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. Ultrasound (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 common 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 was noted to have ampullary distortion secondary to malrotation following presentation with an intestinal obstruction. Pruritus and pancreatitis have not been previously reported with malrotation, although cholestatic jaundice has been described. The diffculties in diagnosis and the role of US are discussed. Ladd's procedure is the appropriate treatment for this condition.