(Multiple Evanescent White Dot Syndrome)

BASICS


DESCRIPTION


• Multiple evanescent white dot syndrome (MEWDS) is an idiopathic inflammatory disease of the choroid that causes mild to moderate acute vision loss (1).


• It is usually self-limited, with recovery to normal vision within several weeks without recurrence.


• It is usually unilateral.


EPIDEMIOLOGY


Incidence


• Affect younger individuals, usually in the second to fifth decades of life


• 90% of patients are female


RISK FACTORS


50% of cases are associated with a viral prodrome.


Genetics


Possible association with HLA-B51 (2).


PATHOPHYSIOLOGY


Unclear.


ETIOLOGY


• Unknown


• It is presumed to be caused by a virus.


• Genetic predisposition combined with an environmental trigger may cause the disease.


COMMONLY ASSOCIATED CONDITIONS


Acute idiopathic blind spot enlargement syndrome (AIBES)-some feel that MEWDS and AIBES (as well as a number of other “white dot” syndromes are a spectrum of the same disease) (3).


DIAGNOSIS


HISTORY


• Sudden onset of decreased central vision in one eye


• Occasionally it may be bilateral, and may occur asynchronously


• May have central scotoma or photopsias


• May have a history of a recent viral illness


PHYSICAL EXAM


• Patients often have mild myopia


• There may be a mild afferent papillary defect


• Patients may have a mild vitreitis


• Optic disc edema


• There are ill defined small white lesions in the posterior pole at the level of the RPE/outer retina


• “Orange” foveal granularity


DIAGNOSTIC TESTS & INTERPRETATION


Lab


None usually necessary as MEWDS is a clinical diagnosis.


Imaging


Initial approach

• Fluorescein angiogram shows early hyperfluorescence with late staining in a cluster or wreath-like pattern around the macula.


• OCT shows subtle disruptions of photoreceptor inner and outer segment junction (4).


Follow-up & special considerations

• ICG is usually not needed for diagnosis.


• ICG angiography usually demonstrates more numerous hypofluorescent spots than seen on fluorescein angiography.


Diagnostic Procedures/Other


An electroretinogram (ERG) may be abnormal.


DIFFERENTIAL DIAGNOSIS


• Acute idiopathic blind spot enlargement


• Acute multifocal posterior pigment epitheliopathy (AMPPE)


• Acute macular neuroretinopathy


• Multifocal choroiditis


• Birdshot retinochoroidopathy


• Primary intraocular lymphoma may rarely appear similar to MEWDS, especially in patients >50 years old (5).


TREATMENT


MEDICATION


• Observation


• Symptoms usually resolve in 1–2 months.


ADDITIONAL TREATMENT


Issues for Referral


Consider referral to a vitreoretinal specialist.


ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


Monthly until symptoms resolve, then as needed.


PROGNOSIS


• Visual prognosis is good with almost all patients recovering vision >20/40.


• Recurrence is rare.


COMPLICATIONS


Choroidal neovascularization is extremely rare.



REFERENCES


1. Jampol LM, Sieving PA, Pugh D, et al. Multiple evanescent white dot syndrome. I. Clinical findings. Arch Ophthalmol 1984;102:671–674.


2. Borruat FX, Herbort CP, Spertini F, et al. HLA typing in patients with multiple evanescent white dot syndrome (MEWDS). Ocular Immunol Inflamm 1998;6:39–41.


3. Gass JD. Are acute zonal occult outer retinopathy and the white spot syndromes (AZOOR complex) specific autoimmune diseases? Am J Ophthalmol 2003;135:380–381.


4. Shah GK, Kleiner RC, Augsburger JJ, et al. Primary intraocular lymphoma seen with transient white fundus lesions simulating the multiple evanescent white dot syndrome. Arch Ophthalmol 2001;119:617–620.


5. Nguyen MH, Witkin AJ, Reichel E, et al. Microstructural abnormalities in MEWDS demonstrated by ultrahigh resolution optical coherence tomography. Retina 2007;27:414–418.

Only gold members can continue reading. Log In or Register to continue

Nov 9, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on (Multiple Evanescent White Dot Syndrome)
Premium Wordpress Themes by UFO Themes