Submacular choroidal neovascularization at the margin of staphyloma in tilted disk syndrome.

PURPOSE: To clarify the nature of submacular choroidal neovascularization (CNV) and underlying choroidal circulatory disturbances in tilted disk syndrome.

METHODS

We retrospectively examined patients with submacular CNV associated with tilted disk syndrome using fluorescein angiography and indocyanine green angiography in this observational case series. The axial lengths were measured by ultrasonography. Eyes with myopia exceeding -6.0 diopters were excluded.

RESULTS

Eleven consecutive eyes (mean patient age ± standard deviation, 60.5 ± 12.1 years) were included. The axial lengths ranged from 21.7 mm to 24.6 mm (mean, 23.61 ± 0.95 mm). Classic CNV in nine eyes and polypoidal choroidal vasculopathy in two eyes developed at the macula along the superior margin of the inferior posterior staphyloma that crossed the macula in all eyes. The superior margin of the inferior posterior staphyloma was hyperfluorescent on fluorescein angiography and hypofluorescent on indocyanine green angiography, with the latter larger than the former. The inferior staphyloma contained fewer and smaller choroidal arteries and veins. During follow-up, the submacular CNV and polypoidal choroidal vasculopathy spontaneously regressed and the subretinal hemorrhage resorbed, leaving a fibrotic scar in eight eyes. Newly developed CNV at the initial no-leakage site on fluorescein angiography developed in one eye and the CNV located in the area of band-shaped hypofluorescence on indocyanine green angiography.

CONCLUSION

Stretching of the ocular wall might result in occluded choriocapillaris and degeneration of the retinal pigment epithelium. These changes along the border of the inferior posterior staphyloma predispose to submacular CNV and polypoidal choroidal vasculopathy in tilted disk syndrome.