Tilted disc syndrome.
BACKGROUND: Tilted disc syndrome (TDS) has a varied morphological appearance which can be difficult to differentiate from acquired optic nerve pathology. In addition, there are visual deficits and potential complications associated with this syndrome. Failure to recognize features of the TDS frequently leads to unnecessary medical evaluation or improper examination for possible associated ocular complications. METHODS: The literature is reviewed to examine the embryonic developmental defects that create TDS. Characteristic ophthalmoscopic features of the disc, visual field deficits, electrofunctional abnormalities, retinal pigment epithelial and choroidal hypoplasia, refractive error, and choroidal neovascular development are examined from a clinical standpoint. RESULTS: The literature clearly identifies visual deficits and ocular complications, which must be understood in the context of TDS. CONCLUSIONS: TDS is a congenital anomaly that has visual deficits and an appearance that can mimic serious neurological disease. Also, there are potential vision-threatening complications that are typically not associated with a congenital anomaly.