Clinical Practice and Cases in Emergency Medicine

Introduction: Hepatopulmonary syndrome is a rare but serious cause of pediatric hypoxemia.

Case Report: A 10-year-old obese male with obstructive sleep apnea and asthma presented to the emergency department with low baseline oxygen saturations and exertional hypoxia, despite normal physical exam and outpatient pulmonary function testing. Workup revealed liver cirrhosis, hepatosplenomegaly, gastroesophageal varices, and an elevated alveolar-arterial gradient, raising concern for hepatopulmonary syndrome. He was diagnosed with this disease at a tertiary hepatology center and underwent liver transplantation.

Conclusion: Emergency physicians should consider hepatopulmonary syndrome in children with unexplained hypoxia and liver disease, especially in a child with a normal lung exam and no bronchodilator response.