History of present illness
A 20-year-old male patient presents with 3 days of experiencing a “black spot” in the center of his vision in the left eye. The patient states that the onset of symptoms was sudden. He denies flashes, floaters, or eye pain. The patient denies any ocular or medical history or any ocular surgical history. One week before the onset of symptoms the patient experienced an upper respiratory tract infection; he has now developed a papular rash on his hands with desquamation on his fingers.
Ocular examination findings
Best-corrected visual acuity was 20/15 in the right eye and 20/80 in the left eye. Intraocular pressure was normal in both eyes. The anterior segment was normal in both eyes. On dilated fundus examination, the right eye was unremarkable. The left eye showed perifoveal and parafoveal intraretinal hemorrhages with subtle subretinal fluid involving the fovea.
Imaging
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Fundus photograph: color fundus photograph of the left eye shows intraretinal hemorrhages in the macula with an area of subretinal fluid involving the fovea ( Fig. 51.1 ).
Fig. 51.1
Color fundus photograph of the left eye at initial presentation. Intraretinal hemorrhages adjacent to the area of macular subretinal fluid.
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Optical coherence tomography (OCT): OCT of the left eye shows subretinal fluid with some hyperreflective material at the level of the outer retina and irregularity of the retinal pigment epithelium (RPE) ( Fig. 51.2 ).
Fig. 51.2
Optical coherence tomography of the left eye at initial presentation. Significant subretinal fluid involving the fovea with hyperreflective material. There is also hyperreflective material at the outer retina and retinal pigment epithelium irregularity.
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Fundus autofluorescence (FAF): FAF of the left eye shows small areas of hypoautofluorescence correlating to areas of intraretinal hemorrhage and small punctate areas of hyperautofluorescence at the edge of the subretinal fluid location ( Fig. 51.3 ).
Fig. 51.3
Fundus autofluorescence of the left eye at initial presentation. There is mild hypoautofluorescence in the central macula with faint hyperautofluorescence ring surrounding the area of subretinal fluid.
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Fluorescein angiogram (FA): FA of the left eye shows multifocal areas of hyperfluorescence at the level of the RPE ( Fig. 51.4 A) that progresses to hyperfluorescence in a pooling pattern with areas of hypofluorescence due to blockage from the intraretinal hemorrhages ( Fig. 51.4 B).
Fig. 51.4
Fluorescein angiogram (FA) of the left eye at initial presentation. (A) FA at 47 seconds showing early hyperfluorescence at multiple points at the level of the retinal pigment epithelium. (B) FA at 6 minutes showing significant hyperfluorescence in pooling pattern.
Questions to ask
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Does the patient use any steroid medications? In an otherwise healthy male with macular subretinal fluid, it would be important to ask about steroid medications to investigate the possibility of central serous chorioretinopathy.
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Has the patient had a recent upper respiratory tract infection? Preceding viral illness with symptoms of upper respiratory infection has been implicated in multiple disease processes, including white dot syndrome and Vogt-Koyanagi-Harada syndrome, that would be included on the differential diagnosis for this patient.
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Does the patient have a history of sexually transmitted illnesses, or does the patient engage in high-risk sexual practices? Syphilis is a great mimicker and should always be assessed for, especially in a young patient.
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What is the patient’s refraction? Pathological myopia with choroidal neovascular membrane and subsequent exudation of fluid into the subretinal space can cause this sort of presentation.
Assessment
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The patient is a 20-year-old male patient presenting with acute onset painless vision loss in the left eye with a preceding viral illness that is found to have parafoveal and perifoveal intraretinal hemorrhages with subretinal fluid in the macula.
Differential diagnosis (see algorithm 51.1 for full algorithm)
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Central serous chorioretinopathy
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Pathological myopia
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Syphilis chorioretinitis
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Harada disease
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Idiopathic choroidal neovascularization
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Acute posterior multifocal placoid pigment epitheliopathy
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Unilateral acute idiopathic maculopathy (UAIM)
Working diagnosis
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UAIM
Multimodal testing and results
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Fundus photographs
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On fundus examination, patients are found to have neurosensory detachment of the retina in the macula, intraretinal hemorrhages near the neurosensory detachment, and an irregular white/gray thickening to the RPE. A “bull’s eye” appearance may occur late in the disease.
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OCT
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OCT shows fluid accumulation in the subretinal space with hyperreflective material in the subretinal and accumulation in the outer retina with RPE irregularities.
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FAF
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Early on, FAF shows a mixed pattern of alternative hyperautofluorescence mixed with hypoautofluorescence in the fovea or peripapillary areas. Later in the disease there is more confluent hypoautofluorescence, indicating RPE cell death. The FAF may also show hyperautofluorescent “satellite” lesions that do not appear on near-infrared imaging or fundus photography.
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FA
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Early FA shows mild subretinal hyperfluorescence, with late stage angiogram showing hyperfluorescence at the level of the RPE and pooling in the subretinal space.
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Management
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Patients with UAIM are typically observed because good visual acuity recovery spontaneously occurs. Some reports have shown that treatment with oral corticosteroids may hasten visual recovery.
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Our patient was observed and, over the course of 2 weeks, had significant improvement in visual acuity to 20/20 and improved imaging findings ( Figs. 51.5–51.7 ).
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