Surg Radiol Anat (2008) 30:285290

DOI 10.1007/s00276-008-0332-7

R EV IE W

Abnormalities of the gallbladder, clinical eVects

J. P. Faure C. Doucet M. Scepi P. Rigoard
M. Carretier J. P. Richer

Received: 30 April 2007 / Accepted: 25 February 2008 / Published online: 11 March 2008
Springer-Verlag 2008

Abstract The aim of this review of the literature was to

present and discuss the anatomical and embryological basis
of congenital abnormalities of the gallbladder, based on a
case of volvulus. In the rare cases of ectopic gallbladder,
diagnosis of a biliary disease could be diYcult. In such
cases surgery can also be dangerous, especially when it is
associated with abnormalities of the intra-hepatic biliary
and vascular tree. This study, based on the embryology of
the extra hepatic bile duct, focused on the most frequent
gallbladder abnormalities to keep them in mind.
Keywords Ectopic gallbladder Gallbladder volvulus
Congenital anomaly Anatomic variation

Introduction
Volvulus of the gallbladder is a rare phenomenon, which
may mimick cholelithiasis disease. Based on one reported
case, the aim of our study was to present and discuss the
anatomical and embryological basis of congenital abnormalities of the gallbladder. We focused on ectopic gallbladder
to highlight the associated abnormalities of the intra-hepatic
biliary and vascular tree when they are present.

J. P. Faure (&) C. Doucet M. Carretier J. P. Richer

Service Chirurgie Viscrale Digestive et Endocrinienne,
Hpital Jean Bernard, CHU Poitiers, Universit de Poitiers,
2 rue de la Miletrie, BP 577, 86021 Poitiers Cedex, France
e-mail: j.p.faure@chu-poitiers.fr
J. P. Faure C. Doucet M. Scepi P. Rigoard J. P. Richer
Laboratoire Anatomie, Centre de Don de Corps,
Facult de Mdecine et de Pharmacie, Universit de Poitiers,
6 rue de la Miletrie, BP 199, 86034 Poitiers Cedex, France

Materials and methods

Case report
A 84-year-old woman was admitted to our hospital with the
complain of right upper quadrant pain of approximately
48 h in duration. She had no relevant past history. Pain was
not preceded by jaundice. Murphys sign and a palpable
mass were detected in the abdominal examination. Laboratory data only showed a leukocytes count of 10,300/mm3.
Abdominal ultrasound (US) demonstrated a distended,
thick-walled gallbladder, but no stones. A computed
tomography (CT) examination of the abdomen demonstrated a large dilated gallbladder, measuring approximately 67.9 mm 47.4 mm. Additionally, the enhanced
CT was characterized by the non-enhanced wall of the gallbladder (Fig. 1). The diagnosis of gallbladder volvulus was
supposed. She underwent a laparoscopy, Wndings included
a distended and gangrenous gallbladder, and closer examination revealed a clockwise torsion of 360 on the gallbladder mesentery. De-torsion and cholecystectomy were easily
performed. The histopathology report showed necrosis and
hemorrhage of the gallbladder without evidence of lithiasis.
Two days after the operation she was discharged with no
complications.

Discussion
First reported in 1898 by Wendel [41], volvulus of the gallbladder is a relatively uncommon phenomenon, with no
more than 300 cases reported in the literature. It occurs in all
age groups, with the highest incidence in elderly women,
and a female-to-male ratio of 3:1. The incidence could
increase with a longer life expectancy rate [12, 22, 36].

