S5724 Cirrhosis with a Bias—Unilateral Hepatic Involvement in Secondary Sclerosing Cholangitis

Introduction: Secondary sclerosing cholangitis (SSC) is a progressive biliary disease that can result from ischemia, infections, and surgical complications, often progressing to cirrhosis involving both hepatic lobes. However, segmental SSC with unilateral cirrhosis is rarely reported. Case Description/Methods: A 36-year-old woman presented in 2018 with biliary colic. Initial laboratory tests revealed alanine aminotransferase 101 U/L, aspartate aminotransferase (AST) 150 U/L, and alkaline phosphatase (ALP) 150 U/L with normal total bilirubin. A HIDA scan showed cystic duct obstruction with associated cholecystitis. Following laparoscopic cholecystectomy, she developed a biliary leak that proved refractory to multiple endoscopic retrograde cholangiopancreatographies (ERCPs) with common bile duct stenting and required percutaneous drain placements and serial external stent exchanges for 3 years. Although the biliary leak eventually resolved and her AST and alanine aminotransferase normalized, ALP remained persistently elevated in the 200s-300s U/L range. In 2021, magnetic resonance cholangiopancreatography (MRCP) revealed chronic right hepatic duct occlusion with segmental atrophy of the right hepatic lobe and compensatory hypertrophy of the left lobe. ERCP demonstrated a normal common bile, common hepatic and left hepatic ducts without opacification of the right posterior duct. In 2023, bilobar liver biopsies showed chronic cholestatic features with bile duct loss and cirrhosis confined to the right lobe and mild steatohepatitis with F1 fibrosis in the left lobe. Due to her right lobe cirrhosis, she is now undergoing surveillance for hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA). Discussion: Biliary related cirrhosis often results from primary chronic liver diseases such as primary sclerosing cholangitis (PSC) and usually affects both hepatic lobes. SSC shares similar clinical and radiological findings as PSC but often has an identifiable etiology such as ischemia, infections, drugs, or biliary injuries. This case illustrates that cirrhosis may occasionally present in an isolated lobar distribution, as seen here with right lobe involvement and left lobe sparing. In patients with a history of complex hepatobiliary surgery, bilobar liver biopsy should be considered to better characterize the extent and pattern of hepatic injury. While the long-term implications of unilateral cirrhosis are not well established, it may be reasonable to consider surveillance for hepatocellular carcinoma and cholangiocarcinoma, particularly in the context of chronic biliary disease.