Two Cases of Topless Optic Disc Syndrome

ABSTRACT

Topless optic disc syndrome is considered as congenital optic nerve hypoplasia.The risk factors of this syndrome are female sex, prematurity and history of maternal diabetes.Normal central visual acuity, thinning of superior peripapillary nerve fiber layer and corresponding inferior visual field defects are the characteristic features of this syndrome.Relative superior entrance of central retinal vessels and superior scleral halo can be found in patients with this syndrome. We experienced two cases of topless optic disc syndromeone was unilateral and the other, bilateral. They had no history of maternal diabetes. Diffuse superior retinal nerve fiber layer atrophy and inferior visual field defects were observed.Pallor of superior optic neural rim, relative superior entrance of central retinal vessels and superior scleral halo were also observed. It will be necessary to differentiate these findings from glaucomatous optic disc damage and visual field defects.