Idiopathic localized bile duct strictures: relationship to primary sclerosing cholangitis

Two patients have been described who presented with localized hilar bile duct strictures initially diagnosed as cholangiocarcinoma. Resection of the strictures showed benign disease. The subsequent development of further independent benign strictures was consistent with sclerosing cholangitis. Problems in diagnosis and management of this form of primary sclerosing cholangitis have been discussed.