Technical note on complete excision of choledochal cysts

Tan To Cheung and Sheung Tat Fan
Hong Kong, China
 
Author Affiliations: Department of Surgery (Cheung TT and Fan ST) and State Key Laboratory for Liver Research (Fan ST), The University of Hong Kong, Hong Kong, China
Corresponding Author: Sheung Tat Fan, MD, Department of Surgery, The University of Hong Kong, 102 Pokfulam Road, Hong Kong, China (Tel: 852-22554703; Fax: 852-29865262; Email: stfan@hku.hk)
 
© 2013, Hepatobiliary Pancreat Dis Int. All rights reserved.
doi: 10.1016/S1499-3872(13)60035-1
 
Contributors: CTT and FST drafted and revised the manuscript. FST approved the manuscript.
Funding: None.
Ethical approval: Not needed.
Competing interest: No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.
 
 
BACKGROUND: holedochal cysts are congenital cystic dilatations of the extrahepatic or intrahepatic portion of the biliary tree. Complete excision of choledochal cysts is currently regarded as the gold standard treatment, while less extensive procedures including cystoduodenostomy have become obsolete due to the potential for malignant change in the remnant cyst. For type-1 choledochal cysts, which sometimes extend to the main pancreatic duct closely, some surgeons may adopt a less aggressive approach in order to avoid damage to the main pancreatic duct as such damage can lead to serious consequences. However, incomplete excision of choledochal cysts may also cause problems.
 
METHOD: ere we report on a reoperation treating incomplete excision of a choledochal cyst with focus on the technical aspect.
 
RESULTS: n the reoperation, meticulous dissection of the liver hilum which had been previously operated on was performed. The hepaticojejunostomy was left intact. With the assistance of intraoperative cholangiography, the residual pancreatic portion of the choledochal cyst was completely excised. The pancreatic opening and the lower end of the common bile duct were reconstructed. Whipple operation was avoided.
 
CONCLUSION: areful planning with the aid of precise imaging before and during the operation largely enhanced the accuracy of the excision of the choledochal cyst.
 
(Hepatobiliary Pancreat Dis Int 2013;12:218-221)
 
KEY WORDS: choledochal cyst; re-excision; incomplete excision
 
 
Introduction
Choledochal cysts are a congenital condition commonly found in the young population. The incidence is around 1 in 5000 with a high frequency in Asia.[1, 2] The diagnosis is usually made in the first few years of life but many cases are detected in early adulthood. Choledochal cysts are classified according to Todani modification of the Alonso-Lej classification.[3] Type-1 choledochal cysts are the most common presentation, which account for 67.9% of all cases.[4] Apart from Caroli disease (type-5 choledochal cysts) which may require liver transplantation, choledochal cysts are best treated with complete excision, followed by hepaticojejunostomy when technically feasible. It is well recognized that patients with choledochal cysts have a higher risk of cholangiocarcinoma, and cystojejunostomy (or cystoduodenostomy), which was once a popular operation, has become an inferior treatment option in the modern era.[5] Excision of choledochal cysts can be a technically challenging operation, particularly when the lesion is intrahepatic or involves the distal end of the common bile duct. Intrahepatic choledochal cysts may require a partial or an anatomical hepatectomy for complete removal.[6] Lesions involving the pancreas are even more challenging, as injury to the main pancreatic duct may lead to serious complications. Surgeons who do not handle this condition frequently may try to avoid dissection towards the pancreatic duct, but incomplete excision may lead to problematic symptoms posing the risk of malignancy and cause dense adhesion that hinders subsequent operation.
 
 
Case report
The patient was a 27-year-old female who presented with epigastric pain. She had no jaundice but her serum alkaline phosphatase level was 540 µmol/L. Magnetic resonance imaging of the liver revealed a type-1 choledochal cyst 3×3.5×7 cm in size involving the proximal bile duct (Fig. 1A) and the pancreatic portion of the distal common bile duct (Fig. 1B). Magnetic resonance cholangiogram showed a tapering of the distal common bile duct making virtually a two-chamber configuration of the choledochal cyst (Fig. 1C). Open excision of the choledochal cyst and hepaticojejunostomy were performed in another hospital. She had uneventful recovery and was discharged a week after the operation.
 
She developed recurrent episodes of epigastric pain, fever, chills and rigors two months after the operation. She was referred to our hospital for further management. Computed tomography showed a 3×3.5×3 cm residual component of choledochal cyst at the distal common bile duct with filling defects compatible with stones (Fig. 2A). Reconstruction computed tomography showed that the lesion was mainly inside the head of the pancreas (Fig. 2B). Complete excision of the residual choledochal cyst was indicated due to the symptoms and potential of malignancy.
 
