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1011

Oriental

Cholangiohepatitis:

Correlation Between and Hepatic Atrophy

Shoichi

Kusan&’2

Yoshitaka

The

Okada1

Takashi End& Hisaaki Yokoyami’ Harumi Ohmiya3 Hideo

Atari4

angiographic

features

of the

Portal

hepatic

Vein Occlusion

vasculature

in patients

with

oriental

cholan-

giohepatitis and their relationship to liver atrophy remain unclear. We studied I 1 patients with oriental cholangiohepatitis to define the spectrum of portal vein, bile duct, and parenchymal involvement by correlating findings on cholangiography, sonography, CT, and angiography. The portal veins appeared normal in five cases, pruned in four, and completely obstructed in two. This spectrum of appearances was found to correlate with the severity of liver atrophy. No massive arterioportal shunting was found in any patients in this series. The two patients with complete obstruction of the portal veins

showed no evidence It is concluded

degree

of malignancy that

the

at histologic examination of

portal

vein

of the resected

obstruction

correlates

of liver atrophy in patients with oriental cholangiohepatitis

complete

central

portal

obstruction

158:1011-1014,

AJR

degree

Oriental

May

do not necessarily

indicate

specimen.

well

with

the

and that findings of

associated

malignancy.

1992

cholangiohepatitis

(OCH)

with intrahepatic

bile duct

stones

is commonly

seen in Asia, particularly in China, Hong Kong, Korea, and Japan [1 ]. Differentiating between cholangitis in ordinary patients with intrahepatic bile duct stones and OCH in those

with

intrahepatic

bile duct

stones

is very

difficult,

especially

when

the

extrahepatic bile ducts, both proximal and distal to the stone, are diffusely dilated. Hepatic atrophy is also a common feature of OCH. Atrophy has been attributed to diminished

portal

chymal

venous

flow,

but the relationship

loss is not well understood.

unclear, but the presence of clinically been considered to be an important this disorder [2].

In this report, in patients Received March 8, 1991 ; accepted December ii, 1991. 1

Department

of Radiology,

East Hospital, 2-i -1 , Asamizodai, agawa 228, Japan. 2 Present address: tional

Defense

Medical

Department Kitasato,

of Surgery, Sagamihara,

of Radiology.

these

features

findings

flow and paren-

of OCH remains

of the portal veins the pathogenesis

of the hepatic

with the degree

vasculature

of liver atrophy

of arterioportal shunting, and discuss and the clinical role of angiography.

has of

and

the etiologic

3-2, Namiki,

Toko-

reprint re-

Kitasato University Sagamihara, Kan-

0361 -803X/92/i 585-i Oi i © American Roentgen Ray Society

and

Methods

Na-

Kitasato University, 1Kanagawa 228, Japan.

Department of Pathology, East Hospital, 2-1-i , Asamizodai, agawa 228, Japan.

occult derangement factor in understanding

the angiographic

correlate

the presence or absence mechanism of this disorder Materials

Department College,

we describe OCH,

portal

mechanism

Kitasato University Sagamihara, Kan-

rozawa, Saitama 359, Japan. Address quests to S. Kusano. 15-i,

after revision

with

between

The etiologic

From April 1 980 to July 1989, 1 1 of 20 patients with a primary diagnosis of OCH who required surgery because of repeated episodes of biliary infection and jaundice were referred for diagnostic arteriography before hepatic resection. The group comprised seven women and four men 25-81 years old (average, 53 years). The diagnosis was proved histologically from

the

Six

patients

resected

with T-tube

had

specimen had

drainage),

prior

whereas

Before angiography, transhepatic evaluated

in 1 0 cases, biliary

together

the remaining

all patients

cholangiography at the

while

surgery

of angiography.

case

it was

suggested

cholecystectomy

and

intraoperatively.

two

cholecystectomy

five had had no prior surgery.

had abdominal

or endoscopic time

in one

(four

sonography,

retrograde The

CT, and either percutaneous

cholangiography. studies

were

evaluated

These

studies

retrospectively

were as

1012

KUSANO

ET

AL.

AJR:i58,

Fig. 1.-46-year-old

May 1992

woman with ori-

ental cholangiohepatitis. A, Unenhanced CT scan. Amorphous calcified stones in bile ducts of right hepatic lobe are associated with

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beaded dilatation and moderate loss of liver parenchyma. B, Digital subtraction hepatic arteriogram shows fine neovascularity in right hepatic lobe and third-order branches of right posterior segmental artery (arrows). C, On venous phase of B, parenchyma of right hepatic lobe is densely opacified with hepatic venous shunting. D, Arterial portogram shows poor fill-

ing of third-order

or smaller

intrahe-

patic branches of right portal vein parallel to corresponding arterial branches (as shown in B, arrows). This finding represents “pruned tree” appearance.

A

B

C

D

follows. Initially, the first observer

reviewed

all studies independently.

