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April 1967

Picture of the Month

Am J Dis Child. 1967;113(4):469-470. doi:10.1001/archpedi.1967.02090190115012

Denouement and Discussion 

Oculoauriculovertebral Dysplasia  (Goldenhar's Syndrome, Oculoauriculo Dysplasia, Mandibulofacial Dysostosis With Epibulbar Dermoids, First and Second Branchial Arch Syndrome)

MANIFESTATIONS  The major manifestations are found in the eyes, ears, and vertebrae. An epibulbar dermoid, unilateral or bilateral, but most frequently bilateral, is usually located at the limbus or corneal margin of the lower outer quadrant of the eye. Occasionally a lipo-dermoid is found in the upper outer quadrant and may occur alone or accompany a dermoid. There is frequently a unilateral coloboma of the upper eyelid, in contrast to the Treacher Collins syndrome in which the coloboma, when present, involves the lower lid. Ears may be small, low-set, or abnormally shaped with stenosis or atresia of the external auditory meatus. A constant finding is the presence of bilateral auricular fleshy appendages which are usually multiple, often pedunculated, located anterior to the tragus and found along the line from tragus

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