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Case Report

RAPIDLY PROGRESSIVE NEOVASCULAR GLAUCOMA FROM CYTOMEGALOVIRUS RETINITIS IN A NON–HUMAN IMMUNODEFICIENCY VIRUS PATIENT

Cho, Jane MD; Siegel, Nicole MD; Subramanian, Manju L. MD; Ying, Howard MD, PhD; Ness, Steven MD

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Retinal Cases & Brief Reports: Winter 2021 - Volume 15 - Issue 1 - p 62-64
doi: 10.1097/ICB.0000000000000734
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Cytomegalovirus (CMV) retinitis in human immunodeficiency virus (HIV)-negative patients is becoming more prevalent with increasing use of systemic immunosuppression therapy for various reasons. We present a case of a non–HIV-related CMV retinitis with severe ischemia atypical of the classic CMV retinitis complicated by neovascular glaucoma.

Case Report

A 71-year-old man presented to the emergency department to be evaluated for 2-week history of right eye photophobia, floaters, and decreased vision. The patient's medical history was significant for multiple myeloma diagnosed 10 years previously with history of stem-cell transplant, currently on monthly infusions of daratumumab (monoclonal CD38 antibody) in combination treatment with dexamethasone. His visual acuity in the right eye was 20/70 and the left unaffected eye was 20/20. His intraocular pressure in the right eye was 18 and the left eye was 15 mmHg. On examination of the right eye, there was 1+ cell in the anterior chamber, 2+ cell in the anterior vitreous with strands of vitreous debris overlying the optic nerve. There was a granular yellow retinal lesion with central clearing in the superotemporal periphery, scattered dot hemorrhages, and diffuse arterial vascular sheathing surrounded by hemorrhage in all quadrants (Figure 1). The examination of the left eye was unremarkable with no cell in the anterior chamber, vitritis, nor retinal lesions.

Fig. 1.
Fig. 1.:
Initial wide-field fundus photograph of the right eye. Presence of granular retinal lesion with central clearing in the superotemporal periphery, vitritis, scattered retinal hemorrhages, and diffuse arterial vascular sheathing surrounded by hemorrhage in all quadrants.

Because of a high suspicion for viral retinitis in the setting of immunosuppression, the decision was made to treat with an intravitreal injection of foscarnet (2.4 mg/0.1 mL). A vitreous tap was sent for polymerase chain reaction to evaluate for CMV, varicella zoster virus, and herpes simplex virus. Infectious disease was consulted and the patient was admitted for rapid induction of intravenous ganciclovir until the infectious workup was finalized. The vitreous sample polymerase chain reaction was positive for CMV and negative for varicella zoster virus and herpes simplex virus. The remainder of the infectious workup was negative including HIV Ag/Ab with CD4 count of 450/mm3.

Over the next 10 days, the vision continued to deteriorate, eventually falling to hand motion, despite treatment with repeated intravitreal foscarnet injections and intravenous ganciclovir. Examination revealed regression of the peripheral retinitis (Figure 2), but a fluorescein angiogram revealed severe and diffuse arteriolar nonperfusion (Figure 3). The patient was discharged to continue intravenous ganciclovir 5 mg·kg−1 per dose q12h at home for a total of 3 weeks followed by 4 weeks of oral ganciclovir 900 mg daily.

Fig. 2.
Fig. 2.:
Wide-field fundus photograph of the right eye 11 days after onset of treatment. The retinitis appears less active but there is persistent vitritis, increased perivascular retinal hemorrhages, and macular whitening from ischemia.
Fig. 3.
Fig. 3.:
A late-phase fluorescein angiography of the right eye. There is scattered blockage from retinal hemorrhages, poor vascular perfusion with incomplete filling only up to the proximal arcades, and superotemporal granular hyperfluorescence at area of retinitis.

At follow-up examination about 2 weeks after presentation, he was found to have vitreous hemorrhage with concerns for possible peripheral retinal detachment, so vitrectomy surgery was recommended. On the day of surgery, the patient complained of worsening eye pain and intraocular pressure was measured to be 60 mmHg. Vitrectomy revealed dense vitreous hemorrhage without detachment and was complicated by anterior chamber bleeding because of neovascularization of the iris which was treated with panretinal photocoagulations and intravitreal injection of bevacizumab. Postoperatively, his intraocular pressure remained elevated despite maximum medical therapy and his visual acuity remained to be hand motion.

