THE

SKELETAL

MANIFESTATIONS

OF

CONGENITAL

SYPHILIS
A

REVIEW

M.

N. RASOOL,

From

In a retrospective

review

of

OF

302 clinically

infants;

have

12 of

these

were

found

to

without
residual
should consider

had

pregnancy,

though

clinical

symptoms

may

Congenital
of age) and

pathological

not appear

for

syphilis
is divided
late. The disease
is

seldom
seen in North
America
and in some
hospitals
routine
serological
tests have
been discontinued
(Wilkinson
and Heller
1971 ; Rosenfeld,
Weinert
and Kahn
1983; Toohey
1985).
In the United
Kingdom
about
10
cases are seen each year (Ewing
et al 1985).
Primary
skeletal
involvement
is rare (Levin
1970;
Toohey
1985). It is a bilaterally
symmetrical
polyostotic
condition
which
affects
mainly
tubular
bones,
but any
bone may be affected
(McLean
1931). Diagnosis
may be
difficult
when
the classical
symmetrical
osteoperiostitis

PATIENTS

presentation
is absent.

AND

of

1989

be sent

to Mr

British
Editorial
Society
ofBone
030l-620X/89/5
I 75 $2.00
J Bone Joint Surg [Br] 1989 :7 I-B :752-5.

752

fractures

syphilis,

and

59 cases

retrospectively;

bone

changes

changes
sign in 34

four

Complete

had

dactylitis.

were recalled
for review.
The
destructive
lesions
of bone are

197 cases

with

radiologically

infants.

There

was

cases

seen

with

1984,

49 in 1985,

a gradual

skeletal

increase

lesions

53 in 1986

in the

number

of Natal,

cases
were
recalled
and
radiographs
taken
to assess
healing
of the lesions.
Clinical
presentation.
Anaemia,
hepatosplenomegaly,
skin lesions
and rhinitis
were the predominant
clinical
features
(Table
I). Gastro-enteritis,
bronchopneumonia,
septicaemia
and meningitis
were found
in 75 cases;
34
cases with pseudoparalysis
were referred
for orthopaedic
assessment
(Table
II), withjoint
swelling
as the common

I. Clinical

presentation

Hepatosplenomegaly
Anaemia
Desquamating
skin
Fever
Erythematous
rash
Oedema
of feet
Rhinitis
(snuffles)
Bronchopneumonia
Pseudoparalysis
Jaundice
Gastro-enteritis
Septicaemia
Conjunctivitis

lesions

on hands

and

soles

48
43
34
26
16
10
8
7
6
4
4
2

Meningitis
Epistaxis
Lymphadenopathy
Craniotabes

THE

157
I 25
81
61
58

53

Purpura

Surgery

of

over the 4 years

(38 in
and 57 in 1987). Some
59

bilaterally

1987 302 infants

syphilis
were seen.
were
reviewed

Joint

established

skeletal
lesions
were found and analysed.
There
were 102
boys and 95 girls. The ages ranged
from 0 to 4 months
with
an average
of 2.5 months;
19 were
premature

Number
of cases

M. N. Rasool.
and

were

(20), and metaphyseal

was a presenting

that
when

syphilis

Table

M. N. Rasool,
FCS (Ortho)
(SA), Consultant
S. Govender,
FRCS,
Senior
Surgeon
and Senior
Lecturer
Department
ofOrthopaedics,
Faculty
of Medicine,
University
P.O. Box I 7039, Congella
401 3, Republic
of South Africa.
should

of congenital

METHODS

Between
January
1984 and December
with clinically
suspected
congenital
The
clinical
records
and
radiographs

Correspondence

cases

(102 cases),
osteitis
in 61. Pseudoparalysis

changes
was seen in all
the diagnosis
of congenital

Unfortunately,
congenital
syphilis
is still a problem
in
some countries.
The disease
is an intra-uterine
infection
which
usually
manifests
shortly
after birth.
Spirochaetes
cross
the placental
barrier
after
the fourth
month
of
several
weeks
after birth.
into early (before
2 years

of Natal

suspected

were periostitis
lesion were found

CASES

S. GOVENDER

the University

found in 197. The skeletal

manifestations
(71). Combinations
of more than one
radiological
healing
orthopaedic
surgeon
seen in an infant.

197

JOURNAL

OF BONE

AND

JOINT

SURGERY

THE

SKELETAL

MANIFESTATIONSOF

CONGENITAL

SYPHILIS

753

mode
of presentation.
Four
children
presented
dactylitis
with
swelling
of the hands,
six mimicked
brachial
plexus
type of lesion
and 12 had a pathological
fracture
the

of a long

tibia

with

bone.

