A case of distal bile duct cancer in a patient with polysplenia syndrome and ventral pancreatic malrotation.

Polysplenia syndrome is a rare congenital anomaly characterized by visceral malposition associated with multiple spleens. In relation to the pancreas, reported abnormalities include the annular pancreas, as well as defects in the body, tail, or uncinate part of the pancreas. Here, we present a case of polysplenia syndrome with ventral pancreatic malrotation and distal bile duct cancer. The patient was a 64-year-old man who presented to our hospital with obstructive jaundice. After endoscopic drainage and an anatomical evaluation of the vasculature, a subtotal stomach-preserving pancreaticoduodenectomy was performed for distal bile duct cancer. Polysplenia syndrome is associated with malposition of various organs and blood vessels, which varies from case to case. Therefore, understanding the anatomical variations in each patient is crucial before surgery. The accumulation of case reports will provide valuable information for the treatment of future cases. We report this valuable case along with a literature review.