Cytomegalovirus (CMV) retinitis is usually an opportunistic viral infection, found in individuals with low immunity. This condition results in the inflammation of the retina, and can eventually lead to blindness.
Presentation
Cytomegalovirus infection commonly occurs in adults during their fourth decade of life. The retina can be affected when the virus spreads via the bloodstream [1]. People infected with the human immunodeficiency virus (HIV) are at a higher risk of acquiring cytomegalovirus retinitis, as the former organism compromises the endothelial integrity of vasculature, and blood flow, in the retina [2]. Retinitis may involve inflammation as well as bleeding of the retina, often in the ocular fundus. Lesions such as cotton wool spots can be visualized on the retina.
HIV further predisposes individuals to cytomegalovirus retinitis due to its immunosuppressive effects. This results in activation of latent infection after the host immunity decreases [3]. Various conditions, such as diabetes mellitus, malignancy, and organ transplantation, impart the same effect [4] [5]. Although cytomegalovirus retinitis in immunocompetent individuals is rare, cases have been reported. It has also been described in patients with systemic lupus erythematosus and other autoimmune diseases, with concomitant use of immunosuppressive drugs [6].
Cytomegalovirus retinitis can be asymptomatic. In symptomatic infection, patients may experience various visual disturbances, including floaters, photopsia, narrowed visual fields, blurred vision, and scotoma [7]. They do not usually complain of red or painful eyes. Blindness can ensue within months of symptom onset. Furthermore, cytomegalovirus retinitis is often initially unilateral before becoming bilateral.
Notably, immunocompromised children do not seem to have increased susceptibility to this disease [8]. Cytomegalovirus retinitis can also be congenital, occurring in 25% of babies already exhibiting other features of CMV infection, and does not usually worsen after birth.
Eyes
- Retinal Hemorrhage
The patient was diagnosed to have acute onset unilateral CMV retinitis. Retinal examination uncovered classical appearance of retinal whitening and retinal hemorrhages with extensive macular involvement. [ncbi.nlm.nih.gov]
The retinitis begins as small, white perivascular retinal infiltrates or dot-blot hemorrhages. The classic lesion is a hemorrhagic necrotizing retinitis that follows the retinal vasculature. [eyerounds.org]
File Size: 766 KB Ocular Pathology/Oncology Cytomegalovirus (CMV) retinitis. A, Retinal hemorrhages and areas of opaque retina are present. Note the vascular sheathing in the superotemporal arcade and hard exudates in the macular region. [aao.org]
The retinitis is detected with ophthalmoscopy as either a perivascular yellow-white retinal lesion frequently associated with retinal hemorrhage or as a focal white granular infiltrate, often without hemorrhage. [annals.org]
- Retinal Pigmentation
(n = 3 eyes) characterized by inner retinal hyperreflectivity, large empty spaces in outer nuclear layer, and bridges of retinal tissue but retinal pigment epithelium and choriocapillaris sparing. [ncbi.nlm.nih.gov]
- Retinal Scar
Fibrovascular changes occurred in the CMV retinitis scar, were misdiagnosed as CMV retinitis reactivation, and were treated with anti-CMV medication. [ncbi.nlm.nih.gov]
Two patients were not treated (patients 1 and 8), one of whom (patient 8) had received an autologous transplant and was diagnosed with atrophic retinal scarring compatible with previous CMV active retinitis after spontaneous resolution of the acute retinitis [academic.oup.com]
Clinical manifestations of CMV retinitis had altered after treatment comparing to clinical signs pre-treatment. In recovered patients, hemorrhage and exudation were disappeared completely and yellow-white retinal scars were formed in 10 eyes. [bmcinfectdis.biomedcentral.com]
- Unilateral Blindness
However, there is a risk of unilateral blindness in severe cases. [patient.info]
Neurologic
- Radiculitis
[…] to treat affected patients with systemic anti-CMV medication, has an unfavorable impact on AIDS-related morbidity and mortality, because CMV is a systemic disease [ 42 ], and the extraocular forms of CMV, including colitis, esophagitis, encephalitis, radiculitis [doi.org]
[…] to treat affected patients with systemic anti-CMV medication, has an unfavorable impact on AIDS-related morbidity and mortality, because CMV is a systemic disease [42], and the extraocular forms of CMV, including colitis, esophagitis, encephalitis, radiculitis [ncbi.nlm.nih.gov]
Workup
The ophthalmologic examination is the diagnostic procedure employed. This involves dilating the pupils to visualize internal ocular structures through ophthalmoscopy.
