Cases

of the Day

Pediatric1 M.

Teresa

Brooks,

Edward

MD

M. Burton,

U HISTORY A 1 4-year-old

plaints

girl

sharp

malaise,

dia

and

had

my

and

tympanoplasty

#{149} F.

and period. with

for chronic

undergone

1 b)

as splenomegaby

She multiple

rim

with was

performed

diagnosis

examination

a well-

healed, night lower but no organomegaly

quadrant laparotomy or palpable mass.

scar The

patient

was

and

Lab-

oratory crocytic

work-up anemia

afebnile

nommotensive.

Further

sonography raphy (CT) (Fig 3).

(US) (Fig 1), computed tomog(Fig 2) , and renal scintigraphy

studies

consisted

kidney,

and

lion

of the might kidney

(Fig

2a);

these

upper

were

findings,

pole

of ultra-

Photopenia

also

along

with

the

Children,

I

From

(M.T.B., the and and C

1096

U

the

1990; Department

H.L.M.,

Department accepted

S.L.H.,

genitourinary

system.

RadioGrapbics

#{149} Retroperitoneal

space.

high-grade

fibrosis,

kidney. was

con-

hydronephmosis.

maintained, on the 30-

80.893

10:1096-1100 ofDiagnostic

Imaging.

B.D.F.);

Department

of Radiology’. University Januan’ 3 1 , 1 990 . Supported

b’ the American RSNA, 1990

8 1 .369

delayed

parenchyma

Function of the left kidney was but hydronephnosis was evident minute image (Fig 3d).

terms:

evident

with

by the night

within

sistent

RadioGraphics

of the

.

radiopharmaceutical

Index

.

surrounding

excretion of contrast material (Fig 2d) , are indicative of chronic obstruction. Several calyces of the left kidney were dilated with abnormal tissue adjacent to the renal hilus (Fig 2b, 2c) Delayed contrast enhancement was seen in the tissue surrounding the right kidney and spleen (Fig 2d). Renal scintigrams obtained with technetium-99m DTPA (Fig 3) showed marked reduction in flow, uptake, and excretion of the

revealed hypochmomic, miwith normal bone marrow

cellularity.

right

(Fig

tissue

.

chronic

revealed

of hypoechoic

and hy1 a, 1 c) A thin the

(Fig

kidney was also seen (Fig 1 b- 1 d). CT scans optimally demonstrated encasement of the right kidney and spleen by abnormal tissue (Fig 2a-2c) Panenchymal loss, marked hydronephnosis, and decreased func-

appendicitis.

Physical

echogenicity night kidney

left

at age

was

as well

,

spleen,

me-

MD.

Fletcher,

FINDINGS

an 8-10-

otitis

D.

MD

increased of the

laparotomy, Pathologic

#{149} Barry

Seidel,

dmonephnosis

a right at age

MD

Glen

pain,

of adhesions,

1 0 years.

L. Hanna,

MD

US revealed

mastoidecto7 years. An cxappendectomy

pboratory

#{149} Sobeil

com-

with

amenorrhea,

of antibiotics

lysis

MD

F. Boulden,

epigastnic

boss over a 5-month treated since infancy

courses

Magill,

presented

of intermittent,

lb weight had been

Lynn

#{149}Thomas

U

white

increasing

and

#{149} H.

MD

Lebanese

Syrian

U

StJude ofRadiolog’,

Children’s

Research

of Tennessee College of Medicine, in part by the National Cancer

Associated

Brooks

Charities.

et al

Hospital.

Le BonheurChildren’s

Address

reprint

332

N Lauderdale,

MedicalCenter, Memphis. lnstitute requests

,

From Cancer to

Memphis.

Memphis the 1 989 RSNA Center Support

(E.M.B., scientific (CORE)

TN

38101.0318 T.F.B.,

F.G.S.):

and

assembly. Received grant P3OCA2 I ‘65

H.L.M.

Volume

10

Number

6

a.

b.

d.

C.

Figure

1.

kidney

(b),

left kidney

Prone left

transverse coronal

sonogram

of the right kidney (a), supine longitudinal sonogram of the right of the spleen (C), and coronal image of the spleen and upper pole of the

sonogram

(d).

