Hepatobiliary and Pancreatic: Tiny Pigmented Intra-Hepatic Ducts Stones As the Cause of Jaundice and Liver Failure

An 80-year-old male was referred to our hospital with jaundice and fever. A laboratory examination on admission showed elevated serum levels of aspartate aminotransferase 382 IU/L, alanine aminotransferase 338 IU/L, alkaline phosphatase 1418 U/L, direct bilirubin 4.7 mg/dL, and C-reactive protein 8.16 mg/dL. Carbohydrate antigen 19–9 was 89.1 U/mL, and serum immunoglobulin-G4 was 65 mg/dL. Contrast-enhanced computed tomography (CECT) scan on admission showed mild dilatation of intrahepatic bile duct (Fig. 1a), without obvious tumor or stone. Ultrasonography similarly delineated just dilatation of intrahepatic bile duct (Fig. 1b). Endoscopic retrograde cholangiography (ERC) revealed normal common bile duct, and hepatic bile duct was not described (Fig. 1c). In consideration of biliary tract cancer, cytologic examinations of bile and biopsy for hilar bile duct were performed; however, malignant cell was not detected. Regardless of nasobiliary drainage for common bile duct, direct bilirubin was elevate up to 21.6 mg/dL. Then given the poor health condition, with the patient's consent, supportive care without aggressive medical treatment was offered. He died approximately 2 months after jaundice, and autopsy was performed under informed consent from his family. What was the cause of hepatic disorder? Histologically, autopsy findings revealed bilirubin pigmentation of all organs and multiple small black pigment stones in the intrahepatic bile duct (Fig. 2a,b). Malignant or benign stricture of the biliary tract and the pathological findings such as primary sclerosing cholangitis or IgG4-related sclerosing cholangitis were not observed, then the small stones most likely caused fatal liver injury and liver failure. The blockage of the bile ducts with small stones seems to have caused poor endoscopic cholangiography for hepatic ducts or intrahepatic bile ducts. The black pigment or small stone is difficult to detect in ultrasonography due to the vague acoustic shadow, which seems to have described unidentified intrahepatic bile duct dilatation. In the case of jaundice and cholangitis with diagnostic difficulties in image and cytologic examinations, the existence of the small black pigment stones should be considered.