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D. G. Birch, K. G. Locke, Y.-Z. Wang, A. O. Edwards; mfERG and OCT in Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM). Invest. Ophthalmol. Vis. Sci. 2008;49(13):6099.
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© ARVO (1962-2015); The Authors (2016-present)
Acute exudative polymorphous vitelliform maculopathy (AEPVM) was first described in two male adults with acute vision loss in both eyes associated with headaches. Both subsequently showed multiple, round, yellow-white subretinal lesions at the level of the RPE (Gass et al, Trans Am Ophthalmol Soc, 86/1988). We previously reported the first case of AEPVM in a male teenager (Kozma et al, Retina, 27/2007). Here we present further follow-up data and two new cases, including the first report in a child, in order to document the evolution of fundus appearance, retinal anatomy and visual function.
Ages at initial visit were 6, 13, and 31 yrs. Patients were followed for 3, 5 and 1 yr, respectively. All patients were male. Full-field electroretinograms (ffERG) and multifocal electroretinograms (mfERG) were recorded using standard ISCEV protocols. Optical coherence tomography (OCT) was obtained with a Zeiss Stratus OCT.
The two older patients reported vision loss immediately after a flulike illness with headache. The two youngest patients were first seen while acuity was reduced (20/40 to 20/60), the ffERG was borderline reduced, and the mfERG was severely reduced throughout the macula. Multiple oval, yellow subretinal lesions associated with shallow macular serous detachment were observed by ophthalmoscopy. Subsequently, the subretinal lesions evolved to large, confluent, meniscus-like yellow subretinal deposits near the inferior arcades and parafoveal regions, while parallel mfERG responses showed remarkable improvement as visual acuity returned toward normal (20/30-20/15). The two older patients have essentially resolved and focal deposits in the subretinal space seen by OCT have decreased in size. Both retain some subfoveal thickening at the RPE level and geographic atrophy by OCT but show minimal defects in the mfERG. The youngest patient still has multiple yellow deposits and grossly abnormal mfERG responses.
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