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378
GASTROINTESTINAL IMAGING
Imaging of Pancreaticobiliary
Maljunction
Ayako Ono, MD, PhD
Shigeki Arizono, MD, PhD
Hiroyoshi Isoda, MD, PhD
Kaori Togashi, MD, PhD
Abbreviations: ERCP = endoscopic retrograde
cholangiopancreatography, JSPBM = Japanese
Study Group on Pancreaticobiliary Maljunction,
MIP = maximum intensity projection, MPR =
multiplanar reconstruction, PBJ = pancre-
aticobiliary junction, PBM = pancreaticobiliary
maljunction, 3D = three-dimensional, 2D =
two-dimensional
RadioGraphics 2020; 40:378–392
https://doi.org/10.1148/rg.2020190108
Content Codes:
From the Department of Diagnostic Imaging
and Nuclear Medicine, Kyoto University Gradu-
ate School of Medicine, 54 Kawahara-cho,
Shogo­in, Sakyo-ku, Kyoto 606-8507, Japan. Pre-
sented as an education exhibit at the 2018 RSNA
Annual Meeting. Received April 12, 2019; revi-
sion requested June 26 and received July 19; ac-
cepted July 30. For this journal-based SA-CME
activity, the authors A.O. and S.A. have pro-
vided disclosures (see end of article); all other
authors, the editor, and the reviewers have dis-
closed no relevant relationships. Address cor-
respondence to A.O. (e-mail: nanohananooki@
gmail.com).
© ©
RSNA, 2020
SA-CME LEARNING OBJECTIVES
After completing this journal-based SA-CME
activity, participants will be able to:
„ Recognize the morphologic characteris-
tics of PBM.
Identify the key points of diagnosing
„ reported (1,2).
PBM.
Describe the management and treat-
„ case in Germany. Since then, cases of PBM have been reported
ment of PBM.
See rsna.org/learning-center-rg.
Pancreaticobiliary maljunction (PBM) is a congenital malformation
in which the pancreatic and bile ducts join outside the duodenal
wall, usually forming a long common channel. A major issue in pa-
tients with PBM is the risk of biliary cancer. Because the sphincter
of Oddi does not regulate the pancreaticobiliary junction in PBM,
pancreatic juice frequently refluxes into the biliary tract and can
cause various complications, including biliary cancer. Most cancers
arise in the gallbladder or dilated common bile duct, suggesting
that bile stasis is related to carcinogenesis. Early diagnosis and pro-
phylactic surgery to reduce the risk of cancer are beneficial. The
diagnosis of PBM is made mainly on the basis of imaging findings.
The development of diagnostic imaging modalities such as multide-
tector CT and MR cholangiopancreatography has provided radiol-
ogists with an important role in diagnosis of PBM and its complica-
tions. Radiologists should be aware of PBM despite the fact that it
is rare in non-Asian populations. In this review, the authors present
an overview of PBM with emphasis on diagnosis and management
of PBM and its complications. For early diagnosis, the presence of
extrahepatic bile duct dilatation or gallbladder wall thickening may
provide a clue to PBM with or without biliary dilatation, respec-
tively. The pancreaticobiliary anatomy should be closely examined
if imaging reveals these findings. Radiologists should also carefully
evaluate follow-up images in PBM patients even years after prophy-
lactic surgery because residual bile ducts remain at risk for cancer.
RSNA, 2020 • radiographics.rsna.org
Introduction
Pancreaticobiliary maljunction (PBM) is a congenital malformation
in which the pancreatic and bile ducts join outside the duodenal
wall, usually forming a long common channel. Whether PBM is
hereditary remains unclear, although a few familial cases have been
PBM was first described in 1906 by Arnolds (3) in an autopsy
more frequently in Asian countries, particularly in Japan (4). The
estimated prevalence of PBM in Asia is 100 to 1000 times higher
than in other parts of the world (5). In one study in South Ko-
rea, patients with PBM accounted for 4.1% of the 10 243 patients
who underwent endoscopic retrograde cholangiopancreatography
(ERCP) (6). In the United Kingdom, the estimated prevalence is
about one in 53 000 births (7). PBM occurs more frequently in
women than in men, with a female-to-male ratio of 3:1 (8).
RG  •  Volume 40  Number 2 Ono et al  379
TEACHING POINTS
„ In patients with PBM, the sphincter of Oddi does not regulate
the pancreaticobiliary junction (PBJ), and reciprocal reflux of
pancreatic juice and bile can occur. Refluxed pancreatic juice
injures the biliary epithelium and promotes cancer develop-
ment. Therefore, once PBM has been diagnosed, risk-reduc-
ing surgery is recommended to help prevent development of
biliary cancer.
„ Congenital bile duct stricture of the distal duct or hilar duct
is common in patients with PBM with biliary dilatation.
Radiologists should recognize this characteristic finding of
PBM to differentiate malignant from benign strictures.
„ Multidetector CT and MR cholangiopancreatography can
now obtain high-resolution images of the pancreaticobiliary
anatomy and have become important in diagnosis of PBM
and its complications.
„ Although US alone may not be sufficient to depict an anoma-
lous junction of the pancreaticobiliary duct, it is a useful non-
invasive tool to help screen for PBM. Extrahepatic bile duct
dilatation or gallbladder wall thickening can be seen at US,
and this screening may be the first opportunity to diagnose
PBM with or without biliary dilatation.
„ We should carefully evaluate follow-up images of patients
with PBM even years after prophylactic surgery because the
remaining bile ducts are still at risk for biliary cancer.
The pathogenesis of PBM is controversial.
However, several studies have suggested that it
involves anomalous development of the ven-
tral pancreas (9,10). Because small pancreatic
branch ducts arising from the common channel
have been seen in some patients with PBM, it
is hypothesized that the origin of the common
channel is related to the ventral pancreatic duct
(9). In rare cases, PBM is associated with other
congenital pancreatic anomalies, such as annular
pancreas or pancreas divisum (11).
A high risk of biliary cancer is a clinically
important issue in patients with PBM (8). In pa-
tients with PBM, the sphincter of Oddi does not
regulate the pancreaticobiliary junction (PBJ),
and reciprocal reflux of pancreatic juice and bile
can occur. Refluxed pancreatic juice injures the
biliary epithelium and promotes cancer develop-
ment (Fig 1) (12–14). Therefore, once PBM has
been diagnosed, risk-reducing surgery is recom-
mended to help prevent development of biliary
cancer (15).
PBM is diagnosed on the basis of imag-
ing findings or anatomic findings at surgery or
autopsy (16). With the development of imaging
modalities, radiologists have come to play an
important role in diagnosis of PBM. Radiolo-
gists should be aware of this entity to ensure
early diagnosis. PBM may be uncommon
outside of Asia, but characteristics of PBM in
other populations are similar to those in Asian
populations (5).
Figure 1.  Pathophysiology of PBM. PBM is a
congenital malformation in which the pancreatic
and bile ducts join outside the duodenal wall, usu-
ally forming a long common channel. In PBM, the
sphincter of Oddi does not regulate the PBJ, and
reciprocal reflux between pancreatic juice and bile
occurs. This results in diseases such as biliary cancer
and pancreatitis.
In this review, we present an overview of
PBM with emphasis on its diagnosis, manage-
ment, and complications.
Embryology
An understanding of normal development of
the bile duct and pancreas is helpful to better
understand PBM.
The hepatobiliary system and pancreas origi-
nate from the embryonic foregut. Early in the
4th week of gestation, the hepatic diverticulum
initially appears in the ventral wall of the fore-
gut, which later becomes the duodenum. At this
time, the lumen of the hepatic diverticulum is in
the solid stage. The liver diverticulum separates
into caudal and cranial portions. The cranial
portion becomes the liver and extrahepatic
biliary tree. The caudal portion develops into
superior and inferior buds. The gallbladder and
cystic duct develop from the superior bud, and
the right and left ventral pancreas develop from
the inferior bud.
During the 5th week, the proximal portion of
the hepatic diverticulum elongates, forming the
common bile duct. The dorsal pancreatic bud ap-
pears from the dorsal side of the foregut opposite
the liver diverticulum. As the foregut rotates and
the duodenum begins to form, the ventral pan-
creatic bud and bile duct rotate 180° around the
duodenum to join the dorsal pancreatic bud.
Recanalization of the common bile duct starts
in the 6th week and moves slowly distally. The
ventral duct joins the common bile duct, and they
drain into the duodenum via the major papilla
(Fig 2). When the ventral and dorsal pancreatic
380  March-April 2020 radiographics.rsna.org