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286

Fig. 1 Computed tomography of the abdomen, sagittal view gating on

the gallbladder hilum. a Dilated gallbladder but a non-enhanced gallbladder wall. b Cystic artery

Gallstones are unlikely to be the cause of gallbladder torsion, as gallstones are not uniformly present in all patients
reported with torsion: only in 24.4% of the cases [27]. In
the literature, 51% of cases developed a clockwise torsion
rotation. Although supportive evidence is lacking, inferences have been made in the literature linking gastric peristalsis to clockwise gallbladder torsion and colonic
peristalsis to counterclockwise torsion [17].
This congenital anomaly of the gallbladder can result in
the development of an abnormally long mesentery suspending the gallbladder from the liver bed, thus increasing the
propensity for rotational instability. In conjunction with
this anomalous anatomic conWguration, a subsequent triggering event must occur to initiate torsion. Theories have
focused on liver atrophy, loss of visceral fat and elasticity,
weight loss as causes, particularly found in the aging population, as impetuses for gallbladder torsion [32]. Spinal
deformities are also associated with volvulus of the gallbladder, in such cases we can hypothesized that peritoneal
tensile strength on the liver and also the gallbladder in the
abdominal cavity could increase the risk of volvulus.
Further suppositions include arteriosclerosis and tortuosity of the cystic duct acting as rigid fulcrums for torsion
[32].
Other anomalies of the gallbladder
Gallbladder ectopia has been classiWed into four types:
transverse, intrahepatic, left-sided, and retroplaced [6, 12].
Retroposition of the gallbladder may also be found: it may

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Surg Radiol Anat (2008) 30:285290

be retroperitoneal or retrohepatic. This position might be

produced by hypoplasia of the right hepatic lobe. The
abnormal gallbladder might have originated from abnormal
migration of the gallbladder bud. Another possibility is that
it might have originated from a second bud with atrophy of
the Wrst. Triplication of Gallbladder is an extremely rare
congenital anomaly (only eleven reported cases to date)
[24]. In such cases there was no intra hepatic biliary duct
(IHBD) abnormality. So diagnosis of a biliary disease and
surgery may be a challenge.
The absence of gallbladder (AG) is an unusual anomaly,
mimicking a biliary stone disease in 23% of cases. This
condition must be kept in mind in the diVerential diagnosis
of ultrasonographically undetected or scleroatrophic gallbladder, this congenital malformation should be suspected
[29].
Absence of gallbladder can be observed in both children
and adults, with a median age of 46 at the time of diagnosis
[29]. It is almost always a fortuitous Wnding during abdominal surgery or at autopsy. The prevalence range is 0.007
0.13%. The incidence of this malformation is slightly lower
in surgical cholecystectomy series (0.0070.027%) [29]
than that in autopsy reports (0.040.13%) [23]. The male to
female ratio is equal in autopsy reports [23] but seems to
reach the same ratio (23 women for 1 man) in the clinical
group as other biliary tract diseases [29]. AG occurs in
association with other malformations in 4065% of cases
[23]. It is then found above all in children, leading in most
cases to death in the Wrst year of life [23]. Two congenital
syndromes with multiple anomalies including gallbladder
agenesis have been described: cerebrotendinous xanthomatosis is a rare, inherited lipid-storage disease characterized
clinically by progressive neurologic dysfunction, premature
atherosclerosis, and cataracts. Large deposits of cholesterol
and cholestanol can be found in virtually every tissue [42],
and the G syndrome or Opitz BBB/G syndrome is characterized by association of crania-facials and urogenitals
abnormalities. Two types of this syndrome are described:
one autosomic dominant and one associated to X [39]. AG
has been occasionally mentioned with trisomy 18 and the
KlippelFeil syndrome witch is a rare disorder characterized by failure in the normal segmentation of the cervical
vertebrae during the early weeks of fetal development.
Associated abnormalities may include scoliosis and spina
biWda, anomalies of the kidneys and the ribs, cleft palate,
respiratory problems, and heart malformations [29]. Some
authors [15, 33] reported this anomaly in children with congenital malformations caused by thalidomide (up to 10% in
Mnnls series [20]). This anomaly is explained by an
embryological disturbance occurring during the third week
of gestation and concerning the caudal portion of the anterior diverticulum of the primitive gut. It is an anomaly of
the development of vessels located on each side of the

