Byung
Ihn
Caroli
Choi,
MD
#{149} Kyung
Mo Yeon,
Disease:
Two adults with communicating cavernous ectasia of the biliary tract (Caroli disease) are described. Both patients had the pure form of the disease, characterized by saccular dilatation of intrahepatic bile ducts, multiple intrahepatic calculi, absence of portal hypertension, and associated cystic renal disease. Computed tomographic (CT) scans of the liver showed tiny dots with strong contrast enhancement within dilated intrahepatic bile ducts (the central dot sign). These intraluminal dots on CT scans corresponded to intraluminal portal veins on sonograms, findings indicating portal radicles surrounded by dilated intrahepatic bile ducts. Index
terms:
Bile ducts, CT, 765.1211 #{149} Bile diseases, 765.28 #{149} Bile ducts, US studies, 765.1298 #{149} Kidney, CT, 81.1211 . Kidney, cysts.
ducts,
81.312
Radiology
1990;
174:161-163
MD
#{149} Seung
Central
C
Hyup
Kim,
Dot
OMMUNICATING sia of the biliary
MD
Sign
cavernous tract, also
known as Camoli disease, congenital abnormality
ecta-
is a rare and is almost
with cystic le(i-4). Preopenative noninvasive diagnosis of this entity is impossible with conventional radiography (2,3,5); however, accurate detection of the primary disease process is necessary to ensure prompt therapy of secondary complications such as cholangitis, lithiasis, liver abscess, and septicemia (2). Sonography (6,7) and computed tomography (CT) (8,9) have been shown to be useful in detecting segmental dilatation of intrahepatic bile ducts. However, in the past, specific diagnosis was not always possible by means of sonography or CT, and CT cholangiography was advocated to make a specific diagnosis (iO,ii). Recently, Marchal et al (12) descnibed some particular sonogmaphic features of Caroli disease in three patients. Besides the well-known charactenistic of nonobstructive segmental dilatation of the bile ducts, they
portal
radicles
that
were
scribe CT
the
and
tion
‘ From the Department of Radiology. College of Medicine, Seoul National University. 28 Yeongun-Dong, Chongro-Ku, Seoul 110-744, Korea. Received May 26, 1989; revision requested June 27; revision received July 31; accepted August 4. Address reprint requests to B.I.C. c RSNA. 1990
on CT scans correlation
de-
of findings
sonognaphy
at
in the
identifica-
of intraluminal
portal
radicles.
CASE
REPORTS
Case 1.-A 32-year-old history of chronic renal followed up for 2 years. to have
and
and
liver
findings sentation. intermittent
polycystic
on the obtained The
woman with a failure had been She was thought
disease
basis
kidney
of sonographic
at the
current tremors
of the time
of first
admission of the face
Han,
tremities, calcemia. palpable
MD
which were attributed Physical examination liver 3 cm below the
margin, neys
without were
not she
years died
following
pre-
was for and ex-
The
Past history treated for
hen teenage had
and
that
obtained: hemoglobin, serum urea nitrogen,
(39.3
mmob/L
of urea);
biliary
tree
with
in
dilated
of
both
lobes
with
multiple
central
demareas
consistent
mgI
mg/dL
la-ic)
throughout
Multiple
identified
4.5
(Fig
(72
9.1
calcium,
scans
findings
stones.
7.2 g/dL 110 mg/dL
branching
bow attenuation ed
The values
creatinine,
and
extensive
liver,
younger
laboratory
dL (804 zmoi/L); (1.12 mmoi/L). Abdominal CT
was that during
problem.
were g/L);
the
her
a
kid-
spleen
indicated nephritis
of a renal
abnormal
onstrated
Both
palpable.
palpable. had been
sister
to hyporevealed right costab
tenderness.
also
of
a dilat-
intrahepatic
tiny
dots
were
intrahepatic
bile
ducts with hancement,
strong contrast material enfindings consistent with por-
tab nadicles
surrounded
hepatic
bile
across
the
by
ducts.
dilated
Bridge
dilated
intra-
formation
lumina
of the
bile
ducts
was also seen. Both kidneys were enlarged and were replaced by numerous cysts.
