Oculoauriculovertebral dysplasia was first described by von Arlt in 1845, and the eponym dates from a description published by Goldenhar in 1952. Gorlin and associated suggested the more descriptive title of oculoauriculovertebral dysplasia, which delineates the three major components of the syndrome and the pathologic process.
The three major manifestations of this syndrome are eye, ear and vertebral anomalies.
Recently Tessier proposed a rather detailed classification in 1973. the cleft are numbered from 0 to 14 and extended along constant lines or axis through the eyebrows or eyelids, the maxilla, the nostril and the lips. According to Tessier classification, a frontozygomatic cleft(No. 8) is characteristic of oculoauriculovertebral dysplasia.
We observed a patient of 20 year old male patient, who has an epibulbar dermoid, lipodermoid, preauricular appendage, ear deformities, vertebral anomalies and many other abnormalities.
The multidiscriminatory treatment of the eye, ear and oral anomalies is discussed.
|