MPSC: MediSPA

TUBEROUS SCLEROSIS: Treating the Skin
ANGIOFIBROMAS
Since 1989 Dr. Mausner has treated hundreds of children and adults with Tuberous Sclerosis. Not satisfied with the results of treatment and the high complications rate associated with earlier techniques, Dr. Mausner has developed a unique treatment regimen. Using a series of lasers both the vascular (red color) and the fibrous component (raised portion) of the Angiofibromas of the face can be successfully treat at one time!

Treatments are either performed in our medical suite or in an out-patient surgical setting under anesthesia. The treatment usually takes about one hour. The patient goes home (or to a hotel for our out of town patients) the same day and can resume normal meals that afternoon. Post-operative pain is minimal and care of the face is usually simple, requiring Aquaphor followed by over the counter topical medications.

Healing Time
Vascular Laser - no downtime; immediate return to work/school; occasional minor irritation for 2 - 3 days

Carbon Dioxide (CO2) Laser - approximately 10 days of downtime until new healthy skin has grown in; 2- 4 months for the “pinkness” to subside

Evaluation by Dr. Mausner
Each person’s unique situation must be evaluated, a treatment plan created with the child / adult and family and the timing of the treatment discussed thoroughly. Symptoms might include bleeding, itching, cutting of growths during shaving and the unsightly nature of the angiofibromas on the face.

Other Skin Manifestations
Ash Leaf Spots
There is currently no treatment available for ash leaf spots, or lightly colored skin patches that resemble ash tree leaves. Although there are normal numbers of pigment-producing cells in the spots, they are unable to produce sufficient amounts of pigment to create normal skin tone. New treatments are on the horizon for stimulating pigment production in the skin, and some of these treatments may eventually be applicable for the lightly colored skin patches of tuberous sclerosis (TS) patients. There are special beauty aids available to cover de-pigmented skin.

The Shagreen Patch and Forehead Fibrous Plaque
Both of these less common skin lesions consist of an excess amount of fibrous tissue, similar to that found in scars. The shagreen patch is a section of thickened, elevated pebbly skin (like an orange peel) usually found on the lower back and nape of the neck. The forehead plaque is similar but is found on the forehead or scalp. Neither growth tends to cause problems (such as bleeding). Both of these skin lesions can be surgically removed but because of the resultant scar, it is usually not advised. There is currently no non-scarring method for the removal of these lesions and they tend to reoccur after removal. It is therefore recommended that the fibrous skin thickenings not be removed, unless they are causing severe problems.

Angiofibromas of the Face
Small flat red spots forming on the face, or a more generalized redness of the cheeks, nose and chin, are the first symptoms of angiofibromas. The redness is due to an excess number of blood vessels in the superficial part of the skin. Later, the lesions thicken and elevate, forming reddish-pink bumps, or angiofibromas. Although in some individuals the growths never develop past the red lesions, in most cases, the red lesions do develop into true, fibrous angiofibromas. There is currently no way to prevent the formation of the angiofibromas but there are promising ways to treat them.

Angiofibromas are best and most easily treated when they are in the early flat red spot stage. Treatment reduces or eliminates the red appearance of the skin and may decrease the likelihood of the growth getting larger. While a few individuals have reported no reoccurrence of their angiofibromas following treatment during this stage, there is good clinical evidence that removal of early, flat, red angiofibromas decreases the chance for the development of full-fledged, fibrous angiofibromas at the treated location.

A vascular (blood vessel) laser is the best choice for treating the flat red spots. This laser is designed to destroy the blood vessel feeding the growing lesions in the skin with low risk of scarring. The laser light does not “see” the surrounding normal skin and is only absorbed by the red pigmentation. As a result, it is a good choice for the removal of early red angiofibromas. This laser treatment can either be performed in the office (if limited or if the patient is cooperative) or as an outpatient procedure. Treatment, which usually takes 10-20 minutes, causes moderate discomfort during the treatment. Topical anesthetics or oral sedation can be used in the office to minimize discomfort. The laser treatment can also be performed in conjunction with the sedation used for MRI evaluation. Following the treatment there is rarely any discomfort and usually no wound to care for. The full effect of a given treatment can be judged 6 - 8 weeks later and frequently if a lesion does not disappear, it can be retreated. Although there is no limit to the number of treatments that can be done, generally speaking, if no noticeable improvement is apparent after three treatments, one has to reassess either (1)the lesion that is being treated, (2)the laser chosen or (3)the laser setting used.. There is no age restriction for vascular laser treatment, but clinically it’s been noted that the younger the child, the better the success of treatment.

