ABSTRACT
Portosystemic shunt is a rare congenital vascular anomaly. The shunt is hard to clinically suspect because its symptoms develop insidiously and affect various systems at different time points. We report a case of an 11-year-old girl who presented with brain abscess and hypoxemia without respiratory distress. No abnormality related to the hypoxemia, such as intracardiac shunt, was found on the initial chest radiograph and echocardiogram. However, computed tomography showed a portosystemic shunt. An intrapulmonary shunt was demonstrated by bubble contrast echocardiography, indicating a grade 2 hepatopulmonary syndrome, a complication of portosystemic shunt. This case describes the diagnostic process of an unusual case presenting neurological abnormalities and hypoxemia without respiratory distress. Portosystemic shunt with hepatopulmonary syndrome should be considered in the differential diagnosis of unexplained hypoxemia.