Your Questions
Your Questions
Q: Dr. Eppley, I am interested in correcting my coup de sabre involving my forehead and eye. What would be an approximate cost of correcting my linear scleroderma.
A: There are three approaches to treating your left forehead/orbital scleroderma (linear scleroderma en coup de sabre); forehead bone augmentation with bone cements, fat injections and the insertion of a dermal-fat graft. Which one would be appropriate for you would depend on how the tissues feel (skin stuck to bone with complete loss of fat) and whether there is an underlying bone defect on the orbital rim and in the frontal bone. (which almost always there is) Since there is usually both fat and bone defects along the line of scleroderma the most common surgical approach would be bone augmentation by bone cements combined with fat injections, either done together or in two separate stages.
To determine the ideal treatment needed for your linear scleroderma, a combined physical examination and a 3D CT scan is the best way to know exactly what to do. In many cases, these procedures are covered by insurance. But, at the least, fat injections can be done on a cosmetic fee basis and this is the most economical approach and would be part of any ideal surgical approach anyway.
Dr. Barry Eppley
Indianapolis, Indiana
Q: Dr. Eppley, In elementary school I was diagnosed with Linear Morfia but I have also heard the doctors call it scleroderma as well. I am now in college and it effects the right side of my face. I notice it on my forehead, under my eye continuing down to my cheek and a little on my nose, on the corner of my lip, and also some places under my chin and on my neck. The places on my neck are barely noticable so I’m not sure if they are even fixable but all other places I think would be able to be improved. I’m not sure what procedures would be needed but I’ve heard a lot about fat grafting. I would love to hear from you on what you could possibly do for me to make me feel better about it. I am attaching a picture of the left side of my face to compare to the picture of the right side of my face. Thank you for your time.
A: Fat grafting is the best treatment that we currently know for the soft tissue atrophy that linear scleroderma causes. Since fat loss is a big part of the tissue thinning effect it creates, it is logical that fat replacement would be a key part of its treatment. Harvesting fat by liposuction and then processing it for concentration is how injectable fat grafting is done. Injectable fat grafting is very versatile so it can be placed almost anywhere on the face.I have done this many times for linear scleroderma and it is certainly the one treatment that can help. While historically any treatment for linear scleroderma was recommended to be done once the disease processhad burnt itself out, my feeling is that fat grafting should be done even if the atrophic process is ongoing. It may help abort further tissue atrophy. Sinjce fat grafting is harmless since one’s own tissues are used, there are no adverse effects with its use and it can be repeated as many times as is necessary for optimal soft tissue volume restoration.
Dr. Barry Eppley
Indianapolis, Indiana
Q: Dr. Eppley, I am interested in a cranioplasty. I have a depression in my forehead for the past twelve years. I am twenty-two years of age and this depression started showing around age ten and has just been there ever since. It makes me feel alien, I don’t like taking pictures, I only comb my hair in one style to cover it kind of and I really think that I would be a more confident person had I have a normal forehead. I desire a “normal” forehead, without a sink in it :(. I have attached pictures of what it looks like.
A: In looking at your pictures, what you have is a classic case of what is known as linear scleroderma which creates a deformity known as the ‘coup de saber’ (cut of the saber) effect when it appears on the forehead. It is a condition that usually develops as a child and causes a loss of fat and an indentation in the bone. It is progressive and the tissue atrophy effect eventually burns itself out by the time one is a young adult in most cases. Its causes is not really understood and is currently felt to be related somehow to the nerves. When it appears on the forehead, it usually follows along the line of the first division of the trigeminal nerve. (supraorbital nerve which comes out of the brow bone and extends vertically upward into the forehead) This is why you have a very groove going upward from your brow bone, hence the description ‘cut of the saber’.
I can not tell completely from the pictures about the quality of the overlying skin, which usually is thinned and mottled in color although your skin along the groove does not appear so. (but the pictures are fuzzy) Treatment could consist of fat injections, a minimal incision endoscopic cranioplasty for bone augmentation or a combination of both. I would know exactly what to do by feeling it but I suspect it ail requires a combined fat and bone augmentation technique for optimal forehead augmentation contour improvement.
Dr. Barry Eppley
Indianapolis, Indiana
Q: Dr. Eppley, I have linear scleroderma which affects my forehead and would be interested in discussing how it could be treated. I’m 44 and balding, so the scar is much more prevalent than it had been when I had more hair. According my doctor out here, it’s burned out. I’d like to learn more about your experience with this problem and how you might look to correct it.
A: Your linear scleroderma involvement of the forehead and scalp is fairly classic, going down (or up) along the pathway of the 1st division of the trigeminal nerve. At your age it would be fairly common that the disease process has burned itself out. There are numerous approaches to the reconstruction of the aesthetic forehead defects from it, most of which focus on volume restoration using various forms of fat grafting. The problem with that exclusive approach is that it can not restore the quality of the overlying skin which is usually thinner and discolored to some degree. Even with volume enhancement, the vertical orientation of the involved skin is still very apparent.
In your linear scleroderma case, I would use two different surgical techniques which are different. On the forehead, I would excise the entire length of the involved skin and put the closure back together in a broken line closure methods due to the numerous horizontal wrinkle lines that you have. This would be an ideal way to really hide the scar well. This would not only ensure a permanent and assured resolution to the forehead soft tissue defect but also gets rid of the deformed skin. On the scalp, however, a different approach would be used as it is smooth skin with no wrinkles lines to help hide the scar. I would do fat injection grafting to restore the indentation only.
Dr. Barry Eppley
Indianapolis, Indiana
Q: I have scleroderma which over time has caused atrophy and asymmetry to my face. I’m interested in plastic surgery to help fill my face out and not make it look so narrow. So if you could please just send me some more information about this that would be great. Thank you.
A: Scleroderma that affects just one side of the face (most commonly) is known as linear scleroderma or Romberg’s disease. It is a rare facial condition that often does not start until late childhood or early adolescence and then burns itself out by early adulthood. It is not known what causes it or why it stops. It is currently thought there is a neurogenic basis for it. It results in soft tissue atrophy, with loss of subcutaneous fat, thinning of the overlying skin, and occasionally loss of some of the underlying bone. (most notably the mandible with loss of the jaw angle and shortening of the jaw line) This creates one-half of the face that is thinned and asymmetric. The forehead may have just a vertical line atrophy. Romberg’s disease comes in all variations from just a single area of atrophy to an entire facial half that is severely withered.
The key to reconstruction in Linear Scleroderma is soft tissue replacement or augmentation. I have done numerous Romberg patients and have used allogeneic dermal grafts, dermal-fat grafts, fat injections and even vascularized free flaps. Since the problem is largely soft tissue loss, the focus on reconstruction should be soft tissue-based. Occasionally, I have used a synthetic implant in the jaw angle but one should generally avoid placing implants is areas of thin soft tissue coverage. Which one of these soft tissue replacements is best on based on the location and degree of the facial atrophy. Each of their own advantages and disadvantages and combinations of two or more of them are usually needed.
Indianapolis, Indiana