Figure 2.  Normal development of the bile duct and pancreas. (a) The ventral pancreatic bud and biliary
system arise from the hepatic diverticulum. (b) The ventral pancreatic bud and bile duct rotate around
the duodenum to join with the dorsal pancreatic bud during the 5th week of gestation. The ventral duct
joins with the common bile duct, and they drain into the duodenum via the major papilla.

Figure 3.  Normal and abnormal PBJ anatomy. (a) Coronal two-dimensional (2D) MR cholangiopancreatogram in a 53-year-old
woman with PBM with biliary dilatation shows an abnormal PBJ (solid arrow) with a relatively long common channel (dotted arrow)
and dilatation of the extrahepatic and intrahepatic bile ducts (arrowhead). (b) Coronal maximum intensity projection (MIP) image
from three-dimensional (3D) MR cholangiopancreatography in a 64-year-old man with PBM without biliary dilatation shows an ab-
normal PBJ (solid arrow) with a long common channel (dotted arrow). Significant dilatation of the common bile duct is not present
(Courtesy of Toshihide Yamaoka, MD, PhD, Kyoto-Katsura Hospital, Kyoto, Japan). (c) Coronal MIP image from 3D MR cholangio-
pancreatography in a healthy 60-year-old man shows normal PBJ anatomy.

