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Author: Michael AJ Sawyer, MD, Director, Videoendoscopic Surgical Institute of Oklahoma, Consulting Staff, Department of Surgery, Comanche County Memorial Hospital; Consulting Staff, Great Plains Surgical Clinic, Lawton, Oklahoma

Michael AJ Sawyer 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

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

Editors: Mounzer Al Al Samman, MD, Department of Internal Medicine, Division of Gastroenterology, Assistant Professor, Texas Tech University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; BS Anand, MD, Department of Internal Medicine, Division of Gastroenterology, Professor, Baylor University College of Medicine; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; 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

Author and Editor Disclosure

Synonyms and related keywords: bile duct cysts, congenital bile duct anomalies, biliary tree, extrahepatic biliary radicles, intrahepatic biliary radicles, upper abdominal mass, jaundice, Caroli disease, choledochocele, acholic stools

Background

Choledochal cysts are congenital bile duct anomalies. These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic biliary radicles, or both. In 1723, Vater and Ezler published the anatomical description of a choledochal cyst. Douglas wrote the clinical report involving a 17-year-old girl presenting with jaundice, fever, intermittent abdominal pain, and an abdominal mass.3 The patient died a month after an attempt at percutaneous drainage of the mass.

In 1959, Alonzo-Lej produced a systematic analysis of choledochal cysts, reporting on 96 cases. He devised a classification system, dividing choledochal cysts into 3 categories, and outlined therapeutic strategies. Todani has since refined this classification system to include 5 categories. This article reviews the incidence, pathophysiology, diagnosis, and management of choledochal cysts.

Pathophysiology

No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably multifactorial. In many patients with choledochal cysts, an anomalous junction between the common bile duct and the pancreatic duct can be demonstrated. This occurs when the pancreatic duct empties into the common bile duct more than 1 cm proximal to the ampulla. Some series, such as the one published by Miyano and Yamataka in 1997, have documented such anomalous junctions in 90-100% of patients with choledochal cysts.16 This abnormal union allows pancreatic secretions to reflux into the common bile duct, where the pancreatic proenzymes become activated, damaging and weakening the bile duct wall. Defects in epithelialization and recanalization of the developing bile ducts and congenital weakness of the ductal wall also have been implicated. The result is formation of a choledochal cyst.

These anomalies are classified according to the system published by Todani and coworkers. Five major classes of choledochal cysts exist (ie, types I-V), with subclassifications for types I and IV (ie, types IA, IB, IC; types IVA, IVB).

  • Type I cysts are the most common and represent 80-90% of choledochal cysts. They consist of saccular or fusiform dilatations of the common bile duct, which involve either a segment of the duct or the entire duct.
  • Type IA is saccular in configuration and involves either the entire extrahepatic bile duct or the majority of it.
  • Type IB is saccular and involves a limited segment of the bile duct.
  • Type IC is more fusiform in configuration and involves most or all of the extrahepatic bile duct.
  • Type II choledochal cysts appear as an isolated diverticulum protruding from the wall of the common bile duct. The cyst may be joined to the common bile duct by a narrow stalk.
  • Type III choledochal cysts arise from the intraduodenal portion of the common bile duct and are described alternately by the term choledochocele.
  • Type IVA cysts consist of multiple dilatations of the intrahepatic and extrahepatic bile ducts. Type IVB choledochal cysts are multiple dilatations involving only the extrahepatic bile ducts.
  • Type V (Caroli disease) consists of multiple dilatations limited to the intrahepatic bile ducts.

Frequency

United States

Choledochal cysts are relatively rare in Western countries. Reported frequency rates range from 1 case per 100,000-150,000 to 1 case per 2 million live births.

International

Choledochal cysts are much more prevalent in Asia than in Western countries. Approximately 33-50% of reported cases come from Japan, where the frequency in some series approaches 1 case per 1000 population (as described by Miyano and Yamataka).16

Mortality/Morbidity

The morbidities associated with choledochal cysts depend on the age of the patient at the time of presentation. Infants and children may develop pancreatitis, cholangitis, and histologic evidence of hepatocellular damage. Adults in whom subclinical ductal inflammation and biliary stasis may have been present for years may present with one or more severe complications, such as hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis. Cholangiocarcinoma is the most feared complication of choledochal cysts, with a reported incidence of 9-28%. Wu and colleagues recently exposed cells from a cholangiocarcinoma cell line to bile from patients with choledochal cysts and from controls with structurally normal biliary systems.29 The bile from the patients with choledochal cysts produced significantly more mitogenic activity in the cancer cell line than the bile from the controls.

