Case Presentation - Goldenhar's Syndrome

This very interesting case was presented by Dr. Vinay (PG student NSCB-MCH) under Dr. Mrs. Meeta Shrivastava (Professor and HOD Department of Ophthalmology NSCB-MCH) with adequate audio-visual aids. 30-5-2004, 5 PM, Hotel Krishna Conference room, sponsored by JAWA.

Images awaited.

Goldenhar's syndrome

It is a craniofacial anomaly and represents a variation of hemifacial microsomia with the additional findings of epibulbar dermoids and/or lipodermoids and vertebral anomalies.

The case presented was a young female.

According to various texts, epibulbar dermoids are almost uniformly located in the inferotemporal quadrant and extend over the limbus. They are bilateral in about 25% of patients and present in more than 75% of patients. The lipodermoids are less frequent, occuring in less than 50%, and are located sub-conjunctivally some distance from the limbus in the temporal quadrants. Upper lid colobomas occur in nearly 25% of the cases and are rarely bilateral. Less frequently reported anomalies include microphthalmia, cataract, iris anomalies, decrease in corneal sensation and anophthalmia. Duanne retraction syndrome and other ocular motility disturbances have been noted in a number of cases.