When findings were difficult to categorize, the opinion interpreter was solicited to achieve a consensus.

The findings on sonography and cholangiography assess the site of bile duct stones and the presence of Both enhanced and unenhanced CT scans were patients. None had dynamic bolus CT. The CT findings with

regard

to the presence

of bile duct

stones,

of a second

were used to biliary stricture. obtained in all were reviewed

the degree

of bile

duct dilatation, and changes in hepatic segmental anatomy. Biliary dilatation was classified into three degrees by CT: slight tubular dilatation,

moderate

beaded

dilatation,

and

marked

cystic

dilatation.

Atrophy was diagnosed on CT as the presence offocalloss of hepatic parenchyma and was categorized as slight (identifiable loss), moderate, or marked (almost complete loss). Celiac and superior mesenteric arteriography was performed in all patients: hepatic

1 0 patients arteriograms.

also

had

Conventional

supplementary film-screen

common angiography

used to evaluate

per-

formed in four patients; 30-40 ml of 76% Urografin (Schering AG, Germany) was injected into the superior mesenteric artery at a rate of 10-15 mI/sec immediately after intraarterial injection of a single 10-pg bolus of prostaglandin E1. In the remaining seven patients, who were evaluated most recently, digital subtraction angiography was

the hepatic

(Schering

vasculature.

AG) at a rate

We injected

of 4-5

mI/sec

20 ml of immediately

after injection of a single 10-tg bolus of prostaglandin E1 into the superior mesenteric artery. Angiograms were assessed for (1) changes in hepatic arterial blood flow and staining of the liver parenchyma and (2) segmental obstruction of the portal vein. According to the site and extent of occlusion of the portal veins as documented

by arterial portography, patients were classified into one of three groups: (1 ) no abnormality, (2) occlusion of third-order or more distal branches

of the portal

veins (“pruned

tree”

plete obstruction of first- or second-order branches of the portal veins.

appearance),

or (3) com-

(main lobar or segmental)

Results

or proper was

370

lopamiron

The portal in

veins

appeared

normal

four (Fig. 1), and completely

five patients

in five patients,

obstructed

with normal-appearing

portal

phy of the liver. Of the four patients appearance,

Both patients

all had slight

with complete

pruned

in two. None of the veins showed

atro-

with the “pruned

tree”

to moderate

obstruction

atrophy

of the liver.

of the portal

veins

AJR:i58,

HEPATIC

May 1992

VASCULATURE

IN ORIENTAL

CHOLANGIOHEPATITIS

1013

Fig. 2-81-year-old woman with oriental cholangiohepatitis. A, Percutaneous transhepatic cholangiogram shows stricture of right hepatic duct and cystic ductal dilatation

stones distal to sten-

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with intraluminal

otic segment (arrow). B, Enhanced CT scan

dilatation

of right

shows

hepatic

cystic

ducts

with

multiple radiopaque stones and marked loss of affected liver parenchyma. Note compensatory hypertrophy of caudate lobe. C, Cellac arteriogram. Arrow mdi-

cates right hepatic artery. No evidence of arterioportal shunting was seen. D, Arterial portogram. Obstruction of right branch of portal vein is demonstrated by absence of portal vein branches running parallel to corre-

sponding arterial branches.

had marked vein occlusion

atrophy

of the liver (Fig. 2). The extent

was found

to correlate

of portal

with the degree

of liver

atrophy. In patients

with portal

vein obstruction,

neither

branches

of the portal

veins,

of filling of the right branch patency

of the portal

CT confirmed

of the portal

the absence

vein; in addition,

vein of the unaffected

lobe was

found

(Fig. 2D). Hypervascularity of the liver (capillary staining of the parenchyma) was found in the affected segments (Figs. 1 B and 1C) as well as in other segments in all degrees of liver atrophy. of major

No massive arterioportal shunting with opacification branches of the portal veins was found, but hepatic

venous shunting was seen in one patient (Fig. 1C). All 1 1 patients had dilatation of the intrahepatic ducts intrahepatic

bile duct

mildly

dilated

cases,

and markedly

stones.

in three

cases,

dilated

The

intrahepatic

moderately

in one case.

dilatation was unrelated to the degree volvement or hepatic atrophy.

ducts

dilated

The degree

of portal

formed

and were

of ductal

in-

of the

correlation obstruction

because

typical

in this series, of the portal

specimens

of OCH.

re-

Radio-

including two cases veins, was not per-

angiography

as a

findings at arterial portography with features. There was no evidence of examination of the resected speci-

men in the two patients portal

10 resected

changes

of the lack of specimen

“road map” for comparing anatomic and histologic malignancy at histologic

with

complete

obstruction

of the

vein.

