title: Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation.
abstract: A seven-yr-old boy presented with persistent oxygen requirement following a respiratory infection. Physical exam was remarkable for orthodeoxia and digital clubbing. Laboratory evaluation showed elevated A-a oxygen gradient of 48 mmHg and mildly elevated transaminases. Sonography showed a 13 cm multilobulated liver mass and a biopsy revealed histological findings consistent with focal nodular hyperplasia. MAA scan revealed 23% right to left shunting. Abdominal CTA and MRV demonstrated the absence of the intrahepatic portal vein with an extrahepatic portocaval shunt. Abernethy malformation is a rare anomalous intra- or extrahepatic communication between portal blood flow and systemic venous return. In rare cases, Abernethy malformation results in HPS. Ours is the sixth case report to describe the co-existence of these two entities. Surgical correction of anomalous hepatic vasculature or liver transplant is imperative to restoration of lung function and also to prevent progression of possible malignant liver tumors. We describe the second patient with Abernethy and HPS who underwent liver transplant with complete resolution of HPS.
authors.affiliation: Pediatrics and Child Neurology Resident PGY3, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA 15224, USA. joana.osorio@chp.edu
authors.forename: Maria Joana
authors.initials: MJ
authors.lastname: Osorio
authors.forename: Alison
authors.initials: A
authors.lastname: Bonow
authors.forename: Geoffrey J
authors.initials: GJ
authors.lastname: Bond
authors.forename: Marcus R
authors.initials: MR
authors.lastname: Rivera
authors.forename: Kevin G
authors.initials: KG
authors.lastname: Vaughan
authors.forename: Amisha
authors.initials: A
authors.lastname: Shah
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Shneider
mesh_terms: D001706:Biopsy / Q000379:methods; D002648:Child; D020518:Focal Nodular Hyperplasia / Q000473:pathology; D020065:Hepatopulmonary Syndrome / Q000150:complications / Q000601:surgery*; D006801:Humans; D008099:Liver / Q000002:abnormalities* / Q000473:pathology / Q000601:surgery; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008642:Mesenteric Veins / Q000601:surgery; D010100:Oxygen / Q000378:metabolism; D011169:Portal Vein / Q000601:surgery; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D010100:Oxygen