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

• Published in: 日本口腔外科学会雑誌 Vol. 60; no. 4; pp. 219 - 224
• Main Authors: 井上, 裕美子, 横江, 義彦, 森, 宏樹, 花井, 眞希, 山本, 学
• Format: Journal Article
• Language: Japanese
• Published: 社団法人 日本口腔外科学会 20.04.2014
• Subjects: Goldenhar症候群; 下顎骨形成不全; 顔面非対称; 骨延長術
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.60.219

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.