what-when-how
In Depth Tutorials and Information
Treatment
Treatment options for scoliosis and kyphosis in OI
patients are similar to those observed in treating idio-
pathic cases. Traditionally, brace and surgical treat-
ment were both considered viable options for treatment.
Unfortunately, recent research has shown that brace
treatment results in rib cage deformities and is usually
unable to stop progression.
22,24,36,37
It is thought that brac-
ing for scoliosis in OI patients has a low chance of halting
curve progression in types III-IV, because of the patho-
logical laxity of the rib head-vertebral body attachment
and the fact that ribs may deform.
38,39
Type I OI patients
may derive some palliative benefit from using brace
treatment to delay progression of curves less than 35°.
40
Complications from brace treatment in OI patients include
pressure sores, rib deformity and malocclusion of teeth.
Spinal fusion with instrumentation is an option for
progressive scoliosis and kyphosis in some patients
with OI (
Figure 43.3
). Unfortunately, in some OI
patients, excessive correction may cause vertebral frac-
tures, a direct result of the force of corrective instru-
mentation overwhelming structurally weak bones. As
correction of deformity varies significantly with disease
severity, the main goal of treatment is the support and
stabilization of the scoliotic spine.
41
Current techniques include posterior fusion with seg-
mental pedicle screw instrumentation, using appropri-
ately sized rods and screws.
24,41,42
Additionally, some
surgeons have advocated partially correcting severe
curves with halo-gravity traction prior to posterior
fusion in order to minimize stress exerted on implants.
43
Although these techniques are fairly straightforward
in idiopathic scoliosis patients, the unique anatomy
of OI patients can provide technical challenges. First,
intra-operative exposure can prove difficult because OI
ribs tend to flare posteriorly on either side of the spine,
with the posterior spine elements resting in a deep “val-
ley.” In some cases this may limit the surgeon's ability
to insert pedicle screws. Challenges may also be posed
FIGURE 43.3
OI patient with severe scoliosis. (A and B) Coronal (A) and sagittal (B) preoperative radiographs show severe scoliosis. In this
image, the patient demonstrates right thoracic scoliosis of 68° and left thoracolumbar scoliosis of 48°. (C and D) Coronal (C) and sagittal (D)
postoperative radiographs 20 months post-surgery.
FIGURE 43.4
OI patient with severe scoliosis. The patient was started on cyclic pamidronate (bisphosphonate) therapy prior to surgery.
(A and B) Coronal (A) and sagittal (B) preoperative CT show severe scoliosis and low quality bone. The major curve would progress to be 75° at
the time of surgery. (C and D) Coronal (C) and sagittal (D) postoperative radiographs 5 months after surgery.
