and perforated peptic ulcer, it is the third most common cause of an acute abdomen in Chinese people requiring emergency ad- mission.#{176}The disease.
5974
OCTOBER,
RECURRENT
By
PYOGENIC CHOLANGITIS CHINESE IMMIGRANTS* M.B.B.S., F.R.C.P. (C),t and D. TORONTO, ONTARIO, CANADA
C. S. HO,
ECURRENT
widened
pyogenic cholangitis, also known as cholangiohepatitis, onental cholangiohepatitis, and Hong Kong disease, is prevalent in South East Asia. In
Hong
Kong
after
acute
OF
appendicitis
CASES
CASE I. S.C.L. A 4I year old man presented with acute right upper quadrant pain. He had had similar pain in the past and had had a cholecystectomy and exploration of the cornmon bile duct 2 years previously. On admission, he had scleral icterus and a temperature of 38#{176}C.Abdominal examination revealed right upper
quadrant
organs
or masses. hemoglobin cell count
were:
but
tenderness,
Pertinent gm.
15.1
no
and
normal
and perforated peptic ulcer, it is the third most common cause of an acute abdomen in Chinese people requiring emergency admission.#{176}The disease is occasionally seen in North America and we have encountered it in 5 Chinese immigrants over the past 3 years. In 2 patients it was diagnosed preoperatively on the basis of cholangiographic findings. REPORT
C
I,
the
II.
F.T.W.
(1-36);
and
large
soft
stones
the
common
bile
duct
in diameter. It contained up to 2.5 cm. in diameter.
was many Open
markedly
a
stones ducts.
A
laboratory *
From
standards
often
given
changed
the Departments
difficult
in brackets. during of Radiology,t
to These the
vary period and
find. slightly
In
operation,
CASE
attacks
her
she
Surgery,
M.C.
A 67 year
of
severe
epigastric
for
many
clinically
had
below were:
General
20).
showed
the
of
and bile
recurrent
and
confirmed
(Fig.
2B).
to
have
found
old woman pain
years.
chole-
and
cu.
had
fever
of right
involuntary
edge
margin. 11.2
phosphatase (1-36); and
a
she
revealed with
mm.:
to
admission,
liver
costal
had had
radiating
On
hemoglobin
per
duct
common
made
a palpable
23,900
(48-I 211)
bile
tenderness
breadths
S.U. (Fig.
examination
quadrant
2
finger-
Laboratory
gm. bilirubin
ioo serum
per cent; 7.6 mg.
1.1]. (15-50); amylase i6o
(4o-r’o).
At operation
368
woman
and
jaundiced
and
to
was
III.
guarding
as the
Toronto
plates.
a markedly dilated common of bile grew E. co/i. Choleside-to-side choledochoduoperformed.
per cent; alkaline SGOT 200 l.U.
of study.
was
were
back
was
6
cholangiography
denostomy
values
the
pre-
was
diagnosis
docholithiasis and bile duct. Cultures cystectomy an d
S.C.
and range
cell
old
hepatic
cholangitis
operative
were
small
year
common
common
preoperative
pyogenic
WBC
Normal
69
amylase
dilated
in the
liver biopsy showed the intermediate and large portal tracts to be notably widened and prominent in contrast to the terminal triads which *
liver
cholangiogram
upper
cholangitis.
terminal
A
serum
Intravenous
rent
operation to 3 cm.
was
rant abdominal tenderness, but no palpable organs or masses. Laboratory work-up revealed hemoglobin 12.6 gm. per cent; WBC 1,600 per cu. mm.; bilirubin 3.7 mg. per cent; alkaline phosphatase 100 I.LT. (I#{231}-#{231}o); SGOT 53 I.U.
