Ultrasound Brian

D. White,

Beatrice

Case MD

#{149} Kostaki

L. Madrazo,

of the G. Bis,

MD

1, 2. (1) Sagittal (a) and coronal (b) echoic midline mass (curved arrow) indenting a) . Arrowhead = hypoechoic tubular structures (b) color Doppler images demonstrate turbulent also contain flow (arrowhead).

infants

and

Aneurysm, vein Infants, newborn,

#{149}

R.SNA,

396

U

1992;

the

A. Cacciareii,

MD

ofGalen, central

1765. nervous

gray-scale sonograms ofthe brain demonstrate a 4-cm hypothe posterior aspect of the third ventricle (straight arrow in near the right choroid plexus. (2) Sagittal (a) and coronal flow in the cystic mass. The tubular hypoechoic structures

1494 #{149} Ancnovcnous system, 1765.1494

malformations, Ultrasound

#{149}

(US),

cerebral, Doppler

1765. 1494 #{149} Cerebral studies, 1765.12984

blood vessels, #{149} Ultrasound

US, (US),

From

1991

in

children

RadloGraphics I From scientitic

#{149} Alexander

MD

Figures

Index terms: 1765.12984

Day1

I)epartment assembly.

12:396-400 ofl)iagnostic Received I)ecember

Radiology, William Beaumont 10, 1991 ; accepted December

Hospital. 3601 20. Address

W 13 Mile Rd. Royal Oak, MI 48073. reprint requests to B.L.M.

the

R.SNA

1992

RadioGraphics

U

White

et al

Volume

12

Number

2

a.

b.

3.

Figure Galen

c.

(a) CT scan obtained with contrast material enhancement reveals the markedly dilated vein of (b) Another scan obtained at a lower level shows the dilated straight and transverse dural sinuses

(*).

(arrows) and the choroidal and thalamic feeding dilated straight and transverse sinuses (arrows). ally, more prominent on the right (arrowhead).

ate

U HISTORY A male fetus

had

in whom

revealed

prenatal

an intracranial

cystic

respiratory

dilated and flex. A bruit auricular

episodes

ing

that

mass

By day

accentuation

oped, and performed

Soon

after

consistent

was

of the

computed (Fig 3).

with were

birth,

the

patient

and

postur-

output

seizure

cardiac

cranial

bruit

tomography

failure

had

and

devel-

(CT)

was

the

obtained

marked dilatation ated with dilatation torcula herophili, tiple

serpentine

and

A 4-cm

hypoechoic

after

noted

ventricle

Color Doppler flow in the mass. in the exhibited

ante-

images Multiple region flow

intravenous

material

mild

mid-

mass, third

hypoechoic tubular structures the right choroid plexus that were noted. of contrast

shows the bilater-

ventricles

ventricle.

displaced

CT scans

1,

scan again is present

lateral

third

supnatentonial

istration

activity.

on day 2 (Figs

of the

niorly and superiorly. revealed turbulent

The

pupils

Subsequent hemorrhage

of the

posteriorly,

1

rigidity with

performed

3, high

His

line

was

were

he had no sucking reover the right posterior

of muscular

were

Sonography 2).

fixed, and was heard

region.

had

effort.

dilatation

dilatation

sonography

delivered at term. The Apgar scores and 2, at 1 and 5 minutes respectively. infant was initially limp and cyanotic minimal

vessels (arrowheads). (c) Dependent intraventricular

of

admin-

demonstrated

of the vein of Galen, associof the transverse sinuses, and the straight sinus. Mulstructures

were

seen

in the

region of the thalamus and lateral ventricles, extending into the right chonoid plexus.

U FINDINGS Gray-scale real-time and color Doppler images of the brain were obtained through the anterior fontanelle (Figs 1, 2). There was moder-

March

1992

White

et al

U

RadioGraphics

U

397

Figure echoic

4. Cerebral AVM in another neonate. (a) Coronal gray-scale right-sided posterior supratentonial mass (solid arrow). Open

transaxial CT scan demonstrates a large high-attenuation right-sided noted on the left (arrow). The vein ofGalen is not involved. There due

to previous

Arteniovenous

of the

vein

venous

cation

eurysmal

of vascular malformations AVMs, capillary telangiecta-

angioma,

dysplastic

cavernous

vascular is based

angioma,

malformation on

the

of

(1).

histologic

and

dilatation

rapid

Classifi-

shunting

arteries

appearance

and

eurysms transverse There

ofvessels

terns

surrounded

by

normal

Occasionally, when ischemia In the fetus

brain panenchyma on hemorrhage and the neonate,

AVMs

the

involve

vein

brain

(2).

