Cir Ciruj 2010;78:57-61

Choledochal cysts in adults

Jos Luis Martnez-Ordaz and Javier Nio-Sols
Abstract
Background: Choledochal cysts are usually diagnosed during childhood. They mainly affect females. Abdominal pain
and jaundice are the two most common symptoms. Our objective was to report the experience of a third-level referral
center in the management of choledochal cysts in adults.
Methods: We performed a 17-year retrospective review of patients with choledochal cysts who underwent surgical interventions in a gastrointestinal surgery department.
Results: There were 23 patients with a median age of 26 years; 16 were females. The most frequent symptoms were
abdominal pain and jaundice. Only four patients had an abdominal mass. The most frequently used diagnostic studies
were abdominal sonography and endoscopic retrograde cholangiopancreatography. According to the classification of
Todani, most cysts were type I and were found in 17 patients, two patients had type II, three patients had type III (choledocele) and one patient had type IVA. Eighteen patients underwent complete removal of the cyst with a concomitant
hepatic-jejunostomy, three patients had transduodenal sphincteroplasty and two patients had only cystectomy. Seven
patients developed postoperative complications, representing a morbidity rate of 35%. None of the patients died during
the first 30 postoperative days. Cholangiocarcinoma was found in the resected specimen in only one case.
Conclusions: The surgery of choice for choledochal cysts in the adult is hepatojejunostomy , which has a low surgical
morbidity and mortality.
Key words: choledochal cyst, common bile duct diseases.

Introduction
Choledochal cyst is a rare disease, and is reported mainly in
the Japanese literature. Its incidence varies from one in 50
thousand to one in 2 million live births, mainly in females.1-8
There are two theories about their formation: a) congenital
stenosis that results in distention, b) maljunction between
the bile duct and pancreatic duct with resulting pancreatic reflux, increased choledochal intraluminal pressure and
distention.9 In 1959, Alonso-Lej10 published and proposed
a classification based on a review of 94 cases; however, the
one most used currently is by Todani et al. in 1977, who
Servicio de Gastrociruga, Hospital de Especialidades, Centro Mdico
Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mxico, D. F.
Correspondence and reprint requests to:
Jos Luis Martnez-Ordaz
Servicio de Gastrociruga
Hospital de Especialidades , Centro Mdico Nacional Siglo XXI
Av. Cuauhtmoc 330, tercer piso, Col. Doctores
06725 Mxico D.F.
Tel.: (55) 5627 6900, ext 21529 and 21531
E-mail: jlmo1968@hotmail.com
Received for publication: 1-28-2009
Accepted for publication: 10-28-2009

divided it into five types:1 I: saccular or cystic dilatation of

the extrahepatic bile duct, II: choledochal diverticulum, III:
dilatation of the intraduodenal portion of the bile duct (choledochocele), IV-A: multiple intra and extrahepatic cysts,
IVB: multiple extrahepatic cysts, V: intrahepatic cysts. The
most common are type I and type IV.1,3-4 ,6-8, 11,12
Approximately 40% of cases involve patients >10 years
of age and the risk of complications, particularly malignancy, increases with age.13 Symptoms that provoke most
patients to seek evaluation are very vague (the most common are pain and jaundice). In a large number of adults,
diagnosis is an independent finding because it is rarely suspected at first consultation. The most common laboratory
results are elevated total and direct bilirubin and transaminases and alkaline phosphatase in 60% of cases. Up to one
third of patients have elevated amylase.5 7
Some patients, mainly adults, experience some complications, cholangitis and pancreatitis being the most
frequent, as well as biliary cirrhosis, portal hypertension,
rupture and malignant degeneration. In addition, ~ 25% of
patients had gallstones or bile duct at the time of diagnosis,4,7,12,14-18 which is based mainly on results from imaging
studies. Ultrasound (US) and computed tomography (CT) of
the abdomen suggest the diagnosis by demonstrating dilated
bile duct, and both studies are complementary.7,19,20 Nuclear

