Chauhan Dinesh Singh, Guruprasad Yadavalli
Department of Oral & Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur, 584103 Karnataka India.
J Maxillofac Oral Surg. 2015 Mar;14(Suppl 1):42-6. doi: 10.1007/s12663-011-0279-9. Epub 2011 Sep 1.
Goldenhar syndrome, a variant of hemifacial microsomia, is a well-known developmental anomaly of maxillofacial skeleton that is apparent at birth. The first and second branchial arch involvement during early embryonic development results in a wide spectrum of anomalies that may include macrostomia and lateral facial clefts. Though clefts of the orofacial region are among the most common congenital facial defects, the occurrence of lateral facial clefts (Tessier 7 cleft) in conditions such as the Goldenhar syndrome, is very rare (<5%). The lateral facial cleft, which results because of improper development of the perioral muscles of the face, gives an appearance of macrostomia giving rise to potential psychological, aesthetic and feeding problems. This clinical report describes the closure of a Tessier 7 cleft and the use of distraction osteogenesis to treat mandibular asymmetry in an 11-year-old female patient with Goldenhar's syndrome.
Goldenhar综合征是半侧颜面短小畸形的一种变体，是一种出生时就明显可见的颌面部骨骼发育异常。胚胎早期发育过程中第一和第二鳃弓受累会导致一系列异常，可能包括巨口症和面部侧裂。尽管口面部区域的裂隙是最常见的先天性面部缺陷之一，但在Goldenhar综合征等病症中出现面部侧裂（Tessier 7裂）的情况非常罕见（<5%）。面部侧裂是由于面部口周肌肉发育不当所致，会呈现出巨口症的外观，从而引发潜在的心理、美学和喂养问题。本临床报告描述了一名11岁患有Goldenhar综合征的女性患者的Tessier 7裂修复及使用牵张成骨术治疗下颌不对称的情况。
