IMAGES FOR SURGEONS
Pancreaticobiliary maljunction: a rare finding on a routine
intra-operative cholangiogram with significant implications
A 30-year-old female underwent elective laparoscopic cholecystec-
tomy for biliary colic. Prior to surgery, she had experienced recur-
rent epigastric pain for 2 months necessitating one presentation to
the emergency department. Biochemistry showed normal inflamma-
tory markers, liver function tests and amylase. Abdominal ultra-
sound demonstrated cholelithiasis without signs of cholecystitis.
The patient had no medical comorbidities and had not undergone
previous abdominal surgery.
The cholecystectomy was performed without complications.
Routine intraoperative cholangiography was performed which sur-
prisingly revealed pancreaticobiliary maljunction (PBM), a rare
congenital anomaly where the bile duct and pancreatic duct con-
verge outside the duodenal wall (Fig. 1). A long common channel
measuring 3 cm can be seen draining into the third part of duode-
num; a smaller accessory pancreatic duct (duct of Santorini) drains
into the second part of duodenum. The bile duct and common chan-
nel are not dilated, however there is a possible stricture of the mid
common bile duct. Considering the non-dilated state of the biliary
tree, this is a Type-B PBM as classified by the Japanese Study
Group on Pancreaticobiliary Maljunction (JSGPM).1
Fig. 1. Intraoperative cholangiogram performed during laparoscopic cho-
lecystectomy. Blue arrow: common bile duct, white arrow: common
channel, white triangle: main pancreatic duct, blue triangle: accessory
pancreatic duct.
ANZ J Surg 93 (2023) 1106–1107
PBM is a rare entity in the Western population with an estimated
incidence of 1:50 000–150 000.2 It is far more common in Asia,
particularly Japan, where the incidence can be as high as 1:1000.2
PBM is thought to develop due to misarrangement of the embry-
onic connections in the pancreaticobiliary ductal system and can
occur either with or without concomitant congenital biliary dilata-
tion (CBD). PBM is divided into four subtypes based on the config-
uration of confluence between the distal bile duct and pancreatic
duct.1
Clinically PBM is associated with cholangitis, pancreatitis and
biliary tract malignancy likely due to pancreaticobiliary reflux.3
Pancreaticobiliary reflux leads to chronic inflammation of the bili-
ary system which precipitates carcinogenesis via the hyperplasia-
dysplasia-carcinoma sequence. It may also result in the formation
of protein plugs and subsequent obstruction of both bile and pan-
creatic juice.
Patients may present with intermittent abdominal pain, jaundice
or an elevated amylase level.1,4 When associated with CBD a clas-
sical triad of abdominal pain, jaundice and an abdominal mass has
been described.3 Children are more likely to be symptomatic from
PBM regardless of subtype as are those with associated CBD.4
PBM without biliary dilatation, (Type-B, as seen in our patient),
are often asymptomatic and, therefore, usually diagnosed inciden-
tally during adulthood, often concurrently with advanced biliary
tract malignancy.4
Due to its association with biliary cancers, the treatment recom-
mendation for adults with PBM and CBD is extrahepatic bile duct
resection.3 The treatment recommendation for PBM without biliary
dilatation remains contentious. A consensus meeting from 2012
recommended prophylactic cholecystectomy due to the high risk of
developing gallbladder cancer (37.2%), but no recommendation
was made regarding management of the extrahepatic bile duct as
malignancy was found in only 4% of patients.3,5
Despite being one of the most diverse nations in the world, PBM
remains a rare condition in Australia.6 Recognition of PBM is
important due to its association with malignancy and need for pro-
phylactic surgical intervention. The abnormal anatomical arrange-
ment may also lead to confusion during operative cholangiography
and potentially increase the risk of major bile duct injury.
Author contributions
Min Yien Tan: Data curation; investigation; writing – original
draft. Daniel James Vagg: Conceptualization; supervision; writing
– review and editing.
© 2022 Royal Australasian College of Surgeons.
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Images for surgeons 1107

References 5. Kamisawa T, Ando H, Suyama M, Shimada M, Morine Y, Shimada H.

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maljunction. J. Gastroenterol. 2012; 47: 731–59.
creaticobiliary maljunction and clinical features in children.
6. Free J, Wang F, Williams N et al. Gallbladder mucosal lesions associated
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with high biliary amylase irrespective of pancreaticobiliary maljunction.
2. Hyvärinen I, Hukkinen M, Kivisaari R, Parviainen H, Nordin A,
ANZ J. Surg. 2018; 88: E517–21.
Pakarinen MP. Increased prevalence of pancreaticobiliary maljunction in
biliary malignancies. Scand. J. Surg. 2019; 108: 285–90.
3. Kamisawa T, Ando H. Pancreaticobiliary Maljunction and Congeni- Min Yien Tan,* MBBS
tal Biliary Dilatation. Singapore: Springer Singapore Pte. Lim- Daniel James Vagg,*† MBBS, FRACS, PhD
ited, 2018.
*Department of Surgery, Nepean Hospital, Sydney, Australia and
4. Yoshimoto K, Kamisawa T, Kikuyama M, Kuruma S, Chiba K,
†Faculty of Medicine and Health, Nepean Clinical School, The
Igarashi Y. Classification of pancreaticobiliary maljunction and its
clinical features in adults. J. Hepatobiliary Pancreat. Sci. 2019; 26:
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doi: 10.1111/ans.18144

© 2022 Royal Australasian College of Surgeons.