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Recurrent Caffey’s Cortical Hyperostosis and Persistent

Deformity

Eugene Blank, M.D.

From the Department of Diagnostic Radiology, University of Oregon Medical School, Portland

ABSTRACT. A boy, age 12 years, who had infantile cortical hyperostosis has continued to have occasional aches and new

cortical thickenings in his arms and legs. His mandible is

undergrown and his ribs have an abnormal slope.

Recurrence

of Caffey’s

cortical

hyperostosis and persistent

deformity have been observed in

other

children

and

young

adults.

Some unexplained cases of late cortical thickening

and pain may be due to Caffey’s disease. Pediatrics, 55:856,

1975, cmy’s INFANTILE CORTICAL HYPEROSTO5IS, LATE

RECURRENCES OF CAFFEY’S DISEASE, PERSISTANT DEFORMITY IN

CAFFEY’S DISEASE.

Late

recurrences

of

Caffey’s

disease

are

not

common.

Once

the

swelling

of the

bones

and

the

surrounding

soft

tissues

of the

infant

has

ceased,

remodeling

usually

occurs.

The

bones

are

left

with

a normal

appearance

and

grow

at a normal

rate.

Occasionally,

cortical

thickening

may

recur

and

deformity

may

persist

during

childhood

and

beyond.

CASE REPORT

The patient, a boy, weighed 3.9 kg and was the result of a

normal pregnancy. His mother, age 30, father, age 38, and two siblings were well.

During the third month of life, he became irritable, and an arm swelled. When he was 4 months of age,

roentgeno-graphic examination showed cortical hyperostosis of the mandible, clavicles, ribs, and bones of the arms (Fig. 1). He

was treated with aspirin. Although at 1 year of age he no

longer seemed ill, he weighed 5 kg. He

sat

at 14 months and walked alone at 2years. He fed himself at 1year. He used the

toilet consistently at 3 years. He had occasional respiratory infections.

At the age of 3 years, he still chose soft foods because of difficulty chewing. His mandible was small. His weight was

11.4 kg

height,

0.86 meter; and blood pressure, 100/60 mm Hg. He had

impetigo.

Although his teeth were normal, he

had malocclusion.

At age 4#{189}years, he began to have pain

near

the left knee

without

fever

and without

other

signs of infection. White

blood

cell

and

differential counts were normal. Erythrocyte sedimentation rate was 18 mm/hr. The cortex of the left

femur was thickened (Fig. 2, A). Six months later, when he

was 5 years

of age, his

weight, 14.1 kg, and height, 1 meter,

were below the third percentile, and both approximated the

median for a boy 3 years of age. Head circumference, 48.2 cm, was at the median for an infant 16 months of age.

At

about

6 years of age, he was awakened during several

nights

by pain in the right arm. The arm was not swollen; its motion was free. Nine months later pain in the right arm

forced him to feed himself with the left hand for several weeks. The right upper arm was tender and swollen; the overlying skin was normal. Strength was normal. Roentgeno-graphic examination showed cortical

thickening

of the

right humerus (Fig. 2, B). At age 8 years he had pain in the arms

and

legs

for a short time. He did not have fever. The

erythrocyte sedimentation rate was normal. Two months

later the

right upper arm hurt

for four

days; in

another

two months the left upper arm hurt for three days. Again, at 10 years

of age, he had

pain in the left arm. Sedimentation rate was normal. Roentgenograms did not show cortical

thicken-ing.

At age 11#{189}years he weighed 30.1 kg. the 16th percentile,

and

was

1.4 meters tall, the 10th percentile. His small mandible caused malocclusion. Blood pressure was normal,

and

the remainder of the physical findings were normal. Two

months later, pain in the left upper arm caused him to cry out

at

night.

The

arm

was tender. White blood cell

and

differential

counts

were normal. Sedimentation rate was 29 mm/hr; serum calcium, 10.4 mg/100 ml; phosphorus, 5.8

(Received June 28; revision accepted for publication August 5, 1974.)