buds fuse, their ducts also fuse in the region of
the pancreatic neck. The proximal portion of the
dorsal pancreatic duct usually degenerates, leav-
ing the ventral pancreatic duct, which ultimately
becomes the major pancreatic duct (17–19).
Although the pathogenesis of PBM is still
controversial, several studies have suggested that
it involves anomalous development of the ventral
pancreas (9,10). It is hypothesized that the origin
of the common channel in patients with PBM
is related to the ventral pancreatic duct because
small pancreatic branch ducts arising from the
common channel have been demonstrated in some
patients (9). Ando et al (17) proposed the hypoth-
esis that abnormal fusion may occur between the
bile duct and branches of the ventral pancreatic
duct in PBM.
Morphologic Characteristics
PBM with versus without Biliary
Dilatation
PBM is divided into that with and without biliary
dilatation on the basis of dilatation of the common
bile duct (Fig 3) (15,20). Distinguishing between
these two types is important to understand the
diagnosis, pattern of complications, and treatment
strategy for PBM. PBM with biliary dilatation
(found in 77% of patients) is more common than
PBM without biliary dilatation (23%) (8).
To assess biliary dilatation, the maximum
diameter of the common bile duct is measured
by using nonpressure imaging modalities such
as US, CT, and MR cholangiopancreatography
(21). Because the standard diameter of the bile
RG  •  Volume 40  Number 2 Ono et al  381
Table 1: Diagnosis of Bile Duct
Dilatation at US according to Age
Dilatation
Patient Age (y) (mm)
0 ~3.1
5 ~4.0
10 ~4.6
15 ~5.1
20–29 ~6.0
30–39 ~6.4
40–49 ~6.8
50–59 ~7.3
60–69 ~7.8
~70 ~8.6
Adapted and reprinted, with permis-
sion, from reference 21.
duct is correlated with age, the diagnosis of bili-
ary dilatation should depend on patient age (21).
In children, the upper limit of the common bile
duct is 3.0 mm at 0 years, 3.9 mm at 5 years,
4.5 mm at 10 years, and 5.0 mm at 15 years. In
adults, this limit is 5.9 mm at 20–29 years, 6.3
mm at 30–39 years, 6.7 mm at 40–49 years, 7.2
mm at 50–59 years, 7.7 mm at 60–69 years, and
8.5 mm at 70 years or older (Table 1) (21).
In the clinical practice guidelines for PBM of
the Japanese Study Group on Pancreaticobiliary
Maljunction (JSPBM), PBM with biliary dilata-
tion is considered identical to congenital biliary
dilatation (15). Congenital biliary dilatation, which
is also known as a choledochal cyst, was initially
recognized as a congenital anomaly characterized
by local dilatation of extrahepatic or intrahepatic
ducts regardless of the presence of PBM (22).
Congenital biliary dilatation was subse-
quently redefined as a congenital malforma-
tion involving common bile duct dilatation and
PBM. This was done because congenital biliary
dilatation involving common bile duct dilata-
tion was found to be accompanied by PBM in
almost all cases (21). Whether congenital biliary
dilatation involving common bile duct dilata-
tion unaccompanied by PBM exists remains
controversial, although several cases have been
reported (23,24).
Other Morphologic Characteristics
Since Alonso-Lej et al (25) first described the
classification of congenital biliary dilatation, vari-
ous classifications of PBM and congenital biliary
dilatation have been proposed on the basis of
factors such as the site of dilatation and the form
of the PBJ (11,15,22,26).
In 1977, Todani et al (22) reported a classifi-
cation of congenital biliary dilatation. Todani’s
classification is now widely used, but it does not
include the concept of PBM. While Todani types
Ia, Ic, and IVa are accompanied by PBM, Todani
types Ib, II, III, IVb, and V are not.
In 2015, the JSPBM proposed a new PBM
classification, which is based on the formation
of the PBJ (11). This new classification divides
PBM into the following four types: (a) stenotic
type, in which the distal common bile duct with
stenosis joins the common channel, (b) nonste-
notic type, in which the distal common bile duct
without stenosis joins the common channel,
(c) dilated channel type, in which the common
channel is dilated, and (d) complex type, in
which the PBJ has formed in a complicated con-
figuration (Fig 4) (11).
Another classification of PBM that has been
classically used is the B-P (or C-P) type and
P-B (or P-C) type, in which the bile duct joins
the pancreatic duct (B-P) and the pancreatic
duct joins the bile duct (P-B), respectively (15).
However, because a thorough understanding of
these classifications for use in the clinical setting
is difficult, we describe the individual morpho-
logic features of PBM that are clinically impor-
tant to radiologists.
Primary Ductal Stricture.—Congenital bile duct
stricture of the distal duct or hilar duct is com-
mon in patients with PBM with biliary dilatation
(Fig 5) (27–29). Radiologists should recognize
this characteristic finding of PBM to differenti-
ate malignant from benign strictures. Among pe-
diatric patients, stricture of the distal common
bile duct is seen in 76% of patients with PBM
with biliary dilatation and 21% of those without
biliary dilatation (11). Wall thickening at the
stricture site may suggest malignancy because
primary ductal stricture lacks wall thickening
(Figs 6, 7).
Intrahepatic Biliary Dilatation.—PBM with
extrahepatic and intrahepatic biliary dilatation
is seen in nearly 50% of patients with PBM with
biliary dilatation (Figs 3, 5) (30). Intrahepatic bil-
iary dilatation is often accompanied by stricture
of the hilar duct (27,28,31). Stricture of the hilar
duct is seen in 56% of pediatric patients with
PBM with intrahepatic biliary dilatation (31).
There is a hypothesis that congenital ductal
stricture causes the biliary dilatation seen in
patients with PBM (28,29), but the mechanism is
unclear. Intrahepatic biliary dilatation can persist
even after extrahepatic bile duct resection and
can be associated with postoperative cholangitis
and intrahepatic stones (31).
382  March-April 2020 radiographics.rsna.org

Figure 4.  Classification of PBM proposed by the JSPBM. Type A = stenotic type, in which
a distal common bile duct with stenosis joins the common channel. Type B = nonstenotic
type, in which a distal common bile duct without stenosis joins the common channel. Type
C = dilated channel type, in which the common channel is dilated. Type D = complex type,
in which the PBJ has formed in a complicated configuration. (Reprinted, with permission,
from reference 11.)

Complex Type of Maljunction.—Complicated

union of the pancreatic and bile ducts is a rare
variant (5.7%) (Fig 8) (8). This variant contains
PBM associated with an annular pancreas or
pancreas divisum (11,32). A complicated conflu-
ence pattern may be difficult to depict completely
with imaging modalities other than direct cholan-
giography (20). Radiologists should pay attention
to the abnormal junction of the bile and pancre-
atic ducts at CT or MR cholangiopancreatogra-
phy to identify PBM.

