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Hepatopulmonary syndrome in children – is conventional liver transplantation always needed?
Author(s) -
Willis Asha D.,
Miloh Tamir A.,
Ar Ronen,
Iyer Kishore R.,
Suchy Frederick J.,
Kerkar Nanda
Publication year - 2010
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/j.1399-0012.2010.01378.x
Subject(s) - medicine , hepatopulmonary syndrome , liver transplantation , cirrhosis , biliary atresia , portal hypertension , splenectomy , transjugular intrahepatic portosystemic shunt , portal vein thrombosis , surgery , transplantation , liver disease , gastroenterology , spleen
Willis AD, Miloh TA, Arnon R, Iyer KR, Suchy FJ, Kerkar N. Hepatopulmonary syndrome in children – is conventional liver transplantation always needed?
Clin Transplant 2011: 25: 849–855. © 2010 John Wiley & Sons A/S. 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). Aims: 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.