eMedicine Specialties > Pediatrics: Surgery > General Surgery

Choledochal Cyst: Surgical Perspective: Treatment

Author: Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Coauthor(s): Heather N Paddock, MD, Pediatric Surgery Fellow, Department of Pediatric Surgery, University of Florida; Luong Tuyen Nguyen, MD, Associate Professor, Department of Surgery, Department of Pediatric General Surgery, McGill University; Consulting Surgeon, Montreal Children's Hospital; Saundra M Kay, MD, FRCSC, Consulting Staff, Rocky Mountain Pediatric Surgery
Contributor Information and Disclosures

Updated: Sep 30, 2008

Treatment

Surgical Therapy

Treatment of choledochal cysts is surgical, except in type V multiple intrahepatic cysts, which can benefit from medical management for variable periods of time. In the past, operative aspiration and external drainage were used extensively because most patients were quite sick, and a simple quick procedure was convenient. These external drainage procedures of the biliary tree were unsuccessful because of numerous complications, including repeated cholangitis and biliary fistulae. Mortality rates were high.3,50,51,5,8,7 Today, in the setting of acute severe disease, percutaneous cholecystostomy drainage can be performed prior to the definitive procedure. This is safe and generally well tolerated; however, it is not necessary in most patients.

Internal drainage, either with cystoduodenostomy or cystojejunostomy with Roux-en-Y biliary reconstruction, was used in the past. These procedures left the cyst behind, and the free reflux of pancreatic enzymes into the cyst via the anomalous pancreaticobiliary junction resulted in a high incidence of calculi, recurrent cholangitis, anastomotic strictures, and carcinoma arising from the cyst. Of patients treated with either cystoduodenostomy or cystojejunostomy, 65% remained symptomatic, and 40% required repeat surgery at a later date.52,53 Recurrent cholangitis and chronic inflammation in the remaining cyst eventually produces metaplasia that leads to malignant transformation.

Total excision of the cyst in types I, II, and IV followed by reconstruction of the biliary tree with hepaticojejunostomy in a Roux-en-Y fashion has been widely accepted as the procedure of choice in treating choledochal cysts and has been found to be superior to hepaticoduodenostomy.54 This procedure implies excision of the distal common bile duct (CBD). Consequently, it blocks the reflux of pancreatic enzymes into the biliary tract, thereby decreasing the incidence of carcinoma of the bile duct.

Total excision of the cyst is possible in virtually all infants and young children. In older patients with repeated cholangitis and marked pericystic inflammation, this disease may be best managed with resection of the anterolateral part of the cyst followed by an endocystic resection of the lining, leaving the back wall adjacent to the portal vein in place, as reported by Lilly in 1977.55 This technique also appears to be most useful in patients who have previously undergone cystoenterostomy and who require repeat surgery because of recurrent cholangitis. This technique makes the dissection less hazardous.

Several groups have successfully performed laparoscopic-assisted and laparoscopic total cyst excision with Roux-en-Y hepatoenterostomy with complication rates comparable to those of the open procedure. Li and colleagues (2004) performed laparoscopic cyst excision with laparoscopic-assisted Roux-en-Y hepatoenterostomy in 35 children (33 cyst type, 2 fusiform) without conversion to the open procedure and with postoperative stays of 3-5 days.56 The procedure is described in detail for both choledochal cyst and biliary atresia surgery by Martinez-Ferro et al (2005).57

Intraoperative cholangiography obtained via puncture of the cyst or via the gallbladder is always obtained. It outlines the exact anatomy of the choledochal cyst and its relationship with the pancreas. Cholecystectomy is routinely performed at the same time.

Biliary reconstruction can be performed with a Roux-en-Y hepaticojejunostomy as high as possible, near the hilum of the liver.58,51,5,24 Some authors, including Raffensperger and Shamberger, have interposed a reversed segment of jejunum to prevent reflux.59,60,40 This idea has not been universally accepted. No stents are routinely necessary.

With regard to type II choledochal cysts, a simple excision of the diverticulum with ductoplasty for reconstruction of the CBD is all that is required. Laparoscopic excision has been successfully performed in this rather rare disease.61

With type III choledochal cysts, the general approach is one of lateral duodenotomy with unroofing of the choledochocele to drain the bile duct and pancreatic duct directly into the duodenum. The two ductal openings should be carefully examined to determine whether ductoplasty is required.51,22,62

In patients with type IV choledochal cysts with intrahepatic cysts, each case is individually evaluated, and the principle of adequate bile drainage is taken into account. Excision of the dilated extrahepatic bile ducts as far as the porta hepatis, with hepaticojejunostomy at the level of the hilum, may provide good biliary drainage and effective decompression of the intrahepatic cysts. If the intrahepatic cysts are localized in a small portion of the liver, partial hepatectomy may be required.63,64,9

With regard to type V choledochal cysts, patients with localized disease may benefit from a hepatic lobectomy. If the disease is diffuse, involving both lobes of the liver, treatment is palliative and liver transplantation may be required.51,7,65,21

Complications

Complications after surgery have been mainly observed with types I, IV, and V choledochal cysts. They are much less common in excisional procedures. The overall morbidity rate is less than 10%. Mortality and repeat surgery rates are low after excision, compared with those associated with internal drainage operations.