Diagnosis
Diagnostic Criteria for PBM
According to the JSPBM’s committee on the
diagnostic criteria for PBM, PBM is diagnosed
on the basis of imaging features or the results
of anatomic examination. The presence of an
abnormally long common channel or an abnor-
mal union between the pancreatic and bile ducts
or a PBJ outside the duodenal wall confirms the
diagnosis (Table 2) (16).
There is no clear definition of a long common
channel, although a common channel longer than
10 mm is usually present in patients with PBM
(33). The length of the normal common channel
varies from 1 to 12 mm (34). While some authors
suggest 8 mm or longer as a long common chan-
nel (35), others suggest longer than 15 mm (36).
Diagnostic criteria for PBM were proposed in
the Japanese language in 1987 and were slightly
revised and published in English in 1994 (37,38).
These criteria underwent thorough revision in
2013, mainly because recent advances in diag-
nostic modalities such as multidetector CT and
MR cholangiopancreatography were taken into
account (16). Because CT and MR cholangio-
pancreatography were not effective in depicting
an anomalous PBJ a few decades ago, PBM was
diagnosed mainly with direct cholangiography
such as ERCP.
Figure 5.  Primary ductal stricture in a 64-year-
old man with PBM with biliary dilatation. Coro-
nal MIP image from 3D MR cholangiopancrea-
tography shows an abnormal PBJ (solid arrow). A
hilar bile duct stricture (dotted arrow) and dila-
tation of both the extrahepatic and intrahepatic
ducts are also depicted.
Multidetector CT and MR cholangiopan-
creatography can now obtain high-resolution
images of the pancreaticobiliary anatomy and
have become important in diagnosis of PBM and
its complications. However, in patients with a
relatively short common channel, direct cholangi-
ography is needed to demonstrate that the effect
of the sphincter of Oddi does not extend to the
junction (16).
Diagnosis of PBM
Radiologists now have more opportunities to
diagnose PBM because of advances in diagnos-
tic imaging modalities. When should we suspect
PBM and take a closer look at the pancreatico-
biliary anatomy?
PBM with Biliary Dilatation.—Approximately
50% of cases of PBM with biliary dilatation are
diagnosed in children (8). PBM with biliary dila-
tation tends to have a higher incidence of symp-
RG  •  Volume 40  Number 2 Ono et al  383
Figure 8.  Complex-type PBM in a 72-year-
old woman. Coronal MIP image from 3D MR
cholangiopancreatography shows the dilated
common bile duct and pancreatic duct joining
at two points (arrows) to form a complicated
union.
toms in childhood than PBM without biliary
dilatation (8). The main symptoms are abdominal
pain, vomiting, jaundice, and fever (8). In par-
ticular, abdominal pain occurs regardless of age
or the presence of biliary dilatation (8).
Figure 6.  Primary ductal stric-
ture in a 50-year-old woman
with PBM. Coronal contrast-en-
hanced multidetector CT mul-
tiplanar reconstruction (MPR)
image (a) and coronal 2D MR
cholangiopancreatogram (b)
show a distal bile duct stricture
near the abnormal PBJ (arrow).
No wall thickening is seen at
the stenosis site (arrow in a).
Figure 7.  Distal bile duct cancer in a 71-year-
old man with PBM. (a) Coronal contrast-en-
hanced multidetector CT MPR image shows a
distal bile duct stricture with focal wall thickening
and enhancement (arrow). The pancreatic duct
is not well depicted on multidetector CT images.
(b) Coronal ERCP image depicts the abnormal
communication of the pancreatic and bile ducts
(solid arrow) and a short common channel (dot-
ted arrow).
At imaging, common bile duct dilatation is
a characteristic finding suggestive of PBM with
biliary dilatation. Depicting dilatation of the com-
mon bile duct at US may be the first opportunity
to diagnose PBM with biliary dilatation (Fig
9) (15). Further study with MR cholangiopan-
creatography is recommended as a noninvasive
investigation in such patients.
PBM without Biliary Dilatation.—Abdominal
pain is also a major symptom of PBM without
biliary dilatation in pediatric and adult patients
(8). However, because of less frequent symptoms
in childhood and less remarkable imaging findings
than in patients with PBM with biliary dilatation,
PBM without biliary dilatation is more difficult to
diagnose. Many patients with PBM without biliary
dilatation are not diagnosed until adulthood (8).
Accordingly, many adult patients are diag-
nosed on the basis of clinical signs of associated
biliary cancer (39). Biliary cancer develops at an
average age of 60 years in patients with PBM,
which is 15–20 years earlier than in those with-
out PBM (8). For this reason, we should pay
attention to the pancreaticobiliary anatomy of
relatively young patients with biliary cancer.
384  March-April 2020 radiographics.rsna.org

