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The cause of hemifacial atrophy or Romberg’s disease remains unknown. A vasculitis similar to that found in collagen vascular disorders such as Lupus, Scleroderma or polymyositis is most probable. There is no known genetic predisposition to the disease. The onset of Romberg’s disease is typically during the first two decades of life. However, I have reconstructed patients who’s disease began in infancy as well as older patients with the onset of facial atrophy typical of Romberg’s in their 60’s. In large series of patients the left side of the face is more common than the right and women outnumber men. Medical treatment of early cases or rapidly progressing cases ranges from observation to aggressive intervention with chemotherapy usually involving methotrexate. Pediatric Rheumatologists vary in their recommendations. The surgical reconstruction of hemifacial atrophy or Romberg’s disease depends on the severity and distribution of facial wasting present. The severity of disease ranges from mild to severe and may involve only a small area on the face or the entire hemiface including the scalp to the neck. Rarely skip areas or uninvolved areas separating areas of subcutaneous fat wasting are found. In most cases these skip areas are actually involved with some atrophy just to a much less extent than the adjacent areas. The disease process begins with atrophy of subcutaneous tissue and may involve adjacent overlying or underlying tissues in severe cases. The dermis of the overlying skin thins and the skin darkens. The underlying muscle and bone may be involved resulting in scarring and wasting of the bone especially alveolar bone, which holds the tooth roots in the bone. In severe cases, exposure of tooth roots with loss of teeth may result. In cases with an early age of onset, growth of the underlying facial skeleton is likely to be impaired. As a result the involved side of the face is short and the bite is adversely affected. Reconstruction involves the microvascular transfer of customized soft tissue flaps containing fat and thin layers of fascia as thin as tissue paper which are transferred to the face from areas of the body not involved with the atrophy process. The transferred tissues are taken along with their nourishing blood vessels usually from the flank or groin. The blood vessels contained with the transferred tissues are then reconnected to blood vessels in the face in order to transfer living tissues with intact blood supply. The tissues are then sculpted and inset into the face to restore the facial shape which has been altered by the disease process. The timing of reconstruction of the facial asymmetry and deformity due to Romberg’s disease has classically been to wait until the facial wasting has stopped and been stable for at least two years. Having dealt with literally hundreds of patients with Romberg’s disease my perspective has changed. By intervening earlier perhaps the biology of the disease can be changed. Is it because we introduce well vascularized tissues with rich blood supply? Is it because we bring in a rich capillary bed to promote healthy differentiation of tissues in the same area? I don’t know the answer. But, I do know that microvascular transfer of well vascularized tissues from an uninvolved area of the body has never been attacked by the wasting process. Even more importantly in literally hundreds of patients the transferred tissue has reversed overlying skin atrophy and color and made the entire region healthier in appearance. In three doctors children, who had onset of disease in early childhood I have performed microvascular free tissue transfer early in the disease process. In these three cases the disease process seemed to stop and there was no associated growth disturbance of the underlying jaws. I know that in most cases with the onset of disease in early childhood and similar severity have all progressed to severe deformity with marked restriction of skeletal growth on the affected side of the face. I have performed a second flap in only one patient of mine about ten years after the initial microvascular facial reconstruction. I perform minor revisions or ancillary procedures on all patients about six months following microvascular reconstruction. These consist of minimal tissue rearrangements, lip reconstructions, autologous fat injections or standard cosmetic procedures such as eyelid surgeries or facelifts on older patients. I have published dozens of articles or chapters in textbooks related to the microsurgical reconstruction of facial asymmetry. To date I have performed almost 400 microvascular free flap reconstructions for patients with facial asymmetry. I’ve included several examples of typical patients with Romberg’s hemifacial atrophy, linear scleroderma and bilateral facial atrophy for your review. I’m sure you will agree that this type of reconstruction is life changing and allows these patients to live their lives with minimal stigmata of facial deformity. Professor, Division of Plastic and Reconstructive SurgeryG5/360 Clinical Science Center Appointments: Office: (608) 265-8072 Education
Clinical SpecialtiesDr. Siebert is certified by the American Board of Surgery and the American Board of Plastic Surgery . His practice focuses on microsurgical reconstruction, general reconstructive surgery and esthetic surgery. Recent Publications
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RESTORATIVE PROCEDURE BEFORE AND AFTER IMAGES APPEAR BELOW
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