Figure 1. This fundus photo of the right eye shows a large area of retinal whitening with hemorrhages in the superotemporal macula 1 disc-diameter from the fovea, scattered dot blot hemorrhages and exudates.
Figure 2. A Fundus photo of the left eye shows retinal pigment epithelial changes, few punctate foci of retinal whitening, peripheral retinal whitening, and a few dot blot hemorrhages.
Figure 3: An OCT of the right eye reveals temporal and superotemporal retinal thickening and loss of layers.
Figure 4: This OCT of the left eye reveals inferior inner retinal irregularities.
A 64-year-old man presented to the emergency department with eye pain and central vision loss in the right eye and new floaters in the left eye for 2 weeks. He had a history of a renal transplant for IgA nephropathy 20 years prior to his presentation, for which he was managed using infusions of mycophenolate mofetil, prednisone, and belatacept. His past medical history was significant for chronic kidney disease stage IV, hypertension, hyperlipidemia, type II diabetes, heart failure with preserved ejection fraction, and atrial fibrillation for which he was treated with apixaban.
On examination, his Snellen visual acuity was 20/30 in both eyes. His anterior segment exam was significant for 2+ nuclear sclerosis with no anterior cell. Dilated fundus exam of the right eye showed a large area of retinal whitening with perivascular hemorrhages in the superotemporal macula (Figure 1). Left fundus exam showed retinal pigment epithelial changes, peripheral retinal whitening, and a few dot blot hemorrhages (Figure 2). Both eyes had sclerotic vessels in the macula.
Optical coherence tomography (OCT) was notable for temporal and superotemporal full thickness retinal inflammation with some areas of thinning in the right eye (Figure 3), and inferior inner retinal irregularities in the left eye (Figure 4).
Cytomegalovirus (CMV) retinitis is a devastating ocular manifestation of CMV and the most common cause of retinitis in an immunocompromised individual.1,2 Patients with CMV retinitis are usually asymptomatic but can present with floaters, paracentral scotomas, and decreased visual acuity, as...
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Cytomegalovirus (CMV) retinitis is a devastating ocular manifestation of CMV and the most common cause of retinitis in an immunocompromised individual.1,2 Patients with CMV retinitis are usually asymptomatic but can present with floaters, paracentral scotomas, and decreased visual acuity, as in our patient who was immunosuppressed after his renal transplant.3 Bilateral involvement can occur in 40% of cases, as it did our case.3 Fundoscopic appearance is key to the diagnosis of CMV retinitis. Classically, the patient will have yellow-white retinal necrosis with undefined edges.3,4 Perivascular sheathing with associated hemorrhage is a common clinical manifestation, as seen in this patient.3,4
A broad differential diagnosis of CMV retinitis includes HIV retinopathy, toxoplasmosis, syphilis, tuberculosis, pneumocystis, cryptococcus, choroidal bacterial seeding, acute retinal necrosis syndrome, and progressive outer retinal necrosis .2
Progressive outer retinal necrosis is a common masquerader of CMV, but its clinical progression is more rapid and it usually spares the retinal vessels, unlike CMV.4 Toxoplasmosis retinochoroiditis may also mimic CMV. Features that distinguish toxoplasmosis from CMV include prominent anterior chamber and vitreous inflammation and absence of retinal hemorrhages.5 Toxoplasmosis retinal lesions classically have smooth demarcated borders while CMV lesions will display ill-defined “granular” borders.5
An anterior chamber paracentesis with viral PCR testing is a valuable diagnostic measure to confirm the diagnosis of CMV and rule out infectious etiologies that may mimic CMV retinitis.6 Our patient underwent anterior chamber paracentesis in both eyes sent and aqueous polymerase chain reaction returned positive for CMV (96,000 copies/mL in the right eye, and 80,000 copies/mL in the left eye).
Primary treatment of CMV retinitis is oral valganciclovir, with either intravitreal ganciclovir or intravitreal foscarnet if the CMV retinitis is sight-threatening.5 This patient was treated with intravitreal injections of ganciclovir 2mg/0.1mL and oral valganciclovir 450 mg. On his most recent follow-up visit, his vision was stable, 20/50 in the right eye and 20/40 in the left eye.
This case was submitted by Sloane McTavish, BS and Sandra Hoyek, MD. Nimesh A. Patel, MD was this case’s clinical editor.
Sloane McTavish is a medical student at the University of Virginia, Charlottesville, Virginia.
Dr Hoyek is a postdoctoral research fellow in Ophthalmology at Massachusetts Eye and Ear and Harvard Medical School, Boston.
Dr Patel is an Instructor of Ophthalmology at Massachusetts Eye and Ear and Harvard Medical School and Director of Pediatric Retina at Boston Children’s Hospital, Boston
1. Munro M, Yadavalli T, Fonteh C, Arfeen S, Lobo-Chan AM. Cytomegalovirus retinitis in HIV and non-HIV individuals. Microorganisms. 2019;8(1):55. doi:10.3390/microorganisms8010055
2. Cytomegalovirus retinitis: Differential diagnosis. American Academy of Ophthalmology. Retrieved July 13, 2022. www.aao.org/focalpointssnippetdetail.aspx?id=dec9f690-e681-456e-8f2d-3f57f33ddb93
3. Friedman NJ, Kaiser PK, Pineda, R. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. 5th ed. Elsevier; 2019.
4. Jacobson M. Pathogenesis, clinical manifestations, and diagnosis of AIDS-related cytomegalovirus retinitis. UpToDate. Published November 2, 2021. Accessed July 13, 2022. www.uptodate.com/contents/pathogenesis-clinical-manifestations-and-diagnosis-of-aids-related-cytomegalovirus-retinitis
5. Elkins BS, Holland GN, Opremcak EM, et al. Ocular toxoplasmosis misdiagnosed as cytomegalovirus retinopathy in immunocompromised patients. Ophthalmol. 1994;101(3):499-507. doi:10.1016/s0161-6420(13)31267-6
6. Smith IL, Macdonald JC, Freeman WR, Shapiro AM, Spector SA. Cytomegalovirus (CMV) retinitis activity is accurately reflected by the presence and level of cmv dna in aqueous humor and vitreous. J Infect Diseases. 1999;179(5):1249–1253. doi:10.1086/314710