38.2#{176}C. Abdominal
pyogenic
fibrosis
choledochoduodenostomy
blood (WBC) 12,300 per cu. mm.; bilirubin 5.9 rng. per cent; alkaline phosphatase 180 I.U. (I5_8o);* SGOT 151 1.L. (1-36); and serum amylase 55 S.U. (4o-17o). The intravenous cholangiogram (Fig. i’l) and the T-tube cholangiogram (Fig. IB) obtained after his cholecystectomy 2 years previously were reviewed. The abnormalities in the biliary tree were recognized as those of recurAt dilated
the
had a cholecystostomy 40 years ago and had been troubled with recurrent bouts of epigastric pain since. On admission she was afebrile and clinically anictenic. She had night upper quad-
At
laboratory findings per cent; white
tracts,
including
CASE
M.D4
periductal and was accompanied inflammatory cell infiltrate (Fig. D). The hepatic parenchyma was
An end-to-side performed.
by
palpable
E. WESSON,
portal
dominantly by a chronic
IN
contain Hospital,
the common biliary Toronto,
sludge Ontario,
bile
duct
mixed Canada.
was with
found foul,
VOL.
No.
122,
Recurrent
2
Pyogenic
Cholangitis
Chinese
in
Immigrants
I. Case I. (A) Intravenous cholangiogram shows dilated common A large nadiolucent stone (arrow) is demonstrated in the latter. (B) bile duct and intrahepatic duct (lower arrow) are dilated. The upper duct with short branches arising at right angles to the duct.
bile duct and common ‘F-tube cholangiognam.
FIG.
bile,
murky
cultures
gallbladder
was
ectomy
was
explored
of which
small
and
performed
and
maintained
and
irrigated.
6
for
weeks.
changes
of
recurrent
E.
the
bile
11
duct and
pyogenic
Her
WBC
was
cu. mm. Her Other values I.L’. (15-50);
revealed
CASE
back. years
A
A 55
epigastric
and was
Abdominal
old
woman
radiating had
Shortly
fever
year pain
cholecystectomy
previously.
developed temperature jaundiced.
Y.W.
IV.
with
before
been
preinto
her
done
12
admission
she
chills and on admission her 39.7#{176}C. She was clinically examination
was
unre-
sinensis
S.C.
were
mon
bile
her
common
ograms tree
Ova in her
mucoid changes
4)
cent,
and
to
18,900
per
re-
duct
was
typical
C/onorchis
At
of the comoperation,
found
material. revealed
blood
of
stools.
and T-tube drainage were performed.
(Fig. with
per
Repeated
(40-170).
present
bile
inspissated
gm.
rising
negative.
duct
bile
rose to 3.3 mg. per cent. alkaline phosphatase 435 164 I.U. (1-36); and serum
SGOT
Exploration
dilated
investigations 8..
subsequently
were
hepatic duct. The common
moderately
of
bilirubin were:
2,600
cultures sented
shows
laboratory
of6,6oo
amylase
cholangitis.
arrow
a hemoglobin
was
T-tube
B)
and
markable. vealed
drainage
Operative
3,
The
co/i.
A cholecyst-
T-tube
(Fig.
cholangiograms
grew fibrotic.
369
to
T-tube an
abnormal
of recurrent
be
full
cholangibiliary pyogenic
of
C. S. Ho
370
and
OCTOBER,
phosphatase (1-36); and
..._.sL
7
D. E. Wesson 150
and
was ‘‘::‘.::;: -
..
-.-‘
SGOT 200 I.U. S.U. (4o-I7o). grew gram negative bacilli. the gallbladder contained many
serum
Blood cultures At operation, stones
I.U.
(i-o);
amylase
frank
pus.
indurated.
abnormal
3974
270
The
head
of the
Cholangiograms
biliary
tree.
A
pancreas
revealed
an
cholecystostomy
was
%-
..
performed.
Three
months
was removed and ( Fig. ) revealed
later
the
the T-tube mild changes
gallbladder
cholangiogram of recurrent
-
pyrogenic
eL-j’#{149}
.
cholangitis.
.‘-.
DISCUSSION
t’
?
‘-
‘-
#{149}.,
V
,..,J,,..
.,
.
.. #{149}
“I
.‘
‘.i.’
CLINICAL
The
,
#{149}
\
-
‘a’
4.
,
.
‘,
.
:‘
j!,,:
‘C
,
#{149}5IC’
C,
1#{149} I,,.
4
,‘
#{149}
I,
#{149}1e
V
FIG.
I .
