Goldenhar 症候群患児の下顎骨単独延長術術後に長期観察した1例 − 10年経過時の評価 −

Abstract

Goldenhar syndrome is a disease characterized by a mandibular hypoplastic defect as the main sign, associated with a bulbar conjunctiva epidermoid tumor and backbone malformation resulting from the first and second branchial arch syndrome. Approximately 70% of patients present with unilateral symptoms, resulting in facial asymmetry. Since hypoplasia of the mandible is the main feature of this syndrome, mandibular distraction osteogenesis is suitable for treatment of this syndrome.We describe an 8-year-2-month-old boy with Goldenhar syndrome. His mandible was Type IIa according to the Pruzansky-Kaban classification. We initially perfomed mandibular distraction osteogenesis to acquire facial symmetry. Then, occlusal plane inclination due to the secondary maxillary deformation was prevented by orthodontic treatment. He was followed up for 10 years with no particular relapse.