Spontaneous Biloma Resulting from Intrahepatic Bile Duct Perforation Coexisting with Intrahepatic Cholelithiasis and Cholangiocarcinoma: A Case Report and Literature Review.

BACKGROUND Spontaneous biloma is a rare non-traumatic disease in which an extrahepatic or intrahepatic bile duct perforates spontaneously with no discernable cause. We present the details of a patient with spontaneous biloma resulting from intrahepatic bile duct perforation with concurrent intrahepatic cholelithiasis and cholangiocarcinoma. CASE REPORT A 74-year-old woman was admitted to our hospital with symptoms of abrupt epigastralgia, nausea, and fever. Physical examination revealed epigastric tenderness, guarding, and rebound tenderness. Laboratory test results were normal, except for elevated leukocytes, and C-reactive protein, total bilirubin, and blood urea nitrogen concentrations. Carcinoembryonic antigen and carbohydrate antigen 19-9 concentrations were also elevated. Abdominal computed tomography revealed perihepatic fluid and ascites, with common bile duct dilatation and localized cholangiectasia of B2 with areas of slight high density, which indicated an intraabdominal abscess and intrahepatic cholelithiasis. Spontaneous intrahepatic bile duct perforation was subsequently diagnosed by cholangiography via endoscopic nasobiliary drainage. Left hepatic lobectomy was performed to treat the intrahepatic cholelithiasis and spontaneous biloma. Intraoperatively, a perforation was identified at the edge of the lateral segment of the left triangular ligament, through which bile had been leaking. Histopathology revealed intraductal cholangiocellular carcinoma with intrahepatic cholangiolithiasis. The patient's postoperative course was excellent, and she was discharged on postoperative day 16. However, cancer dissemination to the peritoneum was identified 8 months after surgery. CONCLUSIONS Treatment for patients with intrahepatic cholelithiasis should involve aggressive surgery because of the associated carcinogenicity. This approach reduces the risk of dissemination secondary to intrahepatic bile duct perforation.