CMV infection can be detected through biochemical analysis of blood and urine samples. Furthermore, a biopsy would reveal viral organisms in the tissue, however, this is not a routine procedure.
It is good practice to screen all patients at risk of cytomegalovirus retinitis, including those that lack symptoms, because early stage diagnosis and treatment are uncomplicated, and in addition, a significant proportion of individuals are asymptomatic at the time of diagnosis. Furthermore, intervention prevents blindness, which is a highly debilitating sequela of cytomegalovirus retinitis. Screening via testing visual acuity of patients using a Snellen chart is insufficient, as only a small portion of the retina, the fovea, is assessed in this way. The use of questionnaires specifically tailored to detect or elicit signs and symptoms of cytomegalovirus retinitis can be a more effective screening tool.
HIV patients have a higher risk of cytomegalovirus retinitis, and it is reported that the latter is the most frequently encountered opportunistic viral infection in the above group, especially when CD4 (cluster of differentiation 4) cell count is extremely low, that is, less than 50 per cubic millimeter [9]. Immunocompetent individuals, especially those who have received steroid injections, make up the minority of cytomegalovirus retinitis patients [10] [11].
Other Pathologies
- Viral Inclusion Body
Two cells (centre-left) have the characteristic (cytoplasmic) viral inclusion bodies (small pink globules ). H&E stain. CMV infection can be demonstrated microscopically by the detection of intranuclear inclusion bodies. [en.wikipedia.org]
Treatment
Treatment was again withheld after diagnosis of retinitis because of immunocompetent status, potential side effects of ganciclovir treatment, and location of retinitis in the retinal periphery of both eyes. [ncbi.nlm.nih.gov]
We believe that systemic treatment with oral valganciclovir should be used routinely as the primary treatment strategy because (1) systemic treatment of CMV retinitis reduces extraocular CMV disease [ 39 ]; (2) systemic treatment reduces mortality [ 40 [doi.org]
Prognosis
CONCLUSION: The overall visual prognosis and the clinical features of CMV retinitis do not differ between HIV and non-HIV patients. The visual prognosis of CMV retinitis still remains quite poor despite advancements in antiviral treatment. [ncbi.nlm.nih.gov]
There is no treatment but the prognosis is excellent. [patient.info]
Treatment and prognosis The treatment for CMV retinitis involves administration of antiviral medications (e.g. valganciclovir, ganciclovir, foscarnet, or cidofovir). [radiopaedia.org]
[…] infection of the eye, a common opportunistic infection in AIDS, a complication of disseminated CMV infection Clinical Retinal inflammation, blindness Management Antivirals–eg, foscarnet, cidofovir, gancyclovir, systemically and as a ganciclovir implant Prognosis [medical-dictionary.thefreedictionary.com]
Etiology
This case provides further evidence implicating CMV infection in the etiology of hypertensive iridocyclitis. [ncbi.nlm.nih.gov]
One or more of the following clinical manifestations may be present: CMV mononucleosis CMV pneumonia: interstitial pneumonitis Etiology: immunocompromised patients (e.g., following bone marrow transplant or in HIV/AIDS patients with CD4 ≤ 50 cells/mm3 [amboss.com]
Etiology Etiology is currently unknown. Genetic studies have investigated numerous genes involved in neuronal development as well as candidate genes of hypothalamic and autonomic dysfunction without identifying any specific mutation. [orpha.net]
Epidemiology
Division of Laboratory Medicine, University Hospital of Geneva and Medical School, University of Geneva, Geneva hDivision of Infectious Diseases and Hospital Epidemiology, University Hospital Basel, Basel, Switzerland. [ncbi.nlm.nih.gov]
Epidemiology Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
