CONGENITAL
PSEUDARTHROSIS
AND
BOWING
B. J. DOOLEY and M. B. MENELAUS,
D. C. PATERSON, ADELAIDE,
with
The purpose
of this paper
bowing
or pseudarthrosis
is to describe
of the fibula.
OF
MELBOURNE,
AUSTRALIA
FIBULA
THE
and
experience
with four children
who presented
These
children
demonstrate
a gradation
in the
severity
and
pseudarthrosis
significance
of this condition.
There
may be : 1) fibular
bowing
without
fibular
(Case
1); 2) fibular
pseudarthrosis
without
ankle
deformity
(Case
2); 3) fibular
pseudarthrosis
with
ankle deformity
but without
the late development
of tibia! pseudarthrosis
(Case
3) ; or 4) fibular
pseudarthrosis
with the late development
of pseudarthrosis
of the tibia
(Case
4).
The aetiology
and
those
of antero-lateral
bones
developed
Thus
area
bowing
of bowing
and
by endochondral
and
pseudarthrosis
pseudarthrosis
of the
ossification-the
first
of the
tibia.
Similar
rib,
an
often
area
bowed
Pseudarthrosis
of mesoderma!
maldeve!opment
and
thickened,
the cortex
or bowing
may
involve
abnormalities
in one or both tibiae.
Langenski#{246}ld (1967)
described
in which
with
the fibula
three
the
a narrowed
alone,
cases
but
or
more
of progressive
with persisting
pseudarthrosis
arthrosis
of the tibia.
Carrel!
of the
(1938),
fibula
Boyd
similar
cases.
Boyd
a normal
tibia.
The
described
leg was
a case of congenital
affected
by pseudarthrosis
and Sage
opposite
following
(1941)
and
CASE
through
femur,
are
similar
affect
radius
and
a congenital
Fracture
may
cyst
also
diaphysis
is tapered,
obliterated
medullary
it is associated
commonly
valgus
successful
Boyd and
fibula
conditions
clavicle,
pseudarthrosis
in the fibula
may occur
after
fracture
of fibrous
dysplasia,
or occasionally
in neurofibromatosis.
through
and
pathology
deformity
bone
Sage
pseudarthrosis
of both
to
other
ulna.
or an
occur
sclerotic
of the
cavity.
with
ankle
grafting
for a pseud(1958)
also mentioned
of the
bones.
fibula
with
REPORTS
girl was first seen in July 1969 at the age of 11 months,
because
of bowing
of the lower
the left leg with prominence
of the fibula just above the anide.
There was no history
of injury.
Radiographs
showed
lateral
bowing
and sclerosis
of the lower shaft of the fibula,
without
obvious
abnormality
in the tibia (Fig. 1). The bowing
diminished
slightly in the ensuing
three
years
(Fig.
2).
At review late in 1972 it was noted that she had developed
soft swellings
on the forehead
and right
side of the back, and also some pigmented
hairy
areas
on the back.
Case
half
1-A
of
Case 2-A
that there
the initial
It had been noted
at birth
at the age of one year.
At
the convexity
of the bowing
being
antero-lateral
at the junction
of the middle
and lower
thirds
of the tibia and fibula.
There
was also
medial torsion
of the left tibia of approximately
30 degrees,
compared
with approximately
10 degrees
of medial
torsion
on the right side.
There were no stigmata
of neurofibromatosis.
Radiographs
showed
a pseudarthrosis
of the left fibula (Fig. 3). There was a mesodermal
defect
of mild degree
at
the mid-section
of the tibia, with increased
density
of the cortex,
narrowing
of the medullary
cavity
and antero-lateral
bowing.
It was feared
that the abnormality
of the tibia might precede
pseudarthrosis
of that bone.
For
this reason a half ring-top
caliper was fitted and worn for a year.
Since then the leg has gradually
straightened
spontaneously.
At the most
recent
review
the child was aged eight years ; she was free
from symptoms
and leading
an active and normal
life. Radiographs
at that time showed
a persisting
pseudarthrosis
of the fibula with improved
appearances
of the tibia (Fig. 4).
girl was
was mild
brought
bowing
examination
the
for
advice
at the
age
of 15 months
of the left leg. The child had begun
left leg was bowed
below the knee,
in 1966.
to walk
Case 3-A
boy aged twelve years attended
in April 1971 with severe valgus of the left ankle.