A sonogram
veabed irregular, trahepatic bile morphologic
partially or completely surrounded by dilated bile ducts as additional typical findings with high-frequency sonogmaphy. We describe two patients with Camoli disease in whom portal madicles surrounded by dilated intnahepatic bile ducts (the central dot sign) were
demonstrated
Chung
in CT’
invariably associated sions of the kidney
observed
#{149} Man
radicles. The
feature
patient
renal
of the
liver
saccular ducts with
(Fig
id)
of intraductab
subsequently
transplantation.
re-
dilatation of inthe particular portal
underwent
Because
of the
typi-
cal nadiologic findings, biopsies of the liver or kidneys were not performed. She is now in good condition.
Case 2.-A rent epigastric admission,
24-year-old
pain. he
man
Two
visited
had
months
another
recur-
before
hospital
be-
cause of epigastnic pain that radiated to the back. Polycystic disease of the liver and kidney was diagnosed on the basis of sonographic findings. Thereafter, he was treated conservatively. The patient had no history of fever or jaundice, and physicab examination abnormality.
showed Laboratory
no significant test findings
were
Abdominal
CT scans
normal.
(Fig
2a, 2b) demonstrated saccular dilatation of intrahepatic bile ducts. Multiple tiny dots were identified in dilated intrahepatic bile ducts by means of contrast materi-
ab enhancement, portal
nadicles
results surrounded
consistent by
dilated
with in-
trahepatic duct walls
bile was
bile
punctate
ducts,
uation sented
regions
of high
were demonstrated, intrahepatic stones.
cortical
and
meduliary
the kidneys. 2c) revealed by
ducts. Bridging of the bile also seen. In the dilated
dilated
cutaneous (Fig
2d)
Caroli stones.
atten-
which repneMultiple small
cysts
were
seen
A sonognam of the liven portal nadicles surrounded intrahepatic
bile
transhepatic
cholangiogram
helped
confirm
disease with The patient
the
multiple is being
ducts.
in
(Fig
A per-
diagnosis
of
intrahepatic followed up.
DISCUSSION Caroli disease was described Canoli as an entity characterized (a) congenital saccular dilatation intrahepatic bile ducts; (b) high quency
angitis; sis
and
by by of fre-
of stone formation and chol(c) absence of hepatic cimrhoportal hypertension; and (d)
association with renal tubular ectasia or other forms of cystic disease of the kidneys and, possibly, of the pancreas (1-3). Cases of this pure form of Caroli disease are uncommon, and most have coexisting hepatic fibrosis (2,4-9,i2,i3). Banros et ab (14) reviewed the literature and analyzed 46 cases of congenital cystic dilatation of intrahepatic bile ducts. Six cases (13.0%) were found to be isolated forms of intrahepatic cystic dilatation, i6 (34.7%) were associated with congenital hepatic fibrosis, and iO (21.7%)
were
found
to be either
a
choledochal cyst or nonobstructive extrahepatic biliary tree dilatation; in 14 cases (30.4%), the three anomalies were found together in the same patient. Therefore, communicating cystic dilatation of intrahepatic bile ducts is considered by many to be present in a spectrum of congenital malformations involving the hepatobiliary
b.
a.
system.
Caroli
disease
d.
C.
Figure
1. Case
1. (a) Unenhanced ducts. Several tiny
trahepatic
bile
tnahepatic
stones
(curved
(b) Contrast-enhanced
enhancement
arrows) CT scan
(arrows)
within
CT scan through dots (arrows) are
are also seen at the
dilated
nowheads) is also seen. (c) Contrast-enhanced polycystic disease. Multiple intrahepatic verse sonognam of the liven demonstrates to the central dots seen on CT scans-in
same
level
bile
ducts.
the seen
in dilated
liver shows sacculan in dilated bile ducts.
left intrahepatic
as in a shows
Complete
tiny
bridging
dots
dilatation Several
of intiny in-
bile ducts. with
strong
of the ductal
contrast
wall
(ar-
CT scan through the kidneys demonstrates stones (arrows) are also seen in the liver. (d) Transsmall portal branches (anrows)-which correspond the center of dilated intrahepatic bile ducts.
is at
one end of the spectrum and is cvident as isolated cystic dilatation of the intrahepatic bile ducts. At the other end of this spectrum is cystic dilatation of intrahepatic bile ducts
Figure 2. Case 2. scan through the
tion
a.
b.
