Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. (October 2014)

• Record Type: Journal Article
• Authors: Hukkinen, Maria; Koivusalo, Antti; Lindahl, Harry; Rintala, Risto; Pakarinen, Mikko P.
• Is Part Of: Scandinavian journal of gastroenterology. Volume 49:Number 10(2014)
• Page Start: 1255
• Page End: 1260
• Publication Date: 2014-10
• Subjects: Gastroenterology -- Periodicals; Digestive organs -- Diseases -- Periodicals; 616.33
• DOI: 10.3109/00365521.2014.946084
• Languages: English
• ISSNs: 0036-5521

Abstract

<abstract> <title>Abstract</title> <p> <bold> <italic>Objective.</italic> </bold> Few reports on choledochal malformations (CMs) in European populations exist. <bold><italic>Materials and methods.</italic></bold> The medical records of pediatric CM patients managed in our nationwide referral center for pediatric hepatobiliary surgery between 1976 and 2013 (<italic>n</italic> = 38; 71% females) were reviewed. <bold><italic>Results.</italic></bold> Over follow-up time, the relative proportion of fusiform CMs increased significantly (<italic>p</italic> = 0.007) and the estimated total incidence rose from 1:128, 000 to 1:38, 000 (<italic>p</italic> = 0.017). Cystic CMs (42%) presented at younger age than fusiform CMs (47%) (0.8 vs. 4.6 years, <italic>p</italic> = 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. <bold><italic>Conclusion.</italic></bold> 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.</p> </abstract>