Choledochal cyst in pediatric patients: a 10-year single institution experience

Choledochal cysts (CCs) are rare congenital dilatations of the biliar tree. The incidence is 1:150,000. A correct diagnosis and surgical treatment are important because of the long term risks of infection and neoplasia. We report our experience with CCs in children.

Patients with CC disease, who were seen at the Hospital Italiano de Buenos Aires between 1999 and 2009, were identified retrospectively from our prospectively acquired hepato-pancreatico-biliary database. Prenatal, clinical, surgical and pathological records were analysed.

We included 12 children (10 girls and 2 boys), all of them affected by a cystic dilatation type I of Todani classification. Between the 4 patients with antenatal diagnosis only one presented symptoms before surgery, expressed in acolic feces. Between the 8 patients with postnatal diagnosis, the most common clinical presentation was abdominal pain, occurring in all patients. Ultrasound was used for the diagnosis of all patients. Mean age at surgery was 74 months. All patients had intraoperative cholangiograms to definitively confirm the diagnosis. Five patients underwent open surgery and 7 laparoscopic approach. From patients with a laparoscopic approach, 1 had to be converted to open surgery due to intraoperative complications and 2 underwent an initial surgery for a cholecystectomy and a CC cyst excision. The complications observed were: anastomotic biliary fistula 1, residual lithiasis in the pancreatic portion of the duct 1 and anastomotic stenosis 1. The last 2 cases required reoperation.

Once CCs are diagnosed, careful treatment decisions need to be taken because both intra hepatic and extra hepatic CCs, if untreated, have an increased risk of cancer. After a careful study of the cyst's characteristics, we recommend a laparoscopic approach when possible, as the best choice, after 3 months of life to increase the success of the treatment according to the size of the anatomic structure. We recommend early treatment, before 3 months of life, only in case of severe symptoms.