Although

referred

to as an

from

be detected

systems.

arises from the dude vasculature and telencephalon.

anterior

or

posterior the

an-

AVM

of the

primitive

sonographic

vein

tentorial third

colon

echoic

U

White

et a!

devel-

(2,5-9). and

the

feeding

Doppler

studies

turbulent

differentiate lesions

such

cere-

of prenatal involving

the

can

after

usually

30 weeks

as midline

supra-

posterior

to the

is not

appreciated

pulsed

Doppler

help

flow

pat-

superior

AVMs

masses

sonograms,

an-

and

depending

reports

seen

Ifflow

of

(4). In neonates, the AVMs usually consist lenticulostniate, thaanterior and posterior

These

are

a

The

of Galen,

sonographically

ventricle.

help

enlarged is noted.

ofAVMs

hypoechoic

gray-scale

and

RadioGrapbics

vein

diagnosis

(2)

velocity

U

of the

ofGalen

gestation

diencephalon, but it can infrom the metencephalon It is thought that the close

approximation

the veins

choroidal, and occasionally bellar arteries. There are several case

terial

commonly,

between draining

in AVMs

arise

the

of Galen

drained by dilated straight dural sinuses. are four prominent angiographic

vein

Most

vein

are

of the vein of Galen, this terminology is considered less precise because the aneurysmal vein of Galen is usually fed by an abnormal collection of vessels (3) . These can either

of the

on the age of the patient feeding vessels of these of the anterior cerebral, lamic perforating, both

degenerates has occurred. almost all

ofGalen

is commonly

tissue.

aneurysm

anatomic

is

oping as the fetus grows (3). Occasionally, cerebral AVM is noted without involvement the vein of Galen (Fig 4). At angiography,

of the abnormal vessels and adjacent brain parenchyma. AVM, capillary telangiectasia, and venous angioma are abnormal collections

the abnormality

398

is cortical

of the brain reveals a hypotentorium. (b) Unenhanced Intraventricular hemorrhage loss on the right, presumably

of Galen with the chonoidal arteries leads to the development of an arteniovenous fistula that evolves into an AVM, with secondary an-

malformation

of Galen.

U DISCUSSION The various types the brain include sia,

=

infarction.

DIAGNOSIS: (AVM)

sonogram arrow AVM (*).

confirm

within

AVMs as arachnoid,

the

from

on on

the

high-

mass

(10)

other

hypo-

colloid,

Volume

on

12

Number

2

fled

as high-attenuation,

structures the AVM

without is often

associated cially

dilated

serpentine

mass effect. small, compared

draining in central

venous or

The

nidus of with the

structures,

thalamic

AVMs

espcthat

are

as-

sociated with a dilated vein of Galen (varix or aneurysm). With ruptured AVMs, the high attenuation of the intracenebral hematoma is noted on unenhanced CT scans. Cystic on atrophic changes near the hematoma can be seen and indicate sequelae of prior hemonrhages. On unenhanced CT scans, the dilated vein

of Galen

uation

is noted

smooth

posterior

aspect

hances

after

Use

as a slightly

midline of the

infusion

high-atten-

mass

that

indents

third

ventricle

of contrast

of magnetic

the and

en-

material.

resonance

(MR)

imaging

for intrauterine diagnosis of fetal AVMs not been described, to our knowledge.

has Gradi-

ent-echo pulse sequences may prove to be ideal for imaging the fetal brain because they enable faster data acquisition and provide flow-related enhancement of vascular structunes

Figure

5.

Intrauterine

MR demonstration

in another patient. Gradient-echo image tion time, 50 msec; echo time, 15 msec; angle) reveals a cerebral AVM (*), which depicted due to flow-related enhancement.

other

(repeti. 50#{176} flip is easily On

cysts.

associated

with

largement, dilated and hydrocephalus Cardiac enlargement

heart

failure.

chanical Sylvius monly, orrhagic

thermore, with

result

from

results

of the aqueduct vein of Galen. results from of cerebrospinal infarction

cerebral

and

from

me-

of Less composthemfluid

leukomalacia

hypoperfusion

5cc-

by the AVM. varies, de-

pending on whether the vessels have cuptuned ( 1 , 1 1 , 1 2) . On unenhanced scans, when there has been no rupture, the abnormal yessels usually have attenuation only slightly higher than that ofadjacent brain; on contrast-enhanced scans, the vessels are identi-

1992

exact

role

for

MR

imaging,

since ultrasonography readily available and less costly. AVMs can be readily diagnosed Doppler

and

obviating

color

further

Fun-

Doppler

imaging

flow

with

MR

in

utero. The provisional in utero diagnosis, however, can be confirmed with CT, MR imaging, on angiography after birth.

sonographic findinclude cardiac enveins of the head and neck, (as was seen in our case). is caused by high output

ondary to a steal phenomenon The CT appearance ofAVMs

March

The

Other

Hydrocephalus

Brain

5).

is uncertain,

pulsed

studies,

AVMs

obstruction by the dilated hydrocephalus impairment

absorption.

