BACKGROUND
Cholestatic jaundice with markedly elevated bilirubin levels usually raises concern for extrahepatic biliary obstruction or malignant hepatobiliary disease. However, marked conjugated hyperbilirubinemia can occasionally occur in patients with cirrhosis despite repeatedly negative biliary imaging, creating substantial diagnostic uncertainty and management challenges.
CASE REPORT
We report a diagnostically challenging case of a 63-year-old Egyptian man presenting with progressive jaundice, dark urine, pale stool, pruritus, abdominal pain, fatigue, anorexia, weight loss, and recent ascites. Laboratory investigations revealed severe conjugated hyperbilirubinemia with total bilirubin level reaching 19.45 mg/dL. Extensive infectious and viral investigations were negative. Autoimmune assessment demonstrated only weak anti-smooth muscle antibody positivity with otherwise negative autoimmune markers. Radiological evaluation including ultrasonography, contrast-enhanced computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography demonstrated cirrhotic liver morphology, portal hypertension, splenomegaly, and gallbladder calculi without evidence of common bile duct obstruction, biliary dilatation, or pancreatic mass. The marked discrepancy between the severity of cholestasis and the absence of obstructive pathology created substantial diagnostic uncertainty, while the marked discrepancy between severe cholestasis and persistently negative biliary imaging raised concern for a possible nonobstructive cholestatic process associated with advanced chronic liver disease. The patient was treated conservatively with supportive medical therapy and careful clinical monitoring.
CONCLUSIONS
This case highlights that severe cholestatic jaundice in patients with cirrhosis does not necessarily indicate extrahepatic biliary obstruction. Integrating clinical, serological, and multimodal radiological findings is essential for excluding alternative etiologies and preventing unnecessary invasive biliary procedures.

Keywords: Jaundice, Obstructive; Case Reports; Hyperbilirubinemia; Gallstones; Hypertension, Portal; Liver Cirrhosis