DR. MEHAKLEEN KAUR GILL

DR. SARITA AGGARWAL, Dr.AKSHITA CHAWLA, Dr.NAMAN SAHNI

Abstract

Introduction-

Goldenhar syndrome is a birth defect resulting from maldevelopment of the first two branchial arches with incomplete development of the ear, nose , soft palate , lip & mandible. It is known as Oculo-vertebral dysplasia.

Clinical Presentation-

It includes limbal dermoid , lipodermoid, pre auricular tags, hemifacial asymmetry & vertebral anomalies. Ocular manifestations are limbal dermoid or lipodermoid & occasional coloboma of Upper lid. Limbal dermoid is more comman then lipodermoid. It is usually present in inferotemporal quadrant & can be bilateral in 25% cases. It has 2 types- small & large, the larger one interferes with visual axis causing astigmatism ,predisposing to secondary strabismus from anisometropic amblyopia.

Conclusion- Goldenhar syndrome is a rare congenital anomaly with cosmetically unacceptable defects whose management having numerous challenges & multi disciplinary approach for its optimal management.