Institute
for Limb Lengthening & Reconstruction
Hospital for
Special Surgery
535 East 70th Street
New York, N.Y. 10021
S. Robert Rozbruch, M.D.
Svetlana Ilizarov, M.D.
Arkady Blyakher, M.D.
Lengthening for Constitutional Short Stature
Lengthening
for stature in normally proportioned individuals with short stature
but without dysplasia (dwarfism) requires a different strategy for
lengthening than in patients with disproportion and dysplasia. Most
patients with constitutional short stature (CSS) or low normal stature
(LNS) require only modest increase in stature compared to the extreme
amount of lengthening required by patients with dwarfism. In fact,
the starting stature of patients with LNS is the final goal of lengthening
in patients with dwarfism.
When assessing distribution of height in the population we consider
the normal bell curve. We divide people by distribution around the
mean (average), and normal height is considered plus/minus 3 standard
deviations from the mean. Short stature below 3 standard deviations
from the mean in individuals without a medical condition such as dwarfism,
growth hormone deficiency etc., is considered constitutional short
stature. The lower limit of so called normal stature for Caucasian
men is 5'5" tall and for woman is 5'0".
While patients with dwarfism are often treated as children, patients
with constitutional short stature or low normal stature are not treated
until they have completed growing. Patients with LNS and CSS also
do not usually have deformities of the bones. To minimize the treatment
time for lengthening we prefer the lengthening and the nailing technique
(LATN). This method minimizes the treatment time in the external fixator,
limited to the time required to obtain the lengthening desired without
having to wear the external device during the hardening or consolidation
phase. This method also reduces the rehabilitation time, time to return
to full weightbearing, work, school and recreational activities.
A
new fully implanted lengthening nail is available. This approach would
not require an external fixator at all. While this is FDA approved,
it is relatively new. This devise is called the ISKD and is made by
Orthofix Inc.
The usual goal of lengthening for stature for most patients with LNS
is 2-3 inches. More than that amount cannot be achieved safely in
one pair of bones during one lengthening. For more lengthening one
needs to repeat the process in a second pair of bones.
The usual strategy is to lengthen both tibias including the fibula
2-3 inches using the LATN method or the internal lengthening nail.
If a second lengthening is desired then both femurs are lengthened
the same amount. Most patients do not choose to have the femurs done
because of the added time commitment and the doubling of the expense.
Furthermore, doing too much lengthening in the lower limbs in normally
proportioned people leads to the obvious disproportion. Up to three
inches of lengthening produces no obvious disproportion.
Our preference for tibial lengthening for over femoral lengthening
is for several good reasons. First of all tibia lengthening makes
ones legs look longer than femoral lengthening. The reason for this
is that the level of the knees and feet are obvious but the level
of the hips are not obvious. Depending on how we wear our belts, our
hips seem to be higher or lower. When we see long lower legs we extrapolate
that the rest of the person has a normal proportion which gives the
impression that the individual is longer than they really are. Furthermore,
wearing short skirts and dresses shows the lengths of the lower legs
but not the thighs, therefore one gets more mileage in apparent increased
height from tibial than from femoral lengthening. The second reason
we prefer tibia lengthening is that technically it can be performed
all at one operation in a reasonable period of time. Two femoral lengthening
over nails requires two separate set-ups during the same operation
or two different operations. This greatly increases the operating
time and cost. Furthermore there is much less blood loss with tibial
lengthening than with femoral lengthening. The third reason we prefer
bilateral tibial rather than femoral lengthening is that it is much
easier and better tolerated to have two external fixators on both
lower legs than on both thighs.
Most patients are in the hospital between 2-4 days after the procedure.
Physical therapy to stretch the ankles begins in the hospital and
continues after discharge as an outpatient. Patients need to have
physical therapy for 1-2 hours everyday during the 2-3 months of lengthening.
The patients who do best work diligently at stretching their ankle
and knee joints both at home and at therapy. Therapy can be carried
out at any therapy center. The patients from out of town can do their
therapy in their hometown. Remember you cannot do too much stretching.
