title: Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland.
abstract: OBJECTIVE
  Few reports on choledochal malformations (CMs) in European populations exist.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Pediatric Liver and Gut Research Group, University of Helsinki , Helsinki , Finland.
authors.forename: Maria
authors.initials: M
authors.lastname: Hukkinen
authors.forename: Antti
authors.initials: A
authors.lastname: Koivusalo
authors.forename: Harry
authors.initials: H
authors.lastname: Lindahl
authors.forename: Risto
authors.initials: R
authors.lastname: Rintala
authors.forename: Mikko P
authors.initials: MP
authors.lastname: Pakarinen
mesh_terms: D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015529:Choledochal Cyst / Q000175:diagnosis* / Q000453:epidemiology* / Q000601:surgery; D005260:Female; D005387:Finland / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D011183:Postoperative Complications / Q000209:etiology*; D012189:Retrospective Studies
keywords: Choledochal cysts; choledochal malformations