Purpose: To report a case of adenoma of the nonpigmented ciliary epithelium concomitant with marked neovascularization of the optic disk and cystoid macular edema (CME).
Design: Observational case report.
Methods: A 34-year-old woman presented with a nonpigmented, vascularized tumor behind the iris and neovascularization of the optic disk (NVD). Visual acuity decreased gradually as a result of the development of CME.
Results: Iridocyclectomy to excise the tumor was performed 8 months after initial examination. Histopathologic study revealed an adenoma arising from the NPCE. The levels of vascular endothelial growth factor were significantly elevated in both aqueous and vitreous humor obtained at surgery. The NVD and CME regressed after surgery, and the postoperative visual acuity improved to 20/40.
Conclusions: NVD and CME may develop secondary to adenoma of the nonpigmented ciliary epithelium. Increased levels of vascular endothelial growth factor in intraocular fluids may play a role in NVD and CME development.