(C) Portal
portal
area
and periportal
is widened
demarcated
fibrosis
is
periductal and is accompanied by cell infiltrate. The parenchymal normal. (Hematoxylin and Eosin, Higher magnification of a portion
X21o.) (D) shows peniductal The inflammatory cytes and plasma
is intact.
The
edema cells
and
concentric
fibrosis.
are predominantly
cells.
The
(Hematoxylin
and
hepatic
Eosin,
fever,
lent
Liver
plate
X45o.)
showed
acute
had
was
a
Y.G.
and history
anicteric.
tenderness was palpable
right of
hours chills
was
tion
reveals
right
upper
after
and
than
hemoglobin
per
bilirubin
4.0
rose
at that cent;
mg.
WBC per
cent;
time
fe-
alkaline
duct.
epi-
tenderness
is usual
and
this
later,
affected
are
and
jaundice. Serum
are tests
more
delayed
to per
elevated.
are
in most
usually
is
severely
in
In
addi-
Cases
iii,
reflect
the
din-
obstructive
is usually present. alkaline phosphatase
Other
liver
function
Cultures
show
E.
of
bile
co/i infection
patients.
usually seen
cases
unfamiliarity
and
normal.
at operation
Roentgenologic are
sepsis
Leukocytosis bilirubin and
the
over
milder
to emigrate.
of
both
were
(.
patients
was
features
in is
because
unlikely
diagnosis
rigidity liver
disease
is probably
of
examina-
enlarged the spleen is palof patients.3 at a later age
The
in
drainage
Physical
quadrant.
present
were:
14,100
quadrant Each
surgical
bile
This
obtained
he developed
temperature
values
cu. mm.;
quadrant
the liver edge costal margin.
admission
his
39.8#{176}C.Laboratory 13.7 gm.
upper
and the
upper
in about 6o per cent and pable in about 25 pen cent Our patients presented
ical
He
pancreatitis.
right
young and
jaundice.
and v due to our previous with the disease. The laboratory findings
man presented quadrant pain.
upper
guarding cm. below
2
old
recurrent
There with
Twenty-four shaking
An 84 year
right
and
Iv,
puru-
cholangitis.
CASE v. with vomiting
He
biopsy
in
Males
age.
immediate
common
tion, cholangitis.
of
chills
the
55).
lympho-
terminal
occurs of
attacks
pain,
requiring
)
region of liver. The fibrosis and is sharply
the parenchyma.
from
predominantly an inflammatory liver cells are
by
I
#{149}(3
p
FINDINGS
sode may last several days and then subside gradually. However, the initial attack or any subsequent attacks may produce Severe septicemia and obstructive jaundice
,i,’5
#{149}
I’,
1%
usually years
30-40
recurrent
:-
‘4.
LABORATORY
males are affected equally. Both the age and sex incidence contrast to the usual gallbladder disease which occurs more commonly in older people and females. Clinically, the disease is characterized by
%\
#{149}
AND
disease
adults,
-
5,.
#{149}‘,‘,
FEATURES
findings unrevealing.
in
the
biliany
before
operation
Occasionally,
tract
because
air
of
VOL.
322,
No.
Recurrent
2
Pyogenic
Cholangitis
in Chinese
Immigrants
2. Case ii. (A) Intravenous cholangiogram shows a dilated common bile duct one in the common hepatic duct and the other in the common bile duct (arrows). gram shows markedly dilated intraand extrahepatic ducts. Note the typical
with (B)
FIG.
arrow)
and
the
stricture
(long
sphinceither poor function or nonvisualization of the gallbladder because of the elevated serum bilirubin. Intravenous cholangiography sometimes shows a dilated common bile duct with nadiolucent stones. Operative and T-tube cholangiograms are characteristic8 and are described below.
At larged
operation and
AT
the congested.