ADDRESS FOR REPRINTS: Department of Diagnostic

Radiology, University of Oregon Medical School, Portland,

(2)

I

Fig. 1. After he had been irritable for about one month, the patient at 4 months had, in this examination, thickening of the mandible and clavicles (A), most of the ribs (B), and radiuses and

ulnas (C). The right tibia and fibula showed similar changes.

mg/100 ml; and alkaline phosphatase, 67.8 international units. The cortex of the left humerus was thickened (Fig. 2, C). Two weeks later the left arm still hurt. He could move it through a full range, but motion intensified the ache. Hematocrit reading was 39%; white blood cell count, 7,300/ cu mm; differential count, normal; and sedimentation rate, 10 mm/hr. Platelets were 380,000/cu mm. Serum electro-phoresis showed the single abnormality of slight elevation of

the a2-globulin to 0.90 gm/100 ml (normal, 0.36 to 0.78 gm/

(3)

Fig. 2. A, At age 4#{189}years the patient had pain near his left knee; the cortex of the mid-part of the left femur is thickened medially. B, Roentgenographic examination of the right arm when the patient was 6 years of age, after he had had pain there for several weeks, shows thickening of the cortex of the humerus. C, When the patient was 1 1#{189}years of age, his left arm hurt for several weeks. Erythrocyte sedimentation rate was 29 mm/hr. A thin strip of new bone is being

deposited along the medial part of the left humerus.

of the cortex of the right tibia. Six months earlier, roentgeno-grams of the legs had shown normal cortexes.

DISCUSSION

Caffey and Silverman’ in their first report of infantile cortical hyperostosis recognized

persist-ent

deformity.

A boy

at 3 weeks

of age

developed

swelling of the left side of the mandible and inability to suck from a bottle; almost five years later the left side of the face was larger than the

right. “In the roentgen examination the left side

of the

mandible

was

larger

and

more

opaqu,e

than

the right side and the left zygomatic bone was

enlarged and sclerotic.” Another of Caffey’s

patients, a girl, had dislocation of the radiuses at

the

elbows

at

14 months

of age.

Her

right

leg

was

longer

by

2 cm

than

the

left,

and

the

right

foot

was larger. Thirteen months later bony bridges

had

formed

between

the

radiuses

and

ulnas

and

(4)

Fig. 3. At age 11#{189}years the patient has a small mandible.

c7

seventh and eighth ribs.2 Scott3 observed Caffey’s

disease in a boy who, at age 4#{189}years, had a bony

bridge

between

the

right

radius

and

ulna

with

anterior

dislocation

of the

radius

at the

elbow

and

limitation of pronation and supination of the

forearm.

Tampas

and

associates4

reported

a family

in

which

four

children,

their

father,

and

five

of their

cousins had Caffey’s disease; the father, 25 years

old,

had

bowed

long

bones.

Idbohrn

and

asso-ciates,5 cited by Clemett and Williams,6 described

Caffey’s

disease

in first

cousins,

one

of whom,

19

years

old,

had

slight

residual

thickening

of a tibia

and

a radius.

Clemett

and

Williams6

described

a

brother

and

a sister

who

had

infantile

cortical

hyperostosis. The boy, in whom thickening of a

tibia and the mandible were recognized in utero,

had

bowed

and

widened

tibias

at

4#{189}

years

of

age.

(5)

Pajewski and Vure7 observed a boy with cortical hyperostosis. His 31-year-old father had had pains and swellings in his limbs from infancy

until

he was 19 years old; the tibias and femurs

then

were

expanded

with

thin

cortexes

and

inner

ghosts of partially resorbed cortexes. The infant’s

aunt

had

had

recurrences

of

limb

pain

and

swelling in

infancy

and

childhood,

and

at age

15

years she had similar deformities.

Swerdloff and associates8 observed two

chil-dren

who

had

recurrent

Caffey’s

disease.

A girl

at

about

4 years

of age

had

cortical

thickening

of her

tibias; one year later she had pain and cortical thickening in her left arm and leg. A boy at age 12

years

had

as the

first

recurrence

pain

in the

ribs,

left

arm,

and

left

leg,

and

cortical

thickening

of

the

left

tibia

and

left

fibula.

An

Iraqi

youth9

continued to have recurrences throughout adoles-cence; when he was 19 years old, he again had

swelling and cortical thickening in an arm and a

leg.

Staheli

and

associates’#{176} examined

eight

chil-dren,

6 to

13

years

after

the

onset

of

Caffey’s

disease.

The

mandibles

of

two

children

were

asymmetrical; one of these children also had bony bridges between the right 1 ith and 12th ribs and

between

the

left

tibia

and

left

fibula.

