Malrotation with recurrent volvulus presenting with cholestasis, pruritus,and pancreatitis

A patient aged 16 years was referred following numerous episodes of intense pruritus associated with persistently altered liver function tests. Ultras ound (US) demonstrated the superior mesenteric vein positioned more to the left than usual and abnormal orientation of the duodenum, consistent with a midgut malrotation. Endoscopic retrograde cholangiopancreatography the com mon bile duct, suggesting a malrotation with chronic or recurrent volvulus. A classic malrotation with 180 degrees volvulus was noted at operation. A second child previously thought to have recurrent idiopathic pancreatitis w as noted to have ampullary distortion secondary to malrotation following pr esentation with an intestinal obstruction. Pruritus and pancreatitis have n ot been previously reported with malrotation, although cholestatic jaundice has been described. The diffculties in diagnosis and the role of US are di scussed. Ladd's procedure is the appropriate treatment for this condition.