Open Journal of Gastroenterology, 2013, 3, 84-86 OJGas
http://dx.doi.org/10.4236/ojgas.2013.31013 Published Online February 2013 (http://www.scirp.org/journal/ojgas/)
Choledochal cyst: A difficult diagnosis
Abdelmounaim Ait Ali1, Fedoua Rouibaa2, M. Bekkar2, Hicham Baba1, Ibrahima Sall1,
Ahmed Bounaim1, Aziz Zentar1, A. Aourarh2, Khalid Sair1
1Service de Chirurgie Viscérale I, Hôpital Militaire d’Instruction Mohammed V, Rabat, Maroc
2Service de Gastro-Enterologie I, Hôpital Militaire d’Instruction Mohammed V, Rabat, Maroc
Received 17 October 2012; revised 18 November 2012; accepted 25 November 2012
ABSTRACT
Choledochal cyst is defined as a cystic dilatation of
the distal common bile duct protruding into the duo-
denum. It is considered as the rarest congenital cyst
of the biliary tract by 1.4%. We report a 46 y ears old
woman who presented with recurrent jaundice asso-
ciated with abdominal pain of 07 years duration. The
liver function tests showed cholestasis. The abdominal
scan and Biliary MRI revealed a dilatation of intra-
hepatic, pancreatic ducts and a dilatation of the
common bile duct with a stenosis in its the lower part.
The diagnosis of a common bile duct cholangiocari-
noma was mentioned. The gastroscopy revealed a
stenosed duodenal bulb not allowing us to perform an
echo endoscopy and ERCP. An intraoperative cholan-
giography illustrating a cystic dilatation of the papil-
lary region in which exist a sepa rate protrusion o f the
choledochal and wirsung ducts. Therefore, we didn’t
accomplish the cephalic duodeno-pancreatectomy and
we decided to realize a partial resection of the papilla .
The histolological examination proved the absence of
any tumoral lesion and the presence of biliary mucosa
layered the internal surface of the cyst. The patient is
still asymptomatic after one year of the surgery.
Keywords: Choledochal Cyst; Jaundice; Echoendoscopy;
Biliary MRI
1. INTRODUCTION
Choledochal cyst is defined as a cystic dilatation of the
distal common bile duct protruding into the duodenum. It
is considered as the rarest congenital cyst of the biliary
tract by 1.4% [1]. It corresponds to type 3 in Todani’s
classification [2]. Its diagnosis is difficult because of the
absence of specific clinical signs. We report a case of
choledochocele which intraoperative diagnosis, in a pa-
tient presenting obstructive jaundice, enabled us to nar-
rowly avoid her a cephalic duodeno pancreatectomy.
2. CASE REPORT
A 46 years old woman with a history of appendissectomy
20 years ago and chronic duodenal ulcer treated many
times with anti-secretory drugs since 2001 presented with
recurrent, spontaneously resolving jaundice associated
with abdominal pain of 7 years duration. The intensity of
her symptoms increased 4 months before her admission
and was associated with dyspepsia relieved by vomiting,
fever and 8 kg weight loss. At admission, the clinical
examination revealed a subicteric patient with a pain in
the right hypochondrium and in the epigastric region.
The liver function tests showed cholestasis. The ab-
dominal ultrasonography and abdominal scan revealed
an acalculous gallbladder, a dilatation of intrahepatic,
pancreatic ducts. It also revealed a dilatation of the com-
mon bile duct with a stenosis in its the lo wer part. Biliary
MRI confirmed the results of the scan and the diagnosis
of a common bile duct cholangiocarinoma was men-
tioned (Figure 1). Tumor markers (ACE.CA15-3.CA19-
9.CA125) we r e ne gat ive.
The gastroscopy revealed an ulcerated and stenosed
duodenal bulb not allowing us to perform an echo endos-
copy and ERCP.
Therefore our diagnosis was a cholangiocarcinoma of
the distal part of the common bile duct. And we decided
to perform a cephalic duodenopancreatectomy. Surgical
exploration was normal and we couldn’t find any p a lp ab l e
mass in the ampullary region. However we found lymph
nodes along the hepatic pedicle. Their frozen section was
normal.
An intraoperative cholangiography was done illustrat-
ing a dilatation of the intrahepatic bile ducts, common
bile duct; Wirsung duct and a cystic dilatation of the
papillary region in which exist a separate protrusion of
the choledochal and wirsung ducts.
The cyst opens into the internal layer of the duodenal
mucosa without any abnormality in th e duct s layer. Su ch
picture is pathogn omonic for obstructive choledochocele
Type A2 of Tsang and Sarris classification (Figure 2).
The tumor like stenosis seen at scan and biliary MRI

A. A. Ali et al. / Open Journal of Gastroenterology 3 (2013) 84-86 85
Figure 1. Bili-MRI showing a distal common bile duct stenosis.
Figure 2. Intraoperative cholangiogram showing the choledo-
chocele with two separate openings of the pancreatic and com-
mon bile ducts.
corresponds to the joining up of the common bile duct
into the choledo c hal cyst.
Our worry to dismiss a tumoral process in the ampulla
and our wish to treat at the sa me time the bulbar stenosis
made us perform a duodenotomy of the 2nd segment of
the duodenum where we found 3 to 4 cm of flexible and
easily depressive protrusions confirming the cystic na-
ture. At the examination of the internal surface of the
duodenum, we couldn’t find any orifice, but after the
injection of physiological serum under pressure via the
Kehr catheter we noticed the presence of a smal l s t r e t ch e d
papillary opening (orifice) (Figure 3). Therefore, we didn’t
accomplish the cephalic duodeno-pancreatectomy and we
decided to realize a partial resection of the papilla with
preservation of the biliopancreatic ducts. This surgical
intervention ended by a Finney’s pyloroplasty. The his-
tolological examination proved the absence of any tu-
moral lesion and the presence of biliary mucosa layered
the internal surface of the cyst. The patient is still as-
ymptomatic after one year of the surgery.
3. COMMENTS
The choledochal cyst was described initially by Wheeler
in 1940 [3]. It is a rare pathology with an incidence at
ERCP of 0.1 to 2% [4]. We found an increasing number
of published cases in these last years thanks to specific
morphological examinations that help to detect even the
small and asymptomatic lesions [4]. This pathology is
congenital but now, for many authors it’s an acquired
lesion secondary to papillary dysfunction which may
explain the lesions found in our case [4].
In fact, stretched, stenosed papillary orifice stops the
normal dropping of the bilio-pancreatic liquid into the
duodenum. Then, it is followed by a hyper-pressure and
stasis. Therefore, there is a dilatation of water ampulla
and biliary ducts with a risk of stone formation. Schals
et al. have proposed in 1976 an anatomic classification
[3]:
Type 1 = the ampullar type, most frequent in which
the main papilla opens into the choledochal cyst which
then communicates via an opening into the duodenum.
Type 2 = diverticular type in which the choledocho-
cele communicates at the distal part of a channel and
then drains into the papilla.
Figure 3. Intraoperative image showing the chole dochocele aris-
ing from the second portion of the duodenum.

A. A. Ali et al. / Open Journal of Gastroenterology 3 (2013) 84-86 86
Sarris and Tsang proposed in 1988 a more precised
classification [3]:
Type A = it corresponds to the ampullar form; the
most frequent wit h 67 %. It is divi ded into 3 subty pes,
A1 = the choled oque and the wirsung duct meet into a
commun duct that opens into the choledocal cyst;
A2 = the bilio pancreatic anastomoses are distant;
A3 = intramural and small choledochal cyst.
Type B = 21% are close to the diverticular form.
In our case, the adequate analysis of the cholan-
giograpic images showed that both, Wirsung and com-
mon ducts are distant and open separatly into the chole-
dochal cyst which corr esp onds to the ampu llar typ e A2 , a
very rare f orm.
Clinical signs of choledochal cyst are chronic and non
specific dominated by biliary pain (91%); jaundice and
recurrent attacks of acute pancreatitis (30% - 38%). The
association with biliary duct stones is noted in 17% to
21% of cases [1]. The upper obstructive signs of the di-
gestive system were also reported. The risk of degenera-
tion estimated initially to 15% have now decreased to
only 2.5% Intraoperative cholangiography and ERCP are
the main diagnostic examinations of the cyst [3]. They
enable us to define its volume, the state of the biliary
ducts, the presence of stones and the mode of anastomo-
sis of the different biliopancreatic d ucts.
Lateral vision duodenoscopy may show a protruding
formation of the papillary region. Papillary orifice is not
always visible and its catheterisation is quite difficult.
Echo endoscopy may facilitate the finding of a cystic
dilatation and hence eleminating the presence of a solid
tumor beneath the duodenal mucosa, but it does not give
us a good analysis of biliopancreatic ducts. MRI of the
biliary system gives us a precise study of the extrahepatic
ducts with a main performance approaching 90% [5];
however a solid tumor cannot be certainly eleminated.
The clinical signs of our patient were not specific in
the way that she had jaundice, weight loss associated
with the suspicion of a malignant stenosis of principal
biliary duct in the imaging tests. This weight loss is ex-
plained by the ulcerated bulbar stenosis. The choledochal
cyst was discovered intraoperatively, the tumoral-like
stenosis of the common bile duct seen during MRI cor-
responds to the anastomosis of this latter with the cyst. It
is the accurate study of the cholangiographic images, the
macroscopic aspect and examination of the papillary
region after duodenotomy that prevented us from doing a
cephalic duodenopancreatectomy.
However we cannot establish a precise diagnosis even
intraoperatively because we may miss a small neoplastic
lesion in the papilla and the role of echo-endoscopy is
important.
The classical treatment was the resection of the cyst
either partially or totally with reimplantatio n of the bilio-
pancreatic ducts, since it was initially considered as a
high risk of degeneration [1]. Actually, this strategy is
progressively replaced by endoscopic management, and
it became the first choice in the treatment of choledocal
cyst, especially in type A3 or A1 and A2 with a small
size [3,4].
However partial resection of the cyst with the presser-
vation of biliopancreatic ducts is preferred, mainly in
case of a large cyst or a doubtful diagnosis, as in our
clinical case [3].
4. CONCLUSION
The diagnosis of choledochal cyst is very difficult. It has
many similarities with common bile duct cholangiocari-
noma. In our case, the chief complaint of the patient is
recurrent jaundice associated with abdominal pain. The
liver function test shows cholestasis and the MRI shows
a dilatation of intrah epatic, pancreatic ducts and common
bile duct, and there is a stenosis in the lower part of the
common bile duct. The first diagnosis of common bile
duct cholangiocarinoma is under suspicion. The intraop-
erative cholangiography shows a separate protrusion at
the papillary region, and the partial resection of the pa-
pilla shows no any tumoral lesion.
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