The
deformity
had been noticed
by the parents
for six months.
There
were no symptoms,
and there was
no history of any injury.
Radiographs
showed a valgus ankle with a pseudarthrosis
of the lower fibula
(Fig. 5). The remainder
of that bone was tapered
and sclerotic
and the tibia showed
slight thickening
ofthe medial cortex.
Presumably
this thickening
was due to increased
medial
stress
in the weight-beating
bone associated
with the valgus deformity.
Supramalleolar
closed-wedge
osteotomy
was performed
in
October
1971 . Two screws were placed across the fibula into the tibia, one above the osteotomy
site and
one below. The tibial bone removed
in the osteotomy
was used as a graft to join the lower fibula to the
VOL.
56 B,
NO.
4,
NOVEMBER
1974
739
740
B. J. DOOLEY,
M.
FIG.
B. MENELAUS
AND
D.
C. PATERSON
1
FIG.
2
Case I . Figure 1-Antero-posterior
radiographs
of both legs at presentation
at the age of 11 months
in July 1969. Figure 2-Three
years and one month
later
the bowing
FIGs.
Case
2.
Figure
3 AND
of the fibula
has lessened
; the
tibia
remains
normal.
4
3-Antero-posterior
and
lateral
radiographs
at the age of 15
months.
Figure
4-Antero-posterior
radiographs
of both legs at the age of 7
years.
Both leg bones are less bowedthe pseudarthrosis
persists.
THE
JOURNAL
OF
BONE
AND
JOINT
SURGERY
CONGENITAL
Fio.
VOL.
8-Antero-posterior
is difficult
to discern
9-The
56 B,
fracture
NO.
4,
BOWING
OF THE FIBULA
Fio.
at
741
9
radiographs
of both legs at presentation
in June 1960. The fracture
of the
this stage;
however,
the abnormality
of both tibia and fibula is obvious.
is obvious
NOVEMBER
AND
8
Case 4. Figure
fibula
Figure
PSEUDARTHROSIS
1974
in radiographs
five months
callus formation.
after
presentation
and there
is no evidence
of
742
B. J. DOOLEY,
M.
B. MENELAUS
AND
D.
C. PATERSON
tibia. After three months
(Fig. 6) there was solid union
of
the osteotomy
in good position.
The screws were removed
in December
1972 (Fig. 7). Histological
section
of the
pseudarthrosis
showed
fibrous tissue only.
Case
4-A
boy
aged
thirteen
months
was
brought
in
June 1960 with a history
of having caught
his left leg in
the wheel of a bicycle.
As a result, there was a fracture
of the lower shaft of the fibula through
abnormal
bone
and an abnormal
tibia with narrowing
and dense cortex
and
lateral
bowing
in the fibula
December
biopsy
9).
1960,
showed
appearance
an
(Fig.
(Fig.
fragments
A pseudarthrosis
months
the
pseudarthrosis
fibrous
tissue
developed
after
the fracture,
was
explored
in
and
only.
nailing
combined
with
an
inlay
graft, after excision
of the pseudarthrosis
leg was kept in plaster
for four months.
pseudarthrosis
a
Figure
10 shows the
after
biopsy.
In August
1962
to secure
union
of the fibular
five months
was made
by medullary
attempt
iliac bone
11).
The
8).
Five
(Fig.
The
persisted.
In January
1966 the left leg was l#{149}25
centimetres
shorter
than the right.
The ankle showed
Valgus deformity. Radiographs
taken soon after this showed a partial
fracture
of
the lower third of the left tibia, which had
remained
dense
and rather
bowed
while
the fibula
remained
ununited.
The leg was immobilised
in a caliper.
By August
1966 the boy was complaining
of pain in his
left leg. Radiographs
in October
of that year showed
a
definite
FIG.
10
FIG.
4. Figure
10-The
ance oftheleg
in March
radiological
appear1961 , fourmonthsafter
Case
biopsy
ance
of the
fibula.
Figure
11-The
in plaster
after
internal
fixation
iliac
bone
11
graft
in
August
appear-
fracture
of
the
In October
1966
from
with
the right
insertion
tibia
of a block
two
main
tibia
(Fig.
a double
was applied
fragments.
12).
onlay
cortical
bone
graft
by the Boyd
technique,
of cancellous
bone between
the
The
operation
was
successful
in
securing
union.
