Man presents with 2 weeks of bilateral ‘flashes and zigzags’
A 23-year-old man was referred by an outside optometrist to the retina service at Ophthalmic Consultants of Boston for evaluation of 2 weeks of bilateral “flashes and zigzags.”
On evaluation, the patient noted 1 day of bilateral floaters and new onset left eye pain. He reported seeing blind spots while reading. He had no shadow or curtain symptoms, blurred or double vision, eye redness, pain with eye movements or light sensitivity.
Source: Julia Watson, MD, and Lana Rifkin, MD
His medical history included eczema for which he was treated with dupilumab as well as presumed viral meningitis a few months prior. At the time of hospitalization for meningitis, a lumbar puncture was performed and was negative. His family history was noncontributory. He had no known drug allergies. He drank alcohol socially and did not smoke or use other drugs. His ocular history included myopia with astigmatism for which he was refracted annually.
Examination
On exam, best corrected visual acuity was 20/20 bilaterally. Pupils were equally round and reactive to light. IOP was 12 mm Hg in the right eye and 9 mm Hg in the left eye. Color vision was full by Ishihara color plates. Confrontation visual fields and motility were full in both eyes.
Anterior segment slit lamp exam was unremarkable bilaterally, including deep and quiet anterior chambers and clear vitreous without cells or haze. On dilated fundus exam, optic nerves appeared pink and sharp with 0.3 cup-to-disc ratios bilaterally. There were multifocal creamy chorioretinal lesions in the retina midperiphery and macula in both eyes. Some lesions appeared more “fluffy” and active while others appeared flat and inactive. The lesions in the macula of the left eye were encroaching the fovea (Figures 1a and 1b). The vessels were normal in both eyes.
Imaging
Bilateral OCT (Figures 2a and 2b) showed patchy outer retinal disruption. Fluorescein angiography (FA) transit in the left eye (Figure 3) showed early hypofluorescence and later hyperfluorescence of the chorioretinal lesions during the recirculation phase. FA in the right eye during the recirculation phase (Figure 4) showed multifocal staining without leakage or pooling corresponding to the chorioretinal lesions.
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Chorioretinal lesions
In a patient with a recent viral illness presenting with bilateral photopsias and floaters with fundus findings of bilateral multifocal chorioretinal lesions, the broad category of inflammatory choroidopathies should be considered. These entities do not have strict diagnostic criteria and can have overlapping presentations.
This case was felt to most resemble multifocal choroiditis (MFC). Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was also on the differential. This case resembled MFC because of the punched-out appearance of the inactive lesions; however, MFC is more often associated with anterior chamber and vitreous cell. Both conditions are commonly bilateral and affect younger adults. As in this case, APMPPE is often without anterior chamber or vitreous cell, shows early hypofluorescence and later hyperfluorescence on FA, and has a good visual prognosis. With the exception of APMPPE, which is associated with cerebral vasculitis and therefore warrants appropriate neurologic evaluation if suspicion is high, labeling the specific inflammatory choroidopathy often does not change management. The label can be helpful for counseling regarding visual prognosis and risk of recurrence. Before considering a diagnosis of an inflammatory choroidopathy, however, it is crucial to consider the masquerader conditions tuberculosis, sarcoidosis and syphilis, which require prompt and specific treatment and could worsen with systemic immunosuppressive treatment.
Workup, management and follow-up
Workup including ACE, lysozyme, IL-2 receptor antibody, chest X-ray, FTA-ABS and QuantiFERON Gold was ordered, and the patient was referred to the uveitis service. During his initial uveitis evaluation, ocular vitals and anterior segment examination were stable. On dilated fundus exam, more of both flatter, inactive lesions and fluffier, active lesions were found (Figures 5a and 5b). On fundus autofluorescence, the chorioretinal lesions were hyperfluorescent (Figures 6a and 6b). The aforementioned workup was still pending. Additional lab workup with a Coxsackie panel A+B was recommended.
Given the macular lesions, which could become vision threatening, treatment was recommended with valacyclovir 2 g three times daily due to suspicion of coxsackievirus-associated choroiditis and prior treatment success with this regimen. When the workup for tuberculosis, sarcoid and syphilis returned negative, the patient was started on oral prednisone. Over the course of the next few weeks, the chorioretinal lesions stabilized. The systemic steroids were gradually tapered due to poor patient tolerance. Valacyclovir was decreased to 1 g three times daily until it was felt that the patient was stable, with no new active lesions. At this point, the dosing was reduced to 1 g twice daily. The patient is planned to maintain this dosing for at least 6 months of quiescence. The Coxsackie panel resulted positive for Coxsackie B type 2 with a titer of 1:320.
Discussion
Coxsackievirus has been associated with unilateral acute idiopathic maculopathy, a rare entity characterized by significant unilateral vision loss with often spontaneous visual recovery. Although less well described, there have also been reports of an association of coxsackievirus with APMPPE, also not infrequently with spontaneous visual recovery. Given that there are often overlapping features among the inflammatory choroidopathies, evaluation for possible Coxsackie infection was pursued in this case. Additionally, there are reports in the literature of acyclovir being effective against hand-foot-and-mouth disease, possibly due to the antiviral’s effect on a patient’s immune system, therefore valacyclovir was trialed in this case. It is difficult to say definitively whether the valacyclovir changed the course of this patient’s disease; however, valacyclovir is a relatively low risk and anecdotally effective treatment option for patients with a similar presentation.
- References:
- Agrawal R, et al. J Ophthalmic Inflamm Infect. 2015;doi:10.1186/s12348-015-0034-3.
- BCSC. Retina and Vitreous. 2021-2022.
- Beck AP, et al. Arch Ophthalmol. 2004;doi:10.1001/archopht.122.1.121.
- Crawford CM, et al. ISRN Inflamm. 2013;doi:10.1155/2013/783190.
- Faulkner CF, et al. Australas J Dermatol. 2003;doi:10.1046/j.1440-0960.2003.00679.x.
- Li AL, et al. PLoS One. 2020;doi:10.1371/journal.pone.0238080.
- Yanoff M, et al. Ophthalmology. 3rd ed. Elsevier; 2009.
- For more information:
- Edited by William W. Binotti, MD, and Julia Ernst, MD, PhD, of New England Eye Center, Tufts University School of Medicine. They can be reached at william.binotti@tuftsmedicine.org and julia.ernst@tuftsmedicine.org.
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