DR. CHARU ARORA

DR. ANSHU SHARMA, DR. PRIYANKA MOHANBABU CHATURVEDI, DR. MARISHA BISHNOI

Abstract

Goldenhar syndrome is a rare syndrome with an incidence of 1 in 5000 live births and gender predisposition for males. It is characterized by a classical triad of mandibular/malar hypoplasia, ocular & auricular malformation and vertebral anomalies. Hence also known as oculo-auriculo-vertebral dysplasia. Patient with goldenhar syndrome can present with limbal/epibulbar dermoids, upper lid coloboma, disc coloboma and microphthalmos as far as spectrum of ocular manifestations is concerned. We hereby want to report a case of a 10 year old male child who presented with malar hypoplasia , facial asymmetry on left side, mild deviation of angle of mouth, depressed nasal bridge, epibulbar dermoid in left eye, limbal dermoid in right eye, preauricular skin tags in both eyes and facial dysmorphism. Screening for associated anomalies was suggested which includes Chest Xray, 2D echo, CT Brain for further management.