Microsurgery can correct facial deformities

by Ronnie Shulman, Scleroderma Foundation Committee Member (originally published in "Scleroderma Foundation Newsline, Summer 2000)

New developments in microsurgery make it possible to significantly correct facial contour deformities caused by linear scleroderma (SD) and its variant, Romberg's disease.

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.

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."

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.

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.