eMedicine Specialties > Radiology > Gastrointestinal

Choledochal Cyst: Follow-up

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): Manish K Varma, MD, Chief of Interventional Radiology, Department of Radiology, Tripler Army Medical Center; Thomas F Murphy, MD, Chief of Abdominal Imaging Section, Department of Radiology, Tripler Army Medical Center
Contributor Information and Disclosures

Updated: Jul 30, 2009

Intervention

The treatment for choledochal cysts is surgical. The treatment of choice for a type I choledochal cyst is complete excision of the cyst with construction of a Roux-en-Y biliary-enteric anastomosis to restore biliary continuity with the gastrointestinal tract.42

Surgical specimen.

Surgical specimen.

Surgical specimen.

Surgical specimen.



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

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

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

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


 
 

Type II choledochal cysts can usually be excised entirely, and the defect in the common bile duct can be closed primarily over a T-tube. This approach can be used because, typically, type II choledochal cysts are lateral diverticula of the bile duct.

Therapy for type III choledochal cysts, or choledochoceles, depends on the size of the lesion. Choledochoceles with a diameter of 3 cm or less may be approached endoscopically and effectively treated by means of sphincterotomy. Choledochoceles larger than 3 cm in diameter are often associated with some degree of duodenal obstruction. These cysts are excised surgically by using a transduodenal approach. If the pancreatic duct is found to be entering the choledochocele, it must be reimplanted into the duodenum after the cyst is excised.

For type IV choledochal cysts, the dilatated extrahepatic duct is completely excised, and a Roux-en-Y biliary-enteric anastomosis procedure is performed. No therapy is specifically directed at the intrahepatic ductal disease, except if intrahepatic ductal strictures, hepatolithiasis, or hepatic abscesses are present. In these patients, interventional radiologic techniques can be performed. If the disease is limited to specific hepatic segments or a lobe, these may be resected.

A type V choledochal cyst, or Caroli disease, is defined only by the dilatation of the intrahepatic ducts. If dilatation is limited to a single hepatic lobe, usually the left, the affected lobe is resected. Patients who have bilobar disease and signs of biliary cirrhosis, portal hypertension, or liver failure may be candidates for liver transplantation.

Recently, laparoscopic techniques have been successfully applied to the management of choledochal cysts. Good results have been achieved in both pediatric and adult populations and reported by several groups.43,44,45

A case report of embolization of a ruptured hepatic artery pseudoaneurysm was reported (see Angiography). Angiographic techniques with embolization of bleeding vessels seem feasible and can be used in these situations.

Gulati and colleagues described the use of US-guided percutaneous choledochal cystostomy in 2 infants.46 The children presented with severe hepatic function abnormalities, and 1 child had cholangitis. Choledochal cystostomy was successfully performed in each patient to decompress the biliary system until the infants were ready to undergo surgery.

For patients with choledochal cysts, bile duct stone formation or proteinaceous debris collection in the bile ducts is not uncommon because of poor bile flow. Kaneko and co-investigators described their results in connection with external biliary drainage in patients with choledochal cysts and protracted abdominal pain, jaundice, and vomiting.47 Simple external drainage effected resolution of symptoms in several patients. Filling defects were found in the distal bile duct in 22 patients. These resolved spontaneously or with irrigation in 19 patients (86%). In the remaining 3 patients, material in the bile duct was examined after surgery. It was composed mainly of proteinaceous debris (98%). The authors concluded that symptoms and biliary dysfunction are frequently caused by protein plugs or debris in patients with choledochal cysts. They proposed temporary external biliary drainage and bile duct irrigation as a therapeutic strategy to relieve the signs and symptoms.

Patients with choledochal cysts require lifelong follow-up monitoring. They remain at increased risk for development of cholangiocarcinoma, even after complete excision of the cyst.

Medicolegal Pitfalls

  • Misdiagnosis or the inability to make a diagnosis is associated with medical/legal pitfalls. The key to avoiding the pitfalls is not to place complete reliance on a single test or diagnostic modality.
  • Patients with choledochal cysts have a significant and potentially life-threatening anomaly of the biliary tract. With the possible exception of Caroli disease, surgery is always indicated and is the definitive therapy. Detailed information is required to plan the surgical approach.
  • Radiologists, gastroenterologists, and surgeons should work as a team in treating patients with choledochal cyst. The diagnostic workup should not cease until all are satisfied that enough information is available for operative planning. Such information usually includes data derived through a combination of US and CT scanning, MRI/MRCP, and ERCP.

Special Concerns

  • The first major special concern is the neonate presenting with prolonged jaundice. The workup should proceed expeditiously in these patients, especially to investigate the diagnosis of biliary atresia. Rapid identification of this anomaly and corrective surgical therapy are essential to providing an optimal outcome.
  • If a choledochal cyst is identified, definitive surgical therapy should be performed as soon as feasible. Progressive damage to the bile ducts and to the liver parenchyma is well described in association with these anomalies.
  • An association exists between APBJ and choledochal cysts, cholangiocarcinoma, and carcinoma of the gallbladder. The potential exists for imaging modalities to miss small cancers of the bile ducts and gallbladder; however, definitive surgery should be planned in all patients and should include resection of incidental lesions.
 


More on Choledochal Cyst

Overview: Choledochal Cyst
Imaging: Choledochal Cyst
Follow-up: Choledochal Cyst
Multimedia: Choledochal Cyst
References
Further Reading

References

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Keywords

choledochal cyst, bile duct cysts, Caroli's disease, congenital anomalies of the bile ducts, cystic dilatation, extrahepatic biliary tree, intrahepatic biliary radicles, choledochocele, cystic dilation, cyst dilation, cyst dilatation

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)

Manish K Varma, MD, Chief of Interventional Radiology, Department of Radiology, Tripler Army Medical Center
Manish K Varma, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

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

Medical Editor

Neela Lamki, MD, Professor, Department of Radiology, Sultan Qaboos University, Oman; Adjunct Professor, Department of Radiology, Baylor College of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Abraham H Dachman, MD, FACR, Professor, Department of Radiology, The University of Chicago School of Medicine; Director of CT, Department of Radiology, The University of Chicago Hospitals
Abraham H Dachman, MD, FACR is a member of the following medical societies: Radiological Society of North America
Disclosure: iCAD, Inc. Consulting fee Consulting; iCAD, Inc. Grant/research funds Other; GE Healtcare, Inc. Honoraria Speaking and teaching

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

John Karani, MBBS, FRCR, Clinical Director of Radiology and Consultant Radiologist, Department of Radiology, King's College Hospital, London
John Karani, MBBS, FRCR is a member of the following medical societies: British Institute of Radiology, British Society of Interventional Radiology, Cardiovascular and Interventional Radiological Society of Europe, European Society of Gastrointestinal and Abdominal Radiology, European Society of Radiology, Radiological Society of North America, and Royal College of Radiologists
Disclosure: Nothing to disclose.

 
 
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