Back to Annual Meeting Program

Romberg’s Disease and Linear Scleroderma (Coup de Sabre) are progressive, usually unilateral facial atrophies of unknown etiology. The gold standard treatment for these patients has been microsurgical reconstruction following the “burning out” of the facial atrophy and stable contour for at least two years. In our experience, patients treated early in their disease course have immediate and sustained correction of the deformity, with slowing or in most cases cessation of the disease process upon microsurgical transfer. No disease process has recurred to date even in cases with severe progressive disease. Equally impressive is the apparent improvement of underlying skeletal growth seen in young children with severe disease who in previous experience would have gone on to have much more severe skeletal deformities. We report our experience with 130 patients involving 133 free tissue transfers with a minimum of 1 year follow up who were treated from July of 1989 to July 2009.
All cases had severe atrophy. There were 62 males and 68 females in the series. Distribution of disease was coup de sabre or segmental in 28 patients whereas 102 patients had a hemifacial distribution typical of Romberg’s. 128 patients had unilateral disease and 2 patients had bilateral atrophy without diagnosed systemic collagen vascular disease such as lupus erythematosis. The average age of onset of disease was 12.5 years. The average duration of atrophy was 6.3 years. Average age at operation was 29.2 years with a range from 4 to 69 years. Follow up ranged from 1 to 20 years. Complications included hematoma in 9 patients, one partial flap loss, 1 patient with partial facial skin slough treated with local wound care, and cellulitis in 1 patient. One flap was lost at two weeks following surgery when the patient began smoking heavily. A second free flap was successfully preformed six months later with a stable reconstruction now four years later. Flap revisions performed at 6 months following free tissue transfer consisting of tissue rearrangement, debulking procedures, and resuspension when required were completed in nearly all patients and is now a standard for optimal results. Minimal autologous fat transfers of less than 5 cc of autologous fat were used as an adjunct in the periorbita, nasal alar rim, or minimal lip deficiencies. Recurrence of facial atrophy was not seen.
All patients rated improvement as excellent with follow-up as long as 20 years. Microsurgical correction of Romberg’s disease and linear scleroderma produces long-lasting, sustained results, and may slow progression of the disease by transfer of healthy vascularized tissue to affected areas. Stable long-term results were found even in patients with active disease at the time of microsurgical free tissue transfer. In this article, we review the indications, choices, safety, efficacy, complications, and technical refinements derived from our twenty year experience.