Purpose: To describe the clinical characteristics of macular complications on the border of an inferior staphyloma associated with tilted disk syndrome.
Methods: We reviewed retrospectively the medical records of 32 consecutive eyes of 20 patients with tilted disk syndrome and an inferior staphyloma lying across the macula.
Results: In 21 (66%) eyes, fluorescein angiography showed window defects on the border of the staphyloma, where the early phase of indocyanine green angiography showed hypofluorescence due to atrophy of the choriocapillaris. On the late phase of indocyanine green angiography, 19 eyes (59%) showed hyperfluorescence along the border of the staphyloma, which often extended beyond the area of the window defect. Of the 32 eyes, 25 (78%) had macular complications: polypoidal choroidal vasculopathy in 7 (22%), classic choroidal neovascularization in 1 (3%), focal serous retinal detachment without polypoidal choroidal vasculopathy or choroidal neovascularization in 13 (41%), and atrophy of the retinal pigment epithelium alone in 4 (13%). Visual acuity in eyes with polypoidal choroidal vasculopathy or choroidal neovascularization was significantly worse than that in eyes with other complications (P < 0.001).
Conclusions: Eyes with tilted disk syndrome often have macular complications on the border of the inferior staphyloma, which can cause severe visual loss.