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Two-