International Journal of Research in Medical Sciences

Parra SRM et al. Int J Res Med Sci. 2023 Aug;11(8):xxx-xxx

www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012

DOI: https://dx.doi.org/10.18203/2320-6012.ijrms2023????
Case Report

Gallbladder agenesis in a patient with choledochoduodenal fistula,

report of an exceptional case
Samuel Raul Medina Parra1*, Omar Sosa Sánchez1, Rodrígo Migoya Ibarra1,
Cheryl Zilahy Díaz Barrientos1, Carlos Alberto Martínez Gandarillas1,
José Miguel Espinosa González2

1
Department of General Surgery, 2Department of Endoscopy, University Hospital of Puebla, BUAP, Mexico

Received: 15 June 2023

Accepted: 30 June 2023

*Correspondence:
Dr. Samuel Raul Medina Parra,
E-mail: [email protected]

Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Vesicular agenesis refers to the congenital absence of the gallbladder, however, vesicular agenesia is a very scarce
condition worldwide, so there is little information available on its clinical presentation and surgical data. This is a 79-
year-old male patient, with a history of diabetes mellitus of long evolution, without a surgical history, who comes for
colic pain of 15 days of evolution in right hypochondrium, as well as unquantified fever and jaundice, during its
initial evaluation in the emergency room para clinics were requested observing leukocytosis, hyperbilirubinemia and
elevation of liver enzymes, ultrasound of liver and bile ducts reported common anatomical situation gallbladder,
occupied entirely by multiple hyperechogenic images, configuring sign of W.E.S. and common bile duct of 7.2 mm,
concluding gallbladder scleroatrophic and vesicular agenesia during trans-surgery. Vesicular agenesis is an extremely
rare condition that is mostly diagnosed during surgery, which can lead to erroneous diagnosis and unnecessary
surgery in patients with symptoms including biliary colic, choledocholithiasis with or without cholangitis, and an
ultrasound showing a scleroatrophic gallbladder. Surgeons In situations where there is clinical evidence consistent
with biliary colic in a context of gallbladder agenesis, and the symptoms persist without finding any other cause, a
surgical approach to release adhesions could be considered; since, as observed, this may result in improved
symptoms, although the explanation is not yet completely clear.

Keywords: Liver, Agenesis, Gallbladder, Surgery, Leukocytosis

INTRODUCTION inhabitants).1 The reported incidence based on autopsy

findings is approximately one case per 6334 live births,
Vesicular agenesis refers to the congenital absence of the or approximately 0.035% to 0.3%, it is usually diagnosed
gallbladder, however, vesicular agenesia is a very scarce during the transoperative.2,3
condition worldwide, so there is little information
available on its clinical presentation and surgical data.1 In any case, if, during surgery the gallbladder is not
found, the surgeon must take the necessary measures to
Ther vesicular agenesis is rare, no precise prevalence has ensure the safety of the patient. This may include careful
been established in the general population. Most studies examination of the area for possible gallbladder
report isolated cases or case series, which makes it remnants, gallbladder ectopia, as well as evaluation of the
difficult to obtain accurate data, however, they have been bile duct. The rarity of this association is an exceptional
reported in some of these series, an incidence ranging event, since we do not find in the literature the
between 0.01% and 0.075% (10-75 per 100,000 concomitant presentation with choledoco-duodenal
fistula.