For patients who already have well-developed fibrous angiofibromas, current available treatments include surgical removal, laser ablation (destruction) or dermabrasion. If only a few large angiofibromas are present, surgical removal is an option. It can be performed under local or general anesthesia in an outpatient setting. Although a permanent solution, the resultant scarring is also permanent.

If large or limited areas of the face are affected, the most helpful option is carbon dioxide (CO2) laser surgery. This laser uniquely allows the surgeon to fine tune the light energy to remove angiofibromas without penetrating deeply into the dermal layer of the skin. This will help maximize treatment results while improving healing and minimizing scarring. Risk of scarring from laser treatment is lower than dermabrasion, electrodessication (burning), freezing or surgical excision (cutting out) the angiofibromas. Because the CO2 laser destroys the epidermis and superficial dermis, some degree of scarring is possible. Therefore, be sure to seek out an experienced surgeon. Do not be afraid to ask to see before and after pictures of patients the doctor has treated. Ask the surgeon how many patients he has treated specifically with Tuberous Sclerosis.

There are no age restrictions for CO2 erbium laser treatment. Generally younger children do not have many large raised angiofibromas but if they are seen, they can be treated at any age. Clinical evidence has shown that there can be a rapid growth phase of the angiofibromas during puberty therefore it is suggested that the child be seen and evaluated for treatment well before puberty begins.

Treatment with the CO2 is usually an outpatient procedure performed in a hospital or surgery center under general anesthesia under the supervision of an anesthesiologist. The laser treatment with the CO2 laser is associated with minimal postoperative pain when the face is treated open with topical ointments. Clear and detailed postoperative instructions are important and careful attention to wound care is necessary for optimal skin healing. Be sure the doctor you select addresses these issues with you. Laser surgery is generally performed by either plastic surgeons or dermatologists. Recurrence is the greatest problem associated with removal of facial angiofibromas. As a result, laser surgery may need to be repeated.

Periungual Fibromas and Gingival Fibromas
Periungual fibromas are angiofibromas that are located around the fingernails or toenails. People with periungual fibromas can develop pain when wearing shoes and walking or can distort and push up the nail itself causing infection and bleeding. These symptoms will require the removal of these lesions. Surgical excision is the most common technique for removal of these fibromas and can be combined with CO2 laser removal to maximize effectiveness while limiting scarring and damage to the nail.

Gingival fibromas are angiofibromas involving the gingiva (gums) of the mouth. They can cause bleeding or rarely, problems with eating.

All of the concerns about the removal of facial angiofibromas also apply to the removal of periungual and gingival fibromas. Choose your surgeon carefully and do not be afraid to ask questions. Generally, dentists and oral surgeons remove gingival fibromas and plastic surgeons, dermatologists and podiatrists remove periungual fibromas. These fibromas need to be completely excised can reoccur.

The willingness of insurance companies to cover the cost of these procedures is quite variable. Be sure to check with your insurance company before consulting with your doctor. If your insurance company refuses to cover the removal of facial angiofibromas, sometimes a letter from your doctor will help convince your insurance company to pay for the procedure.

In Summary
Although most skin disorders associated with TS are not curable, an experienced surgeon can remove troublesome lesions with favorable results. Additionally, with continued research into the control of blood vessel and fibrous tissue formation, the future looks bright for new, more effective treatments for skin disorders associated with TS.

For more information about Tuberous Sclerosis please visit the Tuberous Sclerosis Alliance (TSA) website www.tsalliance.org.

* The above information (a modification of the Tuberous Sclerosis Alliance Fact Sheet) is intended to provide basic information about TS. It is not intended to, nor does it, constitute medical or other advice. Readers are warned not to take any action with regard to medical treatment without first consulting a physician. Mausner Plastic Surgery Center, Dr. Mausner nor the TS Alliance does not promote or recommend any treatment, therapy, institution or health care plan.

CARING. COMPETENT. CONFIDENTIAL.

Dr. Mark Mausner, nationally and internationally renowned plastic and cosmetic surgeon located in the Washington, D.C. area, specializes in plastic surgery procedures, including breast augmentation, breast lift, breast reduction, liposuction, facelift and body contouring surgery. Mausner Plastic Surgery Center serves the communities of Bethesda, Rockville, Chevy Chase, Silver Spring, Gaithersburg, Germantown, Frederick, Arlington, Fairfax, McLean, Alexandria, Urbana, Mt Airy, Poolesville, Brookville, Kensington, Clinton, Waldorf, Laurel, Potomac, Columbia, Clarksburg, Ijamsville, Tysons and Washington, D.C.