Surg Radiol Anat (2008) 30:285290

gallbladder bud (sinus venosus cordis, omphaloenteric, and

umbilical veins) [5]. This may explain the association of
this anomaly with cardiac, vascular, gastro-intestinal, and
abdominal wall malformations observed in the multiple
fetal anomaly groups. AG may be inherited, with several
familial cases observed, including across two generations.
These series suggest a non-sex-linked heredity with variable penetration [29, 34].
Multiseptate gallbladder, characterized by the presence
of multiple septa dividing the gallbladder lumen, is a very
extremely rare congenital anomaly of the gallbladder. To
our knowledge, only 30 cases have been reported in the literature [44]. A review of the cases showed a female predominance; 21 of the patients were females. A mean age
was 29.4 years (range 370 years), and pediatric patients in
4 cases [30, 37]. Most patients usually present with right
upper abdominal pain, epigastralgia, and back pain, in the
literature, only six cases were asymptomatic [30, 37, 44].
Though the symptoms of multiseptate gallbladder patients
were relieved after cholecystectomy, histological Wndings
of the resected gallbladder in patients were usually of normal size with smooth surface and not always recognized as
acute cholecystitis or cholecystolithiasis, and the exact
mechanism of the biliary symptoms of multiseptate gallbladder is unknown. In nearly all literatures, pathological
Wndings of multiseptate gallbladder reveal numerous septa,
which consisted of anormal muscularis layer of the gallbladder wall, like a honeycomb appearance. Though the
septa usually existed in the whole of the gallbladder, in
some cases, the septations were conWned to the neck, the
body and the fundus alone [1, 28, 44].
Situs inversus totalis (SIT) is a very uncommon entity. It
was Wrst reported by Fabricius in 1600 [43]. The incidence
is thought to be in the range of 1:10,0001:20,000 [2].
Transposition of the organs may also aVect thoracic organs,
besides abdominal organs. Intrahepatic biliary tree, arterial
and venous systems are in the mirror-image conWguration
of the normal anatomy. The hepatoduodenal ligament is the
same as seen in the orthotopic patients [18]. Arterial system
anomalies are in the mirror-image conWguration of the
orthotopic patients [14]. There is no evidence for increased
incidence of cholelithiasis in SIT [8]. Since Mouret Wrst
performed it in 1987, laparoscopic cholecystectomy has
become the standard operative procedure for cholelithiasis
in SIT [3, 10].
Concerning these previous described abnormalities location of the gallbladder, we were unable to Wnd any reference in the literature suggesting increased chances of
anomalous biliary anatomy in patients. But that is not the
case of another interesting abnormality, which is the left
sided gallbladder or right sided round ligament. In such
cases, the gallbladder is located in the plane of the round
ligament and the bifurcation of the portal vein in two main

287

branches may not exist. The Wrst reported cases were

thought to be published in 1886 by Hochstetter [13]. Very
few cases have been described in the literature: Asonuma
et al. [4] reported an incidence of 4 in 379 (1.1%), and
Nagai et al. [25] reported an incidence of 3 in 1,621 (0.2%).
Yamasaki et al. [45] listed 5 patients among 2,210 patients
studied by ultrasonography (0.2%); Maetani et al. [21] 4 in
327 (1.2%); and Kuwayama et al. [16] 7 in 1,025 patients
(0.7%). But recently a new concept for this anatomic entity
named fusion of the plane has been proposed by Savier
et al. [31]: two liver lobes separated by a unique plane containing the portal axis, without true bifurcation. They
hypothesized that the origin of fusion of the planes (Fig. 2),
as observed in adults, would correspond to an embryologic
step of liver development. Portal and hepatic veins of 20
24 mm embryos resemble fusion of the planes [19], the
central portion of the liver develops secondarily between
two primitive lateral lobes [7] and there is variability in the
position of the anterior sectorial branch [7]. In the case of
incomplete development of the central part of the liver, the
left intersectional plane resembles the midplane, which
could not yet be clearly identiWed between the outlines of
the portal branches for the future segment 4 and the future
right sector. The absence of growth of segment 4 prevented
shift of the round ligament toward the left, the formation of
the horizontal part of the left bile duct, the formation of the
quadrate lobe between the round ligament and the gallbladder, and the curving of its anterior branch [31].
When reviewing the literature, with the exception of AG
(in those cases the diagnosis proposed was almost always
cystic duct obstruction or a small contracted gallbladder)
[29], the preoperative investigations always demonstrated
the diagnosis of ectopic gallbladder which automatically
induced an entire exploration of the biliary tree [3, 8, 9, 17,
31, 44].
Ectopic gall bladder is a rare entity mandating caution by
the laparoscopic surgeon. The surgeon must locate the gallbladder accurately and map out the biliary tract preoperatively with appropriate investigations (Ultrasonography,
CT scan, MRI and Endoscopic Retrograde CholangioPancreatography). Appropriate modiWcations of surgical
approach and technique are required in these patients [29].
Ultrasonography is actually the investigation method of
choice for the diagnosis of common gallbladder bile stones,
with a sensitivity of 9598% [29].
Development of the biliary tract [11]
The development of the biliary tract in humans has been
and is still being actively studied [7, 38]. It is well-known
that an endodermal bud develops from the foregut (duodenum) during the Wrst 3 weeks of gestation. The bud separates into two partshepatic and biliary. The hepatic part is

