Abstract

Golden hars syndrome is a syndrome complex characterised by cong presence of a limbal dermoid with cong associated presence of pre auricular sin tag or pre auricular appendage or sometimes squint the condition is also called as oculo auriculo vertebral syndrome or dysplasia their is also associated cranio facial dysplaia involving head and face the commonest structures involved in canio facial dysplasia being

1. Ear

2. Nose

3. Mandible

4. Soft plate

These dysplastic lesions are due to incomplete development of 1st and 2nd branchial arch as the defect is in genes and it is not inherited so one gets following anomalies

a. Impairment of intelligence

b. Hearing defect

c. Dental anomalies

d. Cong heart

e. Squint

f. Defects in limbs and spine

g. Kidney involvement

So one has to do

a. E e g

b. Hearing test

c. Dental examination

d. Echocardiography

e. X-ray spine and limbs

f. Ultra sound abdomen

g. Mri orbits to rule out under lying orbital involvement

However these anomalies occur in only 5 to 15 percent of case as most of cases of g h s have normal vision and intelligence

Keywords: Limbal dermoid; Oculo auriculo vertebral dysplasia; Cranio facial dysplasia; Pre auricular skin tag of pre auricular appendage