A 26-Year Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma.

BACKGROUND: Parry Romberg disease (hemifacial atrophy) and linear scleroderma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been microsurgical reconstruction following the "burning out" of the facial atrophy and stable contour for 2 years. METHODS: The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. A modified parascapular flap based on the circumflex scapular artery was most commonly used. RESULTS: A total of 177 patients were included. The most common complication was hematoma, occurring in 12 patients (7 percent). Follow-up ranged from 1 to 26 years. All patients subjectively experienced improved facial symmetry and aesthetics. No disease process has recurred to date, even in cases of severe, progressive disease. CONCLUSIONS: In the authors' experience, patients treated early in their disease course have immediate and sustained correction of their deformity, with slowing or in most cases cessation of the disease process following free tissue transfer. The authors now advocate for immediate reconstruction for active disease, especially in young children. CLINICAL QUESTIONS/LEVEL OF EVIDENCE: Therapeutic, IV.