LAUSR

A 39-year-old white woman presented in April 2008 with gradual painless decrease in vision in her right eye over the previous 2 months, which she described as "fuzzy." She denied redness, photophobia, or photopsia in her right eye and had no complaints regarding the left eye. She had been previously diagnosed with Human T-Lymphotropic virus type 1 (HTLV-1)–associated adult T-cell leukemia/lymphoma (ATL) and had undergone stem cell transplant 3 years previously and was currently in remission. She had been followed by the cornea service for graft-versus-host disease screening but had no ocular surface involvement. Her medical history was remarkable for multiple infectious complications, including pseudomonal bacteremia in 2004, streptococcal bacteremia in 2005, cytomegalovirus (CMV) reactivation in 2005, alternaria/ moraxella sinusitis in 2007, bronchiolitis obliterans organizing pneumonia in 2007, and a recent sinus infection within the past week, in addition to hypertension, mucocutaneous graft versus host disease (GVHD), and placement of an indwelling pheresis catheter. She was currently taking ceftin for sinusitis, combivent, prednisone (15 mg daily), prophylactic azithromycin, advair and xanax, and hydrocodone as needed. Aside from her ocular symptoms, her only other accompanying symptom was fever to 102.8°F likely from sinusitis, for which she was being treated. On examination, visual acuity was 20/70 in the right eye and 20/ 20 in the left eye, and a relative afferent pupillary defect was noted in her right eye. Visual fields by confrontation revealed nasal and inferior field loss and diminished color vision in her right eye (3/11 on the right vs. 11/11 Ishihara color plates on the left). Slit-lamp examination revealed a normal anterior chamber. Specifically, no cell or flare was noted. On fundus examination, vitritis with membrane formation and optic nerve head edema with perivascular retinal infiltrates in the posterior pole were detected in the right eye (Figures 1 and 2). Fluorescein angiography revealed dilated and leaking vessels on the disk and surrounding areas with minimal nonperfusion in the right eye (Figure 3). The left eye was normal on clinical examination and imaging. Magnetic resonance angiography of the brain and orbits showed normal and stable findings. Multiple cerebrospinal fluid cytology samples, serum toxoplasma and rapid plasma reagin (RPR), and vitreous taps for polymerase chain reaction (PCR) of CMV, herpes simplex virus, and herpes zoster virus performed three days after presentation were all negative. She was started on systemic ganciclovir without noticeable response at 2 weeks. After this, she was placed on 60 mg of oral prednisone with minimal response noted at 2 weeks. With continued worsening of vision and increasing vitritis, a diagnostic vitrectomy with membrane peel was performed at 4 weeks. Bacterial, viral, and fungal cultures of the vitreous were unrevealing, and PCR for parvovirus B19, herpes simplex virus 1, herpes simplex virus 2, herpes virus 6 (H6D), herpes virus 7 (H7D), herpes zoster virus, CMV, and human immunodeficiency virus were all negative. Polymerase chain reaction testing for HTLV-1 and HTLV-2 were unavailable. Cytology showed a mixed inflammatory response thought to be reactive in character. After vitrectomy, the perivascular infiltrates were better visualized (Figure 4). The patient’s visual acuity decreased to 20/100 during this time, and she was started empirically on oral methotrexate (15 mg weekly) for a presumed autoimmune vasculitic process before her leaving town on a trip. When she returned 6 weeks later, her visual acuity was count fingers at one foot.