Herein, we describe the pathogenesis of CMVR and compare clinical features, epidemiology, and risk factors in HIV and non-HIV infected individuals with CMVR. [mdpi.com]
However, there is the lack of epidemiological report on the incidence of CMV retinitis in the AIDS patients in China. [bmcinfectdis.biomedcentral.com]
Pathophysiology
Etiology Pathogen: cytomegalovirus (CMV, human herpes virus 5, HHV-5) Transmission Blood transfusions Sexual transmission Droplet transmission Transplant-transmitted infection (e.g., bone marrow, lungs, kidneys) References:[1][2][3] Pathophysiology Clinical [amboss.com]
Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology. [books.google.es]
Prevalence, pathophysiology and treatment of rhegmatogenous retinal detachment in treated cytomegalovirus retinitis.Am J Ophthalmol. 1987; 103: 527-536 Holland GN Sakamoto MJ Hardy J et al. [amjmed.com]
The pathophysiology of delirium is not fully understood, and the condition might arise through a variety of different pathogenic mechanisms. [ncbi.nlm.nih.gov]
Prevention
Guidelines for the prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: Recommendations from the Centers for Disease Control and Prevention, the National Institutes of Health, and the HIV Medicine Association of [journals.sbmu.ac.ir]
CAN CMV BE PREVENTED? The best way to prevent CMV disease is to get tested and start HIV treatment before CD4 counts are low. Ganciclovir was approved for prevention (prophylaxis) of CMV. However, many health care providers don’t prescribe it. [aidsinfonet.org]
This multicenter, prospective, observational study of patients with AIDS is identifying key combinations of drug treatments to prevent blinding eye disease in AIDS patients.. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. .. . [medschool.lsuhsc.edu]
This study also examines the effectiveness of valganciclovir in preventing the recurrence of CMV retinitis. [clinicaltrials.gov]
Systematic screening of HIV patients with CD4 counts below 100 cells/μl should be carried out to detect disease at an early stage, when blindness can still be prevented. [ncbi.nlm.nih.gov]
References
- López-Contreras J, Ris J, Domingo P, Puig M, Rabella N, Nolla J. Disseminated cytomegalovirus infection in an immunocompetent adult successfully treated with ganciclovir. Scand J Infect Dis. 1995;27(5):523-525.
- Glasgow BJ, Weisberger AK. A quantitative and cartographic study of retinal microvasculopathy in acquired immunodeficiency syndrome. Am J Ophthalmol. 1994;118(1):46-56.
- Britt W. Manifestations of human cytomegalovirus infection: proposed mechanisms of acute and chronic disease. Curr Top Microbiol Immunol. 2008;325:417-470.
- Bhat V, Joshi A, Sarode R, Chavan P. Cytomegalovirus infection in the bone marrow transplant patient. World J Transplant. 2015;5(4):287–291.
- Vertes D, Snyers B, De Potter P. Cytomegalovirus retinitis after low-dose intravitreous triamcinolone acetonide in an immunocompetent patient: a warning for the widespread use of intravitreous corticosteroids. Int Ophthalmol. 2010;30(5):595-597.
- Kelkar A, Kelkar J, Kelkar S, Bhirud S, Biswas J. Cytomegalovirus retinitis in a seronegative patient with systemic lupus erythematosus on immunosuppressive therapy. J Ophthalmic Inflamm Infect. 2011;1(3):129–132.
- Wei LL, Park SS, Skiest DJ. Prevalence of visual symptoms among patients with newly diagnosed cytomegalovirus retinitis. Retina. 2002;22(3):278-282.
- Baumal CR, Levin AV, Kavalec CC, Petric M, Khan H, Read SE. Screening for CMV retinitis in children. Arch Pediatr Adolesc Med. 1996;150(11):1186-1192.
- Jabs DA. Ocular manifestations of HIV infection. Trans Am Ophthalmol Soc. 1995;93:623-683.
- Gupta S, Vemulakonda GA, Suhler EB, et al. Cytomegalovirus retinitis in the absence of AIDS. Can J Ophthalmol. 2013;48(2):126-129.
- Saidel MA, Berreen J, Margolis TP. Cytomegalovirus retinitis after intravitreous triamcinolone in an immunocompetent patient. Am J Ophthalmol. Am J Ophthalmol. 2005;140(6):1141-1143.