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Martinez-Ordaz and Nio-Solis

magnetic resonance (NMR) has been used with good to very

good results; however, there are certain limitations in the diagnosis of small cysts or small ductal abnormalities.21 Endoscopic
retrograde cholangiopancreatography (ERCP), transhepatic
percutaneous cholangiography (TPC) or NMR is the study that
provides the most information. It is very important for diagnosis
and for surgical planning. It shows the type of cyst, intra- or extrahepatic involvement and abnormality of the pancreatobiliary
junction.3,4,6,7,15,20 Treatment is surgical for the most part and depends on the type of cyst. Due to the histopathological changes,
the best approach is biliodigestive resection with anastomosis:
the single drain is associated with development of complications
and does not decrease the malignancy potential of the disease.3-5
The aim of this study is to report the experience in managing this
pathology in adult patients in our department

Materials and Methods

We performed a retrospective, descriptive, observational and
transversal study of patients who had a diagnosis of choledochal cysts managed initially in the Gastrociruga Service,
Hospital de Especialidades, Centro Medico Nacional Siglo
XXI, Instituto Mexicano del Seguro Social (IMSS).
There were a total of 23 patients with this diagnosis between
January 1, 1991 and December 31, 2007. In all patients, definitive management was conducted in our department. None
of the patients were removed or excluded from this review. We
obtained demographic data (age, sex) and data corresponding
with the clinical picture and diagnostic studies performed. Studies included hepatobiliary US, CT scan of abdomen, ERCP
and NMR, in selected cases. All were classified according to
the classification of choledochal cysts by Todani.1
If patients required surgical treatment, the surgical procedure was performed and the evolution and postoperative
complications were recorded. A biliary fistula was defined as
the output of bile through the drain that persisted for more
than a week postoperatively. Patient follow-up after hospital discharge was done in the outpatient service. During
follow-up, laboratory studies were conducted (liver function
tests) and imaging (especially US). The clinical suspicion of
anastomotic stenosis was based on the patients clinical picture (fever, right upper quadrant pain or jaundice) and was
confirmed with radioisotope cholangiogammagraphy and
cholangioresonance NM.
The results are shown as frequency and percentages. Due
to sample size and results it was not possible to make a comparison between patients.

Results
During the study period there were 32,809 patients admitted to the service of the Hospital of Gastrocirugas. Of

these, 23 were diagnosed with bile duct cysts, of which 16

(70%) were women. Average age was 26 years (range: 1643 years). The main symptom was abdominal pain in 20
patients (87%), jaundice in 13 patients (57%), nausea and
vomiting in eight patients (35%), right upper quadrant tumor in four patients (17%) and fever in three patients (13%).
Four patients (17%) had the triad of jaundice, abdominal
pain and palpable mass. Symptoms had a median history of
10 months (range: 3-24 months) (Table 1).
Diagnostic methods most used were abdominal US in
23 patients (100%) patients, abdominal CT in 17 patients
(74%), endoscopic retrograde cholangiopancreatography
in 18 patients (78%) patients (Figure 1) and NMR in five
patients (22%). According to the Todani classification, 17
patients (74%) were type I, two patients (8%) were type II,
three patients (13%) were type III and one patient was type
IV-A. There were no cases of Carolis disease.
All patients underwent elective surgery. The decision to
perform surgery was made after the diagnosis was confirmed, along with the patients clinical picture. Patients with
type I and type IV cysts were treated with cyst resection
and Roux-en-Y hepatojejunal anastomosis (Figure 2). In
the two patients with type II, the cyst was removed and patients with type III (choledochocele) underwent a duodenal
sphincteroplasty. Surgical management usually involves the
placement of an open or closed drain adjacent to the anastomosis. The patients received different antibiotic schemes
during the study period. The most frequently used antibiotics were second- or third-generation cephalosporins or a
quinolone. Postoperative complications were as follows:
three patients (13%) patients had biliary fistula secondary
to leakage of the hepatojejunum anastomosis, necessitating
surgical reintervention for repair. Evolution was towards
improvement and patients were discharged without complications . Three patients (13%) presented data of cholangitis,
managed medically (antibiotics and ursodeoxycholic acid),
two patients (8%) demonstrated pancreatitis (considered
postoperative) and was resolved conservatively. One patient
(4%) had a duodenal lesion during surgery that was repaired
with primary closure. During the postoperative period the
patient developed a duodenal fistula and was managed by
fasting and parenteral nutrition with spontaneous closure in
2 weeks. There were no patient deaths during the first 30
days postoperatively, so there was no operative mortality.
Pathology report for 20 patients who underwent resection
was choledochal cyst. In a 42-year-old male a cholangiocarcinoma was found, which was not suspected during
preoperative evaluation or surgical intervention. Complete
resection of the cyst with hepatojejunal anastomosis was
possible, without complications. Postoperative evolution
was uneventful but the patient died after two months with
evidence of a liver metastasis.

