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Central visual obscurations reported in healthy 37-year-old woman
The right eye showed an enlarged foveal depression and RPE mottling.
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A 37-year-old woman with a history of ocular migraines presented urgently to the New England Eye Center with a 3-week history of seeing a blurred “circle” in the central vision of her right eye. She described the symptom as “looking through water” and felt it had been stable since onset. She noted that when she blinked, the circle seemed to pulse and colors appeared to change. She denied any flashing lights or floaters in her vision.
She endorsed a recent viral upper respiratory tract infection before the onset of her visual symptoms but denied any other systemic symptoms, including headache. She denied any history of tick bites. She had an indoor cat but did not recall any cat scratches. She did recently view the solar eclipse but used NASA-approved lenses. Her symptoms were similar to her prior ocular migraines, but the migraines usually resolved quickly (within minutes), while these symptoms had persisted.
She denied any additional ocular history or other medical problems. She was not taking medications and denied any previous surgical history. Her family history was positive for Fuchs’ corneal dystrophy in her mother and central serous retinopathy in her brother.
Examination
The patient was alert and oriented to person, place and time with normal mood and affect. Visual acuity without correction was 20/40 in the right eye and 20/20 in the left eye. Both pupils were round and briskly reactive to light, measuring 4 mm in dim light and 2 mm in bright light. There was no evidence of an afferent pupillary defect. IOP was normal in both eyes. Visual fields were full to confrontation bilaterally. Extraocular movements were full bilaterally. The external lids and lacrimal system were within normal limits.
The conjunctiva was white and quiet in both eyes. The cornea was clear without scars or guttata bilaterally. There were no iris transillumination defects. The anterior chambers were quiet bilaterally. The lenses were clear. The vitreous was clear without cells. The optic nerves were pink and sharp with small cups in both eyes. No disc edema was appreciated. The macula of the right eye showed an enlarged foveal depression and retinal pigment epithelium (RPE) mottling. The macula of the left eye showed mild mottling of the RPE (Figure 1). The peripheral retina was normal, notably without chorioretinal lesions or vascular sheathing.
Source: Sarah Adelson, MD, and Elias Reichel, MD
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Unilateral vision changes
The differential diagnosis of subacute vision changes with RPE mottling in an otherwise healthy young woman includes inflammatory, autoimmune and infectious etiologies.
The white dot syndromes, a group of idiopathic inflammatory conditions, are important to consider in the differential diagnosis of decreased vision in a young patient with retinal and choroidal pathology. In our patient’s case, the most likely inflammatory conditions include multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multifocal choroiditis and panuveitis (MCP) and acute macular neuroretinopathy (AMN). These conditions can be differentiated by history, funduscopic findings, laterality and appearance on ocular imaging.
MEWDS, APMPPE, MCP and AMN all typically present in otherwise healthy young adults in their second to fifth decades of life. APMPPE affects both men and women equally, while the other listed inflammatory processes are seen predominantly in women. APMPPE and MCP typically present with bilateral inflammation. AMN can present with unilateral or bilateral disease. MEWDS, in contrast, is typically a unilateral disease with patients endorsing temporal or paracentral scotomas, flashes and changes in color vision in one eye. APMPPE has an association with systemic conditions and can present with an associated cerebral vasculitis. A preceding viral prodrome can be seen in APMPPE, MEWDS and AMN. In these syndromes, clinical examination and imaging will help aid in the diagnosis.
On fundus examination, MEWDS characteristically presents with multiple gray-white lesions in the posterior pole at the level of the outer retina or RPE measuring approximately 100 µm to 200 µm in size. These lesions are transient and therefore often not present on initial exam. Thus, a more common finding is macular granularity. Vitreous cell and mild disc swelling have also been seen in patients with MEWDS. Visual field testing may show an enlargement of the blind spot. OCT typically shows disruption of the inner segment/outer segment junction. Fundus autofluorescence (FAF) may help make the diagnosis in clinic because it is both quick and noninvasive. FAF typically shows hyperautofluorescent spots in the posterior pole that correspond to the white lesions seen on fundoscopy. FAF may show these hyperautofluorescent lesions despite the absence of lesions on clinical exam. Fluorescein angiography (FA) shows early hyperfluorescence in a “wreath-like pattern” with late staining of the disc. Indocyanine green angiography (ICG) may demonstrate hypocyanescent spots that outnumber the lesions seen on clinical exam.
In contrast, APMPPE typically presents with multiple creamy placoid lesions measuring 1 to 2 disc areas in size at the level of the RPE. FAF typically shows hypoautofluorescence corresponding to the placoid lesions with variable hyperautofluorescence at the borders of the lesions. FA demonstrates early blockage with late staining of the lesions. ICG shows delayed choroidal filling and nonperfusion.
