Hind
S. Teixidor,
MD
#{149} Thomas
A. Godwin,
Cryptosporidlosis In AIDS’ Cryptosporidiosis tern was studied
with
ciency nosis logic
syndrome was made examination
acquired
studies and the cryptosporidiosis
sysin 13
immunodefi-
(AIDS). The diagby means of histo(n = 9) or imaging
presence
of intestinal
(n = 4). Imaging studies were done in 10 patients. Ultrasound (US) and computed tomography (CT) showed dilatation of the biliary ducts, some with wall thickening, thickening of the gallbladder wall and pericholecystic fluid. Chol-
angiograms
showed
attenuation
pruning of the intrahepatic ducts, some with beading tion of the common bile
patients
had
papillary
merous Cryptosporidium were found in three bladders and in the
seven
patients
resected biiary
gallducts of
autopsy
performed, accompanied by berant inflammatory response. lation of the radiologic and logic findings establishes a etiologic role of Cryptosporidium one of the major infectious cholangitis associated with Index terms: Acquired drome (AIDS), 768.289
and
bile and dilataduct Three
stenosis. Nuorganisms
in whom
was
an
exuCorrepathodirect as agents in AIDS.
immunodeficiency
syn-
#{149} Bile ducts, CT, 768.1211 #{149} Bile ducts, diseases, 768.201 #{149} Bile ducts, stenosis or obstruction, 76.289 #{149} Bile ducts, US studies, 768.1298 . Cholangitis, 768.288 . Cholecystitis, 762.285 ‘ Cryptosporidiosis, 76.2059
Radiology
1991;
#{149} Elizabeth
ofthe
of the biliary retrospectively
patients
MD
C
A. Ramirez,
Billary
has been reported with increasing frequency in patients with acquired immunodeficiency syndrome (AIDS). It is an opportunistic infection that often involves the gastrointestinal tract and produces debilitating diarrhea in immunosuppressed patients (1). Cryptosporidium organisms are reportedly found in 6% of all patients with AIDS (2) and in 21 % of those who have diarrhea (3). Cryptosporidiosis of the biliary systern that causes acalculous biliary disease is rare. Small numbers of patients with AIDS-related cholangitis have been sporadically reported in the literature (1,2,4-12). They all have a common clinical presentation of right upper quadrant pain, nausea, vomiting, fever, and biochemical evidence of anicteric cholestasis. Ultrasound
covered after cholecystectomy, and in seven patients, at autopsy. This large number of cases of cryptospondiosis of the biliary system proved with histologic examination seems to establish an etiologic relationship between this infectious agent and the biliary disease more firmly than has been previously reported.
(US)
cholecystectomy
RYVrOSPORIDIOSIS
and
computed
tomography
sis are similar to those in sclerosing cholangitis; some cholangiograms
show additional papillary stenosis (5-11). The cause of these abnormalities has been variably attributed to a combination of infectious agents, most commonly Cry ptosporidium and/or
cytomegalovirus
13). Immunologic been considered
tis was
From
the
Departments
of Radiology
(H.S.T.,
EAR.) and Pathology (T.A.G.), New York Hospital-Cornell Medical Center, 525 E 68th St. New York, NY 10021. Received November 16, 1990; revision requested January 14, 1991; revision received February 22; accepted February 25. Address reprint requests to H.S.T. C
RSNA,
1991
(CT)
of the upper abdomen show biliary duct dilatation with thickening of the ductal wall and abnormalities of the gallbladder without calculi. Findings on cholangiograms in cryptosporidio-
(CMV)
disturbances additional
The role of Cryptosporidium infectious agent causing the
I
Tract
(1,2,4-
have factors
(5,7).