Burbank

and

ass’

found

residual

asymmetry

in

the

mandibles of eight of 1 1 children who had had

Caffey’s disease; two of the eight had also severe

malocclusion.

Weis

and

Thompson’2

concluded

from a study of ten children who had had Caffey’s

disease

that

residual

mandibular

deformity

was

no

more than would be found in any group.

Some

unexplained

episodes

of pain

and

cortical

thickening in older children may represent

recur-rences

in patients

in whom

the

infantile

phase

of

Caffey’s

disease

has

not

been

severe

enough

to

call

attention

to

the

disease.

Keipert

and

Camp-bell’3

observed

a boy

of 14 years

of age

who

began

to have recurrences of pain and cortical

thicken-ings,

along

with

elevation

of

the

erythrocyte

sedimentation rate and serum amounts of a2,

fl,

and

7-globulin.

Goldbloom

and

associates’4

reported

the

cases

of two

children

who

had

pain,

fever,

and

cortical

thickening

after

respiratory

illness,

which

in one

of them

was

streptococcal.

The

a2-globulins

and

‘y-globulins

in

the

serum

were

elevated;

the

albumin

was

depressed.

The child or adolescent who has illness and

physical

findings

compatible

with

Caffey’s

corti-cal

hyperostosis,

even

though

age

and

remote

history make the diagnosis unlikely, may, in reality, be suffering from late recurrence of

Caffey’s disease.

REFERENCES

1. Caffey,

J.,

and Silverman, W. A.: Infantile cortical hy-perostosis: Preliminary report on a new syndrome. Am.

J.

Reentgenol. Radium Ther. NucI. Med., 54:1,

1945.

2. Caffey,

J.:

On some late skeletal changes in chronic infantile cortical hyperostosis. Radiology, 59:651, 1952.

3. Scott, E. P.: Infantile cortical hyperostosis: Report of an unusual complication. J. Pediatr., 62:782, 1963.

4.

Tampas,

J.

P., Van

Buskirk,

F.

W., Peterson, 0. 5., and

Soule, A. B.: Infantile cortical hyperestosis. JAMA, 175:491, 1961.

5. Idbohm, H., Lindg#{227}rd, B., and Lindquist, B.: Caffey’s syndrom: Infantil cortical hyperostos: RedogOrelse fOr tv#{226}fall fran samma sl#{228}ktSven. Tandlak. Tidskr., 53:3482, 1956.

6. Clemett, A. R., and Williams,

J.

H.: The familial occurrence of infantile cortical hyperostosis. Radi-ology, 80:409, 1963.

7. Pajewski, M., and Vure, E.: Late manifestations of infantile cortical hyperostosis (Caffey’s disease). Br.

J.

Radiol., 40:90, 1967.

8. Swerdloff, B. A., Ozonoff, M. B., and Gyepes, M. T.:

Late recurrence of infantile cortical hyperostosis

(Caffey’s Disease). Am.

J.

Roentgenol. Radium Ther. NucI. Med., 108:461, 1970.

9. Taj-Eldin, S., and al-Jawad,

J.:

Cortical hyperostosis: Infantile and juvenile manifestations in a boy. Arch.

Dis. Child., 46:565, 1971.

10. Staheli, L. T., Church, C. C., and Ward, B. H.: Infantile

cortical hyperostosis (Caffey’s disease): Sixteen

cases with a late follow-up of eight. JAMA, 203:364,

1968.

1 1. Burbank, P. M., Lovestedt, S. A., and Kennedy, B. L.

J.:

The dental aspects of infantile cortical hyperostosis. Oral Surg., 11:1126, 1958.

12. Weis, R. W., and Thompson, M. L.: Infantile cortical

hyperostosis: A study to determine if residual de-formities exist in mandibles.

J.

Dent. Child., 36:441, 1969.

13. Keipert,

J.

A., and Campbell, P. E.: Recurrent hyperos-tosis of the clavicles: An undiagnosed syndrome. Aust. Paediatr.

J.,

6:97, 1970.

(6)

1975;55;856

Pediatrics

Eugene Blank

Recurrent Caffey's Cortical Hyperostosis and Persistent Deformity

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(7)

1975;55;856

Pediatrics

Eugene Blank

Recurrent Caffey's Cortical Hyperostosis and Persistent Deformity

http://pediatrics.aappublications.org/content/55/6/856

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