Heterotaxy syndrome with pancreatic malrotation: CT features

We report the case of an asymptomatic elderly man with heterotaxy syndrome who presented with incomplete pancreatic rotation and an aplastic pancreatic uncinate process, polysplenia, nonrotation of the intestine, a midline liver, a midline gallbladder, a right-sided stomach, and interruption of the inferior vena cava with azygos continuation. To our knowledge, pancreatic malrotation with resultant oblique placement has not been described in patients with heterotaxy syndrome, and we speculate that the pancreatic rotation was interfered with by the left lobe of the midline liver in the embryo.