DR. SUBHRA SARITA BEHERA

Dr. SUMITA MOHAPATRA, Dr. BALAKRUSHNA SAMANTARAY, Dr. SHRUTI SATPATHY

Abstract

INTRODUCTION-Goldenhar syndrome or oculo-auriculo-vertebral dysplasia(OAVS) or hemifacial microsomia is a rare congenital anomaly characterized by Ocular & auricular malformation,Vertebral anomalies,mandible & maxillary hypoplasia.

CASE-A 5month old male baby came to ophthalmology OPD with chief complain of bilateral conjuctival mass associated with right ear malformation,pre-auricular skin tag,macrostomia,soft palate fusion defect,facial asymmetry. On examination-limbal epibulbar dermoid on right eye and lipodermoid superotemporally on left eye conjunctiva. On X-ray, mandibular and maxillary hypoplasia and nonfusion. After correlating the history,clinical findings and investigations,Goldenhar syndrome was diagnosed.

DISCUSSION-OAVS is due to malformation of 1st and 2nd branchial arches.Fast intervention provides fast recovery.

CONCLUSION-Goldenhar syndrome is easy to diagnose with clinical findings.Surgical treatment is complex,multidisciplinary & individualized with age.