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s%.,,_..,...,, .....,

creatinine mg/dl.

.

The

66.8%

of

“‘

was

37.5

Davis,

iothalamate

‘‘“-‘

‘‘“-MI

with a serum

mg/100

ml.

revealed poor visualization of essentially normal size.

California,

sodium

..,

was 69 mI/mm

hematocnit

at another institution thinning in kidneys University

‘‘“-‘

,...,1,

clearance

Medical

(Conray

Excretory

#‘-‘

-._-- . .. .

a ., is unimpressive

‘

of 1 .6

unography

hyaline

with diffuse cortical Repeat unography at

Center

400,

‘

creatinine

used

1 00

Mallinckrodt)

ml

as a rapid

medulla

with

calyceal

cystic

system

Received

appeared

January

I Department

lucencies

1 1

,

of Diagnostic

(fig.

markedly

1 979;

accepted

Radiology,

2).

The

clearly

distorted

after

revision

University

delineated

with areas

April

9,

is rarely

and [2].

disease

at autopsy,

studies

AJR

Department

of Urology,

133:303-305,

August

University 1979;

of California,

0361 -803X/79/1

Davis,

Medical

332-0303

50

cysts and [3]. of five

be considered

is cysts The

could

not be

affected

are

cuboidal

with were

involving

cysts

by simple

patients

Significant

should

[1]. finding

lined

occasional

the 50 sm

epithe-

medullary

cystic

evident

macro-

identified kidneys

only

on

by Sher-

1979.

of California,

Davis,

Medical

Center,

4301

X St.,

Sacramento,

Link. 2

are

cells,

proteinuria.

junction.

of

in 1 8 subjects examination

Microdissection

of stretch-

and

a few

mild and

corticomedullary

studied

scopically microscopic

pelvi-

or

renal disease pathologic

In a series

,

only

encountered

to 2 cm in diameter hum

show

casts,

evidence for other The characteristic

of

may

granular

proteinunia

intravenous bolus. Vascular phase nephrography revealed kidneys of normal size with a uniformly thinned, continuous cortical layer surrounding lucent enlarged medullary pyramids (fig. 1). During the excretory phase, the medullary areas exhibited a mottled pattern of increased density

or

and

Center, $00.00;

Sacramento, © American

CA 9581 7. Roentgen

Ray Society

CA 9581

7. Address

reprint

requests

to D. P.

CASE

304

REPORTS

AJR:133,

August

1979

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Fig. 1 -A, Right kidney. B, Left kidney. Zonogram 1 5 sec after injection. Vascular phase nephrogram with thinned continuous cortex surrounding lucent medullary pyramids (arrows).

B

A

Fig. 2.-A, Right kidney. B, Left kidney. 30 mm zonogram. Lucent medullary cysts (small arrows) surrounded by partly obstructed contrast filled tubules outlining medullary pyramids (large arrows).

,?

A

‘4

B

AJR:133,

man

August

et al. [4] showed

size,

with

cysts

collecting tion

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CASE

1979

numerous

involving

tubules.

They

of the diverticula

in other

types

tubular

only

noted

renal

distal

and

and concentra-

greater

than

The

cortex

disease.

of varying

convoluted

the number

to be much

of

diverticula

the

they

had

may

pathology

has

been

reported

cystic disease as a recessive

pigmentosa,

and

aracts

have

Biedl not

been

observed,

syndrome. been

The renal

tation

of gb-

patients

with

with

when the renal trait [1 ]. Retinitis

as the

with

and cat-

has

course

function to

of the

requiring

and leading

to death

mitigation

disease

eventual of the

is progressive

dialysis

in most

loss

or renal

cases

of

transplan-

[1 ]. Treatment

complications

is

of chronic

renal

failure.

In the past, considered

although described contrast [5].

normal

dose

intravenous

nondiagnostic

in renal

urography

medullary

has been

cystic

disease,

recently a late medullary nephrogram’ has been as a finding in early disease, presumably due to material retained in partially obstructed tubules ‘ ‘

Angiographic

nosis

study

of five

patients

by

Mena

et al.

[6]

although are

cysts

large

not often

enough

present

in a patient

disease

in this

with

but

there

polycystic

entity,

abnormalities

from kidney.

diabetes has

kidneys

with

unique

intravascular

At greatest

in the

in patients with

failure

patients.

suggests that in the high dose urography

cortex

medullary

been

risk

are

Almost

all

mellitus

or

a case

presence of mild can demonstrate clear

to this

of

[9].

visualization

renal the of the

disease.

medullary

biopsy

REFERENCES Gardner KD Jr: Juvenile nephnonophthisis and renal medullary cystic disease, in Cystic Diseases of the Kidney. edited by Gardner KD Jr. New York, Wiley, 1 976, pp 1 73-i 85 2. Lang EK: Roentgenologic assessment of medullary cysts. 1

to

be

detected

radio-

[3].

Computed

.

Semin

3. 4.

tomography

and

ul-

not

1 0: 1 45-1

54,

197

juvenile

nephronophthisis

examined

by

microdissec-

Am J Clin Patho! 55:391 -400, 1971 5. Burgener FA, Spataro RF: Early medullary cystic disease: urographic diagnosis? Radiology 1 30:321 -322, 1979 6. Mena E, Bookstein JJ, McDonald FD, Gikas PW: Angiographic findings in renal medullary cystic disease. Radiology 1 10:277tion.

281,

trasound scanning may be useful in this entity but have been reported and were not performed in our case.

Roentgeno!

Mongeau JG, Worthen HG: Nephronophthisis and medullary cystic disease. Am J Med 43:345-355, 1967 Sherman FE, Studnicki FM, Fetterman EH: Renal lesions of familial

[1].

Tissue diagnosis is usually obtained by open renal biopsy or from postnephrectomy or autopsy specimens due to the technical difficulties and hazards posed by percutaneous renal

vessel

nephropathy,

Our case dysfunction,

‘

identified the presence of a thinned, uniformly smooth acystic cortex as an important differential diagnostic criterion. Demonstration of lucent medullary cysts clinches the diaggraphically

renal

failure

occurred

hypertensive

thinned

disease

oliguric

in renal small

have

oliguria

Laurence-Moon-

hepatic

of acute

media

cases

degeneration,

as well

An association

reports

described.

natural

limited

retinal

been contrast

in association

cases of renal medullary disease was transmitted tapetoretinal

seen

The safety of high dose, 40 g iodine, intravenous urography has been well established [7, 8], providing patients are maintained in proper hydration. Recently, there have

appear

microscopically normal or show a variable degree merular hyalinization or perigbomerular fibrosis [2]. Eye

305

REPORTS

7.

a

1974

Davidson

AJ:

Radiologic

Diagnosis

of Renal

Parenchymal

Dis-

ease, Philadelphia, Saunders, 1977, pp 3-il 8. Link DP, McGahan JP, McMahon J, Teague JH: Routine dose of sodium iothalomate (Conray 400) for urography: 40 g I versus 20 g I. Submitted for publication 9. Heneghan M: Contrast induced acute renal failure. AJR 1 13: 1113,

1978