Table 2: Diagnostic Criteria for PBM

Definition
  PBM is a congenital malformation in which the pancreatic and bile ducts join outside the duodenal wall.
Pathophysiology
  In PBM, the duodenal papillary sphincter (sphincter of Oddi) fails to exert any influence on the PBJ because
of the abnormally long common channel. Therefore, reciprocal reflux between pancreatic juice and bile oc-
curs, resulting in various diseases, such as inhibiting the excretion of bile and pancreatic juice and causing
biliary cancer in the biliary tract and pancreas.
Diagnostic criteria (imaging features or anatomic examination help diagnose PBM)
  Imaging diagnosis
   An abnormally long common channel or an abnormal union between the pancreatic and bile ducts must be
evident at direct cholangiography, such as ERCP, percutaneous transhepatic cholangiography, or intra-
operative cholangiography; MR cholangiopancreatography; or 3D drip infusion CT cholangiography.
However, in patients with a relatively short common channel, it is necessary to confirm that the effect of
the papillary sphincter does not extend to the junction with direct cholangiography.
   PBM can be diagnosed if the PBJ outside the wall can be depicted at endoscopic US or on MPR images
obtained at multidetector CT.
  Anatomic diagnosis
   It should be confirmed at surgery or autopsy that the PBJ lies outside the duodenal wall or that the pancre-
atic and bile ducts unite abnormally.
Supplementary diagnosis (the following findings strongly suggest the existence of PBM)
  Elevated amylase levels in bile
   Pancreatic enzymes, especially amylase, in the bile within the bile duct and gallbladder obtained immediate-
ly after laparotomy (endoscopically or percutaneously) are generally at high levels. However, levels close
to or below the normal serum value are occasionally observed in patients with PBM.
   Clinical features similar to those of PBM, including elevation of pancreatic enzyme levels in bile, are observed
in some cases with a relatively long common channel, showing the effect of the sphincter on the PBJ.
  Extrahepatic bile duct dilatation
   PBM includes one type that is associated with bile duct dilatation (congenital biliary dilatation) and another
that is not (PBM without biliary dilatation). When cystic, fusiform, or cylindrical dilatation is depicted in
the extrahepatic bile duct, careful investigations are needed to determine whether PBM is present.
   Standard values for the maximum diameter of the common bile duct at each age are useful to help diagnose
PBM with or without biliary dilatation.

Source.—Reference 16.

Moreover, gallbladder wall thickening may
provide a clue to early diagnosis before cancer de-
velops (39,40). In patients with PBM, smooth and
irregular patterns of gallbladder wall thickening can
be seen at US (40), and such findings may not be
characteristic of PBM (41). However, the pancre-
aticobiliary anatomy of patients with gallbladder
wall thickening should be assessed by using MR
cholangiopancreatography or multidetector CT
when no specific cause of gallbladder wall thicken-
ing is demonstrated at US (Fig 10) (39,40).
When assessing gallbladder wall thickening in
patients with PBM, it is often difficult to distin-
guish mucosal hyperplasia from early gallbladder
cancer (Fig 10). However, this may not be prob-
lematic because risk-reducing cholecystectomy is
recommended for patients with PBM.
Imaging Modalities
Ultrasonography.—Although US alone may not
be sufficient to depict an anomalous junction of
the pancreaticobiliary duct, it is a useful nonin-
vasive tool to help screen for PBM (40). Extra-
hepatic bile duct dilatation or gallbladder wall
thickening can be seen at US, and this screening
may be the first opportunity to diagnose PBM
with or without biliary dilatation (39,40).
MR Cholangiopancreatography.—MR cholan-
giopancreatography has become a well-estab-
lished noninvasive modality used to demonstrate
pancreaticobiliary anatomy. It has become the
preferred modality over ERCP in imaging many
pancreaticobiliary diseases, including PBM. MR
cholangiopancreatography can be performed
without contrast agent injection or ionizing radia-
tion. Therefore, it is considered a second-line im-
aging modality after US for imaging PBM (42).
Three-dimensional (3D) MR cholangiopan-
creatography can obtain high-resolution images.
Source images and maximum intensity projection
(MIP) images obtained at 3D MR cholangiopan-
creatography are useful to show pancreaticobiliary
RG  •  Volume 40  Number 2 Ono et al  385

Figure 9.  PBM with biliary dilatation in a 40-year-old man who presented with upper abdominal pain.
(a) Sagittal (left) and axial (right) US images show dilatation of the common bile duct (*). (b) Coronal 2D MR
cholangiopancreatogram shows a complex-type PBM, in which the dilated common bile duct and pancreatic
duct join abnormally (arrow).

Figure 10.  Early gallbladder cancer associated with PBM without biliary dilatation
in a 49-year-old woman who had no symptoms. (a) Axial US images show polypoid
gallbladder wall thickening (arrows). (b) Coronal contrast-enhanced multidetector
CT image (left) shows irregular gallbladder wall thickening (arrows). Axial MPR im-
age (right) shows an abnormal PBJ (arrow). (c) Coronal 2D MR cholangiopancrea-
togram also shows an abnormal PBJ (arrow). Significant dilatation of the common
bile duct is not present.

