Progressive hemifacial atrophy (Romberg's disease): skeletal involvement and treatment

Abstract

Progressive hemifacial atrophy (Romberg's disease) manifests variable involvement of the skin, soft tissue and underlying cranio-facial skeleton. Significant bony deformation has been identified in those patients with early onset disease, the result of factors both intrinsic to the disease process and secondary to the abnormal environment in which the skeleton develops (functional matrix). Treatment demands combined osteotomy and augmentation of the skeleton in concert with conventional approaches to soft tissue correction.