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Biliary fistulas are a rare entity, of which common bile preserved intrahepatic bile duct, extrahepatic dilated up to
duct-duodenal fists report the lowest incidence, usually approximately 15 mm, without filling defects, with
congenital or acquired, as a result of inflammatory or adequate contrast passage to the duodenum, unidentified
traumatic processes, gallstones, previous biliary surgery, gallbladder, bile duct (Figure 4), so it is carried out
infections and inflammatory diseases of the biliary tract. coledocorraphy with monocryl and it is placed drainage
These conditions can weaken the wall between the to Morrison’s space, it enters the floor of general surgery
common bile duct and duodenum, allowing the formation where it is observed adequate postoperative evolution and
of an abnormal connection. it is decided to leave after three days with adequate
tolerance to the oral route, without data of biliary
The clinical presentation is very varied and may become leakage, follow-up was carried out a week in an
asymptomatic. complications may occur, however, outpatient clinic where stitches and drainage were
cholangitis being more frequent, followed by biliary removed, ultrasound of the liver and bile ducts was
ileus, by the impact of a lithum at the level of the requested, and no gallbladder was reported in the usual
ileocecal valve, or present as Bouveret syndrome due to topography, with dilated intrahepatic bile duct (Figure 5)
obstruction at the level of the duodenum.2,4,9 A clinical that did not merit management, was revalorized a month
case of a patient with acute cholangitis and later in the office, where he referred asymptomatic so he
choloduodenal fistula in which vesicular agenesis was discharged our service.
diagnosed during the trans operative procedure, also
describes its management, evolution and a review of the
medical literature.

CASE REPORT

This is a 79-year-old male patient, with a history of

diabetes mellitus of long evolution, without a surgical
history, who comes for colic pain of 15 days of evolution
in right hypochondrium, as well as unquantified fever and
jaundice, during its initial evaluation in the emergency
room para clinics were requested observing leukocytosis,
hyperbilirubinemia and elevation of liver enzymes,
ultrasound of liver and bile ducts reported common
anatomical situation gallbladder, occupied entirely by
multiple hyperechogenic images, configuring sign of
W.E.S. and common bile duct of 7.2 mm, concluding
gallbladder scleroatrophic (Figure 1). with high risk for
choledocholithiasis by criteria of ASGE 201917 and with Figure 1: Ultrasound of the liver and bile ducts
data of mild acute cholangitis according to criteria of showing hyperechogenic images, with acoustic shade,
Tokyo 18 with significant adjacent inflammatory process, and which was reported as WES phenomenon,
so endoscopic retrograde cholangiopancreatography concluding the scleroatrophic gallbladder.
(ERCP) was performed observing normal intrahepatic
bile duct, extrahepatic dilated 10 mm in its greater
portion, balloon catheter was scanned with removal of
abundant stones of 3 to 8 mm and bile sludge, finding a
choledooduodenal fistula, (Figure 2) which was handled
conservatively, evolved favorably, so it was decided to
discharge and schedule for elective laparoscopic
cholecystectomy a month later.

During the surgical procedure, diagnostic laparoscopy

was performed, finding an plastron epiploon dependent,
liver and bile duct, adheciolysis was performed with
electrocoagulation and choledoco-duodenal fistula was
observed, which was dismantled without observing
leakage data, the hepatic bed was focused without
observing the gallbladder, so that when it was not found
in the usual position, it is reviewed in possible ectopic Figure 2: Endoscopic retrograde
locations such as the left liver lobe, major omentum, cholangiopancreatography in which the greater
minor and suprahepatic without visualizing it, so it is papilla is observed without alterations, with
decided to perform longitudinal choledotomy and hydatophilic guidance cannulating the
transoperative cholangiography (Figure 3), observing choledochoduodenal fistula.

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DISCUSSION

There is no exact date of publication in the medical

literature describing for the first time vesicular agenesia,
however, the first cases reported are attributed to Lemery
in 1701 and Bergman in 1702, and since then there are
about 430 reports of new cases, however, the number is
increasing as new publications come to light.2,4

Incidence varies between 0.01% and 0.075% (10-75 per

100,000 inhabitants).1 The reported incidence based on
autopsy findings is approximately one case per 6334 live
births, or approximately 0.035% to 0.3%.

Women are most often affected (3:1) and are usually

diagnosed in the second or third decade of life.
Figure 3: Laparoscopic vision of dissected plastron,
dilated common bile duct and absence of gallbladder
It may be associated with other congenital defects in 40-
in hepatic bed, with feeding tube introduced in
65%, such as trisomy 18, thalidomide malformations,
common bile duct, prior to cholangiography.
cerebrotendinous xantomatosis, inheritance not linked to
sex with variable penetrance and alterations of the
primitive intestine, cardiopulmonary defects, in addition
to genitourinary abnormality; however, these alterations
were not found in our patient.9,12

