Dr. JITENDER KUMAR

Dr.Monika Dahiya, Dr. JITENDER KUMAR, Dr. MANISHA NADA, Dr.Priyamvada Yadav

Abstract

We had come across a few cases of Goldenhar syndrome, but bilateral presentation is quite rare, only in 10-15% cases. It is typically a triad of epibulbar dermoid, accessory auricular appendages and aural fistula. Its prevalence ranges from 1:3500 to 1:7000 live births with M:F ratio of 3:2. We report a case of 22 year old male presenting with diminution of vision in both eyes associated with poor hearing since birth. He had a history of some mass excision in both eyes in childhood. On general physical examination, he had microtia and accessory auricular appendages on both sides. On ocular examination, he had a BCVA of 6/12 (+1.5DS ) in both eyes with microcornea on both sides ( Horizontal corneal diameter=9mm and Vertical corneal diameter=8.5mm) with old scar on limbus in inferotemporal area suggestive of epibulbar dermoid excision. Fundus examination was absolutely normal bilaterally. No other vertebral, cardiac, renal and CNS anomaly was found on examination