Adults with cicatricial retinopathy of prematurity (ROP) may develop chronic angle-closure glaucoma1,2 due to steeper corneas, shallower anterior chambers, and larger lens-to-axial length ratios than normal eyes.3,4 We report a case of chronic angle-closure glaucoma with prominent iris vessels mimicking neovascular glaucoma in an adult with cicatricial ROP.
A 38-year-old man, born at 7 months' gestation, had useful vision only in his right eye but was otherwise healthy. Cicatricial ROP and normal intraocular pressure (IOP) were noted on ophthalmic examination at our clinic 5 years earlier. For 1 year he had intermittent right-sided headaches progressing to become daily, and resolving after sleep, that were accompanied by redness and blurred vision in his right eye. His symptoms became constant and his peripheral vision declined; when he was no longer able to read, he was examined. Visual acuity was 20/400 OD and light perception OS. Slitlamp examination of the right eye revealed moderate conjunctival hyperemia, microcystic corneal edema, a peripherally shallow but centrally deep anterior chamber, and a posterior subcapsular cataract. Large arborizing blood vessels were visible on the iris surface. Goldmann applanation tonometry was 44 mm Hg OD, 17 mm Hg OS. Dilated fundus examination of the right eye revealed temporal dragging of the retina, and no apparent macula. In the left eye no details were visible, because the subluxed, cataractous lens filled the irregular pupil.
One day after treatment with isosorbide, acetazolamide, and topical glaucoma medications, the IOP was 10 mm Hg OD. Gonioscopy revealed complete angle closure without neovascularization, but the iris vessels remained prominent. The clearer fundus view confirmed the absence of retinal hemorrhages, detachment, or neovascularization; the optic nerve was dragged temporally and cupped. Blood glucose concentration and carotid pulses and auscultation were normal. Frequent 1% prednisolone acetate was added in anticipation of laser peripheral iridotomy. Two days later the IOP was 50 mm Hg OD, but the iris vessel caliber had decreased greatly (Figure 1). Despite neodymium:YAG laser iridotomy, the IOP became refractory to medical treatment, and trabeculectomy with mitomycin was performed. Six months later the IOP was 12 mm Hg OD without medications; no iris vessels were visible.
Iris vessels of the patient described are greatly decreased in caliber but still visible 2 days after beginning topical corticosteroid treatment.
Rubeosis irides is rare in the absence of retinal or ocular ischemia; active ROP in neonates could conceivably provide the necessary stimulus.5 Michael et al2 reported 2 cases of adult-onset neovascular glaucoma associated with cicatricial ROP. In one patient no fundus examination was described, and the second patient had cicatricial changes without mention of detachment or acute ischemic changes (such as hemorrhages or neovascularization), and may represent a case similar to ours. Our patient's history and ophthalmic examination were consistent with chronic angle-closure glaucoma. Neither retinal detachment nor signs of acute retinal ischemia were evident. The rubeosis responded rapidly to topical corticosteroid treatment and was presumably the result of inflammatory congestion of preexisting vascular channels, perhaps formed in conjunction with "plus" disease in infancy, rather than a true neovascular response to angiogenic factors. Panretinal photocoagulation or cryotherapy usually is considered as part of treatment for neovascular glaucoma but should be deferred in cases similar to ours.
Corresponding author: Philip P. Chen, MD, Department of Ophthalmology, University of Washington, Box 356485, Seattle WA 98195-6485 (e-mail firstname.lastname@example.org).
Chen PP, Kalina RE. Chronic Angle-closure Mimicking Rubeotic Glaucoma in an Adult With Retinopathy of Prematurity. Arch Ophthalmol. 1998;116(9):1248. doi: