{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T18:42:12Z","timestamp":1782931332700,"version":"3.54.5"},"reference-count":11,"publisher":"BMJ","issue":"6","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,5,5]]},"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:p>Hirschsprung disease (HD) is a gut motility disorder usually diagnosed acutely in infancy, although variants of HD may present later in life with indolent symptoms. This report highlights the rarity of diagnosing HD and hypoganglionosis in adulthood and the nuances that need consideration for their surgical management. We present a report of a 49-year-old man presenting with chronic constipation. A full thickness rectal biopsy confirmed aganglionosis, and HD in adulthood was diagnosed. He underwent a defunctioning left-sided colostomy to ensure histological confirmation of ganglia in his left colon, and adequate colonic function via the colostomy.This served also as an assessment of the proximal conduit for any future anastomosis. He later underwent ultra-low anterior resection, coloanal anastomosis and loop ileostomy with subsequent reversal. His final histology revealed hypoganglionosis of the resected segment, with normal innervation to the site of the colostomy. He made full recovery with normal bowel movements.<\/jats:p>","DOI":"10.1136\/bcr-2020-239532","type":"journal-article","created":{"date-parts":[[2021,6,23]],"date-time":"2021-06-23T11:47:30Z","timestamp":1624448850000},"page":"e239532","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Double check: diagnosis and management of adult Hirschsprung disease"],"prefix":"10.1136","volume":"14","author":[{"given":"Niamh","family":"Moynagh","sequence":"first","affiliation":[{"name":"Department of Surgery, St James Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ailin C","family":"Rogers","sequence":"additional","affiliation":[{"name":"Department of Surgery, St James Hospital, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cian","family":"Muldoon","sequence":"additional","affiliation":[{"name":"Department of Histopathology, St James Hospital, Dublin, Ireland"},{"name":"Faculty of Health Sciences, Trinity College Dublin, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul H","family":"McCormick","sequence":"additional","affiliation":[{"name":"Department of Surgery, St James Hospital, Dublin, Ireland"},{"name":"Faculty of Health Sciences, Trinity College Dublin, Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,6,23]]},"reference":[{"key":"2025082623302734000_14.6.e239532.1","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1007\/s00384-019-03442-8","article-title":"A national analysis of operative treatment of adult patients with Hirschsprung's disease","volume":"35","author":"Schlund","year":"2020","journal-title":"Int J Colorectal Dis"},{"key":"2025082623302734000_14.6.e239532.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.anndiagpath.2006.03.017"},{"key":"2025082623302734000_14.6.e239532.3","first-page":"1624","article-title":"Adult Hirschsprung's disease: report of four cases","volume":"6","author":"Qiu","year":"2013","journal-title":"Int J Clin Exp Pathol"},{"key":"2025082623302734000_14.6.e239532.4","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1007\/s10151-019-01999-8","article-title":"Allied disorders of Hirschsprung's disease","volume":"23","author":"Qiu","year":"2019","journal-title":"Tech Coloproctol"},{"key":"2025082623302734000_14.6.e239532.5","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2472070182"},{"key":"2025082623302734000_14.6.e239532.6","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1053\/j.sempedsurg.2017.09.008","article-title":"Hirschsprung disease-Bowel function beyond childhood","volume":"26","author":"Wester","year":"2017","journal-title":"Semin Pediatr Surg"},{"key":"2025082623302734000_14.6.e239532.7","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-010-2694-2"},{"key":"2025082623302734000_14.6.e239532.8","first-page":"371","article-title":"A new surgical treatment for Hirschsprung's disease","volume":"28","author":"Swenson","year":"1950","journal-title":"Surgery"},{"key":"2025082623302734000_14.6.e239532.9","doi-asserted-by":"publisher","DOI":"10.1136\/adc.35.179.38"},{"key":"2025082623302734000_14.6.e239532.10","doi-asserted-by":"publisher","DOI":"10.1007\/BF02554342"},{"key":"2025082623302734000_14.6.e239532.11","doi-asserted-by":"publisher","DOI":"10.1136\/adc.39.204.116"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2020-239532","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T06:30:40Z","timestamp":1756276240000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2020-239532"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6]]},"references-count":11,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2021,6,23]]},"published-print":{"date-parts":[[2021,6]]}},"alternative-id":["10.1136\/bcr-2020-239532"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2020-239532","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6]]}}}