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Unfortunate Co-incidence: Caroli's Syndrome in a Post Renal Transplant Patient


Bawany, Muhammad MD; Youssef, Wael MD; Nawras, Ali MD

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American Journal of Gastroenterology: October 2010 - Volume 105 - Issue - p S199-S200
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Purpose: Caroli's syndrome is characterized by bile duct ectasia in association with hepatic fibrosis. It is usually transmitted in an autosomal recessive fashion and has been well documented to be associated with autosomal recessive polycystic kidney disease (ARPKD) and occasionally with autosomal dominant polycystic kidney disease (ADPKD). However, there has been only few case reports published with Caroli's syndrome diagnosed post renal transplantation. We present a case of 52 year old Caucasian male who was referred to us for evaluation of elevated liver enzymes. His medical history was significant for renal transplant 13 years ago thought to be due to ADPKD. He had no history of smoking or alcohol use. At presentation his vital signs were stable except for the temperature of 99 F. Rest of the physical examination was unremarkable. At his first presentation liver function tests (LFTs) showed albumin of 3.7 g/dl, bilirubin 1.7 mg/dl with direct bilirubin 0.6 mg/dl, alkaline phosphatase 188 IU/L, ALT 50 IU/L and AST 40 IU/L. Patient's prior LFTs were normal. Ultrasound of the liver revealed dilated biliary tree with sludge in gallbladder. CT scan of abdomen revealed marked intrahepatic biliary dilatation with common bile duct measuring 1cm. Patient had ERCP which showed dilatation and stricturing of intra and extra hepatic biliary tree and multiple stones which were removed after performing sphincterectomy, followed by cholecystectomy. Subsequently, patient presented to our institution with complaint of repeated bouts of low grade temperature associated with right upper quadrant abdominal pain and mildly elevated liver enzymes. CT scan of abdomen revealed persistent biliary tree dilatation. ERCP revealed multiple saccular dilatations within the intrahepatic biliary tree. Multiple balloon sweeps were performed; several stone fragments with sludge were removed. Patient continued to have recurrent cholangitis, therefore, he was eventually evaluated for liver transplant and he finally received orthotopic liver from a deceased donor. Pathology of patient's native liver revealed cavernous biliary ectasia with fibrosis consistent with caroli's syndrome. To our knowledge, there are only 5 cases reported of caroli's disease post renal transplant in English literature [Table 1].

Conclusion: Caroli's syndrome in patient's with history of renal transplant carries high risk for recurrent or refractory cholangitis, therefore, surgical therapy, i.e., liver transplantation, needs to be considered early in the course of treatment when conservative therapy with antibiotics fail due to immunosuppressive status of the patient conservative therapy with antibiotics might fail due to immunosuppressive status of the patient.

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