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Choledochal cysts

Excluding this patient, follow-up was on average of

4 years (range: 1-12 years). During follow-up, patients
underwent liver function tests (transaminases, alkaline
phosphatase, -glutamyl transpeptidase and bilirubin) and
US. For suspected dysfunction of the anastomosis (mainly
based on clinical signs of fever or jaundice) the patient was
evaluated with US, radioisotope cholangiogammagraphy
and more recently with MR cholangiography, with the last
two being elective studies to determine the permeability of
the anastomosis. Two patients developed cholangitis that
was managed with hydration and medical treatment with
antibiotics, without complications.

Discussion
Most patients with choledochal cysts are diagnosed during
the first decade of life.19,22 The most common symptoms are
right upper abdominal pain and jaundice. Other symptoms
include nausea, vomiting, fever and weight loss. During
physical examination, it is possible to find an abdominal
tumor in the right upper quadrant.4-8,11,14,15,19,20,23 The classic
triad of abdominal pain, jaundice and tumor is present in
<15% of adult patients 3-7,20,23 and we found this triad in 17%
of the patients. In fact, only 25% of adult patients demonstrate two signs compared with 85% of pediatric patients,
possible due to delayed diagnosis.7 In most cases (>60%),
symptom evolution is >1year.3
Cholangiography is important for diagnosis and surgical
planning. It shows the type of cyst, intra- and extrahepatic involvement, and abnormality of the pancreatobiliary
junction.3-4 ,6-7,15.20 In most patients there is an abnormal

pancreatic-bile duct junction. It is considered abnormal

when the common duct on joining both bile and pancreatic ducts is >15 mm or the junction is 6 mm outside the
wall of the duodenum. With this distance, the junction is
beyond the sphincter of Oddi and this results in pancreatic
reflux, bile duct inflammation and increased pressure.4,24,25
The frequency with which patients have this abnormality
is 80-100%3,4,20,26,27 and is also responsible for the high incidence of pancreatitis.7 In addition, 25% of patients show
some other anatomic abnormalities such as primary biliary
stricture, aberrant ducts, arterial abnormalities, etc.4,28
Histologically, all adult patients have abnormal bile duct
mucosa. The epithelium is ulcerated and eroded (probably due to the presence of activated pancreatic enzymes).
Dense connective tissue and inflamed smooth muscle and
polymorphonuclear infiltrates are found within the abdominal wall as well as decrease of mucosal glands of the
bile duct. The areas without mucosa are responsible for
stenosis and occlusion.2-3,7,11,23,27 All of these alterations
have been found in the mucosa of adult patients. In pediatric cases metaplasia and dysplasia are uncommon.26 The
presence of cancer in the cyst is reported in <1% in pediatric patients and between 10 and 30% in adults, even up to
50% for patients >50 years of age who underwent internal
drainage.1-3, 5,22,14,29-31 Survival is ~12 months once the diagnosis is made.30 Factors involved in carcinogenesis are
chronic inflammation, bile stasis, possible development of
carcinogens and decrease of mucosal glands in the bile
duct.26, 32 Type I seems to be most frequently associated
with cancer, followed by type IV.19,33 The most frequent
site of carcinoma development is the cyst wall, although
it may be at any height of the biliary tree.12,16,33 One study