C.
d.
(a) Contrast-enhanced CT liven shows saccular dilatabiliary tree. Tiny dots in dilated intrahepatic bile
of intrahepatic are seen Bridge formation (arrowheads) across the dilated lumina of bile ducts is also seen.
(arrows) ducts. (b)
Contrast-enhanced
kidneys lary
cysts.
shows (c)
CT scan
multiple
Transverse
through
cortical
sonognam
and
the
medul-
of the
liven demonstrates small portal veins (anrows)-which correspond to the central dots seen on CT scans-surrounded by dilated intrahepatic bile ducts. (d) Percutaneous transhepatic chobangiogram shows sacculan dilatation of right intrahepatic bile ducts with multiple tiny intrahepatic stones. The left intrahepatic bile ducts are not opacified.
162
#{149} Radiology
January
1990
accompanying bnosis
congenital
(2,4-9,i2-i4). the pure form
were and had
all the
that
of this
in the diagManchal et al sonographic
support
normal
trahepatic pathogenesis sonographic dilatation, trusions,
characteristics is useful disease. specific
that
the
fi-
cases disease
the
hypothesis
embryogenesis
of in-
bile
ducts is arrested in the of Caroli disease. These findings were bile duct intraluminal bulbar pro-
bridge
formation
across
attenuation
dilated enhanced
intrahepatic CT scans
strongly
after
tration dots
were
di-
not
veins cating
on portal
madicles
surrounded
174
#{149} Number
1
Portal
that
bile
ducts
the
lumina
radicles
they
appear
of the
bile
tent
with
the
resorbed,
wall
bile 6.
to
ductal
that
10.
1 1.
1 2.
come-
U
Caroli J, Sonpaubt R, Kossakowski J, et al. La dilatation pobykystique congenitale des voies biliaires intra-hepatiques: essai de classification.
Semin
Hop
Paris
1958;
3.
by
CC,
Mall JC, Ghahremani li’s disease associated
Mujahed
13.
50:366-369.
JA,
Mall
JC,
of Caroli report
1979;
Pear patic
JJ. by computed cases. Radiolo-
Salmen
BJ, Parker
disease of two
132:661-664.
BL. bile
Congenital dilatation ducts (Caroli disease).
Sorensen
KW,
Glazer
of cystic
of intraheAJR 1980;
GM,
ectasia
Francis
IR.
Di-
of intrahepatic
bile ducts by computed tomography. Comput Assist Tomogn 1982; 6:486-489. Musante F, Derchi LE, Bonati P. CT cholangiography in suspected Caroli’s disease. J Comput Assist Tomogr 1982; 6:482-485. Marchal GJ, Desmet VJ, Proesmans WC, et a!. Caroli disease: high-frequency US and pathologic findings. Radiology 1986; 158:
and
Z, Glenn
with
renal 1974;
Boyer
patic
34:
JL.
Caro-
congenital
he-
tubular ectasia. 66:1029-1035.
F, Evans
JA.
Commu-
Davies CH, Stringer man A, Mancer K. brosis
with
bile
kidneys.
14.
patic fibrosis Gastroenterology
portal mdi-
Radiology
507-511.
References
2.
1977;
Kaiser
agnosis
the study lated well
1.
disease).
Mittelsteadt C, Volberg F, Fischer G. McCartney W. Caroli’s disease: sonographic findings. AJR 1980; i34:585-587. Bass EM, Funston MR. Shaff MI. Caroli’s disease: an ultrasound diagnosis. Br J Ra-
gy
surrounds the portal nadicles. Sonographic findings in our two cases, which were very similar to those in
scans.