Sex

Choledochal cysts are more prevalent in females than males, with a female-to-male ratio in the range of 3:1 to 4:1.

Age

Most patients with choledochal cysts are diagnosed during infancy or childhood, although the condition may be discovered at any age. Approximately 67% of patients present with signs or symptoms referable to the cyst before the age of 10 years.



History

The history varies according to the age at presentation. Choledochal cysts can present dramatically in infancy. The clinical manifestations in older children and adults are more protean.

  • Infants
    • Infants frequently present with jaundice and acholic stools. In early infancy, this may prompt a workup for biliary atresia.
    • In addition, infants with choledochal cysts often have a palpable mass in the right upper quadrant of the abdomen, accompanied with hepatomegaly.
  • Children
    • Children diagnosed after infancy typically have a clinical picture of intermittent biliary obstruction or recurrent bouts of pancreatitis.
    • Those with a biliary obstructive pattern can still present with a palpable right upper quadrant mass and jaundice.
    • Children whose primary manifestation is pancreatitis may pose some difficulty in arriving at the correct diagnosis. These patients frequently have only intermittent attacks of colicky abdominal pain. Biochemical testing reveals elevated amylase and lipase concentrations, which lead to the proper diagnostic workup.
  • Adults
    • Adults with choledochal cysts can present with one or more severe complications.
    • Frequently, adults with choledochal cysts complain of vague epigastric or right upper quadrant pain and can develop jaundice or cholangitis.
    • The most common symptom in adults is abdominal pain.
    • A classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass has been described in adults with choledochal cysts but is found in only 10-20% of patients.

Physical

A right upper quadrant mass may be palpable. This is observed more frequently in infancy and early childhood. Patients who develop pancreatitis present with nonspecific midepigastric or diffuse abdominal pain.

Causes

No unifying etiologic theory exists for choledochal cysts. The pathogenesis probably is multifactorial.



Bile Duct Tumors
Biliary Obstruction
Cholangiocarcinoma
Pancreatitis, Acute


Lab Studies

  • CBC count: No laboratory studies are specific for the diagnosis of a choledochal cyst. An elevated white blood cell count with increased numbers of neutrophils and immature neutrophil forms may be observed in the presence of cholangitis.
  • Liver function tests may be useful in narrowing the differential diagnosis. Hepatocellular enzyme and alkaline phosphatase levels may be elevated. None of these tests are specific for the diagnosis of a choledochal cyst.
  • Serum amylase and lipase concentrations may be increased in the presence of pancreatitis. Serum amylase concentrations also may be elevated in biliary obstruction and cholangitis.
  • Results of serum chemistry may be abnormal if the patient is vomiting. One might expect to see a hypochloremic, hypokalemic metabolic alkalosis in this clinical picture.

Imaging Studies

  • Abdominal ultrasonography is the test of choice for the diagnosis of a choledochal cyst. Ultrasound is useful in the antenatal period as well, according to Chen and coworkers, and can demonstrate a choledochal cyst in a fetus as early as the beginning of the second trimester.1 Caroli disease has also been detected antenatally with ultrasound by Sgro and colleagues.22
  • Abdominal CT scan and MRI help to delineate the anatomy of the lesion and the surrounding structures. These tests also can assist in defining the presence and extent of intrahepatic ductal involvement. Yu and associates published a series of 64 patients in whom magnetic resonance cholangiopancreatography (MRCP) was particularly valuable in defining anomalous pancreaticobiliary junctions.31
  • Fitoz and colleagues recently described the use of MRCP in 17 children with pancreatobiliary anomalies.6 The reported diagnostic accuracy of MRCP in choledochal cysts in this series was 100%.
  • Invasive diagnostic studies: When noninvasive measures (eg, ultrasound, CT scan, MRI) fail to sufficiently delineate the anatomy, they should be supplemented by the addition of percutaneous transhepatic cholangiography (PTC) or endoscopic retrograde cholangiopancreatography (ERCP). As reported by Nagi and coworkers, these studies are particularly helpful in demonstrating the presence of an anomalous pancreatobiliary junction and in delineating associated extrahepatic or intrahepatic strictures and stones.17

