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(A)
(B)
FIGURE 44.2
Codfish vertebra.
FIGURE 44.1
Infant with type VIII OI treated from 5 days of
age with pamidronate every 2 months. (A) Age 3 months with ante-
rior wedging, platyspondyly and some codfish shape to vertebrae.
(B) Improvement in vertebral height and shape with increased den-
sity of the vertebral end plates.
contribute to coronal deformity. The ligamentous lax-
ity inherent to individuals with OI is also surmised to
contribute to the development of progressive deformity.
Scoliosis and kyphotic deformities typically worsen
with age, and the severity of scoliosis is related to the
severity of the OI.
1-4,10,19
Although the development
of kyphosis has been noted to have an inverse correla-
tion with the severity of scoliosis and the DXA z-score,
this has not, to the best of the author's knowledge, been
correlated with spine fractures, although clinically this
is commonly observed in scoliosis.
20
A key part to the
early treatment and management of a child with severe
OI is teaching parents how to handle the child to avoid
acute flexion to prevent these injuries. Many parents
bring the their children to the clinic the first time on a
pillow, terrified that if they pick up and hold their chil-
dren, they will sustain a spine or extremity fracture. This
greatly contributes to the development of skull defor-
mity, and interferes with the bonding of the child with
his or her parents. It also decreases the normal physio-
logical loads on the spine, inhibits muscle development
and physiologically increases bone density from the nor-
mal axial loading. Earlier ability to develop supported
sitting has been shown to delay the age of developing
scoliosis, but there are no data that this will decrease
fracture rate.
21
It is imperative that the parents be edu-
cated in the appropriate way to lift and handle the child
to prevent fractures and allow optimal physiologic and
psychosocial development. Allowing the child to remain
supine also may contribute to respiratory dysfunction.
22
The types of vertebral body deformities seen in
patients with OI have been reasonably well described.
These include the biconcave or “codfish” vertebrae,
which typically is seen in patients with severe OI
(
Figure 44.2
). This is postulated to occur when the
disease. They also require more significant trauma to
sustain a fracture than individuals with more severe
types of OI, albeit much less than a normal individual.
There are no large series of patients with OI exclu-
sively regarding the treatment of spinal fractures or
dealing with the kyphotic deformity seen as a result of
isolated or multiple compression fractures. There are only
two recent surgical series dealing with spine problems in
OI patients. These deal almost exclusively with scoliosis,
including only 20 and 11 patients, respectively.
15,16
Spondylo-lysis and -listhesis also occur in a very
high proportion of individuals with OI, especially those
with the more severe forms of OI who are now being
medically and surgically treated, which has allowed
them to assume a bipedal posture. The long-term impli-
cations of this increased incidence of lysis and listhesis
is unclear and requires further study.
17
SPINE FRACTURES
Compression fractures of the spine have long been
recognized as a significant part of life for individu-
als with OI and occur in all types of OI. The number of
fractures and severity of deformity have also been rec-
ognized to be related to the severity of the underlying
OI.
10,14,18
The compression of the vertebral body has
been recognized as contributing to spinal deformity,
both in kyphosis and in scoliosis. Asymmetrical com-
pression in the anterior posterior (AP) plane is felt to
