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Choledochal Malformation in Children: Lessons Learned from a Dutch National Study
, Maria H A van den Eijnden1, Ruben H J de Kleine2
1Department of Pediatric Surgery, University of Groningen, University Medical Center Groningen, Hanzeplein 1, HPC BA20 Postbus 30.001, 9700RB, Groningen, The Netherlands.
Insights
Surgery for choledochal malformations (CM) in children has significant short- and long-term complications. Specialized pediatric centers are recommended for these rare hepatobiliary surgeries.
Area of Science:
- Pediatric Surgery
- Hepatobiliary Surgery
- Congenital Malformations
Background:
- Choledochal malformation (CM) is a rare congenital anomaly, particularly in Western populations.
- Understanding the incidence and surgical outcomes of CM in the Netherlands is crucial for improving patient care.
Purpose of the Study:
- To determine the incidence of choledochal malformation (CM) in the Netherlands.
- To evaluate the surgical outcomes and complications associated with CM in pediatric patients.
Main Methods:
- Retrospective analysis of pediatric patients (types I-IV CM) who underwent surgery between 1989 and 2014.
- Exclusion of type V CM cases.
- Data collection on symptoms, surgical details, and short-term (<30 days) and long-term (>30 days) complications.
Main Results:
- 91 pediatric patients underwent surgery for CM at a median age of 2.1 years.
- Short-term complications (biliary leakage, cholangitis) occurred in 22% of patients.
- Long-term complications included cholangitis (13%) and anastomotic stricture (4%); surgery before 1 year and laparoscopic approach were associated with increased long-term complications.
Conclusions:
- Surgery for CM is associated with significant short- and long-term morbidity.
- Hepatobiliary surgery for CM, especially laparoscopic procedures, should be centralized in experienced pediatric surgical centers due to the low incidence and potential for complications.
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.
