Nephrectomy in patients with Caroli’s and ADPKD may be associatedwith increased morbidity

Abstract

Autosomal dominant polycystic kidney disease (ADPKD), characterizedby multiple bilateral renal cysts, is the most commoninherited disorder of the kidney and an important cause of endstagerenal disease (ESRD). Caroli’s disease is a much less frequentcondition with ectasia of the intrahepatic biliary system. A clearassociation between autosomal recessive and Caroli’s disease hasbeen described, but only 4 cases of ADPKD and Caroli’s diseasehave been reported with 2 postoperative mortalities. The aim of thiscase is to increase the awareness of intra-operative and postoperativecomplications. A 66 year-old male was diagnosed with ADPKDand Caroli’s disease with hepatosplenomegaly and 4 episodes ofascending cholangitis. After 3 years of hemodialysis for ESRD, hereceived a cadaveric renal allograft. Subsequently, he developedparoxysmal atrial fibrillation. Upon anticoagulation, he developedmultiple episodes of gross hematuria from the left native kidney.After the anticoagulation therapy was discontinued, he underwentbilateral nephrectomies of his native kidneys. Intra-operatively, asplenic laceration could not be managed conservatively. Therefore,splenectomy was performed. In addition, he developed ascendingcholangitis post-operatively that was treated with antibiotics. Hewas discharged on postoperative day 18. Genetic testing revealedthat the patient is heterozygote for a large deletion in PKD1 gene,which encompasses all tested exons (exons 1-44).