Abstract
Congenital portosystemic shunts (CPS) are rare vascular malformations associated with hepatic and extrahepatic complications, including hepatopulmonary syndrome (HPS). We present a 16-year-old adolescent with no relevant medical history who presented with episodes of abdominal pain, jaundice, and dark urine. Ultrasound revealed a heterogeneous liver with multiple hyperechoic nodules and no visualization of the intrahepatic portal vein. Angiography demonstrated an extrahepatic portocaval shunt at the level of the portal trunk, with no opacification of the intrahepatic portal tree and low portal pressures; liver biopsy revealed regenerative nodular hyperplasia. Subsequently, he developed hypoxemia with orthodeoxygenation and required home oxygen therapy. He was placed on the waiting list and received an orthotopic liver transplant from a deceased donor. This case highlights the need for timely diagnosis, proper anatomical characterization, and individualized treatment planning in CPS associated with HPS.