527

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Case Report

Intrahepatic Jae Hyung

Portosystemic

Park,1

Sang

Hoon

Venous

Koo Han, and Man Chung

Cha, Joon

Among numerous variations of communications between the portal and systemic venous circulations, intrahepatic shunts between portal and systemic veins have seldom been described [1 , 2]. Recently we encountered an intrahepatic portosystemic venous shunt directly connecting the right portal vein to the inferior portion of the vena cava. We describe our case and discuss 14 other previously published cases [110].

A 51 -year-old of altered the

man

was

mental

past

two

which

On liver

status.

were

chest

Emergent Treatment mental

and

blood for

variceal

changes

such

flapping

tremor

Selective

General treatment, mental

portal

vein

lobe (Fig. 1A). Underlying Abdominal

CT

portosystemic posterior vena

surface

cava

just

of the

for

mellitus

he ate protein-rich department,

angioma

signs

on his face of the

did

not

diabetes improve

Endoscopy

the

wrist.

patient’s grade

a hepatic

tubular

II

in the

posterior

of altered

cause

structure

connecting

segment

the

of the right

chronic liver disease was found also. the

sonographic

liver

the

and

from turned

insertions

findings.

The

the right portal medially

veins

unusual

vein to the

to enter

of the hepatic

the

inferior

(Figs.

1 B and

1 C). Arteriography with the catheter tip at the common hepatic artery showed a tortuous, corkscrew appearance of the peripheral branches of

the

hepatic

venous

phase

of superior

artery,

clearly

visualized,

just

was

1 .5 cm in diameter

and

beaded

inferior

vena

Received I

at the cava

advanced

mesenteric

as it was and

posterior (Fig.

February

All authors:

suggesting

was

with

sonography

tubular

surface

liver

cirrhosis.

arteriography, and

shunt

CT.

The

intrahepatically of the

liver

In the

the

and

before

was shunt

irregular

entering

the

1 D).

2, 1990; accepted

Department

of Radiology,

after revision Seoul

March

National

21

,

University

status

September

1990 0361-803X/90/1553-0527

hepatic

veins

to

be

including dietary control, improved the patient’s

and he was discharged.

arbitrarily

into four

different morphologic types. The first and most common type, described in six cases [3, 6, 8, 1 0], is a single large tube of constant diameter that connects the right portal vein to the inferior vena cava. The second type is a localized peripheral shunt in which single or multiple communications are found between peripheral branches of portal and hepatic veins in one hepatic segment [1 , 4, 9]. The third type is aneurysmal: peripheral portal and hepatic veins are connected through an aneurysm [7, 9]. The fourth type has multiple communications

between peripheral lobes [2, 5].

portal and hepatic

veins diffusely

in both

The tubular shunt in our case was of the first type, nearly identical to the other six published cases [3, 6, 8, 1 0]. Most of the patients with this type of intrahepatic portosystemic shunt had clinical evidence of liver cirrhosis and portal hypertension [3, 6, 8, 1 0]. Ohnishi et al. [6] believed this unusual communication of portal vein to the vena cava was a patent ductus arteriosus. However, we believe the typical course of this type of intrahepatic portosystemic shunt is different from that of the patent ductus arteriosus. First, the shunt connects the right portal vein instead of the left to the vena cava [8].

1990. Hospital,

28 Yongon-dong,

Chongno-gu,

H. Park. AJR 155:527-528,

the

hepatic venous shunts can be categorized

of and

mellitus.

revealed

showed

To our knowledge, a total of 1 4 cases of portohepatic venous shunt have been reported [1 -1 0] since the initial report of congenital intrahepatic shunt by Doehner et al. [1] in 1 956. In most reported cases, the patients were over 50 years old (the oldest was 75 years old), and the chief cornplaints were personality changes or abnormal mental status due to portosysternic encephalopathy [2, 3, 5, 6]. Our case and the 14 previously published cases of porto-

liver

drinker

hyperextension

exclude

ran posteriorly

below

of diabetes

uncontrolled

cava

confirmed

shunt

on

unusual

to the vena

alcohol

history

venograms

Discussion

esophagus.

to an

a habitual

emergency

expected.

in the lower revealed

because

hepatic

caused by the shunt.

status.

coma as

and

Hospital

as spider

suggested

sonography

status

right

in the

much

mellitus

admission,

mental

observed tests

as

Abdominal mental

before

altered

hyperosmolar

status

been

diabetes University

a 10-year

days

the

examination

cirrhosis

anterior

He had

Two

of

National

despite

induced

physical

a history

to Seoul

decades

and liver cirrhosis. food,

with

admitted

Han

relatively small, and inferior venacavograms showed normal patency with an inflow defect just below the hepatic vein insertions that was

Case Report

cirrhosis

Shunt

© American

Roentgen

Ray Society

Seoul

1 1 0-744,

Korea.