is reportedly 75% in adults and 44%–65% in

anatomy. Since breath-hold 3D MR cholangiopan-
creatography is not yet widely available, breath-
hold 2D MR cholangiopancreatography can be
performed in patients whose irregular respiratory
rhythms cause artifacts at respiratory-triggered 3D
MR cholangiopancreatography.
In diagnosis of PBM, MR cholangiopancrea-
tography is superior to ERCP for depicting biliary
anatomy, including the intrahepatic bile duct (43).
The sensitivity of MR cholangiopancreatography
children (42,44,45). A disadvantage of MR chol-
angiopancreatography is that image quality can be
deteriorated by motion artifact. It is also difficult
to depict fine structures such as the pancreatico-
biliary anatomy in pediatric patients or a complex-
type PBJ at MR cholangiopancreatography.
With respect to functional imaging of recip-
rocal reflux between pancreatic juice and bile
in patients with PBM, it has been reported that
secretin-stimulated dynamic MR cholangiopan-
creatography and time–spatial labeling inversion
pulse (Time-SLIP) MRI can be used to visualize
pancreaticobiliary reflux and that gadoxetic acid–
enhanced MRI can be used to visualize biliopan-
creatic reflux (46–48).
Secretin stimulates the exocrine pancreas to se-
crete fluid, which improves visualization of the main
pancreatic duct at MR cholangiopancreatography.
386  March-April 2020 radiographics.rsna.org
Figure 11.  Post-ERCP cholangi-
tis in a 47-year-old man with PBM
without biliary dilatation who
presented with elevated levels of
aspartate transferase, alanine trans-
aminase, and alkaline phosphatase
after ERCP. (a) Coronal MPR CT
image before ERCP shows the com-
mon bile duct and pancreatic duct
(solid arrow) joining outside the
duodenal wall. A primary ductal
stricture (dotted arrow) is depicted
near the abnormal PBJ. (b) Coronal
post-ERCP MPR contrast-enhanced
multidetector CT image shows bile
duct dilatation (solid arrow) periph-
eral to the primary ductal stricture
(dotted arrow).
In patients with PBM, secretin-stimulated dynamic
MR cholangiopancreatography shows a retrograde
increase in signal intensity and enlargement of the
common bile duct (46).
The Time-SLIP method is a form of spin label-
ing that can provide selective inflow information
by selectively placing the inversion pulse and sup-
pressing the background. Compared with secretin-
stimulated dynamic MR cholangiopancreatogra-
phy, Time-SLIP MRI allows direct visualization
of pancreaticobiliary flow by placing the inversion
pulse at the pancreatic head and body (47).
Gadoxetic acid is a hepatobiliary-specific con-
trast agent that is taken up by hepatocytes and then
excreted into the bile ducts. Therefore, gadoxetic
acid–enhanced MRI can be used to demonstrate bil-
iopancreatic reflux in patients with PBM, although it
fails to depict reflux in 50% of patients (48).
Multidetector CT.—High-resolution multide-
tector CT provides multiplanar reconstruction
(MPR) images and has become more readily
available. MPR images from contrast-enhanced
multidetector CT can depict details of the pan-
creaticobiliary anatomy and help diagnose PBM.
The sensitivity of multidetector CT is reported
to be 58%–100% in adults and 20% in children
(45,49,50). Contrast-enhanced multidetector
CT can also be used for biliary cancer screening
and surveillance.
Drip-infusion cholangiography is a CT chol-
angiography technique involving intravenous
injection of biliary contrast agent. Drip-infusion
cholangiography can acquire high-resolution im-
ages of the biliary anatomy, may be able to depict
the communication of the pancreatic and bile
ducts, and is less invasive than ERCP. However,
drip-infusion cholangiography sometimes fails to
depict the pancreatic duct, preventing diagnosis
of PBM (43). As a result, the sensitivity of drip-
infusion cholangiography is not high, and it is
25%–38% in children (43,44).
Endoscopic US.—Endoscopic US is the third-line
modality after US, CT, and MR cholangiopan-
creatography. Because high-resolution images are
obtained at endoscopic US, endoscopic US can
help diagnose PBM by depicting the PBJ outside
the duodenal wall (51). Endoscopic US is also
used for screening and surveillance of biliary can-
cer after the diagnosis of PBM (52). Endoscopic
US should be performed by an experienced spe-
cialist because its diagnostic accuracy is operator
dependent (15).
Endoscopic Retrograde Cholangiopancreatog-
raphy.—ERCP can help confirm lack of effect of
the sphincter of Oddi on the PBJ even in patients
with a relatively short common channel (16).
The sensitivity of ERCP is reported to be 75% in
children (43). However, use of diagnostic ERCP
must be carefully weighed against the risk of
complications (20). Complications after ERCP
may occur in 7% of patients, with a fatality rate
of up to 0.3% (53). The major complication
is pancreatitis (occurring in 3.5% of patients),
followed by cholangitis, bleeding, and infection
(53). In patients with PBM, primary ductal stric-
ture is a risk factor for post-ERCP cholangitis
(Fig 11) (54).
Compared with other modalities, one of the
main advantages of ERCP is that it allows bile and
tissue sampling as well as biliary intervention. An
elevated amylase level in bile suggests reflux of
pancreatic juice through an anomalous PBJ (8).
ERCP is also used for histopathologic diagnosis
and to perform therapeutic procedures in patients
