Primary Sclerosing Cholangitis in a Paediatric Patient: An Atypical Presentation

Abstract

Primary sclerosing cholangitis (PSC) is an autoimmune chronic liver disease that studies have shown is rare in children. Here, a challenging case of PSC in a 13-year-old male, without preceding manifestations of inflammatory bowel disease and with evidence of biliary obstruction, is reported. The patient presented with progressive scleral icterus; their total bilirubin and alkaline phosphatase levels were raised, with negative autoimmune work-up, and an ultrasound scan of their abdomen was unremarkable. Magnetic resonance cholangiopancreatography revealed marked dilatation of the intra- and extrahepatic bile ducts, with strictures in the hepatic duct and proximal common bile duct (CBD). Endoscopic retrograde cholangiopancreatography revealed a very narrow CBD with high-grade severe biliary stricture at the common hepatic duct. A cholangiogram revealed the beaded appearance of the intrahepatic ducts, and a left percutaneous external biliary drainage tube was placed. Overall, the findings were suggestive of PSC with high-grade CBD strictures. This case is unique due to the absence of preceding clinical manifestations of inflammatory bowel disease and predominantly obstructive symptoms at the time of presentation, which is highly unusual in the paediatric population.