Address

reprint

requests

to J.

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Fig. 1.-A, Transverse sonogram of right lobe shows curved tubular structure connecting right portal vein to inferior vena cava (arrow). B, Unenhanced CT scan reveals tubular structures of low density in posterior segment of right lobe. Shunt turns acutely to inferior vena cava at posterior aspect of right lobe, showing dilated and beaded appearance. C, Tubular structure enhances homogeneously on enhanced scan. D, Arterial portogram with injection of contrast medium into superior mesenteric artery discloses intrahepatic portosystemic shunt connecting right portal vein to inferior vena cava (arrow). Course of shunt at right posterior aspect of liver is shortened on this oblique view.

Second, the relation of the intrahepatic course of the shunt to the caudate lobe and the fissure for the ductus venosus was different from that of the patent ductus arteniosus. The cause of intrahepatic portosysternic venous shunts is unknown. Kozuka et al. [3] considered the abnormality to be acquired because microscopic examination showed both the muscular layer and the elastic lamellae to disappear abruptly from the wall of the shunt and because cerebral manifestation was not apparent until older age. However, congenital origin of this abnormality also has been postulated [2, 9]. One embryologic explanation for shunts in the right lobe is the persistence of a high-flow communication between the omphalomesentenic venous system and the right horn of the sinus venosus [2]. Decreasing tolerance to toxic metabolites with increasing age may explain the late clinical manifestation [2, 9]. In the aneurysmal type, perhaps a portal aneurysm precedes the venous shunt and then ruptures into the hepatic vein to make the communication, although the cause of portal aneurysm is still uncertain [7]. Treatment including dietary control and surgical removal of the shunt can be used to alleviate symptoms [2, 9]. As an intrahepatic portosystemic venous shunt may cause encephalopathy, correct radiologic diagnosis and proper treatment of this unusual abnormality are clinically important.

REFERENCES

1 . Doehner system:

GA, Ruzicka FE Jr, Rousselot LM, Hoffman on its pathological roentgen anatomy.

G. The portal venous Radiology 1956;66:

206-217 2. Raskin NH, Price JB, Fishman RA. Portal systemic encephalopathy due to congenital intrahepatic shunts. N Engi J Med 1964;270 : 225-229 3. Kozuka S. Sassa R, Kakumu S. An enormous intrahepatic shunt between portal vein and hepatic one. Angiology 1975:26:365-371 4. Okuda K, Kanda V. Fukuyama Y, et al. Hepatic vascular anomalies in non parasitic cysts of the liver. Acta Hepatogastroenterol 1976;23: 110-113 5. Yamashita S, Nakata K, Muro T, et al. A case of hepatic encephalopathy due to diffuse intrahepatic porto-systemic shunts (Japanese). Nippon Naika Gakkai Zasshi 1982;71 :843-850 6. Ohnishi K, Hatano M, Nakayama T, et al. An unusual portal-systemic shunt, most likely through a patent ductus venosus: a case report. Gastroenterology 1983;85:962-965 7. Takayasu K, Moriyama N, Shima V. et al. Spontaneous portal-hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology

1984;86:945-948 8. Kadoya M, Takashima T, Matsui 0, Kitagawa K, Chohtoh S. Unusual portosystemic collateral penetrating the liver parenchyma. Gastrointest Radio! 1985;10:1 15-118 9. Chagnon SF, Vallee CA, Barge J, et al. Aneurysmal portohepatic venous fistula: report of two cases. Radiology 1986;159:693-695 1 0. Mon H, Hayashi K, Eukuda T, et al. Intrahepatic portosystemic venous shunt: occurrence in patients with and without liver cirrhosis. AJR

1987;149:71 1-714