Histologic Findings

Evidence of chronic inflammation is typically observed in the cyst wall. The cyst wall is thin, fibrous, and frequently devoid of a true epithelial surface, although it can be lined by a low columnar epithelium. Note that infants can develop complete obstruction of the distal common bile duct secondary to acute and chronic inflammatory changes. In the liver, ductal fibrosis and portal edema may be present. Changes consistent with biliary cirrhosis may be observed in adults with long-standing disease. The most feared histologic abnormality is the presence of cholangiocarcinoma.



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

The positive results of proper surgical treatment were reinforced by Visser and colleagues.25 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.23 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.13 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).10 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.27 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.14 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.28 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.
  • 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.



Further Outpatient Care

  • Patients need lifelong follow-up because of the increased risk of cholangiocarcinoma, even after complete excision of the cyst.

Complications

  • Patients undergoing excision of a choledochal cyst are subject to the usual complications associated with surgery, including hemorrhage, wound infection, bowel obstruction, and thrombotic complications.
  • Postoperatively, patients are at risk of developing pancreatitis and ascending cholangitis.
  • Late postoperative complications include development of intrahepatic bile duct stones and cholangiocarcinoma.
  • Adult patients with long-standing subclinical ductal inflammation and biliary stasis may develop one or more of the following complications: hepatic abscesses, cirrhosis, portal hypertension, recurrent pancreatitis, and cholelithiasis.

Prognosis

Patient Education



Medical/Legal Pitfalls

  • The major medicolegal pitfall associated with choledochal cysts is failure to diagnose them. Many patients have only subtle manifestations of the disease early in its course, and the clinical presentation may consist solely of intermittent bouts of right upper quadrant abdominal pain or unexplained, self-limited jaundice.
  • However, highly sensitive noninvasive screening and diagnostic modalities, such as ultrasound, are commonly available. Findings are extremely accurate and aid in the diagnosis. Ultrasound has been used to diagnose this condition prenatally.
  • Therefore, it should be extremely rare for patients with choledochal cysts to present with numerous repeated bouts of biliary colic, jaundice, or cholangitis before a definitive diagnosis is made. A proper level of clinical suspicion and the application of diagnostic tests, such as ultrasound, should make it relatively easy to diagnose this entity before development of its most dreaded complications, including cirrhosis and cholangiocarcinoma. 



Media file 1:  Type I choledochal cyst.
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Media file 2:  Type II choledochal cyst.
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Media file 3:  Type III choledochal cyst (choledochocele).
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Media file 4:  Type IV choledochal cyst (extrahepatic and intrahepatic disease).
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Media file 5:  Type V choledochal cyst (intrahepatic, Caroli disease).
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Media file 6:  Nuclear medicine scan of choledochal cyst.
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Media file 7:  Nuclear medicine scan of choledochal cyst.
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Media file 8:  Nuclear medicine scan of choledochal cyst.
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Media file 9:  CT scan of choledochal cyst demonstrating intrahepatic extension involving the main left hepatic duct.
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Media file 10:  CT scan of choledochal cyst involving the common hepatic duct.
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Media file 11:  CT scan demonstrating large choledochal cyst and adjacent gall bladder.
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Media file 12:  CT scan of large, saccular type I choledochal cyst.
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Media file 13:  Diagnostic ultrasound demonstrating type I choledochal cyst in a 4-month-old child presenting with hyperbilirubinemia and transaminase elevations.
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Media file 14:  Intraoperative cholangiogram of type I choledochal cyst.
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Media file 15:  Operative specimen of type I choledochal cyst.
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Media file 16:  Roux-en-Y hepaticojejunostomy to restore biliary-enteric continuity following resection of choledochal cyst.
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Choledochal Cysts excerpt

Article Last Updated: Jun 8, 2007