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

Abstract

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.

Conclusions

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.

Keywords:

• Choledochal cyst
• hepaticojejunostomy
• long-term outcome
• pancreaticobiliary maljunction

Disclosure statement

Authors have no conflicts of interest to declare.

Additional information

Funding

This study was supported by the Sigrid Juselius Foundation, the Finnish Pediatric Research Foundation, and the Helsinki University Central Hospital (grants to M.P.P.).