Walsh & Hoyt: Congenital Tilted Disc Syndrome

The tilted disc syndrome is a nonhereditary, usually bilateral condition in which the superotemporal optic disc is elevated and the inferonasal disc is posteriorly displaced, resulting in an oval-appearing optic disc, with its long axis obliquely oriented. This configuration is accompanied by situs inversus of the retinal vessels, congenital inferonasal conus, thinning of the inferonasal RPE and choroid, and bitemporal hemianopia that does not respect the vertical midline. Histopathologically, the optic nerve enters at an extremely oblique angle, the superior or superotemporal portion of the disc is elevated, and there is posterior ectasia of the inferior or inferonasal fundus and optic disc.