Perforation: a rare complication of choledochal cysts in children.

Purpose: Spontaneous perforation is a rare complication of choledochal cyst (CDC) which is difficult to diagnose due to its nonspecific clinical presentation. The surgical treatment can be either single-staged cyst excision or two-staged procedure with an initial drainage followed by delayed cyst excision. Both biliary duct obstruction and irritation due to refluxed pancreatic juice have been proposed as possible aetiology. In this report, we describe six cases of CDC perforation in hope to have a better understanding on the clinical features and cause of this complication.

Methods: Medical records of six patients whose diagnoses of perforated CDC were confirmed with intra-operative findings were retrospectively reviewed. Clinical data, investigation results and post-operative outcomes were analysed.

Results: Vomiting and abdominal pain were the most common complaints. Few patients present with clinical jaundice. Overt sign of peritonitis was absent. Both single-staged and two-staged approach offered satisfactory outcome.

Conclusion: Hyperbilirubinaemia and remote free intra-peritoneal fluid allude the diagnosis of perforated CDC. When presenting with cholangitis, it warrants timely surgical intervention to prevent perforation. Single-staged or two-staged surgical approach would depend on stability of patient and surgical expertise available. Reversible dilatation of intra-hepatic duct suggests that increased intra-ductal pressure is a contributing factor to the perforation.