Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetic kidney disease and polycystic liver disease is its major extrarenal manifestation, however biliary peritonitis due to a liver cyst rupture is extremely rare.
Case presentation: The patient was a 71-year-old Japanese woman who was diagnosed with ADPKD 3 years previously and developed right abdominal pain suddenly 1 month previously. As abdominal computed tomography (CT) showed a ruptured liver cyst in the right lobe, she was admitted to our hospital. Her symptoms improved with conservative management and she was discharged from the hospital after 1 week. Although she was asymptomatic for a while, she noticed abdominal distension and general malaise at 1 month after hospital discharge. Since abdominal CT showed massive ascites, she was admitted to our hospital again. A physical examination revealed abdominal distention without tenderness. Her serum creatinine, alkaline phosphatase, γ-glutamyl transpeptidase, total bilirubin, and CA19-9 were elevated. Abdominal paracentesis revealed amber transparent ascites and the bilirubin and CA19-9 concentrations were high. She was diagnosed with biliary peritonitis due to a ruptured liver cyst. Hemodialysis treatment was initiated with drainage of the ascites. The outflow of the ascites was no tendency to decrease and drip infusion cholangiography (DIC)-CT revealed a communication between the ruptured cyst and an intrahepatic bile duct. On day 31, she was transferred to a university hospital and abdominal surgery was performed. After removing the necrotic roof of the ruptured cyst on the right liver lobe, the orifice of the bile leakage was sutured. Cholecystectomy was performed and cholangiography showed no stones in the common bile duct. Abdominal CT one month after the operation showed no recurrence of ascites and she was discharged on day 49. Hemodialysis treatment was discontinued immediately after discharge because urine volume increased and her creatinine level decreased. There has been no recurrence of ascites since then.
Conclusions: While rare, biliary peritonitis can occur in association with the rupture of a liver cyst in ADPKD patients due to communication between the cyst and the intrahepatic bile duct, and DIC-CT should be recommended when biliary cyst rupture is suspected.
Keywords: Autosomal dominant polycystic kidney disease; Biliary peritonitis; Liver cyst rupture.