The Turkish Journal of Gastroenterology
Volume 17, No 1, Page(s) 70-73
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|An adult choledochocele case presented with gastric
outlet obstruction: A rare presentation
|Mehmet Fatih CAN, Nihat KAYMAKÇIOĞLU, Gökhan YAĞCI, Semih GÖRGÜLÜ, Turgut TUFAN
|Department of General Surgery, Gülhane Military Medical Academy, Ankara
|Keywords: Bile duct cyst, choledochocele, gastric outlet
Bile duct cyst is a biliary tract disease that is less common
among adults compared to children, and it is accepted to have
a congenital entity. The classical findings constitute a triad including
abdominal pain, jaundice and abdominal mass. However,
infective findings also occur in case of developed cholangitis,
pancreatitis and cholecystitis. Duodenal obstruction leads
to a rarely seen case in patients accompanied by nausea and vomiting,
and it may easily be confused with other obstruction causes.
We present a case report of an adult male patient with
complaints of abdominal pain, and intermittent nausea and vomiting.
Due to failure of attempted endoscopic approach for treatment,
we performed transduodenal cyst excision during surgical
procedure on the patient diagnosed to have type III biliary
tract cyst. The complaints of the patient decreased significantly
after the surgical therapy compared to the previous period and
no complication was observed.
Bile duct cyst is a biliary tract disease which was
first defined by Vater in 1723 (1). It is specifically
prevalent in East Asian countries and is more
common among females compared to males. It
commonly arises as a congenital disease of the
childhood period, when symptoms generally present
(2). However, in recent years, there has been
an increase in the literature regarding cases in
whom symptoms were first observed as adolescents
or adults. Although the classical triad composed
of abdominal pain, jaundice and abdominal
mass is more common among children compared
to adults, in infants in particular, jaundice alone
presents more frequently than the classical state
(3). It is an exceptionally rare case that a symptom
occurs due to obstruction of intestinal passage by
a bile duct cyst. Moreover, most cases with accompanying
duodenal obstruction can be attributed to
a further pathology (i.e. annular pancreas, duodenal
atresia). In this paper, we report an adult male
choledochocele case who presented with symptoms
other than the classical presentation due to
the obstruction of duodenal transit by a type III
A 21-year-old male patient presented to the surgery
department with abdominal pain, and intermittent
nausea and vomiting complaints which had appeared periodically for about six months.
These attacks generally occurred after meals and
repeated a few times once they occurred. Furthermore,
he had persistent blunt pain in the right
hypochondrium that became more severe during
these nausea and vomiting episodes. No positive
finding except sensitivity with palpation on the
right hypochondrium was observed in the physical
examination. Reasonable hematological and biochemical
results were received in the laboratory
examination. Ultrasound (US), stomach-duodenum
radiogram (Figure 1), computerized tomography
(CT) and magnetic resonance cholangiopancreatography
(MRCP) were performed subsequently
and all displayed similar findings in regard
to an intraduodenal cystic lesion in the second
portion of the duodenum. Furthermore,
MRCP showed that the cyst had the same intensity
as the biliary tract and might have originated
on the common bile duct (Figure 2). Endoscopic
retrograde cholangiopancreatography (ERCP) failed
due to bad location of ampulla of Vater. Based
on these findings, exploration of the patient was
decided and he was taken into operation. It was
possible to enter the second part of the duodenum
with anterior duodenotomy after the Kocher maneuver.
A soft and pedicular mass, roughly 3x3x5
cm in size, was observed in the medial wall of the
duodenum. The lesion, which included the ampulla
of Vater, had a smooth surface covered with duodenal
mucosa (Figure 3a). When a silastic tube
was placed in the ampulla, bile was observed
(Figure 3b). After total excision of the lesion, together
with the duodenum mucosa covering it, the intramural pedicle was anastomosed to duodenal
mucosa (Figure 4). Sphincterotomy was not applied
due to the decreased pressure in choledochus by
new orifice on the duodenum. The duodenotomy
incision was primarily closed. The patient experienced
no complication in the postoperative period
and was discharged from the hospital. Endoscopic
follow-up in the second postoperative month revealed
no problems with biliary and intestinal passage.
Figure 1. The appearance of the cyst as intraduodenal filling
defect by stomach-duodenum radiograph
Figure 2. The MRCP appearance of the bile duct cyst
Figure 3: A) Choledochocele and ampulla of Vater after
duodenotomy. B) Orifice of the cyst in pedicle after silastic tube
Figure 4. The schematic aspect of the operation: 1. Exploration;
2. Excision; 3. Anastomosis
The knowledge related to bile duct cysts has significantly
increased since it was first defined. Current
knowledge is based on Todani and colleagues’
modification of the 1977 classification of the extrahepatic
disease, which was primarily made by
Alonso-Lej in 1959, according to cyst locations (4).
Bile duct cysts have been typed to five categories
according to Todani classification. The most common
type among adults is type I when general prevalence
is considered. Furthermore, type IV is
slightly more frequent in adults than in children.
On the other hand, type III bile duct cyst (also
known as choledochocele) is observed in only 4% of
total cases (5). It is generally accepted that the cause
of the disease is congenital disorder development
of the bile duct. However, there is also an
opinion advocating that biliary tree mural defects
are formed due to deterioration caused by reflux of
the pancreatic enzymes into the extrahepatic bile
duct (6). In addition, it is still controversial whether
the disease may occur later in adult patients.
