FP1400 : A RARE CASE OF GOLDENHAR SYNDROME PRESENTING WITH BILATERAL LIMBAL DERMOID

Dr.A.RANI PRIYADHARSINI, R21524

A 13 year old female child presented with bilateral growth near the limbus since birth with no systemic illness or positive family history. O/E her vision in RE was 6/24 and LE 6/12, slit lamp examination showed B/L inferotemporal limbal dermoid with other findings being normal. Fundus BE revealed a clear media with tilted disc and vessels appeared normal. She was found to have preauricular skin tags with high arched palate. With the above findings, this child was diagnosed to be a case of Goldenhar syndrome with B/L limbal dermoid and was subjected to UBM and AS OCT which revealed moderate reflective echoes between 3’O-4’OCP involving sclera, limbus and peripheral cornea. An extensive workup to rule out other systemic and genetic factors has been carried out. The child has been planned for BE Limbal dermoid excision biopsy. This case is reported for its rarity as 50% of Goldenhar syndrome present with unilateral limbal dermoid whereas only 25% present bilaterally.