Choledochal Cysts

Author: Michael AJ Sawyer, MD; Chief Editor: Julian Katz, MD

Practice Essentials
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.
Signs and symptoms
Infants with choledochal cysts can present dramatically with the following:
Jaundice and acholic stools: In early infancy, may prompt workup for biliary atresia
Palpable mass in the right upper quadrant of the abdomen, with hepatomegaly
The clinical manifestations in older children and adults are variable. Children diagnosed with
choledochal cysts after infancy typically present with intermittent biliary obstruction or recurrent bouts
of pancreatitis with the following features:
Biliary obstructive pattern: Palpable right upper quadrant mass and jaundice
Primary manifestation of pancreatitis: May pose diagnostic difficulty; patients frequently have only
intermittent attacks of colicky abdominal pain (elevated amylase and lipase concentrations lead to
the proper diagnostic workup)
The classic triad in adults with choledochal cysts is an abdominal pain, jaundice, and palpable right
upper quadrant abdominal mass. However, this triad is found in only 10-20% of patients.
Adults may present with the following:
Abdominal pain: Most common symptom
Vague epigastric or right upper quadrant pain; can develop jaundice or cholangitis
One or more severe complications (eg, hepatic abscesses, cirrhosis, portal hypertension, recurrent
pancreatitis, cholelithiasis)
See Clinical Presentation for more detail.
Diagnosis
Testing
No laboratory studies are specific for the diagnosis of a choledochal cyst, but some may be used to
narrow the differential diagnosis. The following tests may be helpful:
Complete blood count with differential: Elevated white blood cell count with increased numbers of
neutrophils and immature neutrophil forms may be noted in the presence of cholangitis
Liver function studies: Elevated hepatocellular enzyme and alkaline phosphatase levels are
nonspecific for choledochal cysts
Serum amylase and lipase levels: Both may be elevated in the presence of pancreatitis, but they
can also be elevated in the presence of biliary obstruction and cholangitis
Serum chemistry levels: Results may be abnormal if patient is vomiting (hypochloremic,
hypokalemic metabolic alkalosis)
Imaging studies
The following imaging studies may be used to assess patients with suspected choledochal cysts:
Abdominal ultrasonography: Test of choice for the diagnosis of a choledochal cyst; can be useful for
antenatal diagnosis
[1, 2]

Abdominal computed tomography (CT) scanning and magnetic resonance imaging (MRI): Help to
delineate the anatomy of the lesion and the surrounding structures; can also assist in defining the
presence and extent of intrahepatic ductal involvement
Magnetic resonance cholangiopancreatography (MRCP): Useful for defining anomalous
pancreatobiliary junctions
[3]
and pancreatobiliary anomalies
[4]

Procedures
Percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde
cholangiopancreatography (ERCP) are used to supplement the above noninvasive imaging studies
when those studies fail to sufficiently delineate the relevant anatomy.
See Workup for more detail.
Management
Surgery
The treatment of choice for choledochal cysts is complete excision with construction of a biliary-
enteric anastomosis to restore continuity with the gastrointestinal tract.
[5, 6]

The surgical management for each choledochal cyst type is described as follows:
Type I: 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
[7]