180:51-56
MD
proved
in some
cases
as an cholangi(1,2,4-7)
but was speculative in most others (8-13). We report findings in 13 patients with AIDS who had cholecystitis and/or cholangitis cryptosporidiosis.
associated
PATIENTS
AND
METHODS
In the past 7 years (1984-1990), 13 pawith AIDS who had cholangitis associated with cryptosporidiosis underwent examination in our institution (Table 1). Their medical records, imaging studies of the biliary system, and histopathologic materials were reviewed retrospectively. The diagnosis of cholecystitis and/or cholangitis was established at autopsy in seven patients, after cholecystectomy in three patients (it was established both after tients
and
at autopsy
in one
patient), and by imaging studies in the four other patients. They were 12 homosexual men and one woman who presumably contracted AIDS from her husband, a drug abuser. Their ages ranged from 25 to 46 years (mean age, 36 years). They all had severe diarrhea due to intestinal cryptosporidiosis and some right upper quadrant or epigastric symptoms, including nausea, vomiting, fever, right upper quadrant pain, and tenderness. Abnormal results of liver function tests consistent with anicteric cholestasis
were
cept patients slightly
elevated
present
in all patients
one and two, who serum
bilirubin
ex-
had levels
(3.1
mg/dL [53 moVLJ and 1.3 mg/dL [22 pmol/LJ, respectively) (Table 1). The Serum alkaline phosphatase level was the most persistently elevated one, ranging from 183 to 1,640 U/L (normal range, 30110 U/L), but fluctuant levels were noted
with
Cryptosporidium organisms were recovered from the intestinal tract in all patients and also from the biliary system in nine patients; in three patients they were re-
Abbreviations: AIDS = acquired immunodeficiency syndrome, CBD = common bile duct, CMV = cytomegalovirus, EHBD = extrahepatic bile duct, ERCP = endoscopic retrograde cholangiopancreatography, IHBD = intrahepatic bile duct.
51
a.
b.
Figure 1. Images from the CBD shows dilatation
gallbladder (c) T-tube ues).
(GB) wall and pericholecystic cholangiogram shows pruning
Table 1 Clinical Presenta Biliary Tract
C.
Cryptosporidium
patient 1, a 40-year-old man with AIDS and of the duct up to 1.3 cm with wall thickening
lion,
fluid and
Phosphata
Alkaline
(curved
arrow).
attenuation
se Levels,
dilated
IHBDs
and
Imaging
and
Age/ Sex
1
4WM
Fever,
RUQ
pain
and
ten-
and
=
and cholangitis. (a) Longitudinal portal vein. (b) CT scan shows mildly
arrow)
and
normal-size
irregularity
in 13 Patients
of the
AIDS
with
pancreatic
extrahepatic
1-tube
(U/L)
US
CT
ERCP
212-658
+
+
+
Repeat
Imaging CT 3 d after
888-1640
+
Two
+
Time from Last Imaging Study to Diagnosis
Studies US: worse
31/M 31/M
Fever, RUQ pain
44/M
Fever,
6
483 762-110
Fever, N, RUQ pain RUQ
pain,
severe
Diarrhea,
38fF
COs in gailblad-
Concurrent
CT examinations
chills
1,104
V
+ + +
+ +
390
+
+ + +
Concurrent
Concurrent
ND CT 3 wk after
+
US: no change
Two US examinations mo: no change
at 1
Two
at 2
3 mo later
COs in galibladder, bile, stool
at 7
wk: worse; 2 ERCPs at 6 wk: worse
derness 3 4 5
of the
der; autopsy
Fever, RUQ pain and ten-
311M
(Fig 1 contin-
ducts
an d Cryptosporidiosis
derness 2
US scan of thickened duct to its left.
Studies
Alk Phos
Symptoms
dilatation
Studies
Imaging
Patient
PV CBD (straight
(arrowheads).
Note
of the
cholecystitis
Concurrent
COs COs
in stool in stool
Concurrent
COs in galiblad-
der, bile, stool
US examinations no change
Concurrent
COs
in stool
Concurrent and colon NA
COs in stool biopsy sample
mo: 7
29/M
Diarrhea,
8
2A’M
N, V, diarrhea
9 10
291M 4WM
N, V, diarrhea RUQ pain, diarrhea
11
44/M
N, V, diarrhea
12 13
4WM
RUQ
33’M
Fever, diarrhea
Note.-Alk
phos = alkaline N = nausea,NA
inal findings,
epigastric
pain,
pain
280-561
+
+
ND ND
700-996
1,302 650-4,437
ND Three US examinations at 3 wk and 6 wk: improved ND ND Two US examinations at 7 wk: no change
+
276
diarrhea
250-270 183
-
-
phosphatase, CO = Cry#{216}osporidiumorganism, ERCP = endoscopic retrograde = not applicable, ND = not done, plus sign (+) = abnormal findings, RUQ
on repeat studies. No patient had an elevated serum amylase level. Three patients underwent cholecystectomy. In two patients, the common bile duct (CBD) was completely obstructed at the ampulla at the time of surgery. The
(ERCP) showed papillitis and complete ampullary obstruction. Repeat papillot-
first patient
omy was
also
underwent
nal sphincteroplasty. quadrant symptoms gery,
but
3 months
His
a transduoderight upper
improved later
he died
after
sur-
of pulmo-
nary infections. The second patient underwent choledochoduodenostomy, which resulted in improved symptoms. He had recurrent symptoms and an elevated serum alkaline phosphatase level 3 months later, at which time repeat endoscopic retrograde cholangiopancreatography 52
#{149} Radiology
omy
resulted
symptoms,
in some
but
improvement
no long-term
of his
follow-up
information is available in this patient. The third patient underwent sphincterotand placement left in place.