Discussion

In the age of highly active antiretroviral therapy for treatment of HIV, the prevalence of CMV retinitis has dramatically decreased. Classic CMV retinitis typically presents as a focal or multifocal area of retinal necrosis associated with retinal hemorrhage and nonocclusive phlebitis.1 Neovascular complications of classic HIV-related CMV retinitis have been reported but are unusual.2–5 The granular peripheral retinitis in our case mimicked the classic presentation, but it was differentiated by a panretinal arteritis and significant vitritis. In fact, some of these features seem to fit the criteria of acute retinal necrosis as defined by the American Uveitis Society and this case could well represent a case of CMV acute retinal necrosis.6

Recently, there are increasing case reports of CMV retinitis in non-HIV patients with atypical features.7 Schneider et al8 termed the phrase “chronic retinal necrosis” in their description of five cases of smoldering CMV retinitis associated with significant vitritis and panretinal vasculitis more typical of acute retinal necrosis. These patients were reported to have symptoms for weeks to months and some were observed to have no progression of retinitis even without appropriate antiviral coverage. Neovascular complications were described in four of five patients in this case series.

In our case, however, neovascular glaucoma developed acutely within several weeks of presentation despite early, aggressive treatment with appropriate intravitreal and systemic antiviral therapy. Although treatment was sufficient to control the retinitis, it was not able to reverse the arteritis, suggesting either early permanent vascular occlusion or a separate inflammatory mechanism of vasculitis independent of viral activity. It is recognized that patients with CMV retinitis in the non-HIV group may have higher rates of neovascularization.8,9 In addition, it is possible that our patient's underlying blood dyscrasia may have contributed to retinal ischemia, although there were no perfusion abnormalities found on angiography of the contralateral eye.

In summary, CMV retinitis in immunocompetent or partially immunocompromised patients may have a clinical picture intermediate between herpes simplex virus/varicella zoster virus–related acute retinal necrosis and classic CMV retinitis. Although previous reports have described the chronic development of neovascular complications in these patients, our case demonstrates that in cases of severe retinal ischemia related to CMV retinitis, neovascular glaucoma may develop rapidly, even with appropriate antiviral therapy. These patients should be evaluated with fluorescein angiography, and we would urge early treatment with panretinal photocoagulation before the development of neovascular complications.

References

1. Wiegand TW, Young LH. Cytomegalovirus retinitis. Int Ophthalmol Clin 2006;46:91–110.
2. Babu K, Murthy KR, Sudarshan S, Biswas J. Bilateral arteritis with cytomegalovirus retinitis in a patient infected with human immunodeficiency virus. Retin Cases Brief Rep 2010;4:31–33.
3. Levin AV, Zeichner S, Duker JS, et al. Cytomegalovirus retinitis in an infant with acquired immunodeficiency syndrome. Pediatrics 1989;84:683–687.
4. Bogie GJ, Nanda SK. Neovascularization associated with cytomegalovirus retinitis. Retina 2001;21:85–87.
5. Lee S, Ai E. Disc neovascularization in patients with AIDS and cytomegalovirus retinitis. Retina 1991;11:305–308.
6. Holland GN. Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society. Am J Ophthalmol 1994;117:663–667.
7. Davis JL, Haft P, Hartley K. Retinal arteriolar occlusions due to cytomegalovirus retinitis in elderly patients without HIV. J Ophthalmic Inflamm Infect 2013;3:17.
8. Schneider EW, Elner SG, Van Kuijk FJ, et al. Chronic retinal necrosis: cytomegalovirus necrotizing retinitis associated with panretinal vasculopathy in non-HIV patients. Retina 2013;33:1791–1799.
9. Pathanapitoon K, Tesavibul N, Choopong P, et al. Clinical manifestations of cytomegalovirus-associated posterior uveitis and panuveitis in patients without human immunodeficiency virus infection. JAMA Ophthalmol 2013;131:638–645.
Keywords:

cytomegalovirus retinitis; immunosuppressed; neovascular glaucoma.

Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.