Bilateral nonsimultaneous visual loss associated with idiopathic arterial pulmonary hypertension

To report ischemic central retinal vein occlusion in one eye and recurrent serous retinal detachment in the other eye of a patient with idiopathic arterial pulmonary hypertension.

A 33-year-old woman was referred to our institution 2 months after an episode of central retinal vein occlusion in her right eye. She had arterial pulmonary hypertension for 2 years and was enrolled in a double-blind clinical trial of a novel vasodilator (bosentan) for arterial pulmonary hypertension. At presentation, corrected visual acuity was hand movements in the right eye and 20/20 in the left eye. Intraocular pressure was normal in both eyes. Fundoscopy showed an optic disk with blurred margins, venous engorgement, and diffuse intraretinal hemorrhages in the right eye. The left eye was unremarkable. An afferent relative pupillary defect was also noticed on examination. Fluorescein angiography and electroretinogram confirmed the diagnosis of ischemic central retinal vein occlusion. An intravitreal injection of bevacizumab was then performed in the right eye.

The first day after injection, best-corrected visual acuity in the left eye fell to counting fingers and a serous detachment of the macula was detected. Oral acetazolamide was used, with prompt recovery of visual acuity in the left eye to 20/20 in 2 days. Another intravitreal injection of bevacizumab and panretinal photocoagulation were later performed in the right eye. Although visual acuity in the right eye improved to 20/400 2 months after bevacizumab injection, neovascular glaucoma developed in this eye. Moreover, serous retinal detachment recurred in the left eye. Topical antiglaucomatous drugs, further panretinal photocoagulation, and a new intavitreal injection of bevacizumab in the right eye as well as another course of acetazolamide controlled these complications. Final visual acuity was stable at 20/300 in the right eye and 20/20 in the left eye 2 months thereafter with normalization of intraocular pressure under hypotensive drops.

Arterial pulmonary hypertension is a rare entity, infrequently leading to decreased vision. This report emphasizes the prompt response to acetazolamide as therapy for recurrent retinal detachment in one eye as well as the use of bevacizumab for the treatment of ischemic central retinal vein occlusion in the other eye and as an adjuvant to panretinal photocoagulation for neovascular glaucoma, which later developed. The disease must be remembered in the possible etiology of central retinal vein occlusion and of serous retinal detachment in the young patient.