Choledochal cyst: A difficult diagnosis

doi:10.4236/ojgas.2013.31013

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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

Email: [email protected], [email protected]

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

OPEN ACCESS

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

Sarris and Tsang proposed in 1988 a more precised

classification [3]:

OPEN ACCESS

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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