GET FAMILIAR WITH INFECTIOUS RETINITIS I once heard that an astute clinician can make the right diagnosis 90% of the time based only on a thorough case history. Testing myself to find a diagnosis based on presenting symptoms and clinical data alone is one of my favorite parts of clinical care. Understanding that drifting spots in a patient’s vision are more likely to be caused by vitreous floaters or a posterior vitreous detachment than by an infectious process is just playing the odds and a part of being a good doctor. With that in mind, however, it is important to remember that infectious retinal diseases, although rare, do exist. They may present with common visual symptoms such as blurred vision or floaters. But infectious retinitis can also be present in a completely asymptomatic patient. Because there are so many potential causes for infectious retinal disease— bacterial, viral, parasitic, fungal, and tick-borne—prompt recognition of clinical signs and risk factors, along with proper testing, is necessary for successful diag- nosis and management. SIGNS, SYMPTOMS, AND RISK FACTORS Some causes of infectious retinitis are potentially devastating, not only because these diseases can lead to permanent vision loss, but because they are often the clinical manifestation of a systemic illness. For example, cytomegalovirus (CMV) retinitis classically affects immune-compromised individuals with HIV. 1,2 It is important to remember, though, that infections of the retina can affect immune- competent patients as well, such as in cases of acute retinal necrosis (ARN), cat- scratch disease, and Lyme disease. 3-5 A patient with retinitis often presents with complaints of ocular pain (ARN, toxoplasmosis), vision loss, and/or new floating spots in his or her vision. 6 However, some patients remain completely asymptomatic and may show up only for a routine examination or eyeglass prescription update. 7 Familiarity with key clinical signs and risk factors is the main driving force behind finding a proper diagnosis (see Key Signs, Symptoms, and Risk Factors of Infectious Retinitis). Prompt diagnosis is key for successful management of this rare yet potentially devastating condition. BY JOSEPH J. ALLEN, OD, FAAO COVER FOCUS RETINAL DISORDERS & DISEASES
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28 | SEPTEMBER 2019
GET FAMILIAR WITH INFECTIOUS RETINITIS
Ionce heard that an astute clinician can make the right diagnosis 90% of the time based only on a thorough case history. Testing myself to find a diagnosis based on presenting symptoms and clinical data alone is one of my favorite parts of clinical care. Understanding that drifting spots in a patient’s vision are more likely to be caused by vitreous floaters or a posterior vitreous detachment than
by an infectious process is just playing the odds and a part of being a good doctor.With that in mind, however, it is important to remember that infectious
retinal diseases, although rare, do exist. They may present with common visual symptoms such as blurred vision or floaters. But infectious retinitis can also be present in a completely asymptomatic patient.
Because there are so many potential causes for infectious retinal disease— bacterial, viral, parasitic, fungal, and tick-borne—prompt recognition of clinical signs and risk factors, along with proper testing, is necessary for successful diag-nosis and management.
SIGNS, SYMPTOMS, AND RISK FACTORS Some causes of infectious retinitis are potentially devastating, not only because
these diseases can lead to permanent vision loss, but because they are often the clinical manifestation of a systemic illness. For example, cytomegalovirus (CMV) retinitis classically affects immune-compromised individuals with HIV.1,2 It is important to remember, though, that infections of the retina can affect immune-competent patients as well, such as in cases of acute retinal necrosis (ARN), cat-scratch disease, and Lyme disease.3-5
A patient with retinitis often presents with complaints of ocular pain (ARN, toxoplasmosis), vision loss, and/or new floating spots in his or her vision.6 However, some patients remain completely asymptomatic and may show up only for a routine examination or eyeglass prescription update.7
Familiarity with key clinical signs and risk factors is the main driving force behind finding a proper diagnosis (see Key Signs, Symptoms, and Risk Factors of Infectious Retinitis).
