Acquired varicella-zoster virus (VZV) retinitis produces 1 of 2 patterns of retinal involvement, each with its own characteristic clinical features. In the acute retinal necrosis syndrome, affected patients are typically healthy and are initially seen with vitritis, retinal vasculitis, and confluent necrotizing retinitis preferentially affecting the peripheral retina.1 In the progressive outer retinal necrosis syndrome, patients are immunodeficient and have multifocal areas of deep retinal opacification located in the peripheral retina with or without macular involvement. They usually have a clear vitreous, no hemorrhage, and an absence of vascular inflammation.2
We recently treated a patient with the acquired immunodeficiency syndrome (AIDS) who had unilateral sudden visual loss and dense vitreous hemorrhage obscuring most retinal findings. Vitreous biopsy and laboratory analysis using polymerase chain reaction established the diagnosis of VZV retinitis.
A 28-year-old man with AIDS had a 1-day history of severe visual loss in his left eye. He complained that the vision had been blurred in the eye for several weeks, but that the previous morning he awoke with "no vision." The patient had been aware of his status as positive for the human immunodeficiency virus for 2 years. The AIDS diagnosis was based on a CD4 lymphocyte count of 4 cells per microliter (normal, >500 cells per microliter). His ocular history was unremarkable except for an episode of conjunctivitis 3 years previously.
His visual acuity was 20/20 OD and hand motions OS. The right eye was entirely normal. The left eye had small keratic precipitates and rare aqueous cells. A detailed fundus examination of the left eye was precluded because of a moderately dense vitreous hemorrhage obscuring most details. The macula was largely obscured. The optic nerve head was partially visible and peripapillary retinal edema and hemorrhage could be appreciated (Figure 1). The peripheral retina was visible with difficulty. Isolated areas could be seen with difficulty and showed intraretinal whitening and hemorrhage. No retinal breaks were present in these areas.
Fundus photograph of the left eye at initial examination. There is a moderately dense vitreous hemorrhage obscuring most details. The optic disc is partially visible, as is some peripapillary retinal edema and hemorrhage.
Echographic examination of the left eye showed an attached retina. The patient was treated with induction doses of ganciclovir for presumed cytomegalovirus retinitis. Serum was obtained and tested negative for syphilis, active toxoplasmosis, and sarcoidosis. Skin test results for tuberculosis were negative and a chest x-ray film was normal. His hemoglobin level was 100 g/L (normal, 133-171 g/L) and his platelet count was normal.
During the next few days there was no improvement in visual acuity or in the degree of vitreous hemorrhage. Because of the severe nature of the patient's ocular condition and the lack of a specific diagnosis, he underwent a pars plana vitrectomy and a vitreous biopsy 4 days after the initial examination. After removing the vitreous hemorrhage, widespread retinal opacification, intraretinal hemorrhage, and retinal necrosis were noted (Figure 2). The temporal retina was very necrotic and at the completion of the vitrectomy a large retinal defect was noted. Because of this large hole and a developing intraoperative retinal detachment, an air-fluid exchange was performed and the eye was filled with silicone oil to prevent postoperative retinal detachment.
Postoperative photograph of the left eye. There is widespread retinal opacification, hemorrhage, and necrosis. The vitreous cavity is filled with silicone oil.
Polymerase chain reaction analysis of the vitreous biopsy specimen was positive for VZV and negative for herpes simplex virus, cytomegalovirus, and toxoplasmosis. Postoperatively, the patient continued to receive intravenous ganciclovir. The retina remained attached, and retinal edema and hemorrhages resolved during the course of the antiviral therapy. The optic nerve became pale and the visual acuity fell to no light perception OS. The right eye has remained normal 1 year after the initial examination.
Spontaneous vitreous hemorrhage can occur in a variety of retinal disorders, but it is rare in ocular infections.3 Varicella-zoster virus retinitis in patients with AIDS typically manifests as progressive outer retinal necrosis with minimal hemorrhage. Our patient had some features of the progressive outer retinal necrosis syndrome, including profound immunosuppression, minimal anterior chamber reaction, rapid progression, and a poor visual outcome despite treatment. However, he had a markedly atypical presentation, with an acute, spontaneous vitreous hemorrhage. His human immunodeficiency virus status and the presence of retinal whitening in the periphery suggested an infectious cause and the need for laboratory diagnosis. This hemorrhagic variant of VZV retinitis adds to our knowledge of the various manifestations possible in this recently described condition.
This work was supported in part by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY. Dr Lewis was a Heed Foundation Fellow for 1996-1997.
Reprints: James E. Puklin, MD, Kresge Eye Institute, 4717 St Antoine, Detroit, MI 48201 (e-mail: email@example.com).
John Michael Lewis, James E. Puklin. Varicella-Zoster Virus Retinitis Presenting as an Acute Vitreous Hemorrhage. Arch Ophthalmol. 1998;116(1):104–105. doi: