Figure 26-8 Tilted disc syndrome, right eye.

Patients may demonstrate a bitemporal hemianopia, which is typically incomplete and preferentially involves the superior quadrants. The hemianopia can usually be distinguished from a chiasmal lesion because it does not respect the vertical midline. Refractive correction often results in elimination of the visual field defect. Tilted discs, myopic astigmatism, bilateral decreased vision, and visual difficulty at night suggest the possibility of X-linked congenital stationary night blindness (see Chapter 25). Acquired tilted disc and peripapillary crescent formation have been documented in children with myopic progression.

Kim TW, Kim M, Weinreb RN, et al. Optic disc changes with incipient myopia of childhood. Ophthalmology. 2012;119(1):21– 26.

Bergmeister Papilla

To varying extents, the hyaloid artery may not be resorbed before birth. The entire artery may remain as a fine thread or cord extending from the optic disc to the lens and can be associated with persistent fetal vasculature. The hyaloid artery may be patent and contain blood where it attaches to the posterior lens capsule. In mild cases, the attachment to the posterior lens capsule is located inferonasally (Mittendorf dot) and is usually visually insignificant. Bergmeister papilla is a benign prepapillary glial remnant of the hyaloid artery.

Megalopapilla

Megalopapilla features an abnormally large optic disc diameter and is often associated with an increased cup–disc ratio that can be confused with normal-tension glaucoma. Visual acuity is usually normal or slightly decreased, and visual fields may demonstrate a slightly enlarged blind spot. Megalopapilla can be unilateral or bilateral. The cause is unknown. In rare cases, megalopapilla has been associated with optic nerve glioma.

Peripapillary Staphyloma

Peripapillary staphyloma is a posterior bulging of the sclera in which the optic disc occupies the “bottom of the bowl.” It is nonhereditary and usually unilateral, with rare bilateral cases. The disc may be normal, but it is surrounded by stretched choroid, thereby exposing the white sclera encircling the disc. Visual acuity is usually poor.

Optic Nerve Aplasia

Optic nerve aplasia is rare. The affected eye lacks optic nerve axons and retinal blood vessels, making the choroidal pattern clearly visible. Unilateral optic nerve aplasia may occur with normal brain development. Bilateral optic nerve aplasia is usually associated with other CNS abnormalities.

Melanocytoma

Melanocytoma is a darkly pigmented tumor with little or no growth potential that usually involves the optic disc and adjacent retina (Fig 26-9). Malignant melanoma of the choroid is extremely rare in children.