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Golden hars syndrome is a syndrome complex characterized by congenital presence of a limbal dermoid with cong associated presence of pre auricular skin tag or pre auricular appendage sometimes there may be presence of squint the condition is called as ocular auricular vertebral syndrome or dysplasia the defect is in genes it is not inherited due to incomplete development of 1st and 2nd bronchial arch ome gete defects in head and face called as crania facial dysplasia involving head and face common organs involved are 1 ear 2 2 nose 3 soft plate and mandible it occurs only in 5 to 15 percent of cases so one gete defects ibn 1 spine 2 limbs 3 kidney 4 hearing problem 5 dental an amolies 6 cog heart 6 impartment of memory so one should do following investigations in these cases
1. x-ray limbs and spine
2. Ultrasound abdomen
3. Echo cardio grapy
4. Dental examinations
5. mr I orbits to exclude underlying orbital involvement .
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.
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.
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. 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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Sudarshan P, Gaurkar, Khushboo D, Gupta, Kiti SP, et al. GH syndrome a report of 3 casesindian journal of ophthalmology.
Goldenhar M (1952) Associations Malmormatives de I Oeil Et De I Oreile En Particulier Le Sydrome Dermoid Epibulbarire-Appendices Auriculo Dysostose Mandibulo Faciale. Jounal De Genetique Humaine Geneve 1(2): 243-282.
Araneta MR, Moore CA, Oiney RS (1997) GH syndrome among infants born in miiary hospitals in gulf war veterans sici 10 56: 42-44.
Kokavec R (2006) GH Syndrome with various clinical manifestations cleiff palate craniofacial. Journal Official Association 43(5): 628-634.