Scientific paper
Some considerations for management of choledochal cysts†

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Abstract

Background

There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults.

Methods

We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures.

Results

There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst.

Conclusions

Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy.

Section snippets

Patients

Our management of 16 patients with choledochal cysts was based primarily on a review of the literature. Nine had type I (diffuse dilation of the extrahepatic bile ducts), 1 had type II (saccular diverticulum of the common duct), 1 had type III (choledochocele within the wall of the duodenum (Fig. 2), 3 had type IV-A (similar to type I, but in addition intrahepatic ducts are involved), 1 had type IV-B (multiple dilatations of the extrahepatic ducts and normal intrahepatic ducts), and 1 had type

Results

Diagnosis of choledochal cysts was most simply achieved by US examination; ERCP and percutaneous transhepatic cholangiography were more definitive in displaying ductal anatomy and the existence of an anomalous pancreatic bile duct junction. An anomalous pancreaticobiliary duct junction with a long common channel was identified in 56% (9 of 16) of our patients and 53% of those of Chaney et al [10]. The ductal junction was normal in 2 patients. Demonstration of the pancreaticobiliary junction was

Comments

Most observers agree that choledochal cysts are associated with an abnormal communication between the common bile duct and the pancreatic duct that allows reflux of pancreatic juice into the bile duct [9]. Injury of the bile ducts by activated pancreatic juice is presumed to cause their dilatation. We documented an abnormal junction in 9 patients. All patients with anomalous pancreaticobiliary duct junction do not have a choledochal cyst [13], but 93% of choledochal cysts are associated with an

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    †This study originally appeared in the March issue (Am J Surg 2004;187:434–439) and has been reprinted with corrections.

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