JG.
intrahepatic
134:1291-1292.
plate
by Marchal et al (i2), with findings on CT
(Caroli’s
Diagnosis tomography: 9.
C, Atienza
of the
127:746-778.
diol 8.
JL, Molino
dilatation
ducts
1978;
of an insufficiently
malformed
J, Gomez
Lucaya Congenital
7.
by ab-
ducts. Bridge formation across dilated intrahepatic ducts, a finding mesembling internal septac in intrahepatic ducts, was also seen. It is consis-
4.
Volume
ducts.
normal
depen-
dilated bile ducts. dots on CT scans
to intraluminal sonograms, findings
bile
5.
devel-
128-135.
These
in the
ectatic
enveloped
adminis-
material.
located
dent portion of the These intraluminal corresponded
in
by abnormally
are so completely
bile ducts on unand enhanced
intravenous
of contrast were
visualized
oped
lie within
lated lumina, and portal madicles pantially or completely surrounded by dilated bile ducts and were specific for the diagnosis of Canoli disease. However, specific findings of Canoli disease on CT scans have not been reported, to our knowledge. In our two cases, tiny dots of slightly high
dilated bile ducts. On CT scans, pontal radicles seem to be within the lumina of dilated bile ducts. However, they are not within the bile ducts but
are surrounded
disease. Sonography nosis of Canoli (12) described
findings
hepatic
Our two of Canoli
saccular
ducts Pediatr
DA, Whyte Congenital dilatation
and
infantile
Radio!
1986;
H, Danehepatic fiof intra-he-
pobycystic 16:302-305.
Barros JL, Polo JR. Sanabia J, Garcia-Sabrido JL, Gomez-Lorenzo FJ. Congenital cystic dilatation of the intrahepatic bile ducts (Caroli’s disease): report of a case and review of the literature. Surgery 1979; 85:589-592.
nicating cavernous ectasia of the intrahepatic ducts (Canoli’s disease). AJR 1971; 113:21-26. Boyer J. What is Caroli’s disease? Gastroenterology 1975; 68:417-419.
Radiology
#{149} 163
Ihn
Caroli
Choi,
MD
#{149} Kyung
Mo Yeon,
Disease:
Two adults with communicating cavernous ectasia of the biliary tract (Caroli disease) are described. Both patients had the pure form of the disease, characterized by saccular dilatation of intrahepatic bile ducts, multiple intrahepatic calculi, absence of portal hypertension, and associated cystic renal disease. Computed tomographic (CT) scans of the liver showed tiny dots with strong contrast enhancement within dilated intrahepatic bile ducts (the central dot sign). These intraluminal dots on CT scans corresponded to intraluminal portal veins on sonograms, findings indicating portal radicles surrounded by dilated intrahepatic bile ducts. Index
terms:
Bile ducts, CT, 765.1211 #{149} Bile diseases, 765.28 #{149} Bile ducts, US studies, 765.1298 #{149} Kidney, CT, 81.1211 . Kidney, cysts.
ducts,
81.312
Radiology
1990;
174:161-163
MD
#{149} Seung
Central
C
Hyup
Kim,
Dot
OMMUNICATING sia of the biliary
MD
Sign
cavernous tract, also
known as Camoli disease, congenital abnormality
ecta-
is a rare and is almost
with cystic le(i-4). Preopenative noninvasive diagnosis of this entity is impossible with conventional radiography (2,3,5); however, accurate detection of the primary disease process is necessary to ensure prompt therapy of secondary complications such as cholangitis, lithiasis, liver abscess, and septicemia (2). Sonography (6,7) and computed tomography (CT) (8,9) have been shown to be useful in detecting segmental dilatation of intrahepatic bile ducts. However, in the past, specific diagnosis was not always possible by means of sonography or CT, and CT cholangiography was advocated to make a specific diagnosis (iO,ii). Recently, Marchal et al (12) descnibed some particular sonogmaphic features of Caroli disease in three patients. Besides the well-known charactenistic of nonobstructive segmental dilatation of the bile ducts, they
portal
radicles
that
were
scribe CT
the
and
tion
‘ From the Department of Radiology. College of Medicine, Seoul National University. 28 Yeongun-Dong, Chongro-Ku, Seoul 110-744, Korea. Received May 26, 1989; revision requested June 27; revision received July 31; accepted August 4. Address reprint requests to B.I.C. c RSNA. 1990
on CT scans correlation
de-
of findings
sonognaphy
at
in the
identifica-
of intraluminal
portal
radicles.
