title: Hepatopulmonary syndrome in children - is conventional liver transplantation always needed?
abstract: BACKGROUND
  Hepatopulmonary syndrome (HPS) is the association of liver disease, hypoxemia, and intrapulmonary vascular dilatations. There are little data on the management of HPS in children other than conventional orthotopic liver transplantation (OLT).
  
  
  OBJECTIVE
  To describe the patient characteristics, mode of diagnosis, treatment, and outcomes of children with HPS at our center.
  
  
  METHODS
  Retrospective review of patients diagnosed with HPS between 1997 and 2007 after IRB approval.
  
  
  RESULTS
  There were 10 patients, six females; median age at diagnosis of HPS was 12 yr. Six with cirrhosis underwent OLT and had subsequent resolution of HPS and are stable at last follow-up. Of the remaining four, two had cirrhosis. HPS resolved without conventional OLT in the following four patients: hepatitis C after antiviral treatment, biliary atresia with portal hypertension after transjugular intrahepatic portosystemic shunting, Abernethy syndrome after auxiliary partial OLT, and in a child with splenic vein thrombosis after splenectomy.
  
  
  CONCLUSIONS
  Our series shows resolution of HPS in all patients and 100% survival after conventional OLT. Four children had resolution of HPS after surgical or medical treatments other than conventional OLT. Careful review of clinical status and underlying pathophysiology and anatomy at diagnosis of HPS should inform treatment decisions.
authors.affiliation: Division of Hepatology, Department of Pediatrics and Recanati Miller Transplant Institute, The Mount Sinai School of Medicine, New York, NY, USA.
authors.forename: Asha D
authors.initials: AD
authors.lastname: Willis
authors.forename: Tamir A
authors.initials: TA
authors.lastname: Miloh
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.forename: Kishore R
authors.initials: KR
authors.lastname: Iyer
authors.forename: Frederick J
authors.initials: FJ
authors.lastname: Suchy
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D016031:Liver Transplantation / Q000706:statistics & numerical data*; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome