Sclerosing cholangitis with neonatal onset O. Amedee-Manesme, M.D., O. Bernard, M.D., F. Brunelte, M,D., M. Hadchouel, M.D., C. Polonovski, M.D., J. J. Baudon, M.D., P. Beguet, M.D., and D. Alagille, M.D. From Unite de Recherche d'H6patologie Pr INSERM U 56, and Dr de Pr HOpital de Bic~tre, and HOpital Trousseau, Paris, France Sclerosing cholangitis is characterized by irregular narrowing of extrahepatic or intrahepatic bile ducts, and in adults is defined mainly by radiologic findings. We describe eight children with cholestasis from the first week of life, followed by early cirrhosis and portal hypertension. Histologic examination of the liver showed absence of interlobular bile ducts in the early cholestatic phase in two patients and biliary cirrhosis later in all patients. Radiologic examination by percutaneous cholecystography under ultrasound guidance, carried out at age 8 months to 9 years, disclosed abnormal intrahepatic bile ducts with rarefaction of segmental branches, stenosis, and focal dilation. The extrahepatic ducts were involved in six patients. No intestinal disease has been found in these patients. (J PEDIATR 1987;111:225-9) The clinical diagnosis of biliary atresia is not difficult in most infants with cholestasis. 1,2 The main concern is to identify properly the various causes in the heterogeneous group of infants with intrahepatic cholestasis, because of their different outcomes. Included in this group are infants for whom investigations still fail to identify a defined cause. Some of these children have clinical or histopatho- logic features compatible with bile duct obstruction, but ultrasonography does not show dilation of the bile ducts and laparotomy shows patent extrahepatic bile ducts. In adults, such a pattern is often associated with primary sclerosing cholangitis, a progressive cholestatic disease in which intrahepatic and often extrahepatic bile ducts dis- play short annular strictures with intervening segments of normal or dilated ducts? We report eight children, initially identified as having neonatal liver disease, in whom similar clinical, histopathologic, and radiologic features compati- ble with primary sclerosing cholangitis developed. Submitted for publication June 19, 1986;accepted April 30, 1987. Reprint requests: Olivier Amedee-Manesme, M.D., D+partement de P6diatrie, H6pital de Bic~tre, 78 rue du G6n6ral-Leclerc, 94275 Le Kremlin Bic~tre Cedex, France. Fig. 1. Postoperative cholangiography through cholecystostomy tube performed at age 2l& months in patient 5. Note beaded appearance of right hepatic duct (arrow). 225