Oculo-auriculo-vertebral Spectrum (Goldenhar′s Syndrome) - A Case Report

Abstract

Goldenhar′s syndrome also known as Hemifacial microsomia and Oculo auriculo vertebral dysplasia is a type of mandibulofacial dysostosis, with ocular problems, abnormal pinna, a small mandible and epibulbar dermoids. It represents a heterogenous group of disorders characterized by abnormalities of structures derived from the first and second branchial arches. The case presented here had all the typical manifestations of the syndrome but the facial asymmetry was less pronounced. It presents a diagnostic and therapeutic challenge to the maxillofacial, orthodontic and other dental and medical specialists. The management of this condition requires a coordinated team effort from many specialists. A rare syndrome, it presents with varying features and involves many organs and systems making an interesting study. The features of this syndrome are discussed along with a case presentation. This patient had maxillary hypoplasia and also cleft lip and cleft palate.

Keywords

• Oculo-auriculovertebral syndrome
• Goldenhar′s syndrome
• Cleft lip
• epibulbar dermoids
• facial asymmetry