Choledochal Cyst: Complete Resection Improves Outcomes

Cystic dilatation of the bile duct—usually called choledochal cyst (CC)—is a rare condition that causes numerous complications (eg, cystolithiasis, cholangitis, acute pancreatitis, hepatolithiasis, portal hypertension, and chronic pancreatitis). Up to 30% of CC patients develop cholangiocarcinoma if their conditions are untreated. Asians and women are at highest risk.

Residual pancreatic bile duct cysts often occur if an initial radical resection isn’t complete. The journal Surgery published a study describing treatment of remnant intrapancreatic choledochal cysts (CCs) in adults. It indicates that the best surgical approach is complete resection.

Remnant cysts often lead to cholangitis when reflux of the duodenal juice in the cyst allows bacterial entry and infection. Fewer activated pancreatic enzyme also contribute. If pancreatitis develops, pancreatitis and cholangitis create a vicious cycle. This 12-year, single site, retrospective study included patients with intrapancreatic remnant CCs (N=41) who underwent reoperation. The investigators looked for postoperative complications, surgical outcomes, and malignancies.

After their initial surgical intervention, all patients experienced cholangitis. Sixty-six percent developed cholangiolithiasis, and more than one-half pancreatitis and vomiting.

Thirty-five patients, mean age 49 years, remained cancer-free. Most of these patients (74%) had had a Todani type I cyst and had undergone previously subtotal extrahepatic cyst excision and Roux-en-Y hepaticochojejunostomy.

Among particiapnts, 6 patients or 15% experienced malignant transformation with the average time to diagnosis 140 months from the original incomplete CC excision. These patients tended to be just slightly older, with mean age 51 years. Four of these patients underwent pancreatoduodenectomy, and 2 underwent palliative surgery because the lesions were invading adjacent tissues.

After excision of the remnant cyst, 91% of patients had excellent or good outcomes.

Mortality was high: 5 patients died of cancer within approximately 3 years of reoperation.

The investigators acknowledge that excision of the intrapancreatic portion of a CC is a difficult procedure, but stress that complete resection is critical to improve outcomes.