HPBSurgery, 1995,Vol. 8,pp. 185-186
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Gall BladderTumour, Choledochal Cyst and
anAnomalousPancreatico-BiliaryJunction
T. F. TOUFEEQ KHAN,ZAHEER A. SHERAZI and YAN YEW TAN
Department of Surgery, Department of Radiology, Department of Surgery Hospital Universiti Sains Malaysia,
School of Medical Sciences, Universiti Sains Malaysia
This report highlights the association ofa tumour in an acalculous gall bladder with an anomalous
pancreatico-biliaryjunction (PBJ) and a type IVa choledochal cyst. Cholecystectomy and Roux-
en-Y hepatico-jejunostomy (RHJ) was performed after division ofthe common bile duct (CBD)
and excision ofthe dilated segment. The details ofthe case are presented and the role ofan abnormal
PBJ in gall bladdercarcinogenesis is discussed.
KEYWORDS: Anomalouspancreatico-biliaryjunction gallbladder tumour choledochalcyst.
INTRODUCTION
An abnormal PBJ occurs when the pancreatic duct unites
with the distal CBD proximal to the duodenal sphincter.
This results in the continous reflux ofpancreatic enzymes
into the biliary tree. The repeated inflammation eventually
leads to mucosal changes in the biliary tract. Recently, the
abnormal PBJ has been shown to be associated with mali-
gnant changes in the gall bladder1. In patients with an
abnormal PBJ, the aim ofsurgery is to prevent pancreatic
enzyme reflux by disconnecting this junction from the
biliary tree and to perform a bilio- enteric anastomosis. We
report a case ofa tumour within an acalculous gall bladder
with an abnormal PBJ. The aetiological correlation in the
light ofpresent concepts is discussed.
CASEREPORT
A 49 year old housewife was admitted to Hospital
Universiti Sains Malaysia with acute right upper abdo-
minal pain and vomiting of3 days. She was admitted with
similar complaints andjaundice 2 years ago when ultra-
sound revealed a soft tissue mass in the gall bladder. She
was offered surgery but defaulted.
On examination, mild tenderness was elicited on palpa-
tion in the upper abdomen. She was non-icteric with
Address for correspondence." Department of Surgery, Hospital
Universiti SainsMalaysia, 16150 KubangKerian, Kelantan, Malaysia
normal liver function tests. An ultrasound scan revealed a
hyperechoic soft tissue mass without acoustic shadowing
fillingthe gall bladder (Fig. a &b). Endoscopic retrograde
cholangio-pancreatography (ERCP) showed a grossly dil-
ated cystic duct and a gall bladder with multiple filling
defects. The CBD appeared dilated and was overlapped by
the large calibre cystic duct. The proximal right hepatic
duct was also dilated and an abnormal PBJ was evident
(Fig. 2). A diagnosis of carcinoma of gall bladder asso-
ciated with an abnormal PBJ and type IVa choledochal
cyst was made. Laparotomy confirmed a soft tissue tum-
our filling the gall bladder. The cystic duct measuring 1.2
cm was entering the anterior wall ofthe dilated CBD.
Per-operative cholangiography revealed essentially the
same findings as ERCP. A cholecystectomy and an exci-
sion ofthe supraduodenal CBD was performed. The lower
end was closed behind the duodenum and a 60 cm Roux
loop was used to fashion a hepatico-jejunostomy. She
made an uneventful recovery and was discharged ten days
after surgery. Histology revealed a villous adenoma with
areas ofmetaplasia and some atypical cellswithout stromal
involvement. She remains symptom free at 14 months after
surgery.
DISCUSSION
The abnormal PBJ is thejunction ofthe terminal bile duct
and pancreatic duct proximal to the duodenal sphincter
and enters the duodenum as a single duct or a long
185
186 T.F. TOUFEEQ KHAN et al.
cer-5. The aetiological role of the abnormal PBJ in gall
bladdertumour seems likely in ourpatient especially in the
absence of gall stones and considering her younger age
group. Twenty-five percent ofgall bladdercancers arise in
acalculous gall bladders6. The level ofpancreatic enzymes
in the gall bladder is higher than in the bile ducts in these
patients because ofits concentrating ability. The resulting
long standing inflammation induces mucosal changes
within the gall bladder and the biliary tract. The
association ofmetaplasia with dysplasia and carcinoma is
strengthened by the fact that these latter changes are only
seen in areas ofmetaplasiaT. The association ofmetaplasia
with gall bladder cancer has also been suggested by
others8,9. There has been increasing evidence ofcoexisting
pancreatico- biliary anomalies in patients with chole-
dochal cysts.This infers a collection ofdevelopmental
defects in the pancreatico-biliary ducts ratherthan a single
discrete lesion as demonstrated in our patient.
In gall bladder tumours without gall stones an
abnormal PBJ must be vigorously sought for by ERCP or
per-operative cholangiography. Ifdiscovered, in addition
to cholecystectomy, we advocate separation ofthe biliary
and pancreatic systems by division of the bile duct, to
prevent further long term complications.
Figure a, b Ultrasonogram of the gall bladder, sagittal (a) and
axial scans (b) showing a hyperechoic soft tissue mass without
acoustic shadowing filling the lumen.
Figure 2 ERCP demonstrating the abnormal pancreaticobiliary
junction (arrow) and the dilated cystic duct. Cystic dilation is seen
in the area of the common bile duct (C) and the right hepatic duct
(arrow head).
common channel. Being void ofsphincteric control, pan-
creatic enzymes can reflux into the biliary tree and gall
bladder. It has been proposed that the abnormal PBJ may
be responsible for the development of gall bladder can-
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