Malposition of the Major Duodenal Papilla and Polysplenia.

During her medical checkup, a previously healthy 36-year-old woman underwent esophagogastroduodenoscopy, during which the major papilla on the anterolateral wall of the descending duodenum was found to be in the pushed-forward position (Picture 1, §). The common bile duct (CBD) was detected on the anterolateral-wall of the descending duodenum on contrast-enhanced computed tomography (CE-CT) (Picture 2, arrows: CBD, arrowhead: duodenum). CE-CT revealed two spleens (Picture 3A, arrowhead), the absence of the hepatic portion of the inferior vena cava (Picture 3), the preduodenal portal vein (Picture 3B, arrow), and a short pancreas (Picture2A, 3B). These CT findings are consistent with a diagnosis of polysplenia syndrome (PSS), a rare congenital disease involving organ malposition caused by organ position failure during embryonic weeks 5-6 (1). Only 5-10% of patients who do not have cardiac anomalies reach adulthood (1). PSS can cause late-onset diabetes mellitus and pancreatitis as well as intestinal volvulus (2). When physicians observe the existence of major duodenal papilla malposition, they should suspect congenital abnormalities such as PSS and conduct a screening examination of the whole body.

Picture 1.

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The authors state that they have no Conflict of Interest (COI).