Bogusiak Katarzyna, Arkuszewski Piotr, Skorek-Stachnik Katarzyna, Kozakiewicz Marcin
From the Departments of *Craniomaxillofacial and Oncological Surgery and †Maxillofacial Surgery, Medical University of Łódź, Łódź, Poland.
J Craniofac Surg. 2014 Jan;25(1):177-83. doi: 10.1097/SCS.0000000000000387.
Goldenhar syndrome is a rare congenital defect characterized by ocular symptoms including (epibulbar dermoids, microphthalmia, anophthalmia, eyes asymmetry/dysmorphy, cleft eyelid, exophthalmia, strabismus), auricular symptoms (dacryocystitis), preauricular appendages, preauricular fistulas, ear asymmetry, microtia, atresia of the external auditory canal), craniofacial deformities (cleft face, cleft lip, cleft palate, macrostomia, bifid tongue, hypoplasia of the mandible, hypoplasia of the maxilla, asymmetry of the mandible and maxilla, malocclusion, tooth discrepancies, agenesis of third molars and second premolars, supernumerary teeth, enamel and dentin malformations, delay in tooth development), and skeletal abnormalities (cleft spine, microcephaly, dolichocephaly, plagiocephaly, vertebral defects) or abnormalities of internal organs. The degree of abnormalities vary from severe to mild. In this article, we present a long-term observation of a patient with Goldenhar syndrome. During the patient's life, the intensification of anomalies varied. We describe preoperative and postoperative orthodontic treatment and surgical correction procedures of maxillofacial deformation.
Goldenhar综合征是一种罕见的先天性缺陷，其特征包括眼部症状（眼球皮样囊肿、小眼症、无眼症、眼睛不对称/畸形、眼睑裂、眼球突出、斜视）、耳部症状（泪囊炎）、耳前附件、耳前瘘管、耳部不对称、小耳畸形、外耳道闭锁）、颅面畸形（面裂、唇裂、腭裂、巨口症、舌裂、下颌骨发育不全、上颌骨发育不全、下颌骨和上颌骨不对称、错牙合、牙齿差异、第三磨牙和第二前磨牙缺失、多生牙、牙釉质和牙本质畸形、牙齿发育延迟）以及骨骼异常（脊柱裂、小头畸形、长头畸形、斜头畸形、脊柱缺陷）或内脏器官异常。异常程度从严重到轻微不等。在本文中，我们展示了对一名Goldenhar综合征患者的长期观察。在患者的一生中，异常情况的加剧程度各不相同。我们描述了上颌面部畸形的术前和术后正畸治疗以及手术矫正程序。