with possibly malignant biliary stricture.
Complications
Regurgitation of Pancreatic Juice and
Protein Plugs
In patients with PBM, the sphincter of Oddi does
not regulate the PBJ. Consequently, pancreatic
RG  •  Volume 40  Number 2 Ono et al  387
juice frequently refluxes into the biliary tract be-
cause the pressure is usually higher in the pancre-
atic duct than in the bile duct (55). Regurgitation
is continuous in patients with PBM, but it does
not cause symptoms on its own.
Symptoms such as abdominal pain, vomiting,
and jaundice can arise from increased pressure
in the bile and pancreatic ducts secondary to
obstruction of the common channel or a pri-
mary stricture of the distal bile duct. This can be
caused by impaction from a protein plug and is
often temporary (56,57).
Protein plugs consist mostly of lithostathine,
which is a soluble protein secreted by the pan-
creas (58). Most protein plugs are fragile and re-
solve spontaneously, which explains the transient
and intermittent symptoms of PBM (57). Radio-
lucent protein plugs are depicted at ERCP, but
no reports have described protein plugs shown at
CT or MR cholangiopancreatography (56,59).
Pancreatitis
Acute pancreatitis is more common in children
with PBM (30% of patients) than in adults with
PBM (9%), irrespective of the presence of biliary
dilatation (8). In many cases, pancreatitis is mild
and imaging findings are subtle, but pancreatitis
may be recurrent (60). Protein plugs are thought
to be one cause of acute pancreatitis associated
with PBM (61). Approximately 3% of patients
with PBM develop chronic pancreatitis (8).
Unlike chronic alcoholic pancreatitis, in which
diffuse radiopaque pancreatic calcifications are
usually observed, chronic pancreatitis in patients
with PBM is characterized by radiolucent protein
plugs in the common channel or in the main
pancreatic duct near the common channel (62).
It is unclear whether patients with PBM are more
susceptible to pancreatic cancer (63).
Biliary Stones
Bile duct and gallbladder stones occur more
frequently in adults with PBM (23% of patients)
than in children with PBM (9%). (8). Bile duct
and gallbladder stones were found in 16% and
6% of adult patients with PBM with biliary
dilatation, respectively, and in 6% and 18% of
those with PBM without biliary dilatation (8).
Pigmented stones (47% of stones) are more com-
mon than cholesterol stones (13%) among pa-
tients with PBM with biliary dilatation (64). Be-
cause about half of biliary stones are pigmented,
stone formation seems to be related to bile stasis
rather than pancreaticobiliary reflux (60).
Biliary Cancer and Carcinogenesis
PBM is associated with a high risk of biliary
cancer. Biliary cancer develops at an average age
of 60 years in patients with PBM, which is 15–20
years earlier than in patients without PBM (8).
The location and prevalence of the associated
cancer depend on the presence of biliary dilata-
tion. Gallbladder and bile duct cancers were
found in 13.4% and 6.9% of adult patients with
PBM with biliary dilatation, respectively, and in
37.4% and 3.1% of those with PBM without bili-
ary dilatation (8).
Most cancers arise in the gallbladder or dilated
common bile duct, suggesting that bile stasis is
related to carcinogenesis (8). However, with re-
spect to bile stasis in a dilated common bile duct,
it is unknown whether primary ductal stricture
plays a role in carcinogenesis. Because several
cases of synchronous or metachronous multifo-
cal cancers in the biliary system of patients with
PBM have been reported, radiologists should
keep in mind the possibility of multifocal biliary
cancer (65,66).
PBM is considered to involve a hyperplasia
to dysplasia to carcinoma sequence caused by
chronic inflammation induced by reflux of pan-
creatic juice into the biliary tract (12,13). Re-
fluxed pancreatic enzymes such as phospholipase
A2 (PLA2) are activated in the biliary system
(67). PLA2 is cytotoxic; additionally, it converts
phosphatidylcholine (PC) in bile to lysophospha-
tidylcholine (lysoPC), which is strongly cytotoxic
(67,68).
These cytotoxic substances stagnate in the
gallbladder or dilated bile ducts and irritate the
epithelium. The resulting chronic inflammation
causes epithelial hyperplasia and dysplasia of the
gallbladder and bile ducts (12,69,70).
Diffuse epithelial hyperplasia of the gallblad-
der is an important sign of disease in patients
with PBM (69). Epithelial hyperplasia can be
present in early infancy, and dysplasia develops
with age (71). Gene mutations are simultane-
ously induced in the biliary epithelium, includ-
ing mutational activation of the oncogene KRAS
in the early phase and inactivation of the tumor
suppressor TP53 in the late phase (69,71). These
data support the concept of the hyperplasia to
dysplasia to carcinoma sequence in the biliary
system of patients with PBM (Fig 12).
One report indicated that biliary infec-
tion with Helicobacter bilis, a Gram-negative
enterohepatic Helicobacter species, may play
a role in biliary cancer in patients with PBM
(72). Recent studies have indicated that H bilis
might be associated with biliary carcinogenesis
(73). Although the mechanisms of H bilis–in-
duced carcinogenesis are still unknown, H bilis
colonization is commonly present despite high
amylase activity in the bile of patients with
PBM (72).
388  March-April 2020 radiographics.rsna.org