One

a fluctuant

child

presented

swelling

that

as
a

as osteitis
was

incised

of
and

drained.
Serological
tests. The Wasserman
reaction
was positive
in 165 mothers;
1 1 were negative
and 21 results
were not
recovered.
Of the babies,
1 1 3 had a positive
fluorescent
treponemal
antibody
(absorbed)
Immunoglobulin
M
Immunoglobulin
G test (FTA-ABS
1gM
IgG).
In 72
cases IgG was positive
and 1gM negative.
In 12 cases the
results
were not recovered.
Fig.
Radiograph
metaphysitis,

of left
osteitis

upper
limb
and dactylitis.

Radiological
findings.
were diaphysitis
and

1
with

diffuse

periosteal

reaction,

Joint
ical

Table II. Modes

pseudoparalysis

of presentation

cases had
periostitis.

of

were
Number
of cases

Joint

swelling

Hip
Knee
Shoulder
Elbow

2
2
4
2

Wrist

Swelling

ofthe

hands

Brachial

plexus

type

changes
fractures

found

Osteitis

infants,

dactylitis.

3
12

of Pehu
commonly

reaction

serrations,
(Wegners
callus

(McLean
presented

71-B.

No. 5. NOVEMBER

1989

of the

osteitis
unusual

seen

and
and

in 106 cases

Metaphyseal

lesions

were

frequently

either

1).
the

productive

(Wimbergers
sign, Fig. 2) were seen in 21
changes
at the corners
between
the shaft and
plate occurred
in six cases.
Metaphyseal
the sawtooth
appearance
sign) was seen in four

formation

in three

(Fig.

of the metaphysis
cases,
and exuberant

3).
of two types,
impacted
the fractures
occurred
the
metaphyses.
The

Radiographs
showing
symmetrical
(Wimbergers
sign) in the upper
tibiae
changes
in the distal
femora.

patholog30%

193 1 ; Levin
1 970) (Fig.
as a diffuse
moth-eaten

Pathologicalfractures.
These
were
(infractions)
and displaced.
Often
through
destructive
lesions
in

VOL.

was

of the shaft
(15 cases).
Less
a localised
defect
(5 cases).

medial
tibia
cases.
Focal
the epiphyseal

of tibia

Fig.

12 had
Some

or destructive
and appeared
as lucent
or dense
bands.
Alternating
bands
produced
a sandwich
appearance
on
radiographs
(Caffey
1967).
Erosions
of the metaphysis
were
common
and symmetrical
erosions
in the upper

palsy

Fractures

had

four

in 15 cases.
A periosteal

Metaphysitis.
Wrist drop
Klumpkes
Erbs palsy

in eight

and

and 20 had osteitis.

The periosteal
reaction
was mild (a
single
layer)
in the majority
(72) or severe,
a lamellar
form also known
as cloaking,
coffin
formation,
or the

rarefaction
lesion
was

lesions

occurred

combined
lesions
of metaphysitis,
Asymmetrical
lesions
were not

Diaphysitis.

periostitis
Osteitis

The predominant
skeletal
lesions
metaphyseal
changes
(Table
III).

erosions
and focal

Fig.
Exuberant

callus

formation

3
ofdistal

femora.

754

M. N. RASOOL,

terminal
impacted

fragment
into the

epiphyseal
metaphyseal

with the attached

shaft,
or displaced.

displacements
fragments

(femur,
(upper

(distal
femur
1 , distal radius
Fractures
through
a defect
infants.
Joint involvement.
of which
four

2 and
in the

epiphysis
There

involved
Erosion

the shoulder,
of the articular

one the
surface

the ulna
articular

was seen
in four patients;
surface
involved
in the whole

Dactylitis.

Three
cases were
Two of the hands

seen
had

tibia
1 ) (Fig. 4).
occurred
in four

upper
shaft

radiographs
effusion

feet.

might
be
were two

radius),
two displaced
tibia),
four infractions

Eight
showed

the

showedjoint
and
subluxation;

changes
two

knee
and one
of the sigmoid

the hip.
notch
of

this was the only

series (Fig. 5).

in the hands
and one
unilateral
involvement

(Fig. 6).
Treatment.
All the infants
were kept in hospital
14 days and received
procaine
penicillin
10 000
day for 10 days.
immobilised.
One

Fractures
child
who

fluctuant

of the

abscess

in

for 10 to
units/kg!

and
involved
joints
were
pfesented
with a localised

distal

S. GOVENDER

tibia

had

drained
; a bony
defect
with granulation
was found.
The pus was sterile
on culture.

it incised

and

tissue

pus

and

Fig.
Radiograph
showing
fracture
ofleft
upper

infraction
tibia.

of right

distal

femur

and

RESULTS
Of the
discharged

197

infants
172 improved
after
treatment.
The

concurrent
gastro-enteritis,

infections
meningitis

clinically
remaining

and were
25 died of

including
bronchopneumonia,
and septicaemia.
Ten of these

25 had post-mortem
examinations
and the diagnosis
of
congenital
syphilis
was confirmed
histologically.
The fractures
showed
evidence
of healing
at two to
three
weeks.
All 59 children
who could
be traced
were
recalled
; they
Radiographs
showed
There
fractures

were
were

aged between
taken
of all

completely
normal
bone
was
no residual
evidence
(Fig. 7).

