Add to ORKGOptic nerve aplasia is a rare nonhereditary malformation, that is seen most often in a unilaterally malformed eye of an otherwise healthy person. In its current usage, the term implies complete absence of the optic nerve (including the optic disc), retinal ganglion and nerve fiber layers, and optic nerve vessels. Histopathologic examination usually demonstrates a vestigial dural sheath entering the sclera in its normal position, as well as retinal dysplasia with rosette formation. Some early reports of optic nerve aplasia actually described patients with severe hypoplasia at a time when the latter entity was not clearly recognized