The boy has continued
to wear the caliper;
at the age of thirteen
he remains
well (Fig. 13).
and inlay
1962.
DISCUSSION
Congenital
tO those
pseudarthrosis
of the
Severe
tibia,
but
bowing
abnormality
of
in the
and
not
bowing
so serious
a congenitally
tibia
and
bowing
of the
of the
fibula
leg may
should
abnormal
pseudarthrosis
of the
because
the fibula
with the tibia.
improve
not
be
tibia
is not
and
treated
(Case
are similar
it is not
fibula
in aetiology
a weight-bearing
may
occur
and
of the
deformity
deformity
by
of the
grafting
4; see also
a weight-bearing
fibula
and
of synostosis
Progressive
valgus
ankle
Sage
of the
of
the
fibula
to the
pseudarthrosis
deformity
is thus
ankle
because
bone,
may
failure
Langenski#{246}ld’s
tibia
is advised
and
grafting
prevented.
deformity
is severe enough
after maturity,
supramalleolar
persist
alone
(Case
2).
disability
do not
its integrity
not
pathology
without
obvious
(1967)
(Langenskiold
of
Osteotomy
the
Pseudarthrosis
likely
to
occur
as
experience).
Furthermore,
so necessary
as is the
is not
1967).
The
If the patient
tibia is all that
case
operation
involves
tibia.
if the
presents
with a valgus
is required
(Boyd
and
1958).
THE
JOURNAL
in
valgus
deformity
of the
by splinting,
the operation
distal
fibular
metaphysis
to the
of the tibia is necessary,
in addition,
to warrant
correction.
osteotomy
of the
1).
the
Unlike
congenital
necessarily
ensue.
develop.
is as
:
Pseudarthrosis
of the lower
fibula
may lead to a progressive
nUe (Case 3). Ifthe child is young and the deformity
uncontrolled
excision
and
bone.
the development
ofvalgus
deformity
at the ankle (Case
condition
is analagous
to bowing
of the tibia without
without
Treatment
is not necessary.
The
development
of pseudarthrosis.
A related
condition
is pseudarthrosis
pseudarthrosis
of the tibia,
progressive
The
of the fibula
because
OF
BONE
AND
JOINT
SURGERY
743
t.J.
Case 4. Figure
Figure
1 3-The
Should
(Case
tibia.
pseudarthrosis
4), there
Protective
12
HL.
ii
12-In
October
1966 there is a clearly defined fracture
ofthe tibia.
present
radiological
appearance.
There is sound union of the
tibia and persistent
pseudarthrosis
of the fibula.
of the
is a high
splinting
likelihood
should
fibula
be associated
with
of the subsequent
be worn
until skeletal
obvious
abnormality
development
maturity.
of the
of pseudarthrosis
tibia
of the
SUMMARY
1.
The
cases
only, are
2. There
fibular
of
four
children
described.
is a gradation
bowing
who
in the
without
fibular
fibular
pseudarthrosis
or fibular
pseudarthrosis
3. Proper
management
with
presented
severity
with
and
pseudarthrosis
bowing
significance
; fibular
or
of
pseudarthrosis
this
condition.
pseudarthrosis
without
of
There
ankle
fibula
the
may
be
deformity;
deformity
but without
the late development
f tibia! pseudarthrosis;
with the late development
of tibia! pseudarthrosis.
is dependent
on a knowledge
of this range
of conditions.
REFERENCES
BOYD,
H.
B.
BOYD,
H.
B.,
40-A,
23,
497-515.
and
SAGE,
W.
children
VOL.
56B,
Treatment
by dual
bone
grafts.
Journal
of
Congenital
pseudarthrosis
of the
tibia.
Journal
ofBone
andJoint
(1958):
Bone
and
Joint
Surgery,
B.
Transplantation
(1938):
of the
fibula
of
the fibula
in the
same
leg.
Journal
of Bone
and Joint
Surgery,
627-634.
LANGENSKI#{246}LD,
49-A,
F.P.
pseudarthrosis.
1245-1270.
CARRELL,
20,
Congenital
(1941):
Surgery,
A.
:
(1967)
: treatment
Pseudarthrosis
by fusion
463-470.
NO.
4,
NOVEMBER
1974
of the
distal
tibial
and
and
fibular
progressive
metaphyses.
valgus
Journal
deformity
ofBone
of the ankle
and Joint
Surgery,
in