Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report

Polysplenia syndrome is a rare congenital disorder characterized by multiple spleens and complex visceral and vascular anomalies. We report an adult case admitted for multiple intrahepatic bile duct stones. Imaging revealed multiple congenital abnormalities, including polysplenia, a truncated pancreas, and absence of the superior inferior vena cava segment. Intraoperative findings confirmed extensive intrahepatic bile duct stones and biliary malformations. Surgical treatment included partial hepatectomy, bile duct exploration, and T-tube drainage. Postoperatively, the patient developed bacteremia, which resolved with targeted antibiotics. This case highlights the link between polysplenia syndrome and hepatobiliary anomalies, emphasizing the need for careful anatomical evaluation, early recognition of biliary complications, and close clinical monitoring in affected patients.