Superior segmental optic nerve hypoplasia (SSONH), also known as topless disc syndrome, is a subcategory of optic nerve hypoplasia in which the findings are generally localized to the superior aspect of the disc. In this condition, there is superior disc pallor, a superior peripapillary scleral halo, superior retinal nerve fiber layer thinning, and the central retinal artery has a relatively superior entrance to the eye. The condition often results in a corresponding inferior visual field defect as demonstrated in this Goldmann visual field. A strong associated has been demonstrated between SSONH and maternal diabetes.
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