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Surg Radiol Anat (2008) 30:285290

Fig. 2 According to Savier

et al. [31] relation between fusion of the planes of the liver and
normal anatomy. a Fusion of the
planes. b Development of the
central region separates the medial plane and the left intersectional planes. c The central area
is the place of development of
the horizontal part of the left
portal branch and the right anterior branch, with its known variations. d Normal hepatic
architecture

a collection of rapidly proliferating cells (liver precursor

cells) which penetrate the adjacent mesodermal tissue (the
septum transversum) and are met by an in growing capillary plexus around the bile duct and umbilical veins, followed by the formation of the hepatic lobules and
sinusoids.
Development of the extrahepatic biliary tract [26] (Fig. 3)
The extrahepatic bile ducts (EHBD) develop from the caudal part of the hepatic diverticulum [35, 38]. The lining biliary epithelium is continuous at its caudal end with the
duodenal epithelium and at the cranial end with the proliferating precursor liver cells. The caudal segment develops
into the gallbladder, part of the cystic duct and the common
bile duct. The extrahepatic segments of the right and left
hepatic ducts, which are recognizable from 12 weeks of
Fig. 3 According to Shiojiri
[35] development of the extra
hepatic bile duct. Mid-sagittal
sections of the anterior intestinal
area. The hepatic diverticulum is
formed at 9 days of gestation
and is divided into cranial and
caudal portions at 9.5 days. Hepatic cord from the cranial portion then invades the septum
transversum Mesenchyme

Day 9

gestation and continuous with the common hepatic duct,

and part of the cystic duct, develop from the cranial end of
the diverticulum. The common hepatic duct and distal parts
of both hepatic ducts are known to connect to several converging ductules in the hilar mesenchyme, which in turn are
in direct communication with the ductal plate. Variations in
the remodeling process of the common hepatic duct and
distal parts of both hepatic ducts could explain, in part, the
many recognized branching variants of large bile ducts
[38]. It is well known that bile begins to Xow at about
12 weeks, so that this connection should be completed
before 12 weeks of gestation but not as a solid stage as
primary described but as a functional duct. This fact is well
demonstrated by Vijayan and Tan [40] in a 3D radiological
study of EHBD in embryos and fetuses at 5.516 weeks of
gestation. They were able to show too that the gallbladder
is already present with a lumen. Throughout infancy and

Day 9.5
Hepatic cord

Cranial
Ventral

Mesenchyme

Cranial
Caudal

Hepatic
diverticulum

123

Day 10.5

liver

Extra hepatic
bile duct
Gallbladder

Surg Radiol Anat (2008) 30:285290

early childhood, the lumen (diameter and surface area) and

the wall of the common bile duct increase progressively
and reach mature proportions in adulthood [38].

Conclusion
Congenital anomalies of the gallbladder are a relatively
uncommon phenomenon. But in the rare cases associated
with an intra-hepatic biliary abnormalities, anatomical
knowledge is capital to recognize and understand these
conWgurations. So before all biliary surgery, a good exploration of the extra and intra hepatic duct biliary is required
to avoid injuries.

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