Discussion OCH has three morphologic of intrahepatic

in seven venous

examination

logic-pathologic with complete

hepatopetal

nor hepatofugal collaterals were noted on arterial portography. In the two patients with complete obstruction of firstorder

Histologic

vealed that all had chronic

ducts,

which

features. results

from

The first is dilatation calcium

stones, cellular debris, and mucin substances

bilirubinate

[3]. The second

is stricture of intrahepatic bile ducts, infrequently associated with abscess formation. The third is some degree of atrophy of the affected lobe. Decreased blood flow in the portal veins has been implicated in liver atrophy [4-8]; however, the site

and extent

of portal

vein obstruction

has not been

well

1014

KUSANO

investigated

essentially showed

in patients

a benign

that the degree

with OCH correlated that the appearance Downloaded from www.ajronline.org by 50.106.201.28 on 10/22/15 from IP address 50.106.201.28. Copyright ARRS. For personal use only; all rights reserved

with

OCH,

and relatively of portal

because

this disorder

rare condition.

is

Our study

vein obstruction

in patients

with the severity of liver atrophy, and of portal vein obstruction covered a

spectrum including a normal-appearing liver with no atrophy, the “pruned tree” appearance of portal veins with slight to moderate liver atrophy, and complete obstruction of proximal

portal

veins

with

marked

liver atrophy.

This angiographic

correlation of portal vein involvement has to our knowledge not been reported previously. Two patients with complete obstruction of the portal veins

had no evidence of malignancy on histologic examination of the resected specimen; therefore, OCH should be included in the differential

diagnosis

when

hepatic

atrophy

and portal

vein

occlusion [1 ], which when combined are reported to be reliable signs of cholangiocarcinoma [5-7] and hepatic neoplasm [4, 8], are found.

The finding

of complete

portal

vein obstruc-

tion in the presence of identifiable intrahepatic stones combined with marked liver atrophy does not necessarily indicate a diagnosis of associated malignancy, which occurs in about 5-6% of patients with OCH [9, 1 0]. In 1 953, Benz et al. [11] reported four cases of atrophy of the left lobe of the liver, caused by compression of the left trunk of the portal vein secondary to choledocholithiasis and subsequent dilatation of

the left hepatic duct. However, shunts

major branches

of the portal vein, as is commonly

with obstruction

malignancy.

Freeny

retrograde

no evidence

of arterioportal patients

with

our series showed

of the major

[1 2] reported

opacification portal

one

of the

found

branches

patient

with

in

due to

acute

pyogenic hepatitis in whom diffuse hypervascularity and arterioportal shunting involving the affected lobe were found on selective hepatic arteriography and in whom subsequent tolazoline-augmented arterial portography showed poor filling

of multiple intrahepatic portal veins in this area. In our series, most cases showed intense hypervascularity in the liver parenchyma, but arterioportal shunting was not found in any case. This probably is because all but one patient exacerbation of OCH had long-standing chronic

with intense fibroblastic proliferation. The mechanism of bile duct dilatation

with acute cholangitis

in OCH continues

be controversial.

One explanation

is that biliary

first morphologic

change leading to dilatation

stricture

to

is the

of the bile duct

[1 3]. However, 37% of such patients show dilatation of the bile ducts without biliary stricture [1 4]. From these observa-

tions, it is difficult

to explain

the cause of OCH on the basis

ET AL.

AJR:158,

May i992

of biliary stricture alone. Another possible explanation is that portal vein obstruction triggers dilatation of the bile ducts. In experiments on rabbits that had ligation of the portal branch

of the right posterior

lobe and insertion

of tubing into the bile

duct through the duodenal papilla, marked dilatation of the biliary tree of the affected lobe was observed [2]. Takahashi

et al. [1 5] also frequently found thrombotic obstruction due to cholangitis in the small branches of the portal vein. Our study, however, disease.

does

not clarify

the pathophysiology

of this

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4. Heaston DK, Chuang VP, Wallace 5, de Santos LA. Metastatic hepatic neoplasms: angiographic features of portal vein involvement. AJR 1981;136:897-900 5. Vazquez JL, Thorsen MK, Dodds WJ, Foley WD, Lawson T. the left hepatic lobe caused by a cholangiocarcinoma. AJR 547-548 6. Carr DH, Hadjis NS, Banks LM, Hemingway AP, Blumgart LH. tomography of hilar cholangiocarcinoma: a new sign. AJR 53-56 7. Takayasu K, Muramatsu Y, Shima Y, et al. Hepatic lobar atrophy

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findof

14. Koga A, Miyazaki K, lchimiya H, Nakayama F. Choice of treatment for hepatolithiasis based on pathological findings. World J Surg 1984;8: 36-40 1 5. Takahashi Y, Takahashi T, Ise H, Matsuno S, Takahashi W, Sato T. Threedimensional morphology of intrahepatic bile ducts in patients with intrahepatic gallstones. (Japanese, abstr in English). Nippon Geka Gakkai Zasshi 1990;9i :86-94