Figure 12.  Biliary carcinogenesis in PBM. Pancreatic enzymes such as PLA2 regurgitate into
the biliary tract and are activated. PLA2 is cytotoxic and converts PC in bile to lysoPC, which
is also cytotoxic. These hazardous substances stagnate in the gallbladder or dilated bile duct
and irritate the epithelium. The resulting chronic inflammation causes epithelial hyperplasia
and dysplasia that progress to carcinoma through multiple molecular changes, including KRAS
activation and TP53 inactivation. This pathway is known as the hyperplasia to dysplasia to
carcinoma sequence.

Management and Treatment Postoperative Complications and

Surgical Treatment
Because PBM is a risk factor for biliary cancer,
risk-reducing surgery for patients diagnosed
with PBM is recommended regardless of the
presence of symptoms (15).
In patients with PBM with biliary dilatation,
most biliary cancers are located in the gallblad-
der or dilated common bile duct (8). Therefore,
resection of the extrahepatic bile duct including
the gallbladder is regarded as the standard sur-
gical procedure (15). The hepatic side should be
transected at the confluence of the right and left
hepatic ducts, and the pancreatic side should be
transected immediately above the junction of the
bile and pancreatic ducts (20,74).
Hepaticojejunostomy with Roux-en-Y anasto-
mosis is widely performed and prevents post-
operative gastritis caused by bile reflux, unlike
hepaticoduodenostomy (75,76). Several recent
reports have indicated that partial hepatectomy
at the initial surgery should be considered for
adult patients with PBM with extrahepatic and
intrahepatic biliary dilatation to decrease the
risk of carcinogenesis and cholangitis (77,78).
However, hepatectomy is invasive, especially
for pediatric patients, and no consensus has yet
been reached (20).
For patients with PBM without biliary dilata-
tion, prophylactic cholecystectomy is recom-
mended because most biliary cancers develop
in the gallbladder (15). Whether preventive
resection of the extrahepatic common bile duct
should be performed remains unclear (15,79).
Follow-up
Patients with PBM who have undergone risk-
reducing surgery must undergo imaging periodi-
cally for the rest of their lives. Postoperative com-
plications have been reported, including biliary
cancer arising from the residual bile ducts (15).
Patients with PBM are still at risk for biliary
cancer developing in residual bile ducts, includ-
ing the intrahepatic and intrapancreatic ducts,
even after prophylactic resection of the extrahe-
patic bile duct (Fig 13) (60,80) In patients with
PBM with biliary dilatation, the prevalence of
residual biliary cancer is 2%, and the mean time
until diagnosis of biliary cancer after surgery is
12 years (60).
Repeated cholangitis, intrahepatic stones,
and intrahepatic biliary dilatation are risk fac-
tors for developing cancer of the intrahepatic
residual bile duct (60). Biliary cancer develop-
ing in the common bile duct after cholecys-
tectomy was also reported in five patients with
PBM without biliary dilatation (81). Radiolo-
gists should carefully examine postoperative
follow-up images obtained in patients with
PBM, even years after surgery.
Cholangitis and intrahepatic stones are other
major postoperative complications after resection
of the extrahepatic bile duct. Intrahepatic stones
are seen in approximately 3%–10% of patients
after surgery (82,83). Cholangitis and intrahe-
patic stones often result from cholestasis because
of anastomotic stricture or the remnants of the
primary stricture and dilatation of the hilar bile
duct (82,83).
RG  •  Volume 40  Number 2 Ono et al  389

Figure 13.  Bile duct cancer arising from the re-

sidual intrapancreatic bile duct in a 45-year-old
man with PBM with biliary dilatation who had
a history of extrahepatic bile duct resection. He
initially presented with pancreatitis. (a) Coronal (top) and axial (bottom) MIP images from 3D
MR cholangiopancreatography show the residual intrapancreatic bile duct (solid arrow) com-
municating with the pancreatic duct. The residual intrahepatic bile duct is also shown (dotted
arrow). (b) Axial T2-weighted MR image shows a mural nodule in the residual intrapancre-
atic bile duct (solid arrow) communicating with the pancreatic duct (dotted arrow). (c) Axial
contrast-enhanced multidetector CT image shows enhancement of the solid component in the
residual intrapancreatic bile duct (black arrow). Peripancreatic fluid associated with pancreatitis
is also observed (white arrow). (Case courtesy of Toshihide Yamaoka, MD, PhD, Kyoto-Katsura
Hospital, Kyoto, Japan.)

Imaging Pitfalls gists should recognize this congenital stricture

Diagnosis of PBM without Biliary
Dilatation
PBM without biliary dilatation is difficult to
diagnose because of the lack of remarkable
imaging findings. It is particularly difficult to
identify PBM by using axial CT alone. How-
ever, obtaining MPR images and more closely
examining the bile and pancreatic ducts may be
helpful. Further imaging with MR cholangio-
pancreatography or ERCP should be considered
when imaging features suggestive of PBM are
seen at CT or when a relatively young patient
presents with biliary cancer or gallbladder wall
thickening.
Primary Ductal Stricture Mimicking
Biliary Cancer
Primary ductal stricture of the distal or hilar
bile duct may mimic biliary cancer. Radiolo-
of PBM to differentiate malignant from benign
strictures. Wall thickening at the stricture site
may suggest malignancy because primary ductal
stricture lacks wall thickening (Figs 6, 7).
Biliary Cancer in Residual Intrapancreatic
Bile Duct Mimicking Intraductal Papillary
Mucinous Neoplasm
Patients with PBM are still at risk for biliary
cancer developing in the residual bile ducts,
including the intrahepatic and intrapancreatic
ducts (60,80). Biliary cancer in the residual
intrapancreatic bile duct after extrahepatic bile
duct resection may mimic intraductal papillary
mucinous neoplasm (Fig 13). When examining
postoperative follow-up images in patients with
PBM, the clinician should keep in mind that
biliary cancer can occur in the intrahepatic and
intrapancreatic residual ducts even years after
surgery.
390  March-April 2020 radiographics.rsna.org
Conclusion
Radiologists have come to play an important
role in diagnosis of PBM and its complications
(including biliary cancer) because of progress
in the development of imaging modalities. An
understanding of the morphologic characteris-
tics of PBM helps diagnose this malformation.
Specifically, the presence of extrahepatic bile
duct dilatation or gallbladder wall thickening may
provide a clue to early diagnosis of PBM with or
without biliary dilatation, respectively. We should
carefully evaluate follow-up images of patients
with PBM even years after prophylactic surgery
because the remaining bile ducts are still at risk
for biliary cancer.
Acknowledgment.—We would like to thank the Edanz Group
for editing a draft of the manuscript.
Disclosures of Conflicts of Interest.—A.O. Activities related to
the present article: proofreading by the Edanz Group; received
joint research funds with HIMEDIC. Activities not related to the
present article: disclosed no relevant relationships. Other activi-
ties: disclosed no relevant relationships. S.A. Activities related
to the present article: disclosed no relevant relationships. Activi-
ties not related to the present article: received scientific research
grant from KAKENHI. Other activities: disclosed no relevant
relationships.
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