Tilted Optic Disc Syndrome

Tilted Optic Disc Syndrome
SYMPTOMS Typically asymptomatic but patients can sometimes experience Reduced color vision and Visual field defects
SIGNS Typically bilateral
Optic disc that is typically rotated inferior nasal (65% of the time in this orientation), Chorioretinal atrophy and/or scleral/choroidal crescent, Situs inversus (found in 70% of patients), Inferior staphyloma
Patients with tilted optic discs have an increased risk of developing Choroidal neovascular membranes, Polypoidal choroidal vasculopathy, Sensory retinal detachments at the macula
WORK-UP Pupils (typically normal), EOMs, Full eye exam with dilation, Visual field (Presence of temporal field defects, usually superior temporal, that may mimic a bitemporal hemianopia or quadrantanopia but visual field defects associated with tilted optic discs can cross the vertical midline. Altitudinal or arcuate defects can also be present due to regional myopia that is associated with staphylomas), OCT of the optic nerve (typically see false thickening of the inferior nasal RNFL but false thinning of the inferior temporal RNFL), OCT of the macula (typically GCL is normal), OCT-Angiography, Fundus Autofluorescence, Fluorescein Angiography, Indocyanine Green Angiography, Fundus photos, Infrared photos, Watzke-Allen test, Macular photostress test, Amsler grid
TREATMENT Give take home Amsler grid in order to monitor for macula involvement
Tilted optic disc syndrome with no macular involvement: Monitor. No treatment is needed
Tilted optic disc syndrome with macular involvement (Choroidal neovascular membrane, Polypoidal choroidal vasculopathy, Sensory retinal detachment at the macula): Refer to a retinal specialist ASAP for further evaluation and treatment
FOLLOW-UP If monitoring, the patient should be seen back in 6-12 months
After patient is evaluated and treated by a retinal specialist and the retina/macula is stable, the patient should be seen back every 6 months
ADDITIONAL LAB | TESTS Typically none (May need to consider neuroimaging if the patient presents with a bitemporal hemianopia or quadrantanopia and a chiasmal lesion can’t be ruled out)
ETIOLOGY Congenital anomaly from incomplete closure of the embryonic optic fissure
DIFFERENTIAL DX Optic nerve hypoplasia, Oblique insertion, Glaucoma, Megalopapilla
NOTES Tilted optic discs can be found in 1-2% of the population
Optic discs can rotate or tilt in any direction (typically rotated inferior nasal)
Tilted optic discs can be associated with amblyopia as these patients tend to have high astigmatic and myopic correction (about 18% of patients with tilted optic discs have greater than or equal to 5.00D of astigmatism correction and 20% of patients with tilted optic discs have greater than or equal to 5.00D of myopic correction)