Orthotopic Liver Transplantion

Abstract

Purpose: Liver transplantation (LT) has been the curative therapy for patients with Caroli's disease (CD) having recurring episodes of cholangitis, sepsis and biliary stone formation. There have been no reported cases on the recurrence of intrahepatic bile duct complications in patients with CD who underwent LT. We report a patient with CD who developed a recurrence of intrahepatic biliary dilatation and stricture formation 12 years post – LT. Methods: A 49 year old female was diagnosed with CD in 1992. She had right upper quadrant pain, cholestatic liver disease and was found to have intrahepatic ductal dilatation without portal fibrosis but associated with bilateral multiple renal cysts. She underwent LT with Roux-en- Y anastomosis in 1995 after having recuurent cholangitis with septicemia. In 1999, patient presented with elevated alkaline phosphatase and CT abdomen revealed moderately dilated intrahepatic ducts, patent hepatic-portal veins and a normal common bile duct- CBD. In 2007, she presented with cholangitis and cholangiogram showed a stone in the left bile duct with progressive intrahepatic biliary dilatation. The patient underwent PTC with stent placement followed by stone removal. Since then, the patient has had 2 episodes of right upper quadrant pain with elevated alkaline phosphatase, the last being in 03/2008. On both occasions, the patient underwent PTC with stent exchange and the symptoms improved. Recent abdominal MRI is suggestive of worsening of intrahepatic biliary dilatation with no evidence of biliary mass. CA 19-9 always remained normal. The worsening progression of intrahepatic biliary dilatation leading to recurrent hospitalizations for cholangitis has led to the consideration of retransplantation. Results: Conclusion: Caroli's disease (CD) is a nonobstructive segmental dilatation of the intrahepatic bile ducts. Most common variant, Caroli's syndrome involves bile duct dilatation with hepatic fibrosis. Women are predominantly affected; the average age at diagnosis is 22 years. The diagnosis is established by USG, ERCP and MRCP that demonstrate intrahepatic biliary dilatation with a normal CBD. Medical treatment includes antibiotics for bacterial cholangitis and ursodiol for prevention of bile stone formation. LT is successful with high survival rate for 5 year of 90% and 10 year survival rate of 78%. Patients with Caroli's syndrome due to portal fibrosis develop portal hypertension requiring the need for LT. Most patients with Ischemic-related injury present with complaints within the first 6 months of LT. Another possibility could have been the PKHD1 gene mutation causing the recurrent biliary complications. The etiology for recurrent bile duct complications post LT in our patient after 12 years is unclear.