Young woman has 10-day history of flashes in the left eye

The patient also has a scotoma in the temporal visual field.

Mark A. Patron

Andre J. Witkin

A 24-year-old Caucasian woman presented with a 10-day history of intermittent flashing lights and a dark spot in the temporal aspect of her left eye. There was no associated pain, headache, floaters, shadows or recent viral illness. She had no significant ocular history or medical history, had no known drug allergies and was on no medications.

Examination

On exam, the patient’s best corrected visual acuity was 20/20 in the right eye and 20/30⁺² in the left eye. Her IOP was 16 mm Hg in both eyes. The pupils were symmetric with no relative afferent pupillary defect noted in either eye. Ocular motility and confrontational visual fields were full in both eyes. Ishihara color plates were 14/14 in both eyes. Her anterior segment exam was within normal limits.

The right fundus exam was normal (Figure 1). The fundus examination of the left eye showed granular retinal pigment epithelial (RPE) changes in the central macula and multiple white lesions at the level of the RPE (Figure 2). There was no retinal vessel sheathing, vitritis or significant optic disc edema.

Fluorescein angiogram showed hyperfluorescence of the left optic disc and punctate late stippled wreath-like staining of the fundus (Figure 3). Visual field testing showed an enlarged blind spot in the left eye. Ultrahigh-resolution optical coherence tomography of the left optic nerve showed disruptions in the photoreceptor inner segment/outer segment junction (Figure 4).

Figure 1. Fundus exam of the right eye was normal.

Figure 2. Fundus exam of the left eye found multiple white lesions at the level of the RPE. Images: Nandakumar N, Hedges TR

Figure 3. Early and late fluorescein angiogram of the left optic disc.

Figure 4. Ultrahigh-resolution OCT of the left optic nerve showed disruptions in the photoreceptor inner segment/outer segment junction.

What is your diagnosis?

Flashing in left eye

The differential diagnosis for the patient’s presentation includes acute posterior multifocal placoid pigment epitheliopathy, birdshot chorioretinopathy, multifocal choroiditis, lymphoma, sarcoidosis and multiple evanescent white dot syndrome.

Differential diagnosis

Acute posterior multifocal placoid pigment epitheliopathy is an acquired inflammatory disease affecting the RPE in otherwise healthy young adults. It often presents after a viral prodrome, with symptoms including meningismus, headaches and transient hearing loss. Visual symptoms include acute bilateral painless loss of vision. The fundus lesions are creamy yellow and are located in the choroid. It usually resolves after a few weeks and typically does not recur.

Birdshot chorioretinopathy most commonly occurs in women between the ages of 30 and 60 years. It is characterized by vitritis and numerous orange- to cream-colored lesions that are distributed in the posterior pole and midperiphery. Patients most commonly experience loss of vision and floaters in one eye, with later involvement of the fellow eye. There is a strong correlation with HLA-A29. Like other autoimmune diseases, birdshot chorioretinopathy is characterized by remissions and exacerbations.

Multifocal choroiditis is an inflammatory condition affecting predominantly women in their 30s and is usually bilateral. Patients often experience decrease in vision that is sometimes accompanied by metamorphopsia, floaters and photopsias. Fundus exam reveals several yellow-white lesions that are found more in the peripheral retina than around the macula. Recurrence rates are high, and progressive loss of vision can occur secondary to subretinal neovascular membrane formation.

Patients with primary central nervous system lymphoma usually present in their sixth and seventh decades of life with bilateral, often asymmetric vitritis. Fundus exam shows yellow-white subretinal lesions that represent lymphomatous detachments of the RPE. Fluorescein angiography shows early hypofluorescence with late hyperfluorescence. A vitreal biopsy is often used to confirm the diagnosis. Approximately 20% of patients will have only ocular involvement of primary central nervous system lymphoma. Of these patients, approximately 50% to 80% will go on to develop central nervous system involvement. The prognosis is poor for these patients; therefore, timely diagnosis is critical.

Multiple evanescent white dot syndrome generally affects healthy young women between the ages of 20 and 45 years. Patients present with acute unilateral painless vision loss. Bilateral cases are rare but have been reported. Patients often experience shimmering photopsias and scotomas in the temporal aspect of their visual field. Fundus exam can show multiple small whitish lesions at the level of the RPE scattered in the posterior pole, variable amounts of vitritis and optic disc edema. Fluorescein angiogram shows late staining of the lesions and hyperfluorescence of the disc. Visual fields can often show an enlarged blind spot.

Given the history, funduscopic appearance and fluorescein angiography, a diagnosis of multiple evanescent white dot syndrome was made.

Discussion

Multiple evanescent white dot syndrome is a self-limited disease that resolves in 2 to 6 weeks. Therefore, no treatment is necessary. Although the visual acuity can return to normal relatively quickly, the shimmering photopsias can take several months to resolve. The white dots in the fundus usually resolve completely. However, the foveal granularity that presents initially usually persists. Our patient had OCT findings that showed disruptions in the inner segment/outer segment junction. This has been reported in the literature previously and can be used as another clue to help make the diagnosis.

References:

• Aaberg TM. Multiple evanescent white dot syndrome. Arch Ophthalmol. 1988;106(9):1162-1163.
• Borruat FX, Auer C, Piguet B. Choroidopathy in multiple evanescent white dot syndrome. Arch Ophthalmol. 1995;113(12):1569-1571.
• Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol. 1984;102(5):671-674.
• Li D, Kishi S. Restored photoreceptor outer segment damage in multiple evanescent white dot syndrome. Ophthalmology. 2009;116(4):762-770.
• Nguyen MH, Witkin AJ, Reichel E, et al. Microstructural abnormalities in MEWDS demonstrated by ultrahigh resolution optical coherence tomography. Retina. 2007;27(4):414-418.

• Namrata Nandakumar, MD, and Thomas R. Hedges III, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

• Edited by Mark E. Patron, MD, and Andre J. Witkin, MD. Drs. Patron and Witkin can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.