Cases Genera

V

Marie-Pierre

Cordeau,

MD

of the

#{149} Odile

Prosmanne,

MD

HISTORY

U

white

woman

upper gastrointestinal data indicated severe

was

admitted for Laboratory Results

bleeding. pancytopenia.

Figures

Index

1, 2.

terms:

From

RSNA

1990;

the

Department

annual

RSNA,

(1)

Coronal

Hypertension,

RadioGraphlcs

U

Robillard,

function

nous

wedge

logic vealed

tests were esophageal

MD

studies

pressure

and were

negative, vanices.

the

hepatic

normal.

and

ye-

Parasito-

endoscopy

me-

2a.

1.

114

#{149} Pierre

of liver

A 79-year-old

I

Day

meeting.

splenic

portal,

95.7

sonogram.

11

Pancreas,

#{149}

(2)

Abdominal

CT scans.

77.372

diseases,

10:114-116

ofRadiology. Received

H&tel.Dieu September

29,

de Montr#{233}al,3840 1989;

accepted

Rue October

St Urbain, 3. Address

Montreal, reprint

Que, Canada requests to

H2W P.R.

lT8.

From

the

1989

1990

Ra4ioGrapbics

U

Cordeau

et a!

Volume

10

Number

1

Figures

U

The

3, 4.

(3) Abdominal

RADIOLOGIC sonogram

megaly

with

(4)

Selective

FINDINGS

(Fig

spleno-

echostmucture.

Comput-

(CT)

scans

(Fig

2) showed

a

sels, renal

January

1990

flow,

multiple

and severe artery.

stenosis

venous of the

collateral proximal

yesleft

arteriogram

SURGICAL

Because

normal liver and markedly enlarged spleen. The splenic vein was clearly seen and had numerous collateral vessels, particularly near the medial bonder of the spleen. The abdominal aortogram (Fig 3) and selective splenic arteniogram (Fig 4) revealed a memarkably dilated splenic artery, a patent splenoportal venous trunk with hepatopetal

blood

splenic

U

1) demonstrated

normal

ed tomognaphic

aortogram.

(late

venous

FINDINGS

of her severe

pancytopenia,

tient underwent splenectomy. splenic and portal veins was surgery. The liver was normal

peared

slightly

phase).

granular

the pa-

Patency of the confirmed at in size but ap-

at gross

examina-

tion; analysis of the hepatic wedge biopsy specimen revealed mild portal fibrosis with slight sinusoidal dilatation. The removed spleen was greatly enlarged and showed changes typical of congestive splenomegaly.

The

patient

ten surgery, and normal values.

Cordeau

was asymptomatic hematologic

et a!

data

U

4 months

a!-

returned

to

RadioGrapbics

U

115

DIAGNOSIS: hypertension

Noncimrhotic idiopathic (Banti syndrome).

portal

This

U DISCUSSION Idiopathic portal hypertension consists of splenomegaly, hypersplenism, and portal hypertension in the absence of portal vein obstruction on significant liven disease. This syndrome was originally described in i 882 by Guido Banti and carries his name. This entity

and

is also

known

noncirrhotic Idiopathic

America

and

in India

more

as hepatoportal

Europe

andJapan.

frequently

plaints for trointestinal

enlarged

sclerosis

portal fibrosis portal hypertension but

Middle-aged

in

women

attention or discovery

elements

are gasof an

(3).

of hypersplen-

ism); (1) liver scintigraphic suggestive of cirrhosis; (e) veins with normal to slightly

findings not patent hepatic elevated hepatic venous wedge pressure; (/‘) grossly noncimrhotic but frequently uneven liver surface; (g) hepatic histologic findings not suggestive of cirrhosis; (b) patent extrahepatic portal vein with multiple collateral vessels, as seen at portography or sonography; and (1) elevated portal vein pressure. Although it is

not

necessary

to perform

nomegaly was thought to be the primary event, with splenic hyperfunction mimicka total

arteniovenous

of the splenic in increased portal hypertension. Howev-

(5,6) and

based histologic

U

RadioGraphics

(usually

and

U

Cordeau

slight),

dilatation

of sinusoids,

U CONCLUSION Idiopathic portal order of unknown Diagnosis

hypertension cause with of the

is a rare favorable

condition

the basis of exclusion among causes of portal hypertension.

is made

the

U REFERENCES 1 . Futagawa 5, Fukazawa

patic venography portal hypertension.

M, Musha in noncirrhotic Radiology

on

different

H, et al. Heidiopathic 1 98 1 ; 141:

303-309. Zakim D, Boyer T. Hepatology: a textbook liver disease. Philadelphia: Saunders, 1982.

2. 3

disprog-

.

Rozenbaum A, Atienza J. Primary hepatoportal form of Banti syndrome?

P, Couturier sclerosis: Ann Med

(Paris) 1988; 139:52-53. Futagawa 5, Fukazawa M, Honisawa

4.

Portographic liver changes cirrhotic portal hypertension.

of

D, Guerre current Interne M, et al.

in idiopathic AJR 1980;

non-

134:917-923. 5

.

Okuda

K, Kono

study of eighty-six hypertension and with splenomegaly. 86:600-610.

6.

Ohnishi

K, Saito

K, Ohnishi

K, et al.

cases of idiopathic comparison with Gastroenterology

Clinical portal cirrhosis 1 984;

M, Sato 5, et al. Portal heportal hypertenGastroenterology .

on venofind-

ings suggest that increased portal vascular sistance, rather than increased splenic venous flow, may play an important mole.

116

obliteraportal

intimal thickening with eccentric sclerosis of the peripheral portal vein walls (2,6). The prognosis of idiopathic portal hypertension is much more favorable than that of cirrhosis, but hepatic failure may develop in advanced cases (5) The 5-year survival rate is 90%; the 30-year survival rate is 55% (3).

modynamics in idiopathic sion (Banti’s syndrome) 1987; 92:751-758.

fistula

bed and thus resulting blood flow and portal en, many recent studies graphic, portographic,

and

lim-

all the diagnostic

tests and procedures mentioned above, the essential diagnostic criteria include portal hypertension in the absence of cirrhosis, panasites, and venous occlusion (i ,4). The pathogenesis of portal hypertension in these patients is unknown. Originally, sple-

ing

resistance

fibrosis intrahepatic

branches (5,6). Recognizable pathologic changes are ited to varying degrees of portal fibrosis

nosis.

(as a result

vascular

are

com-

Diagnostic criteria include (a) near normal or normal results from liver function tests; (b) vanices, as seen at endoscopy or madiography; (c) cytopenia of one or more

blood

portal

to portal of the

.

disease

and the chief

seeking medical hemorrhage

spleen

(i ,2). is rare

is a common

affected,

increased

could be due tive venopathy

et al

me-

Volume

10

Number

1