Technical note on complete excision of choledochal cysts

Hong Kong, China

Technical note on complete excision of choledochal cysts

Tan To Cheung and Sheung Tat Fan

Hong Kong, China

BACKGROUND:Choledochal 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:Here we report on a reoperation treating incomplete excision of a choledochal cyst with focus on the technical aspect.

RESULTS:In 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:Careful 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)

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 fi rst few years of life but many cases are detected in early adulthood. Choledochal cysts are classif i ed according to Todani modif i cation of the Alonso-Lej classif i cation.[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 theproximal 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 conf i guration 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 fi lling 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.

Fig. 1.A: Magnetic resonance imaging showing the proximal part of the choledochal cyst (arrow);B: Magnetic resonance imaging showing the distal part of the choledochal cyst inside the head of the pancreas (arrow);C: Magnetic resonance cholangiopancreatography showing a narrowing at the middle of the choledochal cyst (arrow).

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 identif i ed just anterior to the liver hilum. The porta hepatis was dissected until the stump of the distal common bile duct was identif i ed. 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 fl ow 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 def i ne 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.

Fig. 2.A: Contrast computed tomography scan showing a residual choledochal cyst in the head of the pancreas with stones inside (arrow);B: Reconstruction computed tomography scan showing the intrapancreatic portion of the choledochal cyst (arrow).

Fig. 3.The choledochal cyst was identif i ed and the intrapancreatic portion was dissected out. A catheter was placed inside the choledochal cyst for cholangiography.

Fig. 4.A: Intraoperative cholangiogram showing the full conf i guration of the choledochal cyst and contrast fl owing freely into the duodenum via the distal portion of the common bile duct;B: Two large metal clips were applied to the lower transection line of the choledochal cyst (arrow).

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.

Fig. 5.The lower end of the choledochal cyst was cut open, exposing the pancreatic duct opening (arrow).

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 fi rst 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 fi rst operation.

To achieve complete excision of the lesion in areoperation 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 identif i cation of the main portal vein served as an important landmark for searching the truncated common hepatic duct located anteriorly. With the identif i cation of the proximal end of the lesion, operative cholangiography could be performed. It provided the precise anatomy of the operative fi eld, 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 fi stula 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 fi stula formation is controversial, it has been shown that it can effectively decrease the fi stula output and seems to aid fi stula healing.[13-15]

For huge and chronic choledochal cysts, complete excision is not always possible, and pericystic inf l ammation may lead to adhesion of underlying vascular structures including the portal vein. When diff i cult 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.

Contributors:CTT and FST drafted and revised the manuscript. FST approved the manuscript.

Funding:None.

Ethical approval:Not needed.

Competing interest:No benef i ts in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

1 Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst. Ann Surg 1970;172:844-851.

2 Howell CG, Templeton JM, Weiner S, Glassman M, Betts JM, Witzleben CL. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg 1983;18:387-393.

3 Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classif i cation, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263-269.

4 She WH, Chung HY, Lan LC, Wong KK, Saing H, Tam PK. Management of choledochal cyst: 30 years of experience and results in a single center. J Pediatr Surg 2009;44:2307-2311.

5 Yoshikawa K, Yoshida K, Shirai Y, Sato N, Kashima Y, Coutinho DS, et al. A case of carcinoma arising in the intrapancreatic terminal choledochus 12 years after primary excision of a giant choledochal cyst. Am J Gastroenterol 1986; 81:378-384.

6 Todani T, Narusue M, Watanabe Y, Tabuchi K, Okajima K. Management of congenital choledochal cyst with intrahepatic involvement. Ann Surg 1978;187:272-280.

7 Todani T, Urushihara N, Morotomi Y, Watanabe Y, Uemura S, Noda T, et al. Characteristics of choledochal cysts in neonates and early infants. Eur J Pediatr Surg 1995;5:143-145.

8 Liem NT, Dung le A, Son TN. Laparoscopic complete cyst excision and hepaticoduodenostomy for choledochal cyst: early results in 74 cases. J Laparoendosc Adv Surg Tech A 2009;19:S87-90.

9 Tan HL, Shankar KR, Ford WD. Laparoscopic resection of type I choledochal cyst. Surg Endosc 2003;17:1495.

10 Koshinaga T, Hoshino M, Inoue M, Gotoh H, Sugito K, Ikeda T, et al. Pancreatitis complicated with dilated choledochal remnant after congenital choledochal cyst excision. Pediatr Surg Int 2005;21:936-938.

11 Li MJ, Feng JX, Jin QF. Early complications after excision with hepaticoenterostomy for infants and children with choledochal cysts. Hepatobiliary Pancreat Dis Int 2002;1:281-284.

12 Fujisaki S, Akiyama T, Miyake H, Amano S, Tomita R, Fukuzawa M, et al. A case of carcinoma associated with the remained intrapancreatic biliary tract 17 years after the primary excision of a choledochal cyst. Hepatogastroenterology 1999;46:1655-1659.

13 Gans SL, van Westreenen HL, Kiewiet JJ, Rauws EA, Gouma DJ, Boermeester MA. Systematic review and meta-analysis of somatostatin analogues for the treatment of pancreatic fi stula. Br J Surg 2012;99:754-760.

14 Gurusamy KS, Koti R, Fusai G, Davidson BR. Somatostatin analogues for pancreatic surgery. Cochrane Database Syst Rev 2010;CD008370.

15 Gayral F, Campion JP, Regimbeau JM, Blumberg J, Maisonobe P, Topart P, et al. Randomized, placebocontrolled, double-blind study of the eff i cacy of lanreotide 30 mg PR in the treatment of pancreatic and enterocutaneous fi stulae. Ann Surg 2009;250:872-877.

16 Hay SA. Laparoscopic mucosectomy for large choledochal cyst. J Laparoendosc Adv Surg Tech A 2008;18:783-784.

Received June 28, 2012

Accepted after revision August 29, 2012

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10.1016/S1499-3872(13)60036-3)

AuthorAff i liations: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

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