eMedicine Specialties > Gastroenterology > Biliary

Choledochal Cysts: Treatment & Medication

Author: Michael AJ Sawyer, MD, Consulting Staff, Department of Surgery, Southwestern Medical Center; Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Inc
Coauthor(s): Tarak H Patel, MD, Consulting Surgeon, Department of Surgery, Reynolds Army Medical Center, Fort Sill; Thomas F Murphy, MD, Chief of Abdominal Imaging Section, Department of Radiology, Tripler Army Medical Center; Fernando V Ona, MD, Associate Professor, John A Burns School of Medicine, Phillipines; Chief, Center for Digestive and Liver Diseases, Nutrition, University of Hawaii; Professor, St Luke's College of Medicine, Veterans Administration Medical Center
Contributor Information and Disclosures

Updated: Nov 10, 2009

Treatment

Medical Care

The treatment of choice for choledochal cysts is complete excision. Appropriate antibiotic therapy and supportive care should be given to patients presenting with cholangitis. Patients who present at a late stage, after the development of advanced cirrhosis and portal hypertension, may not be good candidates for surgery because of the prohibitive morbidity and mortality rates associated with these comorbid conditions.

  • No medical therapy specifically targets the etiology of choledochal cysts, nor is any drug or any type of nonsurgical modality curative.
  • Patients who present with cholangitis should be treated with broad-spectrum antibiotic therapy directed against common biliary pathogens, such as Escherichia coli and Klebsiella species, in addition to other supportive measures, such as volume resuscitation.
  • Again, it must be emphasized that these means are supportive and that surgery is the only currently available definitive therapy.

Surgical Care

The treatment of choice for choledochal cysts is complete excision of the cyst with construction of a biliary-enteric anastomosis to restore continuity with the gastrointestinal tract. According to Jordan and associates, both partial resection of the cyst and internal drainage procedures expose patients to increased risks of cholangitis, pancreatitis, and cholangiocarcinoma.11,12

The positive results of proper surgical treatment were reinforced by Visser and colleagues.13 These investigators reported a series of 39 adult patients with choledochal cysts. Cholangiocarcinomas or gallbladder cancers were noted in 8 patients (21%) at the initial operation performed by the authors. Seven of these patients had previously undergone a partial cyst excision, drainage procedure, or expectant management. No cancer was noted during the follow-up care of the patients who underwent complete cyst excision.

Shimotakahara and coworkers compared Roux-en-Y hepaticojejunostomy to hepaticoduodenostomy for biliary reconstruction following choledochal cyst excision.14 The authors concluded that hepaticojejunostomy was a better choice because of an unacceptably high rate of duodenogastric bile reflux (33.3%) in the hepaticoduodenostomy group.

Lee and associates reported 3 cases of laparoscopic choledochal cyst excision and Roux-en-Y reconstruction in children.15 One was converted to open operation owing to involvement of the confluence of the lobar hepatic ducts. All children have done well postoperatively.

Jang and coauthors described their experience with laparoscopic surgical management of choledochal cysts in a series of 12 adult patients (mean age=37.3 y).16 Complete cyst excision and reconstruction via Roux-en-Y hepaticojejunostomy was accomplished in all patients using laparoscopic techniques. No mortalities and no anastomotic complications occurred. Mean operative time was 228 minutes. Patients were discharged from the hospital after an average stay of 5.8 days.

Reports of robotically-assisted laparoscopic resection of choledochal cysts are beginning to appear. Woo and colleagues reported such a case in the management of a 5-year-old child with a type I choledochal cyst.17 The cyst was excised successfully. The total robotic operative time was 390 minutes, and the time for the entire procedure was 440 minutes. No complications occurred. The patient was reported well after 6 months of follow-up.

Lee and colleagues reviewed their experience with choledochal cyst excision in 198 children to determine the benefit of operating early in the neonatal period.18 They found a lower complication rate and less hepatic fibrosis in neonates who underwent excision of a choledochal cyst within the first 30 days of life.

Woon and colleagues published their results with the management of choledochal cysts in adults.19 In this series of 32 patients, 84% underwent initial operation with complete cyst excision and Roux-en-Y hepaticojejunostomy. The remaining 16% had revisional surgery for incompletely resected cysts with hepaticojejunostomy. No mortalities occurred. The overall morbidity rate was 44%, with wound infection being most common (19%). Malignancy was found in only one specimen (3%). The authors emphasized the importance of treating cyst-associated complications, such as pancreatitis and sepsis, before attempting to define cyst anatomy with ERCP or MRCP. This aids in delineating the extent of involvement of the biliary tree and the exact type of choledochal cyst. Furthermore, they reiterated the importance of complete cyst excision and reconstruction with Roux-en-Y hepaticojejunostomy.

