Presentation
Abdominal pain, nausea, fatigue and icterus. Laboratory investigations suggested cholestatic jaundice. No evidence of autoimmune, viral or metabolic etiology of hepatitis. US examination of the abdomen was unremarkable.
Patient Data
Maximum intensity projection 3D MR cholangiopancreatographic study shows multiple strictures of the common bile duct interspersed with focal dilatations, with a beaded appearance, along with multiple discontinuous strictures of the intrahepatic bile ducts involving both hepatic lobes as well as slight prominence or dilatation of the intrahepatic biliary radicles. No biliary masses or stones are identified. Axial DWI shows mild hyperintense signal along the intra-hepatic biliary radicals and the CBD.
Normal size and signal intensity of the liver with no focal lesions. Normal MRI of the gall bladder with average wall thickness. Normal MRI signal of the spleen and both kidneys. No ascites or enlarged abdominal lymphadenopathy. Traces of minimal bilateral pleural effusion is noted.
Beaded appearance of the CBD as well as the intrahepatic biliary radicles with multiple strictures alternating with focal dilatations.
Case Discussion
Multiple strictures of the common bile duct interspersed with focal dilatations, with a beaded appearance, along with multiple discontinuous strictures of the intrahepatic bile ducts affecting both hepatic lobes; consistent with primary sclerosing cholangitis in the clinical setting of cholestatic jaundice.