Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland

Scand J Gastroenterol. 2014 Oct;49(10):1255-60. doi: 10.3109/00365521.2014.946084. Epub 2014 Aug 15.

Abstract

Objective: Few reports on choledochal malformations (CMs) in European populations exist.

Materials and methods: The medical records of pediatric CM patients managed in our nationwide referral center for pediatric hepatobiliary surgery between 1976 and 2013 (n = 38; 71% females) were reviewed.

Results: Over follow-up time, the relative proportion of fusiform CMs increased significantly (p = 0.007) and the estimated total incidence rose from 1:128,000 to 1:38,000 (p = 0.017). Cystic CMs (42%) presented at younger age than fusiform CMs (47%) (0.8 vs. 4.6 years, p = 0.001). Two-thirds had abdominal pain and half were cholestatic at presentation. Pancreatitis had occurred in 16%. In addition to ultrasound, 71% underwent magnetic resonance cholangiopancreatography and 39% underwent endoscopic retrograde cholangiopancreatography. Median CM dilatation was 15 (10-28) mm and a 12 (9-13) mm long common pancreaticobiliary channel was confirmed in 61%, increasingly during recent years. Intrahepatic biliary tree was dilated in 19%, whereas main ducts were dilated in 50%. Apart from two operated in the 1970s and one with choledochocele, patients underwent resection of extrahepatic bile ducts with Roux-en-Y hepaticojejunostomy at age of 4.2 (0.8-9.2) years. Postoperative bile leakage and hemorrhage required reoperations in two. At last follow-up 4.8 (1.3-13.2) years postoperatively, none had dilated intrahepatic biliary ducts or elevated plasma bilirubin (5 [3-7] µmol/l). Single cholangitis episodes had occurred in two, whereas others were asymptomatic.

Conclusion: CM incidence has increased significantly in Finland, being currently over threefold higher than previous estimates in the Western world would suggest. Removal of the extrahepatic biliary tree with hepaticojejunostomy for type I and IV CMs yielded excellent results.

Keywords: Choledochal cysts; choledochal malformations.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child, Preschool
  • Cholangiopancreatography, Endoscopic Retrograde
  • Choledochal Cyst / diagnosis*
  • Choledochal Cyst / epidemiology*
  • Choledochal Cyst / surgery
  • Female
  • Finland / epidemiology
  • Humans
  • Incidence
  • Male
  • Postoperative Complications / etiology*
  • Retrospective Studies