10 months
and
the long bones
architecture
of the

4 years.
and all

(Fig.
pathological

6).

Fig.
Erosion

of sigmoid

notch

of ulna.

DISCUSSION
In spite of antenatal
care and screening
for syphilis,
the
incidence
of congenital
syphilis
is high in our environment.
It presents
at two or three
months
of age and is
usually
seen
orthopaedic
paralysis.
Natal.
We

The
find

by paediatricians,
surgeons,
especially
disease
that

the

has
most

a high
helpful

though
if

occasionally
by
there
is pseudo-

mortality
serological

fluorescent
treponemal
antibody
(absorbed)
though
the value ofserology
is controversial

rate

(12%)

in

test

is the

1gM
(Cremin

IgG,
and

Fisher
1970; Ewing
et al 1985; Toohey
1985). Negative
serology
does not exclude
congenital
syphilis
since
the
fetus
infected
late in gestation
may
develop
positive
serology
at variable
times
after birth.
Nor does positive
serology
alone
(Immunoglobulin
G) establish
the diag-

Dactylitis
the same

of left
changes

foot showing
complete
were seen in the right

THE JOURNAL

healing

at two-year

follow-up;

foot.

OF BONE

AND

JOINT

SURGERY

THE
Table

III.

Radiological

SKELETAL

MANIFESTATIONS

Diaphysitis

126

(lamellar)

20

Osteitis
Localised
Diffuse

defect

15

Metaphysitis

71

Dense bands
Lucent
bands
Alternating
bands
Metaphyseal
serrations
Exuberant
callus formation
Wimbergers
sign
Focal changes
Combined
Asymmetrical

Joint

The

articular

ment
The
have

ofa nearby
metaphysis
may lead tojoint
only articular
surface
reported
in the
erosive
changes
is the sigmoid
notch

cortex

is usually

and cancellous

tissue.
Occasionally
fracture.
Dactylitis
radiographically.
syphilis
follow

spared,

though

involve-

distension.
literature
to
of the ulna

bone

by syphilitic

granulation

the diaphysis
of a long bone
may
is rare and may mimic
tuberculosis
The remarkable
feature
of congenital

is that
treatment

complete
(Caffey

Fiumara
1975; Toohey
Radiographically

involvement

cartilage

was also seen

et a! (1983).

ofthe

12

fractures

acute
pyogenic
osteitis
was reported
by Rosenfeld

21

Pathological

(McLean

1970) and we observed

four such cases.
Pathological
fractures
are not unusual
and commonly
occur
through
the metaphysis,
with
subsequent
impaction
or displacement.
They
are due to destruction

15

Dactylitis

tissue

(Levin

61

lesions

granulation

10
15
12
4
3
6

lesions

by syphilitic

defect
resembling
and a similar
case

106
72
34

reaction

755

1931 ; Caffey
1967).
The
Wimberger
sign is the most
common
erosive
lesion.
A localised
fluctuant
area
of
granulation
tissue in the shaft,
with an underlying
osseous

of cases

Mild
Severe

SYPHILIS

of destruction

lesions
Number

Psriosttal

OF CONGENITAL

healing
and normal
growth
1967; Cremin
and Fisher
1970;
1985) (Fig. 7).
the disease
may mimic

many

other

conditions
such
as multifocal
osteitis,
rickets,
scurvy,
neuroblastoma
and the battered
baby syndrome,
so that
a high index of suspicion
is needed
and the orthopaedic
surgeon

should

syphilis

whenever

No benefits
commercial
article.

consider

in any
party

the

the films

possibility

show

form have been

related
directly

of

destructive

congenital

bone

lesions.

received
or will be received
or indirectly
to the subject

from a
of this

REFERENCES
Fig.
Radiograph
femur
with

showing
complete

epiphyseal
resolution

CaffeyJ.Pediatricx-raydiagnosis.

Publishers,

displacement
at three-year

of right
follow-up.

distal

Cremin
Ewing

nosis,

as

there

may

be

passive

transfer

of

maternal

antibodies.
Although
ance
is that
involvement,

the characteristic
radiographic
of bilaterally
symmetrical
diffuse
we have noted several
asymmetrical

appearskeletal
lesions.

In our series
periosteal
reaction
was the most common
radiological
lesion,
but a variety
of metaphyseal
lesions
have been described
(McLean
1931 ; Caffey
1967; Cremin
and Fisher
1970; Levin
1970; Solomon
and Rosen
1975).
Dense
metaphyseal
bands
are a result of accentuation
of
calcified

VOL.

cartilage

matrix,

71-B, No. 5, NOVEMBER

and

I989

lucent

bands

are

a result

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1970;43

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in

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osseous

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