Juniper Publishers- JOJ Ophthalmology

 

An Interesting Case of Golden Hars Syndrome in 3 Years Male Child-Juniper Publishers

Introduction

Now we have defined d h syndrome as a complex of cong limmal dermoid with cong pre auricular skin tag aor appendage limbal dermoids sometimes are bilateral rarely. Usually they are unilateral involving whole of cornea or may be cofined to conjunctiva only incidence of l dermoids are 1 in 10000 or 500 in 2700 they are graded according to involvement of cornea grade 1. when only corneal epithelium is involved 2 grade 2 des membrane 3 grade 3 whole of ant segment of eye is involved inferno temporal involvement of limbal dermoid is the commonest about 70 percent.

Case Report

6 months back a 3 years male child was seen by me in my office with parents having noticed a small palish. White infero temporal limbal region left eye with cong presence of pre auricular skin appendage ft delivered child after lsc s no other cong anamolies seen vision mydriatic refraction ant segment and fundi were normal child had grade 1 limbal dermoid with the commonest site being infer temporal.

Results and Discussion

Golden hars is not a vision threatening disease most of these pts have normal intelligence and vision only in cases where visual axis is involved the we think of surgery conclusion my case was a simple case of gowhar ahmad grade 1 limbal dermoid so it only need observation in cases where visual axis is involved and vision is threatened then the surgical modalities are

1. Visual

2. Cosmotic

We do

1. Lamellar keratoplaty

2. Amionotic membrane graft

3. Stem cell graft

Their families of golden hars syndrome in Greece 2 in middle east in gulf war children born in different military hospital had golden hars syndrome 3 sometimes optic nerve drusen is associated with g h syndrome.

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