{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:12:00Z","timestamp":1753891920901,"version":"3.41.2"},"reference-count":14,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,9,9]],"date-time":"2022-09-09T00:00:00Z","timestamp":1662681600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p>Abernethy malformation is a rare abnormality of the hepatic portal vein system with non-specific and diverse clinical manifestations. Here, we described a case of abernethy malformation with hepatopulmonary syndrome in a 10-year-old girl. On physical examination, cyanosed lips and acropachy could be found. Her oxygen saturation fluctuated at 89\u201394%, and the fasting blood ammonia was 98 umol\/L. Furthermore, there were abnormalities in the imaging. The microbubble test with contrast echocardiography was positive. Computer tomography angiography (CTA) showed the splenic vein, and the superior mesenteric drained directly into the inferior vena cave after confluence. The same result was also observed in delayed splenic arteriography. Then, we discovered a tiny branch of the intrahepatic portal vein by the inferior vena cava balloon occlusion test, which could also show the confluence of the splenic vein and superior mesenteric vein with the inferior venacave. According to the evidence above, we concluded that the girl was a patient of type II abernethy malformation. For the severe dysplasia of the portal vein, the girl accepted partial ligation of portosystemic shunt and Rex shunt, which improved her oxygen saturation and exercise tolerance.<\/jats:p>","DOI":"10.3389\/fped.2022.856611","type":"journal-article","created":{"date-parts":[[2022,9,9]],"date-time":"2022-09-09T06:06:03Z","timestamp":1662703563000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case report: Rare abernethy malformation with hepatopulmonary syndrome in a pediatric patient"],"prefix":"10.3389","volume":"10","author":[{"given":"Lianfu","family":"Ji","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhaoming","family":"Ji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dandan","family":"Xiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuming","family":"Qin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shiwei","family":"Yang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2022,9,9]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"21","DOI":"10.5152\/dir.2021.20474","article-title":"Abernethy malformation: a comprehensive review","volume":"28","author":"Kumar","year":"2022","journal-title":"Diagn Interv Radiol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"003145","DOI":"10.12890\/2022_003145","article-title":"Type II abernethy malformation: a rare cause of hepatic encephalopathy in adulthood","volume":"9","author":"Gon\u00e7alves","year":"2022","journal-title":"Eur J Case Rep Intern Med."},{"key":"B3","doi-asserted-by":"publisher","first-page":"497447","DOI":"10.3389\/fped.2020.497447","article-title":"Treatment option for abernethy malformation-two cases report and review of the literature","volume":"8","author":"Lin","year":"2020","journal-title":"Front Pediatr."},{"key":"B4","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1016\/j.ihj.2020.12.015","article-title":"Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension","volume":"73","author":"Bobhate","year":"2021","journal-title":"Indian Heart J."},{"key":"B5","doi-asserted-by":"publisher","first-page":"266","DOI":"10.1016\/j.jpedsurg.2018.10.083","article-title":"Liver histopathology in patients with hepatic masses and the Abernethy malformation","volume":"54","author":"Lemoine","year":"2019","journal-title":"J Pediatr Surg."},{"key":"B6","doi-asserted-by":"publisher","first-page":"647364","DOI":"10.3389\/fped.2021.647364","article-title":"Case Report: membranoproliferative glomerulonephritis, a rare clinical manifestation of abernethy malformation type II","volume":"9","author":"He","year":"2021","journal-title":"Front Pediatr."},{"key":"B7","doi-asserted-by":"publisher","first-page":"1239","DOI":"10.1007\/s10620-019-05834-w","article-title":"Congenital portosystemic shunts in children: associations, complications, and outcomes","volume":"65","author":"DiPaola","year":"2020","journal-title":"Dig Dis Sci."},{"key":"B8","doi-asserted-by":"publisher","first-page":"658","DOI":"10.1002\/hep.30817","article-title":"Congenital extrahepatic portosystemic shunts (Abernethy Malformation): an international observational study","volume":"71","author":"Baiges","year":"2020","journal-title":"Hepatology (Baltimore, Md)."},{"key":"B9","doi-asserted-by":"publisher","first-page":"203","DOI":"10.14701\/ahbps.2020.24.2.203","article-title":"Living-donor liver transplantation for Abernethy malformation - case report and review of literature","volume":"24","author":"Nam","year":"2020","journal-title":"Ann Hepatobiliary Pancreat Surg."},{"key":"B10","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1016\/j.jvsv.2020.08.001","article-title":"Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children","volume":"9","author":"Zhang","year":"2021","journal-title":"J Vasc Surg Venous Lymphat Disord."},{"key":"B11","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1186\/s42155-021-00279-7","article-title":"A case of two shunts in the endovascular treatment of type II abernethy syndrome","volume":"5","author":"Bombardier","year":"2022","journal-title":"CVIR endovascular."},{"key":"B12","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1097\/MPG.0000000000002263","article-title":"Congenital portosystemic shunts: current diagnosis and management","volume":"68","author":"McLin","year":"2019","journal-title":"J Pediatr Gastroenterol Nut"},{"key":"B13","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1016\/j.ccm.2017.08.002","article-title":"Hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation","volume":"38","author":"Iqbal","year":"2017","journal-title":"Clin Chest Med."},{"key":"B14","doi-asserted-by":"publisher","first-page":"8072","DOI":"10.3748\/wjg.v20.i25.8072","article-title":"Portopulmonary hypertension and hepatopulmonary syndrome","volume":"20","author":"Aldenkortt","year":"2014","journal-title":"World J Gastroenterol."}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.856611\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,9]],"date-time":"2022-09-09T06:06:05Z","timestamp":1662703565000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.856611\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,9]]},"references-count":14,"alternative-id":["10.3389\/fped.2022.856611"],"URL":"https:\/\/doi.org\/10.3389\/fped.2022.856611","relation":{},"ISSN":["2296-2360"],"issn-type":[{"type":"electronic","value":"2296-2360"}],"subject":[],"published":{"date-parts":[[2022,9,9]]},"article-number":"856611"}}