The frequency of the classical triad of the disease
is approximately 40%. While jaundice is the major
complaint among children, adults usually present
with nonspecific complaints related to the right
hypochondrium. However, cholangitis, pancreatitis
and cholecystitis are more common among these
patients compared to the normal population
due to their predisposition. Furthermore, symptoms
and findings of these infections, such as fever,
weakness and tachycardia, are also observed
if these infections are present in the case. Another
case resulting in increased predisposition is the
development of malignancy (7). In these cases,
progressive jaundice, weight loss and findings of
metastasis are also observed. Symptoms due to
symptomatic gallstones or cholecystitis exist in
nearly half of the adults (8). Some of these cases
have cholecystectomy or another surgical operation
There are few data concerning whether symptoms
or findings other than the above-mentioned develop
among adult patients with bile duct cysts. Duodenal
obstruction cases are reported in regard to
children due to additional pathologies such as annular
pancreas or duodenal atresia (9). Duodenal
obstruction is almost impossible for these lesions
except for type III cysts. Theoretically, type III
cysts may lead to findings of the intestinal system
(such as obstruction, bleeding or perforation) as
they protrude to the duodenal lumen. However, as
stated above, choledochocele constitutes only a
small fraction of total cases and there is little information
in the literature regarding different
symptoms and findings, as most of these cysts
may be asymptomatic. Our patient had neither biliary
obstruction findings such as jaundice and
hyperbilirubinemia nor any sign or findings in favor
of infection. Thus, the cause of the nausea and
vomiting complaints, which usually appeared after
meals, was thought to be existence of a duodenal
obstruction. The patient complained of nausea
a few times and once vomited bile after a meal during
the hospitalization period. However, no significant
finding was encountered in the laboratory
examinations of the patient during this period.
There are a few case reports in the literature,
mostly in childhood, regarding choledochocele, also
referred to as gastric outlet obstruction, with
blockage of intestinal passage in the duodenum,
and this is considered exceptionally rare among
the adult population (6, 10-12). The reason why
the complaints of the patient only periodically arose
can be explained by spasm of Oddi’s sphincter
or postprandial increase in the cyst content.
Imaging methods such as prior US and CT are included
in the regular algorithm like in other pancreatobiliary
system diseases (13). MRCP and
ERCP usage has also significantly increased in recent
years. The importance of MRCP in diagnosis
stems from the fact that it has a similar sensitivity
as ERCP as well as a significantly low risk of
complication (8). Evaluation of the suspicious
complaints with MRCP and ERCP in addition to
US and CT, together with the increase in diagnostic
ability, may help to clarify related reasons why
the disease has become more common in recent years.
MRCP was quite important in the differential
diagnosis of duplication cyst of the duodenum in
our case. Nonetheless, it has been suggested that
both diagnosis and treatment of duplication cyst of
the duodenum can be achieved by ERCP (14). The stomach-duodenum radiograph for type III cyst also
contributed to the diagnosis by displaying the
The treatment modalities for bile duct cystic diseases
differ according to cyst type in Tadoni classification.
In recent years, cysto-jejunostomy and
cysto-duodenostomy methods have not been preferred
due to the higher incidence of complication
and continuity of the malignancy risk (15). Total
cyst excision is acknowledged as the best therapy
in type III bile duct cysts. There are also publications
reporting that asymptomatic cases can be observed
without treatment due to the lower malignancy
potential compared to other types of cysts
(16). After excision, anastomosis of the biliary and
pancreatic channels independently to the duodenum
may be necessary due to the location of the
cyst. The regression of saccular structured type III
cyst after endoscopic sphincterotomy is rarely seen
in contrast to other cyst types of the bile duct
(particularly the fusiform ones) (17). On the other
hand, it has been reported in some of the publications
that resolution of the cyst can occur after
sphincterotomy through ease of biliary and pancreatic
drainage (18, 19). Essentially, if the method
can be accomplished in these symptomatic cases,
ERCP seems a preferable application due to both
its diagnostic and therapeutic effectiveness in one
session. In this way, patients may be followed safely
due to low malignancy risk after regression of
the cysts. ERCP was also attempted in our case;
however, cannulation of the ampullary orifice in
the posterior wall of the cyst was not successful.
Thus, transduodenal total cyst excision was applied
to the patient and the orifice in the pedicle of
the cyst was anastomosed to the duodenum at nearly
1.5 cm proximal to the ampulla of Vater.
Enabling the endoscopic approach in later followup
of the patient is also a significant advantage of
this operation technique. Endoscopic evaluation
was possible in our case in the second follow-up
month and bile stream through natural and new
orifices was observed. Although operations of biliary-
enteric anastomosis like hepaticojejunostomy
and choledochojejunostomy are frequently used in
the therapy of other types of bile duct cysts, they
are not regularly employed methods for the therapy
In conclusion, type III bile duct cyst, also known
as choledochocele, is a rarely seen form of an infrequent
disease among adults. The patients may
be asymptomatic or have nonspecific symptoms,
as seen in most cases. We believe that it would be
beneficial in the differential diagnosis to take into
consideration the rarely seen symptoms and findings
related to duodenal obstruction by the cyst
together with the other supporting data.
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IC and IVA cysts with primary ductal stricture. J Hepatobiliary
Pancreat Surg 2003; 10: 340–4.
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