Choledochal Malformation in Children: Lessons Learned from a Dutch National Study

Maria H A van den Eijnden, Ruben H J de Kleine, Ivo de Blaauw, Paul G J M Peeters, Bart P G Koot, Matthijs W N Oomen, Cornelius E J Sloots, W G van Gemert, David C van der Zee, L W E van Heurn, Henkjan J Verkade, Jim C H Wilde, Jan B F Hulscher, Nederlandse Studiegroep voor Choledochus Cysten/malformaties (NeSCHoC)

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INTRODUCTION: A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood.

METHODS: All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (<30 days) and long-term (>30 days) complications were studied retrospectively.

RESULTS: Between January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0-17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up.

CONCLUSION: Surgery for CM carries a significant short- and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.

Original languageEnglish
Pages (from-to)2631-2637
Number of pages7
JournalWorld Journal of Surgery
Issue number10
Publication statusPublished - Oct 2017

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