Acta chir belg, 2007, 107, 338-340

Choledochal Cyst : Diagnosis and Surgical Treatment

A. M. Wolthuis, T. Tollens, C. Aelvoet, J. P. Vanrijkel
Department of General Surgery and Traumatology, A.Z. Imelda, Bonheiden, Belgium.

Key words. Choledochal cyst ; bile duct cyst.

Abstract. A 20-year old woman with a 2-month history of epigastric pain, progressive jaundice and obstructive liver
biochemistry underwent imaging of the biliary tract, carried out using ultrasonography, CT-scan and MRI. Abdominal
ultrasound showed a dilation of the choledochal duct. CT-scan and MRI revealed a cystic dilation of the choledochal
duct.
Because of possible malignant degeneration, en-bloc cholecystectomy and resection of the cyst were performed, with
Roux-en-Y hepaticojejunostomy reconstruction.
This case demonstrates the diagnostic value of MRI and MRCP in a pathology that is rather rare in Western countries.

Introduction
A choledochal cyst is defined as a cystic dilation of the
common bile duct. In 1723, VATER was the first to show
a fusiform dilation of the common bile duct and
DOUGLAS published on the possible congenital origin of
this cyst in 1852 (1-2). The incidence of a choledochal
cyst is about 1:100,000-150,000 with a men to women
ratio of 1:3 (3-4). The aetiology is unknown. It is frequently seen in Asiatic countries, especially Japan. The
authors would like to present a case of this pathology,
rather rare in Western countries.
Case report
A previously healthy 20-year old woman presented with
nausea, progressive jaundice and epigastric pain since
2 months. She also had tea colour urine and clay colour
stools.
At clinical examination, she had right upper quadrant
and epigastric pain.
Liver function tests showed total bilirubin of
12.56 mg/dl (normal 0.20 - 1.10 mg/dl) with conjugated
bilirubin of 9.48 mg/dl (normal 0.00 - 0.30 mg/dl),
alkaline phosphatase of 357 U/L (normal 35 - 104 U/L),
gammaglutamyltranspeptidase of 218 U/L (normal 539 U/L), alanine aminotransferase of 315 U/L (normal
10 - 31 U/L) and aspartate aminotransferase of 149 U/L
(normal 10 - 32 U/L).
Abdominal ultrasound revealed a dilation of the common bile duct with a diameter of 3 cm. On CT-scan the
common bile duct was dilated fusiformly, with normal
diameter at the head of the pancreas (Fig. 1). An anomalous position of the gall-bladder was evidenced. MRI

Fig. 1
CT-scan shows a fusiform dilatation of the common bile duct
(black arrow), well separated from the gall bladder.
Anomalous position of the gall bladder can be seen (white
arrow).

with T2-weighted images and magnetic resonance

cholangiopancreatography (MRCP) showed a massive
dilation of the common bile duct, as seen on CT-scan,
with a maximal diameter of 3.3 cm (Fig. 2). A choledochal cyst was therefore diagnosed. Because of its possible malignant degeneration resection was indicated.
We performed an upper median laparotomy with cholecystectomy and resection of the cyst en-bloc (Fig. 3). A
Roux-en-Y hepaticojejunostomy reconstruction was
made. Anatomopathology showed a choledochal cyst
with a single-layered cylindrical epithelium without

Choledochal Cyst

339

Fig. 3
Peroperative image of gall bladder, cystic duct and choledochal cyst.

Fig. 2
MRCP illustrates the choledochal cyst (arrow) and biliary tract
with opacified gall bladder.

evidence of dysplasia or carcinoma. There were no postoperative complications and the patient was discharged
after 1 week. One year after the operation, she is asymptomatic.
Discussion
The first classification on choledochal cysts dates from
1959 and is from ALONZO-LEY et al (5). Nowadays, different types of choledochal cysts are classified according to the TODANIs classification (6). Type I is a
fusiform dilation of the common bile duct, as seen in our
case. It is the most frequent cyst with 80 to 90% prevalence. Most of the literature relating to diagnosis and
treatment of choledochal cysts refers to type I lesions. It
classically presents as a triad of jaundice, mass and right
upper quadrant abdominal pain ; however this triad is
rarely seen in adults (7). Jaundice and abdominal pain,
together with lightening of stools and darkening of the
urine appeared in the reported case. Other possible
symptoms are nausea, vomiting, abdominal distension,
hepatosplenomegaly, diarrhoea and fever (7).
In 80% of the cases the diagnosis is made before the
age of 10 (4). Diagnosis can be made by ultrasonography, CT-scan, endoscopic retrograde cholangiopancreatography (ERCP) and MRCP (4, 8). Ultrasonography
remains the most useful initial method of evaluating
biliary tract abnormalities. CT-scan can be used to confirm the diagnosis (4). A form of cholangiography is
mandatory to define the precise anatomy and can either

be ERCP or MRCP (3). In the case presented, 3 important investigations were done. Ultrasonography was performed as initial screening method, CT-scan to establish
the diagnosis and MRCP to show the precise anatomy of
the biliary tract. Because abnormal laboratory values
often suggest obstructive biliary disease, initial imaging
should focus on the presence of an obstructing lesion on
the biliary tract and pancreaticobiliary junction.
Therefore a choledochal cyst has to be differentiated
from pancreatic pseudocysts, echinococcal cysts and
cystic biliary neoplasms such as biliary cystadenoma
and biliary cystadenocarcinoma (9).
The most common complications of a choledochal
cyst are : cholangitis, pancreatitis, biliary cirrhosis, liver
abscess, cholelithiasis, portal hypertension, cyst rupture,
portal aneurysm and malignant degeneration (7). The
risk of complications increases with age. The most
important complication is malignant degeneration, with
an incidence of 2.5 to 26% (4). Surgical management
varies according to the type of cyst. Type I cyst requires
surgical resection with Roux-en-Y hepaticojejunostomy
(3-4, 7-8). In the past, the treatment of choledochal cysts
consisted of internal drainage by cystenterostomy.
Because of the increased risk of carcinoma, high rate of
recurrent cholangitis and pancreatitis, drainage was
abandoned in favour of cyst excision (4). Long-term follow-up of patients is necessary after any operation,
because biliary tract carcinoma may be a late postoperative complication of choledochal cysts (10).
Although choledochal cysts are rare in Western countries, the authors would like to stress the need for a good
diagnostic work-up and careful surgical treatment, as
shown in this case.

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References
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A. M. Wolthuis
A.Z. Imelda
Imeldalaan 9
B-2820 Bonheiden, Belgium
Tel.
: +32 15 50 61 95
E-mail : amwolthuis@hotmail.com