of a T tube, His symptoms
and the T tube was removed; later
he underwent
ampulla stent, symptoms
when
resulting that
the stent
None of the cific treatment
patients
balloon
and placement
were
2 months dilation
of the
of an indwelling
in temporary recurred
which persisted,
relief of 2 weeks later,
was removed. other patients of the biliary
given
spiramycin
received system.
speSeven
for intesti-
=
NA mo before
2
autopsy
NA 6#{189} mo before autopsy 2 mo before autopsy
cholangiopancreatogram, right upper quadrant,
minus V
=
sign(-)
nor-
=
vomiting.
nal cryptosporidiosis. The treatment usually resulted in reduction of the intestinal symptoms, biliary mented.
but tract
Preexisting
lated
no
direct
symptoms
infections
and
response was
concomitant
included
ever
of the docu-
AIDS-re-
oral or esoph-
ageal infections due to Candida organisms (n = 9), infections due to herpes simplex virus (n = 5), CMV infections (n = 6), hepatitis B (n = 4), Pneumocystis carinii pneumonia (n = 3), infections due to Mycobacterium avium-intracellulare (n = 2), infections due to Salmonella organisms (n = 2), tuberculosis (n = 2), and syphilis (n = 2). None reported a history of inflammatory bowel disease or prior biliary tract disorders. Of July
1991
and intravenous trast
administration
medium.
the
Olympus
ERCP (Lake
was
of con-
performed
with
NY)
JF-1T-1OL
Success,
endoscope.
RESULTS Imaging
Us
Studies
CT.-Dilatation of the intrabile ducts (IHBDs) was found of the seven patients who unUS and in seven of the eight who underwent CT. The
and
hepatic in two derwent patients CBD
was
dilated
in five
patients
at US
(range, 8-13 mm) (Fig la) and in seven patients at CT (range, 10-17 mm) (Fig lb). In two patients (one who underwent US, and one, CT and US),
no
dilatation
of the
biliary
le.
cystic
fluid
tients
(Fig
was
found
in three
underwent 1g. Figure 1 (continued). (d) Gross specimen of CBD, which has been opened to the ampulla (A). The white-gray ductal wall is diffusely thickened (arrows), the lumen is moderately dilated to a circumference of 1.8-2.0 cm, and the yellow-green mucosal surface is granular to finely nodular. Exposed granular duodenal mucosa (D) is adjacent to the CBD (bar = 1 cm). (e) A low-
photomicrograph
of the CBD wall shows
rows), which were (including glandular
pallor),
and
created by a combination hyperplasia, dilatation,
inflammatory
the mucosal
folds
of inflammatory and tortuosity),
cell infiltrates.
Mild
and
nodules
changes interstitial
postmortem
autolytic
(between
throughout edema
changes
ar-
the wall (intercellular
account
for some
of the epithelial cells along the surface. (Hematoxylin-eosin; original magnification, x40.) (0 Medium-power photomicrograph of a large IHBD that has inflammatory changes similar to those in d. In addition, there is one microabscess (straight arrows). Along the mucosal surface of the ductal lumen is a small, adherent bile plaque (curved arrow). (Hematoxylin-eosin; original magnification, x 100.) (g) Dilated periductal glands are filled with neutrophils. Several Cryptosporidium organisms (arrows) are attached to the surface of epithelial cells. The interstitium is edematous (intercellular pallor) and contains a moderate lymphoid infiltrate. (Hematoxylin-eosin; original magnification, x400.) loss
the
13 patients,
lost
to follow-up,
months
after
by means
have
and the
tients,
three
were
is still alive
diagnosis
imaging
7
tomography ultrasound
were
13 patients (US)
cholangiography
per1);
(CT) in eight
pa-
patients,
in six patients
(ERCP in five patients and T-tube cholangiography in one patient). Two patients underwent two ERCP studies each. In seven
patients,
were
performed
of each
other:
Volume
180
repeat
comparable
within
3 days
US, four
patients;
#{149} Number
1
and
not comparable
ERCP,
one
in another
patient.