Table 1. Clinical characteristics of patients with

choledocal cysts

Sex
Male
Female
Age (average)
Abdominal pain
Jaundice
Nausea and vomiting
Tumor in right hypochondrium
Fever
Triad (jaundice, abdominal pain,
tumor)

7
16
26
20
13
8
4
3
4

30
70
87
57
35
17
13
17
Figure 1. Retrograde endoscopic cholangiopancreatography showing
type 1 choledochal cyst (arrow).

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Figure 2. Intraoperative photo showing biliary vesicle (VB) and

choledochal cyst (CC).

found that 7/8 cancer patients had been managed as follows: three cases with careful surveillance, three cases
with internal drainage, and one case with incomplete resection. This makes evident the fact that once diagnosis
of choledochal cyst is made, adequate surgical treatment
should be evaluated.34
Surgical treatment, in general, is demonstrated by two
procedures: internal drainage without removal of the cyst
(cystoenteroanastomosis) or resection of the cyst with
biliary-enteric anastomosis. Cystoenteroanastomosis is
associated with biliary stasis, secondary biliary calculus,
recurrent cholangitis, hepatic abscess and biliary cirrhosis.4,6,8,15,35 This is secondary to the fact that the anastomosis
should be performed as mucosa to mucosa, whereas the
cyst does not have normal mucosa. The result is stenosis of the anastomosis and recurrent cholangitis.1,12.23 In
addition, cancer risk remains and actually increases after
completion of this bypass.2,36 Reoperation for recurrent
cholangitis secondary to stenosis has been reported to be
from 13-69% .36 It is more frequent for adult patients to
have history of internal drainage or placement of prosthesis for biliary drainage than pediatric patients.31 We do not
report any cases where these procedures were attempted.
For this reason, surgical treatment of choice is cyst
resection1-2-5,7-9,11,14-15,20,22-23,29-31,33,35,37 with variations according to the type. Type I and type IV resection of the cyst
with biliodigestive anastomosis, type II: resection and if
necessary an anastomosis, type III: sphincteroplasty or
sphincterotomy. All extrahepatic cysts should be excised.
Postoperative evolution will be satisfactory if biliary flow
is adequate with symptom resolution in >80% of cases.1
This was the case in 87% of our patients. The remainder
needed to be reoperated due to postoperative complica-

tions (biliary fistula). In patients with intrahepatic cysts

the treatment is controversial. Some authors suggest the
possibility of hepatotomy or hepatectomy, but a wide
hepatojejunal anastomosis is probably useful with close
postoperative monitoring.1,3,4,19,27
After resection, the risk of cancer decreases significantly;6,15,19 however, it continues to be higher than in the
general population. We recommend close monitoring, especially in patients with intrahepatic involvement.3,25
Postoperative morbidity is ~15-30%, mainly due to
surgical wound infection, cholangitis and leakage of the
anastomosis, which in most cases are managed conservatively.34
Approximately 25% of patients are reoperated.35,37 The
most common indications during the early postoperative period are bleeding or anastomic leakage, as occurred in 13%
of our patients. Other reinterventions are performed after an
internal drainage for intestinal obstruction, incomplete resection, recurrent cholangitis, stricture or cancer.2,19,24,28,36,37
In conclusion, choledochal cyst is a congenital disease mainly diagnosed in pediatric patients. When found in
adults, management is surgical in the majority of cases.
Treatment should include resection of the cyst with biliodigestive anastomosis. Internal drain towards a loop of small
bowel is not recommended because of the possibility of
complications and probability of chronic damage to the liver or the development of a malignancy in the cyst remnant.
In our experience, surgical management with resection has
been possible with considerable morbidity but with low
mortality.
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