Multiple Evanescent White Dot Syndrome
|Multiple Evanescent White Dot Syndrome|
|Classification and external resources|
- 1 Multiple Evanescent White Dot Syndrome
- 1.1 Background
- 1.2 Symptoms and Demographics
- 1.3 Course
- 1.4 Exam Findings
- 1.5 Testing
- 1.6 Pathogenesis and Treatment
- 1.7 Additional Resources
- 1.8 References
Multiple Evanescent White Dot Syndrome
Multiple Evanescent White Dot Syndrome (MEWDS) is one of the diagnoses within the family of White Dot Syndromes, first described by Jampol L. M and colleagues. Within this diagnostic group are MEWDS, Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPEE), Mulifocal Choroiditis and Panuveitis (MCP), Punctate Inner Choroiditis (PIC), and Birdshot Retinopathy. The White Dot Syndromes produce yellow-white retinal lesions classically located at the retinal pigment epithelium or outer retina and are found primarily in young adults. Symptoms of MEWDS include blurred vision, visual field loss, photopsias, and floaters. The various diagnoses are differentiated by history, appearance, laterality, and fluorescein angiogram finding.
Symptoms and Demographics
The typical patient with MEWDS is a healthy middle aged female age 15-50. There is a gender disparity as women are affected with MEWDS four times more often than men. Roughly 30% of patients have experienced an associated viral prodrome. Patients present with acute, painless, unilateral change in vision. They may notice photopsias, dyschromatopsia, and temporal or paracentral scotoma.
Though the onset is acute, the disease is typically self limiting and recurrences are rare. Visual prognosis is excellent.
On fundus exam, one sees flat, multifocal, grey-white lesions (100-200 micrometers), appearing to reach as deep as the RPE. Typically lesions are found outside the fovea in the posterior pole. A characteristic finding in MEWDS is an orange-yellow fovea with granularity. One might also note optic disc edema, mild vitritis (usually posterior vitreous cells), mild anterior chamber flare, a relative afferent pupillary defect and an enlarged blind spot. Sheathing of retinal veins and superficial retinal hemorrhages can be seen rarely.
FA reveals early punctate hyperfluorescence in a wreath-like pattern and late staining, in areas corresponding to the white dots. Retinal vascular sheathing and optic nerve staining may be seen in some patients with MEWDS. This early hyperfluorescence and late hyperfluorescence is in contrast to acute posterior multifocal placoid pigment epitheliopathy (APMPPE) where early hypofluorescence is seen followed by late hyperfluorescence.
FAF is a quick and non-invasive method of establishing a diagnosis. Hyperautofluorescent dots correlate with white dots seen on fundoscopy. Even in the absence of white dots on examination, FAF can show characteristic hyperautofluorescent lesions. Recent reports suggest that FAF may be the most sensitive and practical ancillary test to detect MEWDS.
Optical coherence tomography
Discontinuities in inner segment-outer segment junction and mild attenuation of external limiting membrane have been reported in acute phase. Recurrent episodes may result in thinning of outer nuclear layer.
Enlarged blind spot is typically seen. Other features include central or paracentral scotoma. Acute Idiopathic Blind Spot Enlargement Syndrome may represent a form of the same disease process.
Indocyanine green angiography
Hypocyanescent spots, typically outnumbering the clinical lesions are seen.
Changes are reversible. Reduced a wave amplitude may be seen. Early receptor potential may be prolonged.
Reversibe decrease in Arden's ratio may be noted.
Visual evoked response may show a reversible decrease in amplitude and prolongation of latency.
Pathogenesis and Treatment
An infectious etiology is thought to be involved in the pathogenesis of MEWDS since frequently there is a viral prodrome. However, the exact pathogenesis is unknown. Since MEWDS is a self-limited disease, with almost all patients regaining good visual acuity within 3-9 weeks, no treatment is recommended. Photopsias and scotomata gradually resolve and the lesions will disappear and may be replaced by mild pigment mottling or chorioretinal scarring. MEWDS typically is a self-limited disease, however, patients with MEWDS may have persistent blind spot enlargement. While it is uncommon, 10% of patients may also experience a recurrence. That being said, the prognosis is quite good for these patients.
- Basic and Clinical Science Course. United States: American Academy of Ophthalmology, 2010.
- Buggage, R. White Dot Syndrome. Focal Points: Clinical Modules for Ophthalmologists. June 2007. Vol 25(4).
- Emedicine. 13 July 2006. 11 July 2007. www.emedicine.com/oph/topic718.htm
- Friedman, et al. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. Philadelphia: W.B. Saunders Co.; 1998.
- Ho A, Brown G, et al. Color Atlas and Synopsis of Clinical Ophthalmology: Retina. McGraw-Hill publishing. New York. 2003.
- Hy Harvey. White Dot Syndromes. The Ocular Immunology and Uveitis Foundation. http://www.uveitis.org/medical/articles/case/wds.html.
- Jampol LM, Sieving PA, Pugh D, et al: Multiple evanescent white dot syndrome. Clinical findings. Arch Ophthalmol 1984 May; 102(5): 671-4.
- Quillen D, Davis J, et al. Perspective: The White Dot Syndromes. American Journal of Ophthalmology 2004; 137:538-550.
- Quillen D, Blodi B. Clinical Retina. United States: American Medical Association Press, 2002.
- Tewari A, Elliot D. emedicine. 8 June 2005. 11 July 2007. http://www.emedicine.com/OPH/topic749.htm
- Joseph A, Rahimy E, Freund KB, et al. Fundus autofluorescence and photoreceptor bleaching in multiple evanscent white dot syndrome. Ophthalmic Surg Lasers Imaging Retina. 2013 Nov 1;44(6):588-92.
- Images used with permission from emedicine
- American Academy of Ophthalmology. Retina/Vitreous: Multiple evanescent white dot syndrome Practicing Ophthalmologists Learning System, 2017 - 2019 San Francisco: American Academy of Ophthalmology, 2017.
- Jampol, Lee M. “Multiple Evanescent White Dot Syndrome: I. Clinical Findings.” Archives of Ophthalmology 102, no. 5 (May 1, 1984): 671. doi:10.1001/archopht.1984.01040030527008.
- Lavigne, Luciana Castro, David Leonardo Cruvinel Isaac, José Osório Duarte Júnior, and Marcos Pereira de Avila. “Transient Spectral Domain Optical Coherence Tomography Findings in Classic MEWDS: A Case Report.” Arquivos Brasileiros De Oftalmologia 77, no. 3 (June 2014): 185–87.
- Nguyen, My Hanh T., Andre J. Witkin, Elias Reichel, Tony H. Ko, James G. Fujimoto, Joel S. Schuman, and Jay S. Duker. “Microstructural Abnormalities in MEWDS Demonstrated by Ultrahigh Resolution Optical Coherence Tomography.” Retina (Philadelphia, Pa.) 27, no. 4 (May 2007): 414–18. doi:10.1097/01.iae.0000246676.88033.25.