patient
because
of the post-
operative status.) These repeat studies were compared to evaluate progression
(Table
in seven
US, two patients; (Two ERCP studies
were
of cholangitis
studies
in 10 of the
computed
died,
one
of ERCP.
Pertinent formed
and
nine
studies
to 2 months CT and
the
disease. US was
performed
Wideview
with
the
of
Diasonics
400 DR4 (Milpitas, Calif) with a 3.5-MHz transducer and an Acuson 128 (Mountain View, Calif) with a 3.28-MHz transducer. CT was performed
and the Diasonics
with
a General
Electric
8800
or
9800 scanner (GE Medical Systems, Milwaukee). Sections 5-10 mm thick were obtained at 5-12-mm intervals after oral
pa-
ib).
Cholangiography.-ERCP
formed
power
ducts
was demonstrated. Thickening of the wall of the CBD was seen in only two patients (Fig la). The pancreatic duct was prominent (2-3.5 mm in diameter) in four patients. There was mild thickening of the gallbladder wall at US or CT in seven patients, one of whom had sludge in the gallbladder. None had calculi. A small amount of perichole-
in five patients, two
studies
two each.
was perof whom In an-
other patient a T-tube cholangiogram was obtained after cholecystectomy and transduodenal sphincteroplasty. The cholangiographic findings were more specific in demonstrating disease of the biliary ducts than either CT or US. The most consistent abnormality of the IHBDs was attenuation and pruning of the ductular branches (Figs lc, 2a), which was present in all six patients. Focal stricturing and beading were noted in the proximal IHBDs in two patients (Fig 2a). The extrahepatic bile ducts (EHBDs) showed fairly uniform dilatation to the level of the papilla. Papillary stenosis was present in three patients. None showed focal strictures of the EHBD above the papilla. Irregularity and shagginess of the wall of the EHBD was present in four patients, with significant worsening of the mucosal irregularity during an interval of 6 weeks in one of them (Fig 2). Small (2 mm in diameter), smoothly rounded filling defects of uncertain cause were present in the hepatic radicles in three patients. Repeat
comparable
imaging
Seven patients underwent aging studies performed days to 2 months of each
studies.-
repeat imwithin 3 other (Table Radiology
#{149} 53
1). These studies one another to of the disease. mal IHBDs and in diameter) at CT in the same lated IHBDs in of the liver and ber of the CBD tient underwent performed
were compared with evaluate progression One patient had nora dilated CBD (1.3 cm US. Three days later, patient showed dithe right and left lobes an increase in the cali(1.7 cm). A second patwo ERCP studies,
6 weeks
apart,
because
of
worsening symptoms despite the placement of an indwelling stent. The second examination showed increased beading and narrowing of the proximal IHBDs and marked increased irregularity of the wall of the CBD (Fig 2b). A third patient underwent three US examinations. On the first scan the CBD was only slightly dilated and the pancreatic duct was normal. The second scan, obtained 3 weeks later, showed dilatation of both ducts, and the third scan, obtained 6 weeks after the second, showed a return of both ducts to normal caliber. The other four patients underwent repeat CT and/or US examinations performed
within
3-8
weeks
of each
other. Comparable anatomic levels on these scans showed no change in the appearance of the biliary system.
Histopathologic Cryptosporidium
surface
organisms
were
of these
bile
ducts
was
irregular and granular to finely nodular (Fig id). In contrast to the gastrointestinal tract, where infection was patchy and resulted in reactive epithelial changes and mucosal architectural abnormalities, infection of the biliary ducts usually evoked an exuberant penductal inflammatory response with interstitial edema, mild to moderate mixed inflammatory cell infiltrates, and occasional granulation tissue (Fig le, if). The ducts and penductal glands were often dilated and tortuous, and pen54
#{149} Radiology
b.