CASE
REPORTS
Case 1.-A 32-year-old history of chronic renal followed up for 2 years. to have
and
and
liver
findings sentation. intermittent
polycystic
on the obtained The
woman with a failure had been She was thought
disease
basis
kidney
of sonographic
at the
current tremors
of the time
of first
admission of the face
Han,
tremities, calcemia. palpable
MD
which were attributed Physical examination liver 3 cm below the
margin, neys
without were
not she
years died
following
pre-
was for and ex-
The
Past history treated for
hen teenage had
and
that
obtained: hemoglobin, serum urea nitrogen,
(39.3
mmob/L
of urea);
biliary
tree
with
in
dilated
of
both
lobes
with
multiple
central
demareas
consistent
mgI
mg/dL
la-ic)
throughout
Multiple
identified
4.5
(Fig
(72
9.1
calcium,
scans
findings
stones.
7.2 g/dL 110 mg/dL
branching
bow attenuation ed
The values
creatinine,
and
extensive
liver,
younger
laboratory
dL (804 zmoi/L); (1.12 mmoi/L). Abdominal CT
was that during
problem.
were g/L);
the
her
a
kid-
spleen
indicated nephritis
of a renal
abnormal
onstrated
Both
palpable.
palpable. had been
sister
to hyporevealed right costab
tenderness.
also
of
a dilat-
intrahepatic
tiny
dots
were
intrahepatic
bile
ducts with hancement,
strong contrast material enfindings consistent with por-
tab nadicles
surrounded
hepatic
bile
across
the
by
ducts.
dilated
Bridge
dilated
intra-
formation
lumina
of the
bile
ducts
was also seen. Both kidneys were enlarged and were replaced by numerous cysts.
A sonogram
veabed irregular, trahepatic bile morphologic
partially or completely surrounded by dilated bile ducts as additional typical findings with high-frequency sonogmaphy. We describe two patients with Camoli disease in whom portal madicles surrounded by dilated intnahepatic bile ducts (the central dot sign) were
demonstrated
Chung
in CT’
invariably associated sions of the kidney
observed
#{149} Man
radicles. The
feature
patient
renal
of the
liver
saccular ducts with
(Fig
id)
of intraductab
subsequently
transplantation.
re-
dilatation of inthe particular portal
underwent
Because
of the
typi-
cal nadiologic findings, biopsies of the liver or kidneys were not performed. She is now in good condition.
Case 2.-A rent epigastric admission,
24-year-old
pain. he
man
Two
visited
had
months
another
recur-
before
hospital
be-
cause of epigastnic pain that radiated to the back. Polycystic disease of the liver and kidney was diagnosed on the basis of sonographic findings. Thereafter, he was treated conservatively. The patient had no history of fever or jaundice, and physicab examination abnormality.
showed Laboratory
no significant test findings
were
Abdominal
CT scans
normal.
(Fig
2a, 2b) demonstrated saccular dilatation of intrahepatic bile ducts. Multiple tiny dots were identified in dilated intrahepatic bile ducts by means of contrast materi-
ab enhancement, portal
nadicles
results surrounded
consistent by
dilated
with in-
trahepatic duct walls
bile was
bile
punctate
ducts,
uation sented
regions
of high
were demonstrated, intrahepatic stones.
cortical
and
meduliary
the kidneys. 2c) revealed by
ducts. Bridging of the bile also seen. In the dilated
dilated
cutaneous (Fig
2d)
Caroli stones.
atten-
which repneMultiple small
cysts
were
seen
A sonognam of the liven portal nadicles surrounded intrahepatic
bile
transhepatic
cholangiogram
helped
confirm
disease with The patient
the
multiple is being
ducts.
in
(Fig
A per-
diagnosis
of
intrahepatic followed up.