Postsurgical complications include the following:

  • Cholangitis
    • In 1996, Miyano et al reported a 2.3% incidence of cholangitis after cyst excision.24
    • In 1995, Todani et al found cholangitis in 10 patients in their 25-year review of 97 patients who underwent cyst excision.27
    • In contrast, Chijiiwa et al (in 1993 and 1994) found that 88% of patients who previously underwent cystoenterostomy had cholangitis.52,53
  • Biliary stone formation
    • In 1993 and 1994, Chijiiwa et al reported a 25% rate of choledocholithiasis and a 33% rate of hepatolithiasis after cystoenterostomy.52,53
    • In 1997, Yamataka et al reported stone formation in 3 of 18 patients who underwent previous cyst excision.66 Two patients had stones at the porta hepatis, and one patient had stones at both the porta hepatis and within the left lobe of the liver.
    • Stones can be observed in the intrapancreatic bile duct. Usually, cholangitis and stone formation are observed in the same patient. These complications are thought to result from many factors, including the following:
      • Stricture of the anastomosis
      • Residual debris in the intrahepatic bile duct
      • Dilated intrahepatic ducts, especially in type IV and type V choledochal cysts
  • Anastomotic stricture
    • Apart from technical errors, anastomotic strictures may be a progressive phenomenon after surgery.
    • In a 1993 report, Hata et al found a 4.1% rate of anastomotic strictures.58 The diameter of an adequate anastomosis is usually reduced by 20-30% after a few weeks.
    • Such a reduction in the anastomotic diameter may result from excessive devascularization of the duct during dissection. A wide anastomosis as far as the hepatic hilum may prevent anastomotic stricture.
  • Residual debris in the intrahepatic bile ducts: Residual debris is commonly observed in older patients. Debris left within the intrahepatic duct or pancreatic duct during cyst excision may be responsible for postexcisional stone formation and pancreatitis.53,51,63,29
  • Intrahepatic bile duct dilatation: Dilatation usually regresses after cyst excision and hepaticojejunostomy in young patients. In older patients and adults, this dilatation tends to persist. Dilatation and residual debris may cause cholangitis and stone formation.63,64 Some authors recommend endoscopic examination of the duct during surgery to clean out all the debris.67,68
  • Malignancy
    • The risk of carcinoma in the retained cyst approaches 50% in patients treated with cystoenterostomy and is approximately 20 times greater than in the general population.12,53,51,34
    • Total cyst excision had been promising in eliminating the risk of cancer development. However, sporadic cases of carcinoma in the intrahepatic ducts and distal common duct after complete cyst excision have been reported.63,69
    • In a 1997 report, Yamataka et al have recommended excision of the intrapancreatic terminal choledochus.66
    • With regard to intrahepatic ducts, adequate bile drainage may prevent malignant transformation.

In summary, total excision of the cyst with adequate bile drainage is the standard treatment for choledochal cysts. Long-term follow-up is necessary to detect any late complications, especially the development of malignancy.

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References
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Further Reading

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Keywords

choledochal cyst, cystic dilatation of the common bile duct, cystic dilatation of the CBD, cystic dilatation, biliary tract anomaly, cystic disease of the biliary tract, distal CBD obstruction, distal common bile duct obstruction, anomaly of the pancreaticobiliary junction, fibrosis, cirrhosis, portal hypertension, adenosquamous carcinoma, small cell carcinoma, cancer, biliary tract malignancy, jaundice, hepatomegaly, choledocholithiasis, cyst rupture, biliary carcinoma, Caroli disease

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Contributor Information and Disclosures

Author

Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Gail E Besner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Women's Association, American Pediatric Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, and Society of University Surgeons
Disclosure: Trillium Therapeutics, Inc. Consulting fee Consulting; Trillium Therapeutics, Inc. Grant/research funds Other

Coauthor(s)

Heather N Paddock, MD, Pediatric Surgery Fellow, Department of Pediatric Surgery, University of Florida
Disclosure: Nothing to disclose.

Luong Tuyen Nguyen, MD, Associate Professor, Department of Surgery, Department of Pediatric General Surgery, McGill University; Consulting Surgeon, Montreal Children's Hospital
Luong Tuyen Nguyen, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Saundra M Kay, MD, FRCSC, Consulting Staff, Rocky Mountain Pediatric Surgery
Saundra M Kay, MD, FRCSC is a member of the following medical societies: American College of Surgeons, American Pediatric Surgical Association, Canadian Association of Pediatric Surgeons, International Pediatric Endosurgery Group, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Rebeccah Brown, MD, Associate Director of Trauma Services, Associate Professor, Department of Clinical Surgery and Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati Hospital
Rebeccah Brown, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Harsh Grewal, MD, FACS, FAAP, Professor of Surgery and Pediatrics, Temple University School of Medicine; Chief, Section of Pediatric Surgery, Temple University Children's Medical Center
Harsh Grewal, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Surgical Education, Children's Oncology Group, Eastern Association for the Surgery of Trauma, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Laparoendoscopic Surgeons, and Southwestern Surgical Congress
Disclosure: Nothing to disclose.

 
 
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