Risk of biliary cancer in Finnish patients with choledochal malformations: A population-based register study

Abstract

<p><b>Aim</b><br>The purpose of this study was to assess the long-term risk of biliary cancer in patients diagnosed with choledochal malformations (CMs).</p><p><b>Methods</b><br>The patient population was collected from the Helsinki University Hospital registers including all consecutive patients from 1987 to 2020. This data were complemented with the population-based register data from the Finnish Register of Congenital Malformations (FRM) and the Finnish Care Register for Health Care (1987–2020). Patient population was cross-linked with data from the Finnish Cancer Register data and the Cause-of-Death Register.</p><p><b>Results</b><br>There were 209 patients with CM (142 females and 67 males). Most patients (n = 110, 53%) underwent biliary diversion surgery, 18 patients (8.6%) were treated endoscopically, and 57 patients (27%) were managed with observation only. Three patients (1.4%) were diagnosed with biliary cancer at a mean age of 60.3 years, and all of them died of their disease at a mean age of 65.4 years. Cholangitis was diagnosed in 33 patients (16%). Risk of inflammatory bowel diseases (IBDs) appeared higher in CM patients than in general Finnish population as IBD was diagnosed in 10 patients (4.8%) with relative risk (RR) of 4.9 (95% confidence interval [CI] 2.7–9.0).</p><p><b>Conclusions</b><br>CM seemed to be associated with an increased risk of biliary cancer although the prevalence was lower than in previous reports.</p>