DISCUSSION Caroli disease was described Canoli as an entity characterized (a) congenital saccular dilatation intrahepatic bile ducts; (b) high quency
angitis; sis
and
by by of fre-
of stone formation and chol(c) absence of hepatic cimrhoportal hypertension; and (d)
association with renal tubular ectasia or other forms of cystic disease of the kidneys and, possibly, of the pancreas (1-3). Cases of this pure form of Caroli disease are uncommon, and most have coexisting hepatic fibrosis (2,4-9,i2,i3). Banros et ab (14) reviewed the literature and analyzed 46 cases of congenital cystic dilatation of intrahepatic bile ducts. Six cases (13.0%) were found to be isolated forms of intrahepatic cystic dilatation, i6 (34.7%) were associated with congenital hepatic fibrosis, and iO (21.7%)
were
found
to be either
a
choledochal cyst or nonobstructive extrahepatic biliary tree dilatation; in 14 cases (30.4%), the three anomalies were found together in the same patient. Therefore, communicating cystic dilatation of intrahepatic bile ducts is considered by many to be present in a spectrum of congenital malformations involving the hepatobiliary
b.
a.
system.
Caroli
disease
d.
C.
Figure
1. Case
1. (a) Unenhanced ducts. Several tiny
trahepatic
bile
tnahepatic
stones
(curved
(b) Contrast-enhanced
enhancement
arrows) CT scan
(arrows)
within
CT scan through dots (arrows) are
are also seen at the
dilated
nowheads) is also seen. (c) Contrast-enhanced polycystic disease. Multiple intrahepatic verse sonognam of the liven demonstrates to the central dots seen on CT scans-in
same
level
bile
ducts.
the seen
in dilated
liver shows sacculan in dilated bile ducts.
left intrahepatic
as in a shows
Complete
tiny
bridging
dots
dilatation Several
of intiny in-
bile ducts. with
strong
of the ductal
contrast
wall
(ar-
CT scan through the kidneys demonstrates stones (arrows) are also seen in the liver. (d) Transsmall portal branches (anrows)-which correspond the center of dilated intrahepatic bile ducts.
is at
one end of the spectrum and is cvident as isolated cystic dilatation of the intrahepatic bile ducts. At the other end of this spectrum is cystic dilatation of intrahepatic bile ducts
Figure 2. Case 2. scan through the
tion
a.
b.
C.
d.
(a) Contrast-enhanced CT liven shows saccular dilatabiliary tree. Tiny dots in dilated intrahepatic bile
of intrahepatic are seen Bridge formation (arrowheads) across the dilated lumina of bile ducts is also seen.
(arrows) ducts. (b)
Contrast-enhanced
kidneys lary
cysts.
shows (c)
CT scan
multiple
Transverse
through
cortical
sonognam
and
the
medul-
of the
liven demonstrates small portal veins (anrows)-which correspond to the central dots seen on CT scans-surrounded by dilated intrahepatic bile ducts. (d) Percutaneous transhepatic chobangiogram shows sacculan dilatation of right intrahepatic bile ducts with multiple tiny intrahepatic stones. The left intrahepatic bile ducts are not opacified.
162
#{149} Radiology
January
1990
accompanying bnosis
congenital
(2,4-9,i2-i4). the pure form
were and had
all the
that
of this
in the diagManchal et al sonographic
support
normal
trahepatic pathogenesis sonographic dilatation, trusions,
characteristics is useful disease. specific
that
the
fi-
cases disease
the
hypothesis
embryogenesis
of in-
bile
ducts is arrested in the of Caroli disease. These findings were bile duct intraluminal bulbar pro-
bridge
formation
across
attenuation
dilated enhanced
intrahepatic CT scans
strongly
after
tration dots
were
di-
not
veins cating
on portal
madicles
surrounded
174
#{149} Number
1
Portal
that
bile
ducts
the
lumina
radicles
they
appear
of the
bile
tent
with
the
resorbed,
wall
bile 6.
to
ductal
that
10.
