A baby girl with ocular dermoid and preauricular tragi.

Abstract

Goldenhar syndrome or oculo-auriculo-vertebral dysplasia with hemifacial microsomia is a rare development anomaly of the first and the second branchial arches with a prevalence of 1: 5,600 newborns, of unknown etiology, probably with autosomal dominant hereditary transmission, but sporadic in most cases. The most characteristic symptoms, which in most cases affect only a hemiface, include ocular abnormalities such as microphthalmia, epibulbar dermoid, lipodermoid and coloboma, ear abnormalities as preauricular tragi, deafness, microtia and vertebral anomalies such as scoliosis, hemivertebrae and cervical fusion. Other organs such as cardiovascular system, central nervous system and genitourinary tract may be interested. The syndrome has highly variable expressivity as demonstrated by our case that presented only bilateral accessory tragi and right ocular dermoid.