Intussusception of Bypassed Ileal Segment Following Ileal Exclusion for Progressive Familial Intrahepatic Cholestasis

Abstract Progressive familial intrahepatic cholestasis (PFIC) is a rare cause of hepatocellular cholestasis. The disease is typically refractory to medical management and often requires surgical intervention for long-term management. Accepted surgical therapies include biliary diversion, ileal bypass and ultimately orthotopic liver transplantation. Ileal exclusion is felt to be an attractive alternative as it avoids the cutaneous ostomy required with external biliary diversion. We describe a case of ileocolic intussusception following ileal exclusion for PFIC presenting as abdominal pain and hyperbilirubinemia. After reduction of the intussusceptum, the terminal ileum was resected and the ileocolostomy was taken down with re-establishment of bowel continuity. The patient had immediate correction of her hyperbilirubinemia and pruritus post-operatively. This case report describes a unique complication of ileal diversion that has not been previously reported.