OPERATION
liver
is Liver
2 radiolucent Operative
“arrowhead”
stones, cholangio-
sign
(short
arrow).
fistula formation or an incompetent ten.4 Oral cholecystography shows
FINDINGS
371
invariably biopsy
enshows
distinct features. There is a marked increase in fibrous tissues around the portal tracts, especially around the bile ducts. Infiltration by inflammatory cells is seen in the portal tracts and the adjacent liver parenchyma. The gallbladder may be enlarged and may show chronic inflammatory changes. Stones in the gallbladder are seen
The common bile duct is usually dilated. Gross dilatation up to 10 cm. has been reported. The walls are usually thickened by fibrosis, but occasionally are thin and elastic. Stones are usually present and may be found in any in
15-20
part
of
per
the
cent
ofpatients.
biliary
tract. PATHOGEN
ESIS
The exact pathogenesis of the disease is uncertain, although most authors agree that stones are formed primarily in the bile ducts. C/onorchis mary biliary
Stock
sinensis
cause,
resulting
obstruction,
and
Fung6 in stasis
that
believe
infestation
is
stone and
the
pri-
formation, infection.
Ong et al.4 on the other hand believe that the primary cause is portal septicemia resulting from poor eating habits, and that C/onorchis sinensis need not be present in all cases of recurrent pyogenic cholangitis.
C. S. Ho
372
. 3 Case III. (A) dilated intrahepatic
-
radiolucent
Operative radicals
cholangiogram. are
present.
The
and
Note hepatic
that duct
1974
only a few grossly is obstructed by a
stone.
Case iv. T-tube cholangiogram. Note the of branching in the right intrahepatic ducts
and
the
appearance
The
left
intrahepatic
4.
OCTOBER,
(B) T-tube cholangiognam. proximal part of the left
lack
IIG.
D. E. Wesson
of stretching
ducts
are
of these ducts. relatively normal.
___ FIG.
.
common
hepatic
T-tube cholangiogram. bile duct and mild dilatation branches are noted (arrows).
Case
v.
A of
2
dilated intra-
VOL.
122,
No.
2
Recurrent
CHOLANGIOGRAPHIC
Pyogenic
Cholangitis
FINDINGS
Although characteristic, the roentgenologic manifestations of this disease have been neglected in the literature until recently.8 Typical findings include: I. Decrease in arborization of intrahepatic radicals (Fig. iB; 2B; 3, ii and B; and 411). 2. Abnormal branching pattern. The normal branching angles are distorted and may be at right or obtuse angles (Fig. IB). In some cases (Fig. 44) the branches appear to be stretched. 3. Asymmetric changes in the bile ducts. In the same cholangiogram, some intrahepatic ducts may be dilated throughout, while others may show proximal dilatation with rapid tapering towards the periphery. This latter appearance is not unlike that of an arrowhead (arrow in Fig. 2B) and we refer to this as the “arrowhead sign.” 4. Radiolucent stones are usually present. They may be situated anywhere in the biliary tree (Fig. IA; 211; and 3, 11 and B). 5. A dilated common bile duct is usually present. It may measure 3-4 cm. in diameten. The decrease in the number of intrahepatic radicals is probably due to fibrosis in the portal tracts and the abnormal branching patterns to scarring in the liver. The asymmetric dilatations and strictures are a reflection of chronic inflammatory changes in the intrahepatic bile ducts with or without obstruction. As the degree of obstruction and inflammation varies in different parts of the liven, the changes in the bile ducts differ accordingly.
in Chinese iary
tree.
Immigrants
The
full
373
complement
of
intra-
hepatic bile ducts is shown in an operative cholangiogram. Changes of cholangitis may be present, but never to the same degree as in recurrent pyogenic cholangitis, where repeated attacks over a long period of time are the rule. 2. Sclerosing cholangitis.’ This is a disease of unknown etiology associated with submucosal inflammation ofpontions of the bilt ducts and characterized by fibrosis and stenosis of these regions. Clinically, the patient presents with either intermittent or pnogressivej aundice. Many patients also have chronic ulcerative colitis. The condition is usually first diagnosed at operation when the common bile duct feels like
a
thickened
cord.
Operative
or
operative cholangiography shows tent of the disease. A characteristic is decreased ducts
as
arborization
of intnahepatic
in recurrent
pyogenic
post-
the cxfinding bile
cholangitis.
