Primary sclerosing cholangitis in children: utility of magnetic resonance cholangiopancreatography

Abstract

Background: Even when histologic findings are highly suggestive of primary sclerosing cholangitis (PSC), cholangiographic correlation is required for the diagnosis. The present gold standard, endoscopic retrograde cholangiopancreatography (ERCP), is invasive and associated with complications.

Objective: To evaluate the usefulness of magnetic resonance cholangiopancreatography (MRCP) in diagnosing PSC in children.

Materials and methods: MRCP studies were retrospectively reviewed in 20 children with clinical suspicion of PSC and who had undergone liver biopsy. MRCP studies were considered positive or negative for PSC depending on the presence or absence of dilatation, irregularity, multifocal strictures and beading of the bile ducts.

Results: Twenty children (14 boys, 6 girls) with an average age of 13 years qualified for the study. Of 19 diagnostic MRCP studies, 16 were called positive and 3 were called negative. An overall diagnosis of PSC-positive was assigned to all 19 patients based on clinical and laboratory findings, biopsy results and cholangiographic data. Based on this overall diagnosis, MRCP was 84% sensitive and accurate to diagnose PSC in children.

Conclusion: MRCP can be a valuable tool in diagnosing pediatric PSC that can demonstrate major intra- and extrahepatic ducts in most cases. An unequivocally positive MRCP study should not be followed by ERCP for diagnosing PSC in children.