DIAGNOSIS:

Idiopathic

retropenitoneal

fi-

cholangitis, 30%

with mass

cased the spleen and involved the omentum specimens

were

was

found

that

both kidneys and (Fig 4) Multiple .

obtained,

but

no

en-

previous

and

at-

and some received

.

November

70% of adult have been

cases are associated

with systemic bupus erythematosus, thyroiditis, orbital pseudotumor,

Riedel scberosing

1990

cases

gide,

since

of cases

the

fibrosis.

have

malignant

aneurysms,

also bi-

tempt was made at resection. The biopsy samples demonstrated a cellular mesenchymal lesion consisting of proliferative fibroblasts infiltrated by lymphocytes, plasma cells, and eosinophils (Fig 5). Retnopenitoneal fibrosis is rare in childhood, with only 1 3 reported cases in individuals less than 1 5 years of age to our knowledge (1 , 2) The usual age at presentation is in the 5th and 6th decades, with a male-to-female ratio of 3: 1 (2,3). Most pediatric cases and approximately iopathic. Some

occult

aortic

DISCUSSION At surgery, a barge U

opsy

or mediastinal

mately

brosis.

with

surgery,

appendicitis,

ingestion

of various

infancy

and

(2) courses had

abdominal

hemorrhage, diverticubitis,

drugs

methybdopa,

antibiotics) multiple

Approxi-

associated

neoplasms,

trauma

ergotamine,

been

.

(methysen-

hydnabazine, Our patient of antibiotics

undergone

an

had appen-

dectomy with bysis of adhesions. The symptoms in both children and adults with retropenitoneal fibrosis may include abdominal, flank, back, or hip pain and other nonspecific complaints such as weight loss and malaise. Laboratory studies commonly show an elevated erythrocyte sedimentation rate, anemia, azotemia, polycbonal gammopathy, and hypoalbuminemia (4).

id-

Brooks

et al

U

RadioGrapbics

U

1097

a.

b. .-;

,

.‘

d.

C.

Figure minutes

2.

CT scans

afterward

of the abdomen

obtained

during

CT is, at present, the preferred imaging method for patients with suspected netropenitoneal fibrosis. The most common finding is a netmopenitoneal mass at the L-4 and L-5 level (4) The anterior margin of the .

mass

is clearly

delineated

by

the

posterior

penitoneum, whereas the posterior margin is poorly defined and not easily separable from adjacent structures. The inferior vena cava, aorta, and ureters are usually enveloped but not significantly displaced by the mass. Encasement of the umeters may produce varying degrees of hydnonephnosis and, ultimately, renal failure. A less common manifestation of netnopenitoneab fibrosis is extension of the besion into the mediastinum or pelvis, with encasement of regional structures (5) A localized abdominal mass enveloping one on more solid organs (6,7), as was found in our case, .

is an

unusual

the bolus

injection

vealed

a mass

of contrast

media

and 30

(a-C)

(d).

presentation.

CT

clearly

ne-

veboping phrosis

the resulting

of netropenitoneab spleen

and from

ureteropebvic US and renal

junction. scintignaphy

tribute

to the

evaluation

shows

a smoothly

lesion sound

that (4)

mines

ancillary

.

sonographic

and

encasement

en-

hydroneof the

may

also

of these

marginated,

has reduced The location

fibrosis

kidney

con-

patients.

US

hypoechoic

transmission of of the process deterfindings,

such

as

hydronephrosis and encasement of vessels on organs. The impact of retropenitoneab fibrosis on renal function is well demonstrated by renal scintigraphy. When hydnonephnosis is mild or renal function is not severely impained, the administration of funosemide during menognaphy should best demonstrate the obstructive component of the lesion. Experience with magnetic resonance (MR) imaging of netropenitoneal fibrosis is limited (8) The mubtiplanar imaging capability of MR, however, appears to facilitate assess.

ment

1098

U

Ra4ioGrapbics

U

Brooks

et al

of disease

extent.

Retropenitoneal

Volume

fi-

10

Number

6

L

__

_____

b.

a.

C.

