Abstract
Polysplenia syndrome is a very rare congenital disease complicated by variable combinations of thoracic and abdominal anomalies. We report a case of common bile duct cancer with polysplenia syndrome.
The patient was a 48-year-old woman admitted to our hospital for management of common bile duct cancer and duodenal stenosis. Subtotal stomach-preserving pancreaticoduodenectomy was performed.
Preduodenal portal vein, congenital absence of the pancreatic tail, and polysplenia were noted at operation. Moreover, inspection after the operation revealed absence of the hepatic portion of the inferior vena cava with azygos continuation and a dilated azygos vein.
Post operatively, infectious thrombosis developed due to infection of the central venous catheter of the right femoral vein, and antibiotic and anticoagulant therapes were administered. Pathologically, early common bile duct cancer associated with pancreatobiliary anomaly was diagnosed.
