what-when-how
In Depth Tutorials and Information
is especially useful in children who are learning to
walk and in doing so are at risk for falls - the most fre-
quent number of fractures have been found to occur in
the first year of life for OI patients.
4
Ankle foot ortho-
ses (AFOs) can provide support for tibial bowing, and
are much less encumbering for the patient, while pro-
viding excellent support to lax ankle joints. Long leg
braces have the disadvantage of requiring the child to
lift the limb and the brace, and can increase the diffi-
culty of walking. Brace choice for the individual patient
balances minimizing restriction of movement while
providing the most protection. Thus, implementation
of brace treatment requires careful discussion with the
family and patient. There appears to be little downside
to brace treatment, and even the concept that the brace
will support the bone and thereby cause additional
osteoporosis has not been supported. In a study where
braces were removed in half the study population, there
was no increase in fractures comparing the two groups
after the braces were removed.
4
Evidence that bracing may decrease limb deformity
comes from Gerber et al., who reported on 12 patients
with OI who were braced with long leg braces and
received pool and land therapy. Seven patients had
a reduction in the rate of fracture after bracing, four
patients had more fractures per year once braced and
one child had no change in fracture rate after starting
to use long leg braces.
4
Out of the 12 children who were
braced, four had increasing femoral bowing, four chil-
dren did not seem to have a change in femoral bowing
and four children had decreased bowing after the ini-
tiation of brace wear. The numbers of children were too
small for statistical analysis; however, the tibia seemed
to respond better than the femur to bracing, with a
decrease in bowing seen in nine children. An increase
in tibial bowing occurred in two children, and one child
had no change in deformity.
4
Some authors have sug-
gested initiation of braces after surgical intervention
as part of the post-operative treatment plan to protect
the osteotomy sites and allow early mobilization of the
post-surgical patient.
5
Little is known about compliance
with bracing in the OI population.
FIGURE 47.2
(A) Failure to correct the anatomical axis will lead
to progressive deformity, recurrent fracture and often rod migration
out of the bone. (B) Revision of rod with osteotomies to correct the
anatomical axis.
in limb deformity in patients undergoing medical man-
agement of the long bones. In the spine, bisphosphonates
do increase the vertebral body height. Because acute frac-
tures can lead to malunion, fracture prevention would be
expected to reduce the amount of limb deformity occur-
ring in OI patients being treated with bisphosphonates.
Brace Treatment of Deformity
Bracing can provide external support for the limb
and, like medical treatment, can reduce the number
of fractures. Clam shell braces that provide circumfer-
ential support are the most popular style of braces in
children with OI.
4
Whether long leg braces that have
a waistband to support the hip, cross the knee with a
hinge and include the foot are necessary depends on
the ambulation status and muscle strength of the child.
Children who are not independently ambulating and
working on standing can benefit from longer braces
that can be incorporated into a standing frame. Bracing
Surgical Correction of Deformity in OI
The current surgical technique in OI achieves two
important goals. First, realignment of the bone and cor-
rection of deformity help prevent recurrent fractures,
as the bone structurally is able to tolerate greater loads
before failure. In addition, rods inserted into the medul-
lary canal provide a load-sharing device that supports
the bone (
Figure 47.3
). The poor bone quality typically
encountered in OI patients results in a strong prefer-
ence for intramedullary rod fixation, as plates and
screws are load bearing and have been shown to have
