title: Refractory hypoxemia caused by hepatopulmonary syndrome: a case report.
abstract: BACKGROUND
  Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation.
  
  
  METHODS
  We present the case of an 8-month-old Caucasian baby boy with a history of biliary atresia, polysplenia, and interrupted inferior vena cava who presented with hypoxemia and cyanosis that progressed rapidly. A chest computed tomography angiogram revealed significant dilatation of the pulmonary vasculature, prompting further evaluation and diagnosis of hepatopulmonary syndrome with contrast echocardiography. He was maintained on a milrinone infusion while awaiting liver transplantation. His hypoxemia improved slowly following liver transplantation, requiring tracheostomy and prolonged ventilator dependence.
  
  
  CONCLUSIONS
  Hepatopulmonary syndrome should be included in the differential for progressive hypoxemia in children with liver disease, particularly those with biliary atresia. Imaging with chest computed tomography angiogram and contrast echocardiography should be considered in cases of unexplained refractory hypoxemia.
authors.affiliation: Department of Pediatrics and Communicable Diseases, C,S, Mott Children's Hospital, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI, USA. morgenlf@med.umich.edu.
authors.forename: Morgen L
authors.initials: ML
authors.lastname: Govindan
authors.forename: Kevin W
authors.initials: KW
authors.lastname: Kuo
authors.forename: Maryam Ghadimi
authors.initials: MG
authors.lastname: Mahani
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Shanley
mesh_terms: D001656:Biliary Atresia / Q000150:complications*; D020065:Hepatopulmonary Syndrome / Q000150:complications*; D006801:Humans; D000860:Hypoxia / Q000209:etiology*; D007223:Infant; D008297:Male