Findings
recovered from the intestinal tract in all patients and from the biliary system in nine patients; the exact sources of these organisms are listed in Table 2. Other infectious agents in the biliary system or gastrointestinal tract that were present concurrently with the Cryptosporidium organisms and identified in vivo or at autopsy are also listed in Table 2. The three resected gallbladder specimens showed Cryptosporidium cholecystitis. At autopsy, the CBD was dilated to 1.5 and 2 cm, respectively, in two cases. In two other cases, all EI-IBDs had thickened walls. The mucosal
a.
Figure 2. Images from patient 2, a 31-year-old man with AIDS and cholangitis associated with biliary Cryptosporidium organisms. (a) ERCP image shows beading of the proximal left IHBD caused by strictures (arrows), narrowing and irregularity of ductular branches, and dilatation of the common hepatic duct and CBD to the level of the ampulla with irregularity of the wall. Q,) Two spot radiographs of the left LHBD and CBD from repeat ERCP performed 6 weeks after the study in a show marked increase in the irregularity and beading of the visualized IHBDs (long arrows, top) and severe irregularity and shagginess of the CBD (short arrows, bottom). Note slightly dilated and irregular pancreatic duct (arrowheads).
ductal biliary
glands were hyperplastic. epithelium was reactive
casionally
was
frankly
pseudostratified. most
necrotic
Organisms
numerous
in more
The or oc-
bladder only in cases of surgical tion. At autopsy, the gallbladder
or
not
examined
for
examination
were severely
flamed ducts and glands interstitial inflammatory
in-
(Fig ig). The cell infiltrate
was composed predominantly small and intermediate-sized cytes with occasional plasma
of lymphocells and
macrophages. Neutrophils were sprinkled in the interstitium but were more numerous within lumina of dilated glands, forming microabscesses (Fig if). The combination of an interstitial
inflammatory
response
with
and surgically biliary ducts, was edematous
mucosal
right
contained
inspissated
and
bile,
which occasionally formed a plaque that adhered to the eroded mucosal surface (Fig if). In one case, organisms
were
radicles Organisms
traced
to within were
into
smaller
biliary
1 cm of the capsule. found
in the
ducts
left
gall-
in
formed.
distorted
injury
of organisms
Pancreatic
ally
was
Epithelial
Cryptosporidium
hepatic lobes. In some cases, the intrahepatic large duct mucosa was “thrown into folds” by the inflammatory reaction as in extrahepatic ducts. In some cases the lumen of involved ducts
architecture
gregates
creas,
both
absent in one. As in the the entire gallbladder and had moderate
throughout.
the
into
autolyzed
mixed inflammatory cell infiltrates throughout the lamina propria. The
cases
ducts
too
in four cases, was ulcerated in one case, inCandida organisms in one,
extensively fected with
edema accounted for the grossly visible mucosal nodularity and folds (Fig le). Biliary tract infection ascended main
or was
resecwas
were
in four
autopsy was Although infection near
ag-
harbored
organisms
which
in ducts
and patchy.
the
head
perwas
usu-
of the
pan-
one case organisms were also found in the tail. In each case, the associated ductal epithelium had transformed to stratified hyperplastic, in
squamous
metaplasia.
