Surgical problems in primary sclerosing cholangitis.

The characteristics of primary sclerosing cholangitis are a diffuse inflammation with fibrous thickening and narrowing of the biliary ducts. All patients who have stones or have been operated on for biliary disease (risk of traumatic stenosis) must be excluded in discussions of this disease. Two observations illustrating the strict definition of its characteristics are related. From attacks of angiocholitis, the disease generally progresses to a permanent jaundice. Death occurs in a state of biliary cirrhosis. Liver histopathologic changes consist of fibrous rings constricting the segmentary interlobular ducts. Physiopathologic changes of the disease are difficult to define precisely except in cases with coexistent ulcerative colitis (1/3 of cases). The diagnosis is difficult. Illustrative examples are presented: A syndrome of stenosis in a patient in whom no stones were found during the operation but the lesions were typical of the passage of stones. The prognosis was good. One patient seemed rather typical but the rapid evolution and finally a finding of carcinomatous tissue led to the diagnosis of cancer of the biliary duct. One patient had cirrhosis with extrahepatic stenosis. Histologic studies, cholangiolitis and obstructive pericholangiolitis indicated the correct diagnosis. The disease is usually fatal within four years of onset despite administration of corticoids locally and systemically.