1 1.
1 2.
come-
U
Caroli J, Sonpaubt R, Kossakowski J, et al. La dilatation pobykystique congenitale des voies biliaires intra-hepatiques: essai de classification.
Semin
Hop
Paris
1958;
3.
by
CC,
Mall JC, Ghahremani li’s disease associated
Mujahed
13.
50:366-369.
JA,
Mall
JC,
of Caroli report
1979;
Pear patic
JJ. by computed cases. Radiolo-
Salmen
BJ, Parker
disease of two
132:661-664.
BL. bile
Congenital dilatation ducts (Caroli disease).
Sorensen
KW,
Glazer
of cystic
of intraheAJR 1980;
GM,
ectasia
Francis
IR.
Di-
of intrahepatic
bile ducts by computed tomography. Comput Assist Tomogn 1982; 6:486-489. Musante F, Derchi LE, Bonati P. CT cholangiography in suspected Caroli’s disease. J Comput Assist Tomogr 1982; 6:482-485. Marchal GJ, Desmet VJ, Proesmans WC, et a!. Caroli disease: high-frequency US and pathologic findings. Radiology 1986; 158:
and
Z, Glenn
with
renal 1974;
Boyer
patic
34:
JL.
Caro-
congenital
he-
tubular ectasia. 66:1029-1035.
F, Evans
JA.
Commu-
Davies CH, Stringer man A, Mancer K. brosis
with
bile
kidneys.
14.
patic fibrosis Gastroenterology
portal mdi-
Radiology
507-511.
References
2.
1977;
Kaiser
agnosis
the study lated well
1.
disease).
Mittelsteadt C, Volberg F, Fischer G. McCartney W. Caroli’s disease: sonographic findings. AJR 1980; i34:585-587. Bass EM, Funston MR. Shaff MI. Caroli’s disease: an ultrasound diagnosis. Br J Ra-
gy
surrounds the portal nadicles. Sonographic findings in our two cases, which were very similar to those in
scans.
JG.
intrahepatic
134:1291-1292.
plate
by Marchal et al (i2), with findings on CT
(Caroli’s
Diagnosis tomography: 9.
C, Atienza
of the
127:746-778.
diol 8.
JL, Molino
dilatation
ducts
1978;
of an insufficiently
malformed
J, Gomez
Lucaya Congenital
7.
by ab-
ducts. Bridge formation across dilated intrahepatic ducts, a finding mesembling internal septac in intrahepatic ducts, was also seen. It is consis-
4.
Volume
ducts.
normal
depen-
dilated bile ducts. dots on CT scans
to intraluminal sonograms, findings
bile
5.
devel-
128-135.
These
in the
ectatic
enveloped
adminis-
material.
located
dent portion of the These intraluminal corresponded
in
by abnormally
are so completely
bile ducts on unand enhanced
intravenous
of contrast were
visualized
oped
lie within
lated lumina, and portal madicles pantially or completely surrounded by dilated bile ducts and were specific for the diagnosis of Canoli disease. However, specific findings of Canoli disease on CT scans have not been reported, to our knowledge. In our two cases, tiny dots of slightly high
dilated bile ducts. On CT scans, pontal radicles seem to be within the lumina of dilated bile ducts. However, they are not within the bile ducts but
are surrounded
disease. Sonography nosis of Canoli (12) described
findings
hepatic
Our two of Canoli
saccular
ducts Pediatr
DA, Whyte Congenital dilatation
and
infantile
Radio!
1986;
H, Danehepatic fiof intra-he-
pobycystic 16:302-305.
Barros JL, Polo JR. Sanabia J, Garcia-Sabrido JL, Gomez-Lorenzo FJ. Congenital cystic dilatation of the intrahepatic bile ducts (Caroli’s disease): report of a case and review of the literature. Surgery 1979; 85:589-592.
nicating cavernous ectasia of the intrahepatic ducts (Canoli’s disease). AJR 1971; 113:21-26. Boyer J. What is Caroli’s disease? Gastroenterology 1975; 68:417-419.
Radiology
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