Prompt diagnosis is key for successful management of this rare yet potentially devastating condition. BY JOSEPH J. ALLEN, OD, FAAO
� COVER FOCUS RETINAL DISORDERS & DISEASES
SEPTEMBER 2019 | 29
COVER FOCUS RETINAL DISORDERS & DISEASES �
GETTING A GRASP ON THE SITUATION
My first instinct in clinic is to always examine every patient. During the examination, there are key signs you want to identify (or rule out) that could aid you and steer your diagnosis toward the cause of the retinitis. For example, nearly all forms of infectious retinal disease present with some form of uveitis.8,9 With any signs of new or worsening uveitis, a dilated examina-tion is required to evaluate the depth of the inflammation.
Retinitis may also present with simple stellate precipitates in the ante-rior chamber, as in ARN and syphilitic infections, or snowballs in the posterior segment, as in tuberculosis (TB), Lyme, or toxocariasis.10-12 Another classical finding is that of retinal necrosis, which presents with many forms of retinitis, and which can potentially lead to reti-nal detachment.8,13 Severe vision loss from retinal detachment is common in patients with remarkable necrosis, for example in ARN, progressive outer retinal necrosis (PORN), or CMV retinitis.8,13 Retinal hemorrhaging may also be evident and is typical of CMV retinitis.7,8
Once the level of uveitic involve-ment has been determined and the presence of retinal necrosis identified, the next challenge is to narrow down the differential diagnosis. This
is where a solid case history review becomes important.
LEARNING MOREI find myself rechecking the
patient’s review of symptoms and asking additional questions about his or her health status for other possible clues. For example, I review previous notes for mention of herpetic corneal disease, including the presence of ghost dendrites, history of shingles outbreaks, or other signs of preceding viral infection such as a cold sore.8
Because toxoplasmosis causes the vast majority of infectious retinitis, one should check for lymphadenopathy or a history of fever, malaise, ingesting undercooked or raw food, or a feline presence in the household.8,14 I also ask about other signs of skin involvement such as erythema migrans or neurologic changes for possible Lyme disease.15
Any history of a compromised immune system, as in HIV or use of chemotherapeutic agents, may lead to suspicion of CMV retinitis or PORN.1,6 A history of intravenous drug use or pulmonary disease could lead to suspi-cion of fungal infection.16 Asking about recent travel to countries in which TB is endemic may be useful if one sus-pects TB infection.8
Some clinical presentations of reti-nal infections are essentially clinical. For example, in ocular toxoplasmosis, the presence of a focal necrotizing retinochoroiditis with an adjacent or distant retinochoroidal scar or head-light in the fog is a key sign.17 However, appropriate laboratory studies may be necessary to confirm the diagnosis
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Infectious retinal diseases may present with common visual symptoms such as blurred vision or floaters, but they can also be asymptomatic.
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Infectious retinitis can affect not only immune-compromised patients, but also those who are immune-competent.
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Some presentations of retinal infections are essentially clinical, but appropriate laboratory studies are often necessary to confirm a diagnosis and rule out other disease entities.
AT A GLANCE
• Some patients with infectious retinitis notice floaters or decreased vision, whereas others have no symptoms at the time of diagnosis.
• Children with congenital infections and those whose mothers were actively infected during pregnancy or childbirth may have misaligned eyes, involuntary eye movements, or an abnormal white reflex through the pupil.
• Risk factors include active maternal infection during pregnancy or childbirth, exposure to endemic areas, or immunocompromised status.
• Intraocular infections can develop in patients with infected heart valves, gastrointestinal tract infections, or urinary tract infections, and in those who use intravenous drugs or who take antibiotics long-term.
Source: Infectious retinitis. American Society of Retina Specialists. www.asrs.org/patients/retinal-diseases/16/infectious-retinitis. Accessed August 19, 2019.
KEY SIGNS, SYMPTOMS, AND RISK FACTORS OF INFECTIOUS RETINITIS
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and rule out other disease entities. Consider ordering a complete blood count with differential, fluorescent treponemal antibody absorption and rapid plasma reagin tests, erythrocyte sedimentation rate, toxoplasmosis titers, toxocara titer, purified protein derivative, and a chest radiograph to rule out other etiologies.8,9
If the patient could be immuno-compromised (such as in CMV or PORN), consider HIV testing.9 For sus-pected Lyme disease, a Lyme immu-nofluorescent assay or enzyme-linked immunosorbent assay is appropriate.9 Polymerase chain reaction techniques are available for herpes simplex, vari-cella zoster, and other pathogens.
