Eales disease associated with serpiginous choroiditis.

Abstract

Report of a Case. A 49-year-old immunocompetent white man had gradual vision loss in his left eye. Visual acuity was 20/20 OD and 20/100 OS. Anterior segment examination of the left eye demonstrated small, white, central keratic precipitates but no cells or flare. Fundus examination revealed left temporal retinal vascular occlusive disease, arteriolar and venous sheathing, and peripheral retinal ischemia with neovascularization (Figure, A). The right eye was normal. Complete blood cell count, thrombophilia screen, antinuclear antibody, syphilis serology, and QuantiFERON results were normal. An anterior chamber paracentesis was negative for herpes simplex virus, varicella-zoster virus, and cytomegalovirus by polymerase chain reaction. Chest radiograph and tuberculin skin testing results were normal. Eales disease was diagnosed, scatter laser photocoagulation was applied to areas of ischemic retina, and systemic corticosteroids (60 mg/d) and mycophenolate mofetil (1.5 g/d) were prescribed. After 9 months, slowly progressive lobular peripapillary choroiditis (Figure, B), peripheral temporal retinal vascular occlusive disease with vitritis, and keratic precipitates developed in the right eye (Figure, C). Visual acuity remained 20/20. On fluorescein angiography, the area of peripapillary choroiditis revealed hyperfluorescent transmission defect and periphlebitis. Retinal neovascularization was detected at the edge of the capillary closure temporally. Bilateral Eales disease and right serpiginous choroiditis were diagnosed and the ischemic areas were photocoagulated. During the following 7 years, the capillary closure and retinal neovascularization progressed bilaterally, with development of cataract, rubeotic glaucoma, cystoid macular edema, and progressive serpiginous choroiditis with vitritis in the right eye (Figure, D-F). For this reason, bilateral intravitreal bevacizumab and triamcinolone acetonide injections and right intravitreal dexamethasone implants (Ozurdex), peribulbar triamcinolone injections, phacoemulsification with intraocular lens implantation, and pars plana vitrectomy were performed. Polymerase chain reaction results from the vitreous for Mycobacterium tuberculosis, herpes simplex virus, and varicella-zoster virus were negative. Comment. Although tuberculous infection and/or hypersensitivity has been associated with both Eales disease and serpiginous choroiditis, the evidence remains inconclusive. M tuberculosis has been detected by polymerase chain reaction from vitreous biopsies in patients with Eales disease, but the same biopsies were negative for mycobacterial cultures. In patients with systemic tuberculosis, the development of Eales disease is uncommon. A positive QuantiFERON result was detected in 11 of 21 patients with serpiginous-like choroiditis. Choroidal tuberculous lesions mimicking serpiginous choroiditis have been described and named tubercular serpiginous-like choroiditis. Previous authors believe that tubercular serpiginous-like choroiditis may be distinguishable from classic serpiginous choroiditis by the presence of vitritis and smaller, multifocal lesions in the fundus of patients from tuberculosis endemic regions. To our knowledge, the coexistence of Eales disease and serpiginous choroiditis has been reported only once before, in a 35-year-old Pakistani man with bilateral ampiginous chorioretinitis followed by unilateral Eales disease. Mantoux skin test results were positive with no active tuberculosis infection. Although an association between serpiginous choroiditis and retinal periphlebitis and/or vein occlusions was previously reported, testing for tuberculosis in these cases was either not performed or had negative results. Furthermore, in our patient, the area of Eales disease was distinct from the peripapillary serpiginous choroiditis. Our patient does not fit into either category of serpiginous disease previously described. Unlike patients with tubercular serpiginous-like choroiditis, he had a solitary, peripapillary lesion and negative results on extensive investigation for tuberculosis; unlike patients with classic serpiginous choroiditis, he exhibited bilateral intraocular inflammation with vitritis in the right eye and keratic precipitates in both eyes. Because it is improbable that these 2 rare conditions would coexist, it is possible that Eales disease and serpiginous choroiditis represent manifestations of the same underlying inflammatory disease.