Nephrectomy in patients with Caroli’s and ADPKD may be associatedwith increased morbidity
DOI:
https://doi.org/10.5489/cuaj.613Abstract
Autosomal dominant polycystic kidney disease (ADPKD), characterizedby multiple bilateral renal cysts, is the most common
inherited disorder of the kidney and an important cause of endstage
renal disease (ESRD). Caroli’s disease is a much less frequent
condition with ectasia of the intrahepatic biliary system. A clear
association between autosomal recessive and Caroli’s disease has
been described, but only 4 cases of ADPKD and Caroli’s disease
have been reported with 2 postoperative mortalities. The aim of this
case is to increase the awareness of intra-operative and postoperative
complications. A 66 year-old male was diagnosed with ADPKD
and Caroli’s disease with hepatosplenomegaly and 4 episodes of
ascending cholangitis. After 3 years of hemodialysis for ESRD, he
received a cadaveric renal allograft. Subsequently, he developed
paroxysmal atrial fibrillation. Upon anticoagulation, he developed
multiple episodes of gross hematuria from the left native kidney.
After the anticoagulation therapy was discontinued, he underwent
bilateral nephrectomies of his native kidneys. Intra-operatively, a
splenic laceration could not be managed conservatively. Therefore,
splenectomy was performed. In addition, he developed ascending
cholangitis post-operatively that was treated with antibiotics. He
was discharged on postoperative day 18. Genetic testing revealed
that the patient is heterozygote for a large deletion in PKD1 gene,
which encompasses all tested exons (exons 1-44).
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How to Cite
Aguilar, M., Meterissian, S., Levesque, S., & Andonian, S. (2013). Nephrectomy in patients with Caroli’s and ADPKD may be associatedwith increased morbidity. Canadian Urological Association Journal, 5(2), E19-E22. https://doi.org/10.5489/cuaj.613
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