volved pancreas
ducts within generally
The
wall
of in-
the body of the had minimal
changes or inflammatory but infected ducts in the
infiltrates, head and
near the ampulla often had associated periductal interstitial edema and mixed inflammatory cell infiltrates similar to that around the EHBDs. July 1991
Table
2
Sources
Findings
of Cryptosporidium Organisms, Concurrent in 13 Pafients with AIDS and Cnjptosporidium
Sources of
stool, PD
GB, bile ducts,
Rare
Gastrointestinal Tract
in bile ducts
CMV
CMV
Histopathologic Findings
ampullitis,
ileitis,
Severe
CO cholecystitis and cholangitis, COs in PD CO cholecystitis
coli-
fis GB, bile, sample Stool
2t 3
4
duodenal
biopsy
Stool GB, bile, stool
5t
6
Stool
7
Colon biopsy
8*
Bile ducts,
None
Giardia organisms, herpes simplex
None
Tuberculosis lymph nodes
None None
None
stool
Rare
rectal virus in mesenteric
organisms
Herpes simplex None CMV
in bile
virus CMV
Severe
Bile ducts, stool
Several
CMV in bile ducts
Disseminated
CMV
and
10*
Bile ducts,
PD, stool
Candidal
11*
Bile ducts, sample, Bile ducts, sample,
PD, colon biopsy stool colon biopsy stool
None
Bile ducts,
rectalbiopsy
12*
13*
sample,
to moderate CO cholangitis Severe CO cholangitis, COs in PD Moderately severe CO cholangitis, COs in PD Severe CO cholangitis
GB
organism,
=
Cryptosporidium is a protozoan parasite that infects epithelial cells of the gastrointestinal tract in a wide variety of vertebrates (4). It has recently been recognized as a human pathogen, the first case having been reported in 1976 (14). It is transmitted through fecal-oral contamination or through sexual contact in patients with AIDS (15). Although it causes a mild illness in immunocompetent people, cryptosporidiosis can produce severe gastrointestinal illness in patients with a compromised immune system, such as those with AIDS or immunoglobulin deficiency or those receiving
Volume
180
less
#{149} Number
one or-
Aspergillus organisms in gastrointestinal tract, CMV ampullitis
Intestinal
(4).
has been frequently than 1
MAI
=
Mavium-intracellulare,
NA
=
CMV, nodes
MAI
in
not available, PD
Moderate
=
pancreatic
CO
cholangitis
duct
and papillotomv.
DISCUSSION
immunosuppressive therapy Bifiary cryptosporidiosis
gallbladder,
CMV
None
Rare GvfV in bile ducts, focus withAspergillus ganisms None
Six of the seven patients in whom autopsy was performed had disseminated CMV infection. Four of these patients had CMV involvement along the biiary tract; however, infected cells were generally few or rare, involving endothelial and epithelial cells. CMV contributed to the severe ampullitis in one patient. In the three remaining patients, CMV involvement was minimal and distinct from infection with Cryptosporidium organisms.
much
Intestinal
of GB
lymph
cholecvstectomv
underwent
colonization
stool
Note-CO = Cryptosporidium * Data obtained at autopsy.
reported
CO cholangitis
Mild
MA!
Patient
and
papillitis NA NA
colitis
9*
t
CO cholecystitis
Severe
Streptococcusfaecalis,
ducts
NA
NA
Salmonella
None None
stool
sample,
and Histopathologic
Tracts,
Concurrent Infectious Agent in
Infectious Agent in Biliary Tract
Organisms
let
and Gastrointaslinal
Biliary
Cholangitis
Concurrent
Cryptosporidium Patient
in the
Infections
the intestinal disease, either because of its rarity or because of difficulty in diagnosing it. Clinically, its symptoms may be mild or may be obscured by other concomitant features of AIDS that may be overwhelming. At histologic examination the organisms may be overlooked because of their small size, unless they are looked for specifically. In only a few of the patients reported as having biliary cryptosporidiosis were the organisms isolated directly from the gallbladder or bile ducts (1,2,4-7), but in most others the diagnosis was presumptive on the basis of abnormal imaging studies of the biiary tract and the presence of Cryptosporidium organisms in the intestinal tract or ampulla of Vater (813). CMV inclusions in the biliary systern or ampulla were found in combination with Cryptosporidium organisms in some cases, but their etiologic role was not clearly defined (8,9,13). In our
group
cryptosporidiosis
was tion
proved in nine
of i3 patients, of the
biliary
system
with histologic examinapatients in whom Cryptosporidium was the major infectious agent. The organisms were found in large numbers in the gallbladder of three patients who had
undergone cholecystectomy and in the IHBDs and EHBDs of all those in whom autopsy was performed. The presence of Cryptosporidium organisms in the biliary system was always associated with histologic evidence of moderate to severe inflammatory changes. The other four patients had a presumptive diagnosis of the disease on the basis of ERCP studies showing features of sclerosing cholangitis (n = 3) or a CT scan showing dilated biliary ducts (n = 1) in association with intestinal cryptosporidiosis. CMV,
which
may
produce
a clinical