Posterior renal scintigrams um-99m diethylenetriaminepentaacetic flow study (a) and at 1 (b), 5 (C), and Figure

3.

obtained with acid (DTPA) 30 (d)

minutes

technetiduring the afterward.

findings included unilateral or bilateral umeterab narrowing at the L-4 and L-5 level, medial deviation of the uretens, and hydmonephnosis with proximal ureterectasis but nor-

mal-sized

uretens

ureterography grade catheters al narrowing,

in the pelvis.

has also been easily traverse a fact suggesting

Retrograde used. Retroareas of ureterthat hydrone-

phrosis and renal failure result from interference with ureterab penistalsis rather than from mechanical obstruction (3). Imaging findings similar to those of netmo-

L d.

brosis has Ti-weighted to moderate

medium to bow signal intensity on images and heterogeneous low intensity (bess than that of fat

but greater than weighted images. eas of moderate weighted images

tive

inflammation

mass. Before ing, the penitoneal

excretory

November

1990

that of muscle) on T2It is postulated that the amsignal intensity on T2reflect some degree of acwithin the fibrous tissue

pemitoneal fibrosis may be seen with lymphoma, leukemia, retropenitoneal sarcoma, metastatic lymphadenopathy, and metropenitoneal hematoma. Definitive diagnosis must be established by means of histologic evalualion of multiple biopsy samples taken throughout the lesion. Treatment of netropenitoneab fibrosis is palliative, sis with

most often consisting lateral or intrapenitoneal

of the umeters. Occasionally, uneteral involvement is so severe that nephmostomy is necessary. Corticosteroid therapy has been used

with

by on mild

the advent of US, CT, and MR imag. initial radiobogic indication of metrofibrosis was usually provided by urography. Classic urographic

of ureterobyrelocation

tens (5)

varying cases

.

In our

Brooks

success, and

after

patient,

et al

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a trial

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course

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5b.

5a.

demonstrates histologically neal (5a)

(4) Laparotomy

4, 5.

Figures

photograph omental nodules, to sites of retropenito-

multiple similar

fibrosis around Photomicrograph

the

spleen (original

and kidneys. magnifica-

tion, X50; hematoxylin-eosin [H-EJ stain) shows proliferative fibroblasts with inflammatory infiltrate of lymphocytes, plasma cells, and eosinophils. (5b) Photomicrograph

(original

stain)

of another

phoid nodule cells entrapped lion.

magnification,

field

X 1 25;

shows

a small

H-E

lym-

adjacent to retropenitoneal by fibrous tissue prolifera-

fat

4moid therapy olysis

was

to follow

recommended, if steroid

treatment

with

ureter-

5.

Miles

6.

penitoneal fibrosis: a sometime lem. Am Sung 1984; 50:76-84. Inaraja L, Franquet T, Caballero

was

unsuccessful. U

1.

REFERENCES Sninivas V, Dow CanJSurg

2.

Demos T. peritoneal 4 10-4

4.

U

fibrosis.

1982;

7.

145:59-61.

Your diagnosis: idiopathic retrofibrosis. Ill MedJ 1980; 158:381,

12.

Fagan C, Larnieu A, Amparo E. Retropenitoneal fibrosis: ultrasound and CT features. AJR 1979;

1100

Retropenitoneal

Martin

C.

Idiopathic

retro-

surgical

prob-

P, Encabo

B,

Humbert P. CT findings in circumscribed upper abdominal idiopathic retropenitoneal fibrosis. J Comput Assist Tomogr 1986; 10:

27:111-113.

Birnberg F, Vinstein A, Gorlick G, Lee F, Hales M. Retropenitoneal fibrosis in children. Radiology

3.

D.

1984;

R, BrockJ,

8.

1063-1064. Stovall T, Ling F. Retropenitoneal presenting as an abdominopelvic Gynecol 1988; 71:482-483. Mulligan 5, Holley H, Koehler

and MR imaging itoneal fibrosis. 1989;

fibrosis mass. Obstet R, et al.

in the evaluation J Comput Assist

CT

of retroperTomogr

13:277-281.

133:239-243.

RadioGrapbics

U

Brooks

et al

Volume

10

Number

6