what-when-how
In Depth Tutorials and Information
Indications and Timing of Surgical Management
There has been no general agreement as to the opti-
mum age at which to begin operative stabilization in
the long bones of the lower extremity in children with
OI. Traditionally, deformity that resulted after closed
treatment of early fractures has been accepted until the
patient reached the age of 5 years, after which corrective
osteotomy was performed.
52
The underlying principle
for this approach was to minimize the number of opera-
tive procedures that would be required due to growth
over the lifetime of the patient.
The use of intramedullary nails in very young
infants before they begin to stand remains controver-
sial. Operative management of severely involved infants
using “percutaneous” and “semiclosed” intramedullary
rod techniques has been described.
53-55
These authors
argued that, in these very young patients, the benefits
of deformity correction, and fewer and less painful frac-
tures, outweigh the risks of repeated procedures. The
increasing use of bisphosphonates may limit the need for
surgery in this group of patients.
Recently, the trend in the management of OI has
been toward earlier operative intervention. Advocates
of this approach suggest that earlier surgery will lead to
improved gross motor development, self-care and mobil-
ity.
56-58
Using this approach, operative stabilization of
the long bones of the lower extremity generally begins
around the time the infant starts to pull to stand. Zeitlin
et al.
56
observed that the routine use of pamidronate has
led to more rapid motor development in infants with OI,
thus facilitating the use of surgery in patients around
18 months of age. In those infants whose bones are too
thin, they recommend protection with plastic braces until
their bone diameter permits placement of an implant.
It remains unclear as to whether operative man-
agement can influence the walking ability of severely
involved children. Some authors have proposed that sur-
gical treatment will favorably influence motor develop-
ment and will allow previously non-ambulatory children
to walk.
36,58-63
In contrast, Millar
64
and others
14,65-67
have
suggested that the severity of involvement was more
important than surgery in determining the probability of
ambulation. It appears that while operative intervention
may enhance the potential for walking in some children
with OI by correcting deformity and improving the sta-
bility of the long bones, the goal of achieving long-term,
functional ambulation may not be realistic for those with
severe disease.
should be done to detect cardiac and pulmonary prob-
lems that may be associated with OI, such as heart
valve incompetence or restrictive lung disease.
68,69
The
presence of basilar invagination should be considered,
and may be suggested by specific symptoms, such as
occipital headache, and the presence of cranial nerve
abnormalities. If basilar invagination is thought to be
present, preoperative neck radiographs may be indi-
cated. Esposito et al.
70
have suggested that the surgeon
assist the anesthesiologist in stabilizing the neck during
intubation to reduce the risk of injury.
Many patients with OI may tend to run an elevated
temperature during surgery. Although the consensus is
that this is not usually the result of malignant hyperther-
mia, important preoperative assessment should include a
careful personal and family history for this condition.
68,69
Patients generally do not need body warming blankets
during surgery, and occasionally elevated body tempera-
ture can be addressed by use of a cooling blanket during
the procedure.
A tendency to excessive bleeding in patients with OI
is unpredictable and has been attributed to increased
capillary fragility and platelet dysfunction. If a bleed-
ing diathesis is suspected, a hematology consultation to
evaluate platelet function may be warranted. Planning
may include autologous donation or having an adequate
number of units of packed red blood cells and platelets
available. This may be especially important in instances
where surgery involves more than one long bone. In a
recent study, Pichard et al.
71
reported a transfusion rate
of 14% in a group of OI patients undergoing insertion of
a femoral nail. As well, careful handling and positioning
of the patient by the surgeon and operating room staff
is important to reduce the risk of fracture. Use of blood
pressure cuffs with pressures designed for newborns is
appropriate, and occasional use of an intraoperative arte-
rial line to monitor blood pressure is indicated to avoid
iatrogenic humeral fracture.
Selection of Implant
Intramedullary rods have been used for more than
50 years to minimize fracture and correct deformity
in children with OI. The implant stabilizes the bone as
well as sharing the load across the fracture or osteotomy
site thus facilitating union. Plates and screws should be
avoided to avoid the creation of stress risers at either
end of the device.
72
Commonly used implants for stabi-
lization of corrective osteotomy or fracture include solid
nails, telescopic nails and overlapping Rush rods.
In 1959, Sofield and Millar
73
described fragmenta-
tion, realignment and intramedullary nail fixation for the
treatment of children who have OI. The authors found
that this procedure decreased the frequency and discom-
fort of fractures, and corrected deformity. However, in
Preoperative Evaluation
Careful preoperative evaluation is needed for the
child with OI to minimize problems that may occur
around the time of surgery. Preoperative examination
