Isolated intrahepatic biliary dilatation in a patient with acquired immune deficiency syndrome (AIDS): AIDS cholangiopathy versus incidental unilobar Caroli's disease.

We describe a case of possible monolobar Caroli's disease in a patient with acquired immune deficiency syndrome (AIDS) who presented with features of cholangitis. Diagnostic workup, which included endoscopic retrograde cholangiography, revealed focal intrahepatic biliary dilatation confined to the right lobe. The patient subsequently underwent right hepatic lobectomy. Pathology revealed multiple cysts filled with calculi and inflammation in the cyst walls. Special stain results for fungi and acid-fast organisms were negative. The presence of advanced AIDS in this patient raised the possibility of this being a possible manifestation of AIDS cholangiopathy.