J Gastroenterol Hepatol 1999 Jun;14(6):588-93

Primary sclerosing cholangitis in children

Roberts EA

Division of Gastroenterology and Nutrition, Hospital for Sick Children and
University of Toronto, Ontario, Canada

Abstract
Primary sclerosing cholangitis (PSC), a chronic inflammatory process affecting
the extrahepatic and/or medium to large bile ducts, is not rare in children. It
has features suggesting an autoimmune pathogenesis, although the mechanism of
tissue damage remains unknown. The clinical presentation of childhood primary
sclerosing cholangitis is highly variable and frequently without obvious
features of cholestasis. Clinical similarity to autoimmune hepatitis is common.
Association with chronic colitis is less common than in adults. Cholangiography
is essential for the diagnosis and examination of the medium to large
intrahepatic ducts is mandatory, as 40% of children lack extrahepatic duct
involvement. Histological findings may help to distinguish childhood PSC from
autoimmune hepatitis. In children, sclerosing cholangitis may also develop
secondary to other disease processes, notably Langerhans histiocytosis,
congenital immunodeficiencies and cystic fibrosis. Neonatal sclerosing
cholangitis is chronic inflammatory disease of bile ducts which presents
initially with neonatal cholestasis; its pathogenesis remains uncertain and may
not be the same as for primary sclerosing cholangitis. Effective treatment
modalities for childhood PSC remain undetermined. Liver transplantation is
required for children who progress to biliary cirrhosis and hepatic
decompensation