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Hepatopulmonary syndrome in children - is conventional liver transplantation always needed?
Asha D Willis1, Tamir A Miloh, Ronen Arnon
1Division of Hepatology, Department of Pediatrics and Recanati Miller Transplant Institute, The Mount Sinai School of Medicine, New York, NY, USA.
Insights
Hepatopulmonary syndrome (HPS) in children can resolve with orthotopic liver transplantation (OLT) or alternative treatments. This study highlights successful management strategies and outcomes for pediatric HPS patients.
Area of Science:
- Pediatric Gastroenterology
- Pulmonology
- Hepatology
Background:
- Hepatopulmonary syndrome (HPS) involves liver disease, hypoxemia, and intrapulmonary vascular dilatations.
- Limited data exist on managing pediatric HPS beyond liver transplantation.
Purpose of the Study:
- To detail patient demographics, diagnostic methods, treatments, and outcomes for pediatric HPS cases.
- To evaluate management strategies for hepatopulmonary syndrome in children.
Main Methods:
- Retrospective chart review of pediatric HPS patients diagnosed between 1997 and 2007.
- Institutional Review Board (IRB) approval was obtained for this study.
Main Results:
- Ten pediatric patients (6 female, median age 12) were diagnosed with HPS.
- Six patients underwent orthotopic liver transplantation (OLT) with HPS resolution and stable follow-up.
- Four patients achieved HPS resolution through non-OLT interventions: antiviral therapy, transjugular intrahepatic portosystemic shunting, auxiliary partial OLT, and splenectomy.
Conclusions:
- All patients achieved HPS resolution, with 100% survival post-conventional OLT.
- Non-OLT treatments successfully resolved HPS in four pediatric cases.
- Tailoring HPS treatment requires careful assessment of clinical status, pathophysiology, and anatomy.
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.
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