However, there is a lack of dilatation proximal to the stnictured areas and the common bile duct may also have strictures. Both these features distinguish the condition from the Asiatic disease. 3. Caroli’s disease. This is a developmental anomaly in which there is segmental saccular
dilatation
ducts. liver
This abscess
clinical genic
of
results and
picture
the
is similar
cholangitis,
intrahepatic
bile
in stasis, cholangitis, stone formation. The but
to recurrent
it occurs
in
pyo-
a younger
age group. It also carries a much poorer prognosis. Most patients die at an early age as the disease is not amenable to any form of satisfactory treatment. The cholangiographic finding2 of dilated saccules in intrahepatic bile ducts establishes the diagnosis.
DIFFERENTIAL
DIAGNOSIS
the cholangiognaphic changes in recurrent pyogenic cholangitis are quite characteristic, the following conditions, which produce changes similar in some respects, must be considered in differential diagnosis: I. Extrahepatic obstruction. The hallmark of uncomplicated extrahepatic obstruction is generalized dilatation of the entire bil-
MANAGEMENT
Although
In the western of
common
gallbladder,
bile
hemisphere, duct
only
in the ducts.5 The volves exploration duct and extraction is a major surgical drainage
required.
stones a
few
the majority originate
forming
in
the
primarily
surgical treatment inof the common bile of the stones. Seldom procedure for internal
C. S. Ho
374
and
In recurrent pyogenic cholangitis, repeated attacks are the rule and recurrent formation of stones in the ducts is the main problem. Those who fail to respond to conservative treatment usually require a permanent, adequate drainage procedure; e.g., choledochoduodenostomy, choledochojejunostomy or sphincteroplasty.3’7 This is best done at the first operation, but if the patient’s condition is too poor for a major procedure, cholecystostomy or T-tube drainage can be performed, and elective, definitive, surgical drainage postponed until later. CONCLUSIONS
AND
D. E. Wesson
REFERENCES I.
of
Toronto
General
Toronto,
Ontario
Canada
Radiology Hospital
J.,
KRIEGER,
Roen
W. B., and
SEAMAN,
tgenologic
angitis. 2.
appearances
of
M. R. chol-
PORTER, sclenosing
Radiology, 3970, 95, 369-375. Z., GLENN, F., and EVANS,
MUJAHED,
Communicating hepatic GENOL.,
cavernous
ducts
(Canoli’s
RAD.
3. ONG, G. angitis.
J. A.
ectasia
disease).
J.
AM.
&
THERAPY
of
intraROENT-
NUCLEAR
MED.,
2 1-26.
113,
1971,
B.
Study
A.M.A.
of recurrent Surg.,
pyogenic
Arch.
chol84,
1962,
199-
225.
4.
ONG,
G. B., LEE,
Air
in biliany Brit.
T. C., Low, G., and hi, P. Y. passages of choledochoduodenal
7. Surg., 1962, 50, 172-178. D. C. Editor. Textbook of Surgery. W. B. Saunders Company, Philadelphia, 1972. 6. STOCK, F. E., and FUNG, J. H. Oriental cholangiohepatitis. A.M.A. Arch. Surg., 1962, 84, 409origin.
.
SABISTON,
7.
STOCK,
412.
C. S. Ho, M.B.B.S. Department
1974
We thank Dns. R. Stone and D. E. Sanders for encouragement to write this paper, Dr. M. J. Philipps for helping in the preparation of pathology slides, and Dns. M. J. McLoughlin and B. B. Hobbs for their advice and comments.
SUMMARY
Recurrent pyogenic cholangitis is a common disease in the Far East, and is occasionally seen in Asian immigrants in North America. Five such patients are reported. Knowledge of the characteristic cholangiographic appearances will enable the radiologist to make this diagnosis and alert the clinician to the recommended mode of management.
OCTOBER,
F. E., and TINCKLER, duodenostomy in treatment titis.
8.
WASTIE,
Surg., M.
APY&
of cholangiohepa-
Gynec. & Obst., 1955, L., and CUNNINGHAM,
Roen tgenologic cholangitis.
L. F. Choledocho-
AM. NUCLEAR
findings
J.
I. G.
in recurrent
ROENTGENOL.,
MED.,
599-606.
101,
1973,
119,
pyogenic RAD.
71-77.
THER-
E.
