Hepatic duct calculi and Obstructive Biliary Cirrhosis - Erler Zimmer

Hepatic duct calculi and Obstructive Biliary Cirrhosis - Erler Zimmer

Clinical History

An 85-year-old man presented with urinary retention due to benign prostatic hypertrophy. On admission, jaundice with cholestatic derangement of liver function tests was noted. Despite undergoing a transurethral prostate resection, he succumbed to pneumonia 5 days post-operatively.

Pathology

The liver specimen, sliced and displayed, exhibits a slightly thickened capsule and a finely nodular appearance of the liver substance. Intrahepatic bile ducts are dilated. On viewing the posterior or inferior surface, an irregular pigmented calculus, 10 mm in diameter, is impacted in a distended hepatic duct. Another smaller calculus, 3 mm in diameter, has been dislodged. This specimen illustrates secondary biliary cirrhosis resulting from large duct obstruction due to hepatic calculi.

Further Information

Hepatolithiasis is characterized by the presence of intrahepatic gallstones, often leading to cholangitis, progressive hepatocyte atrophy and destruction, and an elevated risk of cholangiocarcinoma. It is prevalent in East Asia but rare in Western countries, with no gender-based incidence difference. The stones primarily consist of pigmented calcium bilirubinate.

These stones cause intrahepatic bile duct obstruction, evident by distension and dilation of the bile ducts proximal to the obstructing stone. Additionally, there is bile duct proliferation at the portal-parenchymal interface with stromal edema and infiltrating neutrophils, indicating acute-chronic inflammation. If untreated, this inflammation leads to periportal fibrosis and eventually obstructive biliary cirrhosis. Microscopic examination typically reveals feathery degeneration of periportal hepatocytes, cytoplasmic swelling often with Mallory-Denk bodies (inclusions with a twisted-rope appearance caused by damaged intermediate filaments within hepatocytes), and bile infarcts from extravasated bile. Chronic inflammation may progress to biliary dysplasia and, in some cases, cholangiocarcinoma.

Patients may present with repeated cholangitis, intermittent abdominal pain, jaundice, or, in some cases, no symptoms. Treatment typically involves surgical removal of the calculi.