FP1763 : Brown’s syndrome:Unusual association with aplasia cutis and ipsilateral myelinated nerve fibre layer

Dr.SIDDHARTH SHETH, S14869

We hereby report a 10 year old boy with both eyes high hyperopia ( +7.00 DS in right eye and +9.00 DS in left eye) associated with left eye amblyopia .Best corrected visual acuity in right eye was 6/9,N6 and left eye was 6/18,N6.

Squint evaluation revealed left esotropia of 16 PD for distance and near.Extraocular movements revealed limitation of elevation in adduction in the left eye and rest ocular movements normal-suggestive of left eye Brown’s syndrome.Fundus evaluation revealed presence of left eye peripapillary myelinated nerve fibre layer in the left eye and rest was unremarkable.External physical evaluation revealed presence of plagiocephaly and a patch of alopecia on the scalp vertex suggestive of aplasia cutis congenita.This is the first time Brown’s syndrome is reported to be associated with ipsilateral peripapillary myelinated nerve fibre layer,aplasia cutis and reverse Straatsma syndrome( amblyopia in more hyperopic eye with myelinated nerve fibre layer).