A right subcostal incision was made along the original wound. Adhesions resulting from the previous operation were taken down. The afferent and efferent limbs of hepaticojejunostomy were identified just anterior to the liver hilum. The porta hepatis was dissected until the stump of the distal common bile duct was identified. The distal component of the choledochal cyst was dissected out from the head of the pancreas until the insertion of the main pancreatic duct was seen. A catheter was inserted into the cyst for operative cholangiography (Fig. 3). Fifteen mL of contrast was instilled into the choledochal cyst and the lower margin of the lesion was shown. Free flow of contrast into the duodenum was observed (Fig. 4A). The distal margin of the lesion was marked by Liga clips (Fig. 4B) in order to define the exact site of bile duct resection. The choledochal cyst was removed by cutting at the lowest end of the common bile duct where the main pancreatic duct opening was also exposed (Fig. 5). The common bile duct stump was closed with 5/0 Prolene suture in one single layer and no pancreatic stent was placed across it.
 
The patient was kept nil by mouth with total parenteral nutrition support for one week. Somatostatin infusion was administered at 250 mcg per hour in the initial postoperative period to minimize pancreatic secretion.
 
Histological examination showed cystic dilatation of the common bile duct compatible with choledochal cyst. There was no evidence of malignancy. The patient made an uneventful recovery and did not complain of any recurrent symptoms after the second operation.
 
 
Discussion
Patients with choledochal cysts can present with jaundice, deranged liver function and cholangitis, which usually lead to early diagnosis in their childhood or even neonatal period.[7] However, the classical triad of presentations is rare and patients may not notice the presence of the disease until their adulthood.
 
Type-1 choledochal cysts can now be excised with the laparoscopic approach although it is technically demanding.[8, 9] The open approach to choledochal cyst excision remains widely practiced although it also requires a lot of expertise. The short-term complication rate for choledochal cyst excision is around 5%. The risk of recurrent cholangitis, pancreatitis, hepaticojejunostomy stricture and intrahepatic stone formation is around 16%.[4, 10] The risk of malignancy after complete excision of a choledochal cyst is minimal,[4, 11] while the remnant of an incompletely excised choledochal cyst still poses a threat of cancer formation.[12] In the case reported herein, it appeared that the incomplete excision of a type-1 choledochal cyst caused recurrent pancreatitis. However, there was the possibility that a co-existing type-3 choledochal cyst that had been left behind from the first operation presented itself as an intrapancreatic lesion.
 
In achieving complete excision, preoperative imaging and planning are crucial. Endoscopic retrograde cholangiopancreatography used to be the gold standard for examination of the biliary system, but with the development of modern technology, magnetic resonance cholangiopancreatography can actually provide images of very high quality without posing the risk of complications from instrumentation. In the present case, magnetic resonance cholangiopancreatography nicely demonstrated the presence of narrowing at the middle common bile duct which separated a single lesion into two components. If this was known beforehand, perhaps the lesion could have been completely excised in the first operation.
 
To achieve complete excision of the lesion in a reoperation setting is a more complicated issue. Since the intrapancreatic portion of the lesion was 3 cm, Whipple operation was a backup option during the operation. Meticulous dissection of the liver hilum without destruction of hepaticojejunostomy was possible. The identification of the main portal vein served as an important landmark for searching the truncated common hepatic duct located anteriorly. With the identification of the proximal end of the lesion, operative cholangiography could be performed. It provided the precise anatomy of the operative field, so a clear margin could be achieved. We did not put in a pancreatic stent across the distal common bile duct stump because there was adequate margin for repair. Alternatively, a 4-Fr pancreatic stent could be deployed to reduce the risk of pancreatic fistula and then be retrieved by endoscopy afterwards. There is a disadvantage of routine placement of pancreatic stent as the stent may sometimes occlude the pancreatic duct, leading to pancreatitis in the early postoperative period. Although the role of somatostatin analogue in pancreatic fistula formation is controversial, it has been shown that it can effectively decrease the fistula output and seems to aid fistula healing.[13-15]
 
For huge and chronic choledochal cysts, complete excision is not always possible, and pericystic inflammation may lead to adhesion of underlying vascular structures including the portal vein. When difficult dissection is anticipated, partial excision of the cyst with mucosectomy of the remaining portion can be employed. The outer wall of the remnant cyst can be left adherent to the portal vascular structure without causing symptoms.[16]
 
Choledochal cyst excision can be a very challenging operation due to the vital structures in the surroundings. Careful planning before operation and the use of operative cholangiography can assure complete excision of the lesions.
 
 
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Received June 28, 2012
Accepted after revision August 29, 2012