HANDLE WITH CAREWith the wide variety of possible
infectious etiologies and masqueraders, knowing the presentations of com-mon culprits is crucial to narrowing down the differentials and initiating
treatment in a timely manner. In many instances of infectious retinitis, depending on the entity involved, management may require immediate referral to a retina specialist, and per-haps even comanagement with an infectious disease specialist. Even with prompt diagnosis and appropriate referral, in many cases, infectious retini-tis can result in devastating outcomes. In the event of significant vision loss, referral of the patient to low vision ser-vices is warranted. n
1. Gupta S, Vemulakonda GA, Suhler EB, et al. Cytomegalovirus retinitis in the absence of AIDS. Can J Ophthalmol. 2013;48(2):126-129.2. Sugar EA, Jabs DA, Ahuja A, et al; Studies of the Ocular Complications of AIDS Research Group. Incidence of cytomegalovirus retinitis in the era of highly active antiretroviral therapy. Am J Ophthalmol. 2012;153(6):1016-1024.e53. Schoenberger SD, Kim SJ, Thorne JE, et al. Diagnosis and treatment of acute retinal necrosis: a report by the American Academy of Ophthalmology. Ophthalmology. 2017;124(3):382-392.4. Chi SL, Stinnett S, Eggenberger E, et al. Clinical characteristics in 53 patients with cat scratch optic neuropathy. Ophthalmology. 2012;119(1):183-187.5. Sanchez JL. Clinical manifestations and treatment of Lyme disease. Clin Lab Med. 2015;35(4):765-778.6. Austin RB. Progressive outer retinal necrosis syndrome: A comprehensive review of its clinical presentation, relationship to immune system status, and management. Clin Eye Vis Care. 2000;12(3-4):119-129.7. Au Eong KG, Beatty S, Charles SJ. Cytomegalovirus retinitis in patients with acquired immune deficiency syndrome. Postgrad Med J. 1999;75(888):585-590.8. Yanoff M, Duker JS. Ophthalmology, 5th ed. St Louis, MO: Elsevier; 2018.
9. Gerstenblith AT, Rabinowitz MP. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Philadelphia, PA: Lippincott Williams & Wilkins; 2012.10. Holland GN. Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society. Am J Ophthal-mol. 1994;117(5):663-637. 11. Borchers AT, Keen CL, Huntley AC, Gershwin ME. Lyme disease: a rigorous review of diagnostic criteria and treatment. J Autoimmun. 2015;57:82-115.12. Padhi TR, Das S, Sharma S, et al. Ocular parasitoses: a comprehensive review. Surv Ophthalmol. 2017;62(2):161-18913. Butler NJ, Moradi A, Salek SS, et al. Acute retinal necrosis: presenting characteristics and clinical outcomes in a cohort of polymerase chain reaction–positive patients. Am J Ophthalmol. 2017;179:179-189.14. Kim SJ, Scott IU, Brown GC, et al. Interventions for toxoplasma retinocho-roiditis: a report by the American Academy of Ophthalmology. Ophthalmology. 2013;120(2):371-378.15. Sathiamoorthi S, Smith WM. The eye and tick-borne disease in the United States. Curr Opin Ophthalmol. 2016;27(6):530-537.16. McCarty TP, Pappas PG. Invasive candidiasis. Infect Dis Clin North Am. 2016;30(1):103-124. 17. Dodds EM, Holland GN, Stanford MR, et al; International Toxoplasmosis Re-search Group. Intraocular inflammation associated with ocular toxoplasmosis: relationships at initial examination. Am J Ophthalmol. 2008;146(6):856-865.
JOSEPH J. ALLEN, OD, FAAOn Optometrist, Buffalo Eye Clinic, PA, Buffalo,