In MCP, acute gray-yellow lesions measuring 50 µm to 350 µm in size are seen at the level of the RPE. Chronic lesions appear as atrophic, “punched-out” chorioretinal scars with pigmented borders. Vitritis is almost always present. FA acutely shows early blockage with late staining of lesions. As the lesions become atrophic, early hyperfluorescence can be seen. ICG demonstrates hypocyanescent spots.
AMN typically presents with one or more reddish-brown “petaloid” lesions surrounding the fovea. Visual field testing often shows a scotoma corresponding to the location of the lesions. FA is typically normal.
Another differential consideration in our patient with a recent history of viewing the eclipse is solar retinopathy. Additional differential considerations include infectious etiologies, such as syphilis, and autoimmune disease, such as sarcoidosis.
Workup and management
For further workup of our patient’s vision loss, we obtained FAF, OCT, FA and ICG of both eyes.
FAF of the right eye showed multiple hyperautofluorescent spots radiating from the optic nerve. FAF of the left eye showed a single hyperautofluorescent spot in the mid-periphery (Figure 2). OCT of the right eye showed disruption of the subfoveal inner segment/outer segment ellipsoid zone (Figure 3). OCT of the left eye was normal. FA of the right eye showed normal filling of the arteries in the early phase, without any defects. In the mid phase, the right eye demonstrated early hyperfluorescent areas around the optic nerve. In the late phase, the hyperfluorescent areas around the optic nerve became more prominent. FA of the left eye showed normal filling in all phases with mild optic nerve staining in the late phase (Figure 4). ICG of both eyes showed hypercyanescent areas of granularity in the late phase (Figure 5).
Our patient’s presentation of unilateral scotoma and color changes with RPE mottling was clinically consistent with a diagnosis of MEWDS. Ocular imaging helped to confirm the diagnosis with the characteristic inner segment/outer segment disruption on OCT, hyperautofluorescent dots on FAF and early hyperfluorescent spots around the optic nerve with late staining of the disc on FA. We did not elect to treat the patient because the outcome is favorable and the symptoms tend to resolve on their own.
Discussion
MEWDS is characterized as one of the white dot syndromes. The white dot syndromes are seen primarily in young adults who present with symptoms such as field loss, floaters, blurry vision or flashes. The annual incidence of white dot syndromes in the United States is 0.45 per 100,000 people among Caucasians. In these syndromes, the pathological changes are located at the level of the RPE or outer retina.
MEWDS patients are typically otherwise healthy young women who present with acute, unilateral, painless changes in vision. They may describe photopsias, scotomas or dyschromatopsia. Women are affected four times more often than men. An infectious etiology is thought to be involved in the pathogenesis of MEWDS because there is a viral prodrome in approximately 50% of patients. However, the exact pathogenesis remains unknown. A detailed clinical examination and ocular imaging can help reach the diagnosis and rule out other conditions, as discussed above.
MEWDS is a self-limited disease. Nearly all patients will regain normal visual acuity within weeks to months. Therefore, there is no recommended treatment besides watchful waiting. The photopsias and scotoma will resolve over time. On exam, the white dots will disappear and be replaced by mild pigment mottling or scarring. Some patients will have a persistently enlarged blind spot. A minority (approximately 10%) of patients may have recurrence, but overall the prognosis remains favorable even in this subset of patients. Few patients may experience decreased vision due to choroidal neovascularization. These patients, although rare in number, have been successfully treated by intravitreal ranibizumab. Treatment is only needed in the case of these rare complications.
Follow-up
Our patient reports that visual acuity has improved in the right eye. She still experiences a “shimmering” in the right eye when going from natural light to artificial light indoors.
- References:
- Abu-Yaghi NE, et al. Ocul Immunol Inflamm. 2011;doi:10.3109/09273948.2011.624287.
- Crawford CM, et al. ISRN Inflamm. 2013;doi:10.1155/2013/783190.
- Gross NE, et al. Arch Ophthalmol. 2006;doi:10.1001/archopht.124.4.493.
- Jampol LM, et al. Arch Ophthalmol. 1984;doi:10.1001/archopht.1984.01040030527008.
- Quillen DA, et al. Am J Ophthalmol. 2004;doi:10.1016/j.ajo.2004.01.053.
- Yanoff M, et al. Ophthalmology. 4th ed. Elsevier Health Sciences; 2014.
- For more information:
- Sarah Adelson, MD, and Elias Reichel, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Aubrey R. Tirpack, MD, and Astrid C. Werner, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.