Oriental Hepatolithiasis in an African Patient Presenting with Subfulminant Hepatic Failure

Abstract

Background: Oriental hepatolithiasis, also known as recurrent pyogenic cholangitis, is a disease characterized by intrahepatic pigment stone formation, dilatation and stricturing of biliary tree. Patients usually present with recurrent episodes of cholangitis leading to chronic biliary obstruction, liver abscess, liver atrophy and eventually secondary biliary cirrhosis. To our knowledge, there are no case reports of Oriental hepatolithiasis in African patients presenting with subfulminant hepatic failure. Methods: We describe a 28 year old African female from Nigeria who presented with a 1 month history of progressive scleral icterus, abdominal distension and lower extremity edema. Liver enzymes were elevated with Bilirubin: 20.8 mg/dl, Alkaline Phosphatase: 514 U/l, AST-297 U/l, ALT-104 U/l, INR-2.03. Workup for chronic liver disease showed ANA>1:160, Anti SM(-), Anti LKM1(-). MRI/MRCP showed cirrhotic liver with moderate intrahepatic biliary dilatation. Patient was given a trial of steroids therapy for possible Auto-immune hepatitis without any clinical response. Transjugular liver biopsy was non-diagnostic. Patient underwent OLT for hepatic failure within 2 months of her presentation. Histopathology of explanted liver showed mixed nodular cirrhosis and diffuse severe intrahepatic bile sludging and hepatolithiaisis, marked cholangiolar proliferation, foamy cells and distended bile ducts suggestive of “oriental hepatolithiaisis”. Conclusion: Although many patients with Oriental hepatolithiasis present with a chronic progressive course, our patient presented with subfulminant hepatic failure. Liver transplantation should be considered as an additional therapeutic modality in these patients.