A rare case of heterotaxy syndrome associated with hepatolithiasis and pre-duodenal portal vein

Polysplenia syndrome is characterised by spectrum of abnormalities which are rarely diagnosed in adults as an incidental finding. The anomalies include multiple spleens, midline liver, gall bladder and biliary tract anomalies, short pancreas, agenesis of pancreas, right-sided stomach, intestinal malrotation, inferior vena cava interruption with azygous or hemiazygous continuation, and a Pre-Duodenal Portal Vein (PDPV). These anomalies may occur isolated or in combination. Since most of the minor anomalies do not cause clinical symptoms, polysplenia syndrome is mostly detected incidentally in the adults because of multi-detector computed tomography which is being used in diagnosis of associated disease process. Therefore, radiologists should become familiar with these rare and peculiar anomalies associated with polysplenia syndrome. In the present report, we describe the case of a 50-year-old male patient with heterotaxy syndrome with polysplenia incidentally diagnosed during an imaging evaluation of an unrelated pathological condition – Cholelithiasis.