Choledochal Cyst: Varied Clinical Presentations and Long-Term Results of Surgery

Abstract

The clinical features and long-term outcome of 21 children with choledochal cyst treated over a 31-year-period is reviewed. All 7 infants ( < 1-year-old) presented with jaundice. Only 3 older children (14%) presented with the classical triad of pain, jaundice and an abdominal mass. Other forms of presentation included pancreatitis (n = 8), cholangitis (n = 3), biliary peritonitis (n = 2) and biliary cirrhosis (n = 2). An incidental diagnosis of a choledochal cyst was made in 3 patients one each with bilateral ureteroceles, renal hypoplasia and meningitis with hepatitis A infection. An elevated serum amylase (SA: mean = 1005 U/L) and intraoperative bile amylase (BA: mean = 16,902 U/L) was observed in all 8 children with pancreatitis. Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy was the primary operative procedure in 18 patients. The remaining patients underwent cystoduodenostomy (n = 2) and cystojejunostomy (n = 1). Recurrent cholangitis and stricture formation complicated cyst enterostomies. In comparison cyst excision with Roux-en-Y hepaticojejunostomy gave excellent long-term results with minimal complication.

A Rare Cause of Neonatal Cholestasis: Type IVA Choledochal Cyst

“…Ayrıca, ilk bir ay içinde opere edilen hastalarda cerrahinin etkinliğinin, bir aydan sonra opere edilenlere göre daha iyi olduğu saptanmıştır (11) . Dolayısıyla koledok kistlerinin erken tanı ve tedavisi önem göstermektedir.…”

Management Dilemmas With Choledochal Cysts

“…This has been reported to be the presenting feature in 10% of children in one series, 34 and in biopsies obtained during surgery, it has been found to occur in 40% to 50% of cases. 37,74 In one series, liver changes, including bile duct proliferation, cholestasis, parenchymal damage, inflammatory cell infiltration, and pericentral fibrosis, were present in all livers on which biopsies were performed, irrespective of liver function and macroscopic liver appearance.…”

“…34,[46][47][48] Among adults, choledochal cysts are quite often incidental findings during investigation for other problems. 34,[49][50][51] Rarer presentations include gastric outlet obstruction, 42 neonatal bleeding tendency, 52 duodenal intusussecption, 53 and portal hypertension. 54 The presentations of choledochal cysts are clearly protean, and prompt diagnosis relies on a high index of suspicion, aided by modern imaging techniques.…”

“…3,17,[33][34][35] It is more commonly seen in cases with onset in childhood rather than in adults, and 85% of children have at least 2 features of the triad at presentation, compared with only 25% of adults. 36 Antenatal diagnoses can be made on ultrasound, 37 although diagnostic accuracy from this technique has been reported to be as low as 15%, 38 and it is not possible to differentiate between biliary atresia and choledochal cysts with antenatal ultrasound.…”

“…Among the more commonly reported presenting features are cholangitis, 34,42,43 pancreatitis, 34,[43][44][45] and biliary peritonitis from cyst rupture. 34,[46][47][48] Among adults, choledochal cysts are quite often incidental findings during investigation for other problems.…”