Microsurgery can
correct facial deformities
by Ronnie Shulman,
Scleroderma Foundation Committee Member (originally published
in "Scleroderma Foundation Newsline, Summer 2000)
Editor's note:
You can skip directly to the subheads that interest you, by
clicking on these links:
New
developments in microsurgery make it possible to significantly
correct facial contour deformities caused by linear scleroderma
(SD) and its variant, Romberg's disease.
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Case
1. A 27-year-old woman
with severe right-side hemifacial atrophy. Her disease
process affects skin, subcutaneous fat, muscle, and
bone. Photographs on the left show her before surgery.
Photographs
on the right show her two years after microvascular
free tissue transfer reconstruction of the right side
of her face. |
What
is linear scleroderma?
Linear scleroderma is a form of localized scleroderma, which
sometimes forms a long crease on the face. It may be referred
to as en coup de sabre because of its resemblance to a saber
or sword wound. Tending to involve deep layers of skin and
underlying subcutaneous fat in a segmental distribution, linear
SD may cause one side of the face to look different from the
other (facial asymmetry).
What
is Romberg's disease?
Romberg's disease, also known as progressive hemifacial atrophy,
in its earlier stages usually involves the skin and subcutaneous
tissue, and later may involve the facial muscles and skeleton.
Both Romberg's and linear scleroderma usually begin in the
first two decades of life.
Liquid
silicone is not a lasting solution
Until recently, procedures to correct facial deformity, or
atrophy, have met with disappointing results. Liquid silicone
injections, used since the 1970s to achieve aesthetic facial
reconstruction, have worked—but often just for a short
time. Because silicone is a foreign substance, the body reacts
by forming scar tissue. The tissue contracts, causing the
skin to break down, essentially melting away what has been
added to correct facial contour.
The
promise of microsurgery
Microsurgery, used originally to reattach limbs—could
prove to be the key to permanent improvements in appearance
and function. One of the plastic and reconstructive surgeons
who has perfected the technique for facial deformities is
Dr. John W. Siebert of New York City. He uses an innovative
technique known as "microvascular free tissue transfer"
to fill in areas of the face that have contour deformities
due to autoimmune diseases, tumors, birth defects or trauma.
Unlike the traditional approach of injecting fat or silicone,
or drafting dermal fat to the site, microsurgery relies on
tissue that has its own blood supply. The living tissue, often
taken from the patient's groin or from under the arm, is sculpted
to replace facial areas that are missing.
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Case
2. A 6-year-old girl
with right-side linear scleroderma, involving right
upper lip, nose, right cheek, right eye, and right forehead.
Photographs on the left show her before surgery.
Photographs
on the right show her two years after microvascular
free tissue transfer reconstruction of the right side
of her face. |
"The transplanted
tissue flap remains healthy, and isn't reabsorbed by the body
as with the other techniques," said Dr. Siebert. "The
result is a 70–80 percent improvement in appearance"
(see photos).
"There are
no reported cases of the scleroderma attacking the transferred
tissue," he added. Dr. Siebert reported that, for reasons
not fully understood, the tissue put in the face behaves forever
like where it came from.
Operating
before the disease does its worst
The classic teaching with facial atrophy was to let it progress
until it stabilized, then wait two years before intervening
with surgery.
Dr. Siebert doesn't
believe it is necessary or advisable to wait. He has operated
on patients as young as four years. "Instead of waiting
until the disease runs its course," he said, "we
think we can change the biology of the surrounding tissues
by bringing in well-vascularized, healthy tissue. We can possibly
prevent some of the secondary changes from the scleroderma,
like changes in the skin color and damage to the bone. We
need many more patients to determine if we're doing that."
So far, results
have been positive. The surgeon estimates he has done over
300 such procedures since the late 1980s, including about
100 people with linear scleroderma and Romberg's disease.
What
is microsurgery?
Microsurgery involves sewing together tiny blood vessels
and nerves under a microscope. It has been used to close large
wounds and for breast reconstructions following mastectomies.
It wasn't until the 1980s that surgeons used microsurgery
for facial reconstruction.
"The surgery
has been perfected in recent years," said Dr. Siebert,
who has written about the technique in prestigious medical
journals. "Now we can bring in tissue as thin as paper
to correct eyelids, lips and other areas that require a subtle
approach."
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Case
3. A 13-year-old boy
with right-side linear scleroderma, involving right
upper lip, nose, right cheek, right eye, and right forehead.
Photographs on the left show him before surgery.
Photographs
on the right show her two years after microvascular
free tissue transfer reconstruction of the right side
of his face. |
Injected fat and
dermal fat grafts tend to fail, on the other hand, because
they do not have an innate blood supply, according to Dr.
Siebert. "Theoretically, blood vessels have grow into
non-living, transplanted tissue [such as fat]. Because it
doesn't happen quickly enough, the tissue dies and melts back
down." Before-and-after photographs of such procedures
may look good because they are taken soon afterward, not a
year or two later when the fat usually has been reabsorbed.
Like all surgery,
of course, the microsurgical flap procedure has risks. Patients
are evaluated pre-operatively to assure their hearts and lungs
are sound enough for surgery that may last 6–16 hours.
Dr. Siebert says the major risk is losing the tissue if the
blood vessels clot off, preventing the donor and recipient
tissue from becoming integrated. In such cases, which are
rare, the transplant can be repeated, using another part of
the body as the donor.
Paying
for the operation
Not surprising, microvascular free tissue transfer is
a costly procedure. Doctors' fees may range from $10,000—$15,000,
plus $25,000–$30,000 for the two to three days in the
hospital. Insurance companies often pay for the procedure,
but sometimes only after the doctor intercedes on the patient's
behalf. "They usually reject it at first," Dr. Siebert
said. "Doctors have to educate the insurance carrier
to show that it's not a surgery done just to help the patient's
appearance.
"We tell them
we are correcting deformity as a result of a disease process,
and that if it isn't done, the patient is likely to have functional
problems.
For example, atrophy
around the patient's eye may threaten sight. Lips that don't
meet properly could cause speaking or eating problems. A deformed
nose could affect breathing.
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Case
4. A 46-year-old woman
with facial atrophy associated with systemic scleroderma.
Photograph on the left shows her before surgery.
Photograph
on the right shows her two years after bilateral microvascular
correction of facial deformity. |
Letters, accompanied
by pictures of pre-and post-operative patients, are very useful
for showing the insurance company the functional problems
patients with facial atrophy have.
Surgeons who have
performed many microsurgical facial reconstructions, particularly
correction of contour deformities, are more likely to have
the edge in the battle against the insurance companies.
More importantly,
surgeons with greater experience in this very exacting procedure
are more likely to have a successful result from the medical
point of view.
Following
up
The American
Society of Plastic Surgeons, based in Chicago, has a list
of doctors who have done microsurgery fellowships. Interested
readers should contact them directly.
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