Type II: Complete excision of the dilated diverticulum comprising a type II choledochal cyst; the
resultant defect in the common bile duct is closed over a T-tube
Type III (choledochocele): Therapeutic choice depends on the size of the cyst; choledochoceles
measuring 3 cm or less can be treated effectively with endoscopic sphincterotomy, whereas lesions
larger than 3 cm (which typically produce some degree of duodenal obstruction) are excised
surgically via a transduodenal approachif the pancreatic duct enters the choledochocele,
reimplantation into the duodenum may be required following excision of the cyst
Type IV: Complete excision of the dilated extrahepatic duct, followed by a Roux-en-Y
hepaticojejunostomy to restore continuity; intrahepatic ductal disease does not require dedicated
therapy unless hepatolithiasis, intrahepatic ductal strictures, and hepatic abscesses are present (in
such instances, resection of the affected hepatic segment or lobe is performed)
Type V (Caroli disease): Hepatic lobectomy for disease limited to one hepatic lobe (left lobe usually
affected); however, carefully examine hepatic functional reserve before committing to such therapy;
patients with bilobar disease manifesting signs of liver failure, biliary cirrhosis, or portal hypertension
may require liver transplantation
Lilly technique: When the cyst adheres densely to the portal vein secondary to long-standing
inflammatory reaction, it may not be possible to perform a complete, full-thickness excision of the
cyst; the Lilly technique allows the serosal surface of the duct to be left adhering to the portal vein,
while the mucosa of the cyst wall is obliterated by curettage or cauterytheoretically, this removes
the risk of malignant transformation in that segment of the duct
Supportive measures
No medical therapy specifically targets the etiology of choledochal cysts, nor is any drug or any type
of nonsurgical modality curative. Patients with choledochal cysts who present at a late stage (ie, after
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.
Patients who present with cholangitis should be treated with broad-spectrum antibiotic therapy
directed against common biliary pathogens, such asEscherichia coli and Klebsiella species, in
addition to other supportive measures, such as volume resuscitation.
See Treatment and Medication for more detail.
[#Treatment] Image library
Operative specimen of type I choledochal cyst.
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.
[8]
The patient died a month after an attempt at percutaneous drainage of the mass.
(See image below.)
Operative specimen of type I choledochal cyst.
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.
Recent research
In a retrospective analysis of 32 children and 47 adults with choledochal cysts, Shah et al investigated
the differences between these 2 groups with regard to the presentation, management, and
histopathology of, as well as the outcomes related to, these lesions.
[9]
The following were among the
authors' findings:
A history of biliary surgery, pancreatitis, cholangitis, early postoperative complications, and late
postoperative complications occurred, respectively, 5.1, 5.4, 6.4, 2.0, and 3.3 times more frequently
in adults than they did in children.
The classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass
occurred 6.7 times more frequently in children than in adults.
Fibrosis of the cyst wall was peculiar to children.
Signs of inflammation and hyperplasia were primarily seen in adults.
Long-term complications occurred in 29.7% of adults but in only 9.3% of children.
The authors concluded that because of such differences, choledochal cysts in children should be
considered separate entities from those in adults.
Pathophysiology
No strong unifying etiologic theory exists for choledochal cysts. The pathogenesis is probably
multifactorial.
[10]
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 commonbile 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.
[11]
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 (see image below) 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 I choledochal cyst.
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 (see image below) 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 II choledochal cyst.
Type III choledochal cysts (see image below) arise from the intraduodenal portion of the common
bile duct and are described alternately by the term choledochocele. Type
III choledochal cyst (choledochocele).
Type IVA cysts (see image below) 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 IV choledochal cyst (extrahepatic and intrahepatic disease).
Type V (Caroli disease) cysts (see image below) consist of multiple dilatations limited to the
intrahepatic bile ducts.
[11]
Type V choledochal cyst (intrahepatic, Caroli disease).
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).
[11]

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 exposed cells from a cholangiocarcinoma cell line to bile from patients
with choledochal cysts and from controls with structurally normal biliary systems.
[12]
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.
[9]

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
o Infants frequently present with jaundice and acholic stools. In early infancy, this may prompt a
workup for biliary atresia.
o In addition, infants with choledochal cysts often have a palpable mass in the right upper quadrant
of the abdomen, accompanied with hepatomegaly.
Children
o Children diagnosed after infancy typically have a clinical picture of intermittent biliary obstruction or
recurrent bouts of pancreatitis.
o Those with a biliary obstructive pattern can still present with a palpable right upper quadrant mass
and jaundice.
o 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
o Adults with choledochal cysts can present with one or more severe complications.
o Frequently, adults with choledochal cysts complain of vague epigastric or right upper quadrant
pain and can develop jaundice or cholangitis.
o The most common symptom in adults is abdominal pain.
o 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. The images below depict nuclear medicine scans of choledochal cysts.
Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst.
Nuclear medicine scan of choledochal cyst.
Causes
No unifying etiologic theory exists for choledochal cysts. The pathogenesis probably is multifactorial.
Differential Diagnoses
Bile Duct Tumors
Biliary Obstruction
Cholangiocarcinoma
Pancreatitis, Acute

Laboratory 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.
[2]
(See image below.)
Diagnostic ultrasonogram demonstrating type I choledochal cyst in a 4-month-
old child presenting with hyperbilirubinemia and transaminase elevations.
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.
[3]
(See images below.) Computed tomography (CT) scan of
choledochal cyst demonstrating intrahepatic extension involving the main left hepatic duct.
Computed tomography (CT) scan of choledochal cyst involving the common
hepatic duct. Computed tomography (CT) scan demonstrating large choledochal
cyst and adjacent gall bladder. Computed tomography (CT) scan of large,
saccular type I choledochal cyst.
Fitoz and colleagues described the use of MRCP in 17 children with pancreatobiliary anomalies.
[4]
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.
[13]

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 asEscherichia 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.
[5, 6]
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.
[14, 15]

The positive results of proper surgical treatment were reinforced by Visser and colleagues.
[16]
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.
[17]
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. See a review of 79 cases by Mukhopadhyay et
al.
[18]

Lee and associates reported 3 cases of laparoscopic choledochal cyst excision and Roux-en-Y
reconstruction in children.
[19]
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).
[20]
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.
[21]
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.
[22]
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.
[23]
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.
[7]
(See
images below.) Intraoperative cholangiogram of type I choledochal cyst.
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.

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
The prognosis after excision of a choledochal cyst is usually excellent (see Further Outpatient Care).
Patient Education
For excellent patient education resources, visit eMedicineHealth's Digestive Disorders Center. Also,
see eMedicineHealth's patient education articlesGallstones, Pancreatitis, Cirrhosis, and Abdominal
Pain in Adults.