(Fig

however, is more

the vein of Galen aneurysm was Arrow = dilated transverse sinus.

porencephalic

also

AVM

images,

identified.

ings

of

U THERAPY High morbidity with ing

all the

and

forms vein

mortality

are

ofmanagement ofGalen

associated

ofAVMs

(13).

In our

involv-

case,

sun-

gery was not performed; the infant was dischanged, received supportive care at home, and died soon after discharge. The decision for surgical on interventional treatment depends on accurate assessment the feeding vessels. Vessels are subsequently ligated and excised (14) or embolized by means of transtonculan or transartcrial techniques. pointing,

The

results

especially

of treatment among

are critically

nates with heart failure. Furthermore, presence of cerebral damage suggests surgical correction of the AVM offers

White

Ct a!

U

of

disapill neo-

the that little

RadioGraphics

U

399

(15). In a series for Sick Children

of 16 neonates in Toronto

the six neonates survived. The

who surviving

hemiparesis.

tolic

pressures

diastolic anesthetics.

from

by the

can

also of the

embolization

optimal

treatment

failure

(16).

Hospital one of

high

poor

partly

reduced to low

condias-

AVM.

Low

large

aggravated

6.

7.

8.

by

neonatal

9.

technique

10.

2.

Haughton VM. Vascular diseases. In: Williams AL, Haughton VM, eds. Cranial cornputed tomography, a comprehensive text. St Louis: Mosby, 1984; 88-147. Comstock CH, KirkJS. Anteriovenous mal-

formations,

3.

4.

5.

locations,

and evolution

VC, DiLeo

PD, Chameides

A.

vein

Computed

CR, Chuang SH. Galen in children:

15.

White

et a!

malPeninatol

Fetal

tomography

Ther

in aneuAssist DC,

Fitz

Aneurysms of the vein of CT and angiographic come20:123-133. F, Peacock

Management

aneurysms:

of vein of Galen

WJ.

combined surgical and endovascular approach. Childs Nerv Syst 1989; 5:208-211. Watson DG, Smith RR, Brann AWJr. Arteniovenous malformation ofthe vein ofGalen. Am J Dis Child 1976; 130:520-525. Norman MG, Becker LE. Cerebral damage in neonates from artemiovenous malformation of the vein of Galen. J Neurol Neurosurg Psychiatry 1974; 37:252-258. Kerber CW, Bank WO, Cromwell LD. Cali-

leak balloon exploration

AiR 1979; 132:207-2

U

Prena-

lations. Neuroradiology 1980; King WA, Wackyrn PA, Vinuela

for arterial

RadioGraphics

ofGalen.

12.

14.

Gy-

1986; 3:209-2 11. Rizzo G, Arduini D, Colosimo CJr, Boccolini MR, Mancuso S. Abnormal fetal cerebral blood flow velocity waveforms as a sign of an

brated

U

L.

of arteniovenous ofGalen. AmJ

rysms of the vein of Galen. J Comput Tomogr 1979; 3:779-782. Martelli A, Scotti G, Harwood-Nash

13.

Inan an-

Am J Obstet

Spalone

16.

400

D. from

1 1.

in the

fetal brain. J Ultrasound Med 1991; 10:361365. O’Brien MS, Schechter MM. Arteniovenous malformations involving the Galenic system. AJR 1970; 110:50-55. Hoffman HJ, Chuang S, Hendrick EB, Humphreys RP. Aneurysms of the vein of Galen. J Neurosurg 1982; 57:316-322. Reiter AA, HugtaJC, Carpenter RJ Jr, Segall 6K, Hawkins EP. Prenatal diagnosis of anteriovenous malformation of the vein of Galen. JCU 1986; 14:623-628.

P, Shah failure

Mendelsohn DB, Hertzanu Y, Butterworth A. In-utero diagnosis of vein of Galen aneurysms by ultrasound. Neuroradiology 1984; 26:4 17418. Vintzileos AM, Eisenfeld LI, Campbell WA,

aneurysm ofthe 1987; 2:75-79.

REFERENCES 1.

D, Roussis ofcardiac

tal ultrasonic diagnosis formation ofthe vein

is success-

be attempted later is more stable.

detection

Herson

through the AVM and the high output car-

If this

90:872-873. Jeanty P, Kepple

eurysm of the vein of Galen. necol 1990; 163:50-51.

mortality the

Mao K, AdamsJ. Antenatal diagnosis of intracranial arteniovenous fistula by ultrasonography: case report. BrJ Obstet Gynecol 1983;

utero

is recommended

ful, surgical correction can when the patient’s condition U

be

during

period to reduce flow subsequently to relieve diac

are

ischemia from flow secondary

Because

surgery,

as the

of surgery

induced

pressures

at the only

were treated surgically patient had residual

Results

due to myocardial onary artery blood

(4),

micro and

catheter: occlusive

a device therapy.

12.

Volume

12

Number

2