":"Long-term surgical and patient-reported outcomes of Hirschsprung disease","volume":"56","author":"J R Davidson","year":"2021","journal-title":"J Pediatr Surg"},{"issue":"12","key":"ref31","doi-asserted-by":"crossref","first-page":"2430","DOI":"10.1016\/j.jpedsurg.2018.08.036","article-title":"The long-term quality of life outcomes in adolescents with Hirschsprung disease","volume":"53","author":"S Sood","year":"2018","journal-title":"J Pediatr Surg"},{"issue":"05","key":"ref32","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1016\/j.jpedsurg.2005.01.048","article-title":"Long-term clinical outcome in patients with Hirschsprung's disease and associated Down's syndrome","volume":"40","author":"M Menezes","year":"2005","journal-title":"J Pediatr Surg"},{"issue":"12","key":"ref33","doi-asserted-by":"crossref","first-page":"3499","DOI":"10.1007\/s00431-021-04129-5","article-title":"Outcomes in Hirschsprung's disease with coexisting learning disability","volume":"180","author":"J R Davidson","year":"2021","journal-title":"Eur J Pediatr"}],"container-title":["European Journal of Pediatric Surgery"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2181-2065.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T13:55:06Z","timestamp":1730814906000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2181-2065"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,9,25]]},"references-count":33,"journal-issue":{"issue":"05","published-online":{"date-parts":[[2024,9,5]]},"published-print":{"date-parts":[[2024,10]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2181-2065","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0939-7248","1439-359X"],"issn-type":[{"value":"0939-7248","type":"print"},{"value":"1439-359X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,9,25]]},"published":{"date-parts":[[2023,9,25]]}}}