The surgical management for each choledochal cyst type is described below.

  • Type I: The treatment of choice is complete excision of the involved portion of the extrahepatic bile duct. A Roux-en-Y hepaticojejunostomy is performed to restore biliary-enteric continuity.20 (See images below and Images 15-16.)

  • Intraoperative cholangiogram of type I choledocha...

    Intraoperative cholangiogram of type I choledochal cyst.

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    Intraoperative cholangiogram of type I choledocha...

    Intraoperative cholangiogram of type I choledochal cyst.


  • Roux-en-Y hepaticojejunostomy to restore biliary-...

    Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of choledochal cyst.

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    Roux-en-Y hepaticojejunostomy to restore biliary-...

    Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of choledochal cyst.

  • Type II: The dilated diverticulum comprising a type II choledochal cyst is excised in its entirety. The resultant defect in the common bile duct is closed over a T-tube.
  • Type III (choledochocele): The choice of therapy depends upon the size the cyst. Choledochoceles measuring 3 cm or less can be treated effectively with endoscopic sphincterotomy. Lesions larger than 3 cm typically produce some degree of duodenal obstruction. These lesions are excised surgically through a transduodenal approach. If the pancreatic duct enters the choledochocele, it may have to be reimplanted into the duodenum following excision of the cyst.
  • Type IV: The dilated extrahepatic duct is completely excised and a Roux-en-Y hepaticojejunostomy is performed to restore continuity. Intrahepatic ductal disease does not require dedicated therapy unless hepatolithiasis, intrahepatic ductal strictures, and hepatic abscesses are present. In such instances, the affected segment or lobe of the liver is resected.
  • Type V (Caroli disease): Disease limited to one hepatic lobe is amenable to treatment by hepatic lobectomy. When this occurs, the left lobe usually is affected. Hepatic functional reserve should be examined carefully in all patients before committing to such therapy. Patients with bilobar disease who begin to manifest signs of liver failure, biliary cirrhosis, or portal hypertension may be candidates for liver transplantation.
  • Lilly technique: Occasionally, the cyst adheres densely to the portal vein secondary to long-standing inflammatory reaction. In this situation, a complete, full-thickness excision of the cyst may not be possible. In the Lilly technique, the serosal surface of the duct is left adhering to the portal vein, while the mucosa of the cyst wall is obliterated by curettage or cautery. Theoretically, this removes the risk of malignant transformation in that segment of the duct.

Consultations

Primary care physicians who encounter a patient with a choledochal cyst should consult a surgeon.

More on Choledochal Cysts

Overview: Choledochal Cysts
Differential Diagnoses & Workup: Choledochal Cysts
Treatment & Medication: Choledochal Cysts
Follow-up: Choledochal Cysts
Multimedia: Choledochal Cysts
References
Further Reading
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References

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Further Reading

Clinical guideline

 ASGE guideline: the role of ERCP in diseases of the biliary tract and the pancreas.
American Society for Gastrointestinal Endoscopy - Medical Specialty Society. 2005 Jul. 8 pages. NGC:004486

Related eMedicine topics

 Choledochal Cyst (Radiology)

Choledochal Cyst, Surgical Treatment (Pediatrics: Surgery)

Cholestasis

Gallbladder Disease

Caroli Disease

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Keywords

choledochal cyst, bile duct, jaundice, bile duct cysts, bile ducts, biliary tree, Caroli disease, choledochal cysts, congenital bile duct anomalies, extrahepatic biliary radicles, intrahepatic biliary radicles, upper abdominal mass, choledochocele, acholic stools

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Contributor Information and Disclosures

Author

Michael AJ Sawyer, MD, Consulting Staff, Department of Surgery, Southwestern Medical Center; Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Inc
Michael AJ Sawyer, MD is a member of the following medical societies: American College of Surgeons, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, and Society of Laparoendoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Tarak H Patel, MD, Consulting Surgeon, Department of Surgery, Reynolds Army Medical Center, Fort Sill
Disclosure: Nothing to disclose.

Thomas F Murphy, MD, Chief of Abdominal Imaging Section, Department of Radiology, Tripler Army Medical Center
Disclosure: Nothing to disclose.

Fernando V Ona, MD, Associate Professor, John A Burns School of Medicine, Phillipines; Chief, Center for Digestive and Liver Diseases, Nutrition, University of Hawaii; Professor, St Luke's College of Medicine, Veterans Administration Medical Center
Fernando V Ona, MD is a member of the following medical societies: American Gastroenterological Association
Disclosure: Nothing to disclose.

Medical Editor

Mounzer Al Al Samman, MD, Department of Internal Medicine, Division of Gastroenterology, Assistant Professor, Texas Tech University School of Medicine
Mounzer Al Al Samman, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

BS Anand, MD, Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine
BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

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