Ankle range of motion is the limiting factor for lengthening. As long
as the patient can stretch the foot beyond 90 degrees, the lengthening
can continue.
Patients are seen in follow up every two weeks during the lengthening
phase. Even out of town patients need to be seen every two weeks.
On these visits we perform nerve testing if needed, exam pin sites
and obtain X-rays. Problems are identified and treated and medication
prescriptions for pain or antibiotics are written. Occasionally adjustments
to the external fixators are required in the office.
One can assume approximately one month of distraction for every inch
(2.5 cm of lengthening). Lengthening does not begin until approximately
one week after surgery. Therefore 2-3 inches of lengthening takes
2-3 months in the external fixator. Weight bearing is allowed within
limits of pain. At the end of the lengthening there is second surgery
to insert the intramedullary nails and the external fixators are removed.
Since the rods only fix the tibias an additional screw is needed for
each fibula to protect the fibula from shifting from the ankle. This
screw can be removed under local anesthesia 1 or 2 months later. After
the external devices are removed the follow up is once a month for
the first 2-3 months. Out of town patients can just send X-rays and
physical therapy reports instead of coming in.
The new internal lengthening nail method does not require the application
or removal of external frames.
Patients remain nonweightbearing until the X-rays show that the bone
is healed enough to allow weightbearing. One or two intact cortices
must be seen on X-ray. This usually takes 1-2 months after nail insertion.
Most patients can begin weightbearing two months after removal. Physical
therapy continues mostly to regain full ankle motion and foot push-off
strength. This can take several more months. Therapy is only 3 days
a week after removal but daily home exercises are recommended. Removal
of rods is recommended in the future. This is not critical and can
be done at anytime once the bones are fully healed. We generally perform
this about one year after the lengthening is completed. Again, this
is an outpatient or one night stay surgery.
While there are many potential complications of lengthening, other
than mild pin infections they are uncommon. Ankle stiffness is a concern
but a rare complication if intense therapy and home exercises are
carried out and if the lengthening does not continue, if the foot
position drops below 90 degrees (equinus contracture). Deep infection
with tibia lengthening over nail is rare. It is resolved by removal
of rod. Nerve injury is also rare. All patients with lengthening for
stature must undergo a psychological evaluation prior to the lengthening.
This evaluation is needed before we will agree to offer the lengthening
to a patient. The fee for initial evaluation is $440.00. Lengthening
for stature is considered cosmetic and will usually not be reimbursed
by insurance carriers.
Therefore, all fees must be self paid in advance of the services contracted
for. We will be happy to prepare a cost estimate for the evaluation,
surgery, anesthesia, X-ray, hospital and physical therapy costs. This
estimate must be paid in advance prior to the surgery. The approximate
current medical costs for bilateral tibial lengthening varies from
$80,000 to $120,000. Lengthening for stature may be reimbursable by
insurance in some cases, especially if there is any evidence of a
hormonal or hormone treated related basis for the short stature, or
if there is associated deformity. Insurance pre-approval is required
prior to acceptance into the program.
Finally the most important issue to consider is that lengthening for
stature in any individual over 5 foot tall is primarily for cosmetic
reasons and does not improve function. It does seem to improve body
image in patients with what we call short person neurosis. Nevertheless,
the issue of function is of greatest concern to us. This procedure
can damage nerves, muscles and joints. If such damage were to occur
it could become irreversible leading to long term problems such as
arthritis, limitation of joint motion and pain. Rare cases can even
develop reflex sympathetic dystrophy which is a chronic pain condition
and which may not be resolvable. Pulmonary embolism and deep vein
thrombosis which are common with other forms of orthopedic surgery
are rare with this surgery, but they can occur and could lead to sudden
shortness of breath, chronic leg swelling and even death. While loss
of life and limb have never occurred to us with this procedure, one
must still weigh the risks of undergoing a major surgical procedure
versus the benefits of increasing ones stature by 2-3 inches. Proceeding
with the surgery is a very personal decision. At the present time
although the procedure is performed for cosmetic reasons, it is not
in the same dimension as other cosmetic procedures such as facelifts,
breast augmentation and nose jobs.
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