Regarding the clinical presentation of vesicular agenesis,

three main groups have been described: 1) those with
multiple fetal abnormalities detected perinatally (15%),
2) asymptomatic cases found at autopsy or incidentally
during surgery and 3) symptomatic cases that usually
occur in the third to fourth decades of life (50%).9

It is estimated that 25% to 50% of patients with

gallbladder agenesis may develop choledocolithiasis
Figure 4: Transoperative cholangiography, after secondary to bile duct stasis8, jaundice is due to
dismantling of choledochoduodenum fistula, where choledocholithiasis associated with or without ascending
intrahepatic bile is observed without alterations, cholangitis.2 which explains the clinical presentation of
dilated extrahepatic up to 15 mm, without filling our patient, when debuting with mild acute cholangitis,
defects, with adequate passage from contrast medium which merited drainage of the bile duct with ERCP,
to duodenum and leakage at choledotomy site, as well however during the procedure the presence of a
as absence of gallbladder. choledocho-duodenal fistula was found, which is reported
with an incidence of 5% of biloenteric fistulas (incidence
of 0.15-8% in the presence of cholelithiasis), however,
there are no reports of the presence of choldocho-
duodenal fistula in the context of gallbladder agenesis.9

Ultrasound is the method of choice for the diagnosis of

biliary pathology, for which the radiologist uses the triad
WES (Wall, Echo, Shadow) which is estimated to have a
sensitivity of 95-98% for the diagnosis of cholecystitis;
some poorly diagnosed acoustic shadows are due to
intestinal gas, duodenum artifacts, peritoneal subhepatic
folds, small or atrophic vesicles, contracted on lithos, so
in many cases of vesicular agenesia, is reported as
scleroatrous gallbladder, making preoperative diagnosis
more difficult.11,13
Figure 5: Ultrasound of the liver and bile ducts, after
a surgical procedure, where the gallbladder is absent In the case of our patient, who underwent acute
in the usual topography, with dilation of the cholangitis, an important local inflammatory process was
intrahepatic bile duct. developed that was observed during the surgical

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procedure when finding adhesions, plastron in the biliary CONCLUSION

tract, as well as the presence of the choledochoo-
duodenal fistula, which was reported in ultrasound as a Vesicular agenesis is an extremely rare condition that is
sclerotrophic gallbladder and according to the mostly diagnosed during surgery, which can lead to
literature.2,4,10 erroneous diagnosis and unnecessary surgery in patients
with symptoms including biliary colic,
In the literature review of Peloponissios et al covering the choledocholithiasis with or without cholangitis, and an
period 1960 to 2003, they indicate that all the cases ultrasound showing a scleroatrophic gallbladder.
analyzed were diagnosed during surgery or in the Surgeons should be alert to the possibility of vesicular
postoperative period, with only two exceptions.12,14 agenesis when faced with a difficult dissection,
Therefore, it is difficult to make a preoperative diagnosis scleroatrophic gallbladder or anatomical variations during
reliably and, in most cases, the diagnosis is established laparoscopic cholecystectomy. Transoperative
during surgery or in the postoperative period.2,4,15 cholangiography is useful to confirm the diagnosis,
address other conditions, and prevent bile duct lesions. In
So far, there are no diagnostic or management guidelines situations where there is clinical evidence consistent with
for gallbladder agenesis, however in 1967, Frey proposed biliary colic in a context of gallbladder agenesis, and the
to meet certain criteria during the transoperative that symptoms persist without finding any other cause, a
consisted in evidencing the absence of inflammatory surgical approach to release adhesions could be
signs or fibrosis in the vesicular bed, convert to considered; since, as observed, this may result in
laparotomy and perform the exhaustive search for an improved symptoms, although the explanation is not yet
ectopic gallbladder, intentionally searching for it completely clear.
intrahepatically, retrohepatically, in the left
hemiabdomen, between the 2 layers of the minor Funding: No funding sources
omentum, in the sickle ligament, retropancreatically, Conflict of interest: None declared
retroperitoneal and in the anterior wall and if not found, a Ethical approval: Not required
transoperative cholangiography with bile duct exploration
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