PARRY-ROMBERG SYNDROME

Parry-Romberg syndrome, also called progressive hemifacial atrophy, is an acquired autoimmune disease that causes progressive atrophy (wasting away) of the tissues underneath the skin of the face. This primarily affects the fat under the skin (subcutaneous tissue), but it also causes changes to the overlying skin that becomes thin and occasionally has increased or decreased pigmentation.

The majority of people who have this problem develop symptoms before age 20 years, but it can affect patients of any age. If the age of onset is during childhood the inflammation and fibrosis caused by Parry-Romberg syndrome can significantly affect the growth of the facial bones that may further worsen facial asymmetry and create abnormal jaw relationships. The most common areas affected Parry-Romberg syndrome are the scalp, forehead, temples, eye socket (orbit), cheek, lips, nose and chin. When the scalp, eyebrows and beard are involved, alopecia (lack of hair growth) of the affected area frequently occurs. Collectively, the changes that Parry-Romberg syndrome create cause an uneven appearance to the face that looks unnatural. The wasting away of the tissues give a skeletonized and very aged appearance to the affected side of the face.

Parry-Romberg syndrome falls under the umbrella diagnosis of Morphea, that is also called localized scleroderma, that is an autoimmune condition that results in atrophy of the subcutaneous tissue and changes in the skin quality and pigmentation in other parts of the body also. Linear scleroderma is a focal atrophy of the skin, muscle and fat of the forehead and scalp heralded by the en coup de sabre (Strike of the Sabre) – a vertical, linear indentation of the forehead. Linear scleroderma also falls under the same umbrella diagnosis of morphea. It is not known why these atrophy conditions occur and why they selective affect different parts of the body and face.

TREATMENT

How and when to treat patients with Parry-Romberg syndrome remains a controversy. The conventional way to approach treatment was to let the disease continue to progress until a final steady state was achieved. This was also called letting the disease “burn out.” Today a number of immune modulators may be used to slow or arrest the progression of the tissue loss. Some recent research studies have demonstrated that reconstruction during the active phase changes the gene expression in the affected tissues. This may cause arrest or decrease in the rate of progression of soft tissue atrophy. Careful coordination with the other providers involved in caring for you is important, including your dermatologist and/or rheumatologist. The 2 main ways to treat the changes in the soft tissues of the face are fat injection or free flap reconstruction. The primary considerations for deciding between fat grafting and free flap reconstruction are related to the amount of donor fat available and what is needed to restore symmetry and normal appearance. For most patients the small amount of fat needed for fat grafting can be harvested easily and the patients prefer the shorter and easier recovery. Occasionally very thin patients who require larger volumes of fat to achieve symmetry are only candidates for free flap reconstruction.

HOW IS THE SURGERY PERFORMED?

Both approaches are performed under general anesthesia. Fat injection uses the patient’s own fat that is harvest with liposuction. The fat is injected under the skin into the areas where thinning of the tissues has occurred. Special thin, blunt injection cannula are used to avoid injury to the skin, muscles, nerves and blood vessels in the treated area. The treatment must be performed 2-4 times, depending on the amount of volume restoration require. Fat grafting is performed with 4-6 months intervals between each treatment.Dr. Derderian prefers performing fat grafting over free flap free flap reconstruction whenever possible. Free flap surgery is described below but the remainder of the information will be for fat grafting as this is used in the vast majority of patients.  

Free flap reconstruction uses a large section of fat and skin from the back – next to the shoulder blade (parascapular flap) – to replace the bulk that was lost due to Parry-Romberg. This approach uses specialized microsurgery techniques to isolate the blood vessels that feed the flap of back tissue that are cut and connected to blood vessels in the face using a surgical microscope. The new blood vessel connections allow the back tissue to survive in the face. The blood vessel connections are monitored for several days in the hospital to make sure that clots do not form that may interfere with blood flow. In 5-10% of cases the blood vessel connections do not remain open and cannot be salvage and the flap of back tissue must be removed without an improvement in appearance. For the majority of patients free flap reconstruction is successful, but patients often need some revision surgeries in the future. The main advantage of free flap reconstruction is that a greater change in appearance can be provided rapidly. The primary downsides are the longer recovery period and scarring on the back from the donor site, and the rare occasion that the free flap fails.  

RECOVERY

Fat grafting is used to treat the majority of patients because the surgery is more straight forward and the recovery is much quicker. Typically patients require prescribed pain medicine for a day or two and then just over the counter pain medications as needed for a week. Patients return back to school, work and activities of daily living in 1 week. They resume normal exercise and sports in 2-3 weeks.

AM I A CANDIDATE FOR FAT GRAFTING?

Common features of Parry-Romberg syndrome treated with fat grafting include:

  • Vertical indentation of the forehead an scalp

  • Thinning of the tissues around the eye socket

  • Thinning of the temples

  • Deflated appearance of the cheek

  • Deepening of the nasolabial fold (fold between cheek and upper lip)

  • Wasting of the tissues of the nostril and nasal asymmetry

  • Thinning of the upper and/or lower lip

  • Asymmetry of the chin and jawline

WHY CHOOSE DR. DERDERIAN?

During his plastic surgery training at New York University Dr. Derderian had extensive exposure to the surgical treatment of patients with Parry Romberg syndrome while working with Dr. John Seibert, a world-renowned expert in free flap reconstruction for treatment of Parry Romberg syndrome. Dr. Derderian is board certified by the American Board of Plastic Surgery. He frequently performs fat grafting for patients with Parry Romberg syndrome at his practice in Dallas, TX. He currently performs clinical research with Dr. Heidi Jacobi who is a dermatologist at UT Southwestern and a recognized expert in treating patients with Morphea, Parry Romberg and Linear Scleroderma. Their clinical outcomes research includes 3D evaluation of changes in facial volume and symmetry that occur with disease progression and after treatment with fat grafting.