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Aim:  We aimed to assess management, comorbidities, biliary histopathology and outcomes of choledochal malformations (CM) in adults. Methods:  All adult CM patients managed during 1987–2021 at the Helsinki University Hospital were retrospectively reviewed. Results:  Of the 39 patients (females 79%) identified, 19 (49%) underwent surgery, 5 (13%) therapeutic ERCP with papillotomy for type 3 CM and 15 (38%) were managed conservatively. Twenty-five (66%) patients had ≥1 comorbidity, and five (13%) patients had associated inflammatory bowel disease (IBD); ulcerative colitis ( n  = 3) and Crohn’s disease ( n  = 2). Most surgically treated patients underwent hepaticojejunostomy ( n  = 13) or pancreaticoduodenectomy ( n  = 3) and they were younger at presentation than other patients [29 (21 – 48) vs. 59 (41 – 71),  p  = 0.001]. Three patients (23%) suffered from pancreatitis following diagnostic ERCP. Early surgical complications occurred in six (32%) patients, including one anastomotic leakage. Biliary epithelial dysplasia was found in three (15%) patients in resected specimens, while no neoplastic changes were observed. Nine patients (47%) had long-term postoperative complications, cholangitis ( n  = 5) and anastomotic stricture ( n  = 3) being the most common ones. No evidence of malignant change was observed after median follow-up of 2.3 (0.60 − 4.5) years included all patient cohorts. Conservatively managed patients remained symptomless with unprogressive biliary tract imaging findings and normal liver biochemistry values during follow-up. Nearly half of operated patients developed long-term postoperative complications. A novel association between CMs and IBD was observed. Although no hepatobiliary malignancies regardless of treatment modality were encountered, the number of patients and length of follow-up remained limited.

Characteristics, management and outcomes of choledochal malformations in Finnish adult patients