and radiologic picture indistinguishable from that of Cry ptos poridium cholangitis (6,10,16), was excluded as a major etiologic infectious agent in our series except in one patient in whom it contributed to a severe ampullitis. Although CMV inclusions were present along the biliary tract in four of the patients in whom autopsy was performed (Table 2), these were few
and
widely
scattered;
therefore,
they were not considered to have contributed to the inflammatory process of the biliary system but were part of disseminated CMV infection that was present in multiple other organs. In the three other patients in whom autopsy was performed, no CMV inclusions were identified in the Radiology
#{149} 55
biliary system. A synergistic role of CMV infection in cholangitis associated with Cryptosporidium organisms has been suggested by some authors (6,10). Tissue injury from ischemia has been suggested as a mechanism resulting from CMV infection. However, in our autopsy series, not enough histologic evidence of CMV infection or “ischemic-type” injury existed to suggest such synergy. Additionally, in three cadavers in which autopsy was performed, Cryptosporidium cholangitis was found in the absence of any biliary CMV inclusions. The four patients without histologic proof of their biliary disease had no clinical or laboratory evidence of CMV infection in other organ systems. Thickening of the wall of the gallbladder and the presence of pericholecystic fluid seemed to be produced by acalculous cholecystitis, as was proved in three of our patients who had undergone cholecystectomy (Fig ib). Irregularity and shagginess of the mucosal surface of the ducts, shown by cholangiography, and ductal wall thickening, shown by US and CT, are probably the result of the exuberant periductal inflammatory response and interstitial edema found at all autopsies (Fig id, le). This response EHBDs
involved
the
IHBDs
and
to various degrees. The filling defects seen in major IHBDs at cholangiography may have been produced by the same inflammatory process
and
formation
of mucosal
folds
or by inspissated bile plaques adherent to the ulcerated mucosa (Fig if). Papillary stenosis, found at surgery in three of our patients, was likely produced by acute papillitis, which resulted in dilatation of the CBD and pancreatic duct in some cases. None of our patients showed strictures of the CBD above the papilla, but such strictures have been noted by other authors (8,9). The pancreatic duct, which contained Cryptosporidium organisms in four of the cadavers in which autopsy was performed, showed minimal periductal inflammatory changes indicative of ductal injury without the production of pancreatitis. Two of these four patients had shown ductal dilatation at US. This suggests that demonstration of
dilatation of the pancreatic duct on imaging studies is more a reflection of edema of the papilla of Vater than of pancreatitis, unless clinical evidence of the latter exists. Clinical and radiologic studies mdicate that cryptosporidiosis of the biliary tract is fluctuant or rapidly progressive. Some authors have reported a change in the size of the bile ducts or gallbladder wall on repeat US or CT scans obtained within a period of 4 days to 3 months (1,9,11). In our study, two of seven patients who underwent repeat imaging studies that
were
gression to 2 months
#{149} Radiology
showed
disease
(Fig
2a,
within 2b).
1.
2.
3. 4.
5.
pro-
3 days
Another
6.
pa-
tient showed an increase in the dilatation of the CBD and pancreatic duct at US
3 weeks
after
tion, but 6 weeks showed that both spontaneously to topsy of the same 2 months
after
the
the
first
examina-
later, repeat US ducts returned normal caliber. Aupatient, performed last
7.
8.
examination
with US, revealed severe cholangitis. The pancreatic duct contained Cryptosporidium
organisms
9.
and
showed periductal inflammation, but no pancreatitis was present. In our study, the acute or subacute nature of the disease is proved by histologic studies in which the inflammatory process was accompanied by edema but little fibrosis. Because of these findings, evaluation of the therapeutic value of papillotomy is difficult. Although some have advocated papillotomy (8,i3), others have found that it is of limited value (6,9) except in the presence of obstruction due to papillary stenosis. Diagnosis of the disease may be suspected by means of US or CT in the proper clinical setting, but cholangiography is more specific. Exclusion of other diseases of the right upper quadrant such as biliary calculous disease, ductal carcinoma, external compression of the CBD by lymph nodes or masses, and pancreatic disease should be attempted with one or more of the three imaging modalities. Exclusion of primary or secondary sclerosing cholangitis can largely be made on a clinical basis. Our case material seems to indicate a direct etiologic relationship between Cryptosporidium
cystitis and/or with AIDS.
56
comparable
of the
References
organisms
cholangitis U
and
10.
I 1.
12.
13.
14.
15.
16.
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in patients
July
1991