{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,23]],"date-time":"2025-09-23T00:14:38Z","timestamp":1758586478890,"version":"3.44.0"},"reference-count":9,"publisher":"BMJ","issue":"9","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,9,9]]},"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:p>We report a rare case of biliary aplasia in an infant who presented with persistent jaundice and elevated direct bilirubin despite having cholic stools. Imaging confirmed the absence of extrahepatic bile ducts, consistent with biliary atresia. Intraoperatively, there was a complete absence of an extrahepatic biliary tree consistent with biliary hypoplasia. We identified a cryptogenic hepatic plate directly fused to the right portal vein on further dissection. Despite the atypical anatomy, a modified Kasai portoenterostomy was successfully performed. The patient showed progressive clinical improvement with complete resolution of jaundice and normalisation of liver function tests over the following months. This case underscores a rare anatomical variant that can still permit a favourable outcome with a timely Kasai procedure. To our knowledge, this is one of the few documented cases of hepatic plate\u2013portal vein fusion in biliary atresia.<\/jats:p>","DOI":"10.1136\/bcr-2025-266981","type":"journal-article","created":{"date-parts":[[2025,9,22]],"date-time":"2025-09-22T04:25:21Z","timestamp":1758515121000},"page":"e266981","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Portohepatic fusion mimics biliary aplasia"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8655-0948","authenticated-orcid":false,"given":"Yousef","family":"El-Gohary","sequence":"first","affiliation":[{"name":"Division of Pediatric General, Thoracic and Fetal Surgery, Chidren's Hospital Univeristy of Missouri, Columbia, Missouri, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher R","family":"Moir","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdalla","family":"Zarroug","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery, Department of Surgery, University of Nebraska Medical Center and College of Medicine, Children\u2019s Nebraska, Omaha, Nebraska, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,9,21]]},"reference":[{"key":"2025092121251464000_18.9.e266981.1","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1001\/jama.2020.0837","article-title":"Diagnostic Yield of Newborn Screening for Biliary Atresia Using Direct or Conjugated Bilirubin Measurements","volume":"323","author":"Harpavat","year":"2020","journal-title":"JAMA"},{"key":"2025092121251464000_18.9.e266981.2","doi-asserted-by":"crossref","DOI":"10.1542\/peds.2020-038596","article-title":"Complete Absence of the Extrahepatic Biliary Tree in a Newborn With Pigmented Stools","volume":"148","author":"Hartmann","year":"2021","journal-title":"Pediatrics"},{"key":"2025092121251464000_18.9.e266981.3","doi-asserted-by":"publisher","DOI":"10.1002\/hep.27191"},{"key":"2025092121251464000_18.9.e266981.4","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(05)80026-0"},{"key":"2025092121251464000_18.9.e266981.5","doi-asserted-by":"crossref","first-page":"10","DOI":"10.21037\/tp-23-504","article-title":"Effect of Kasai procedure on liver transplantation in children with biliary atresia: a systematic review and updated meta-analysis","volume":"13","author":"Liu","year":"2024","journal-title":"Transl Pediatr"},{"key":"2025092121251464000_18.9.e266981.6","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1056\/NEJM196602032740501","article-title":"Metabolism and excretion of C14-labeled bilirubin in children with biliary atresia","volume":"274","author":"Cameron","year":"1966","journal-title":"N Engl J Med"},{"key":"2025092121251464000_18.9.e266981.7","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2025-266941","article-title":"The role of indocyanine green in the diagnosis of biliary atresia: A word of caution","volume":"18","author":"El-Gohary","year":"2025","journal-title":"BMJ Case Rep"},{"key":"2025092121251464000_18.9.e266981.8","doi-asserted-by":"crossref","first-page":"1362","DOI":"10.1016\/j.jpedsurg.2024.03.039","article-title":"A Pilot Study for Biliary Atresia Diagnosis: Fluorescent Imaging of Indocyanine Green in Stool","volume":"59","author":"Lim","year":"2024","journal-title":"J Pediatr Surg"},{"key":"2025092121251464000_18.9.e266981.9","doi-asserted-by":"crossref","first-page":"98","DOI":"10.5114\/ceh.2024.139979","article-title":"Indocyanine green faecal excretion holds potential for diagnosis of neonatal biliary atresia","volume":"10","author":"Murayama","year":"2024","journal-title":"Clin Exp Hepatol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2025-266981","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,22]],"date-time":"2025-09-22T04:25:23Z","timestamp":1758515123000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2025-266981"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9]]},"references-count":9,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2025,9,21]]},"published-print":{"date-parts":[[2025,9]]}},"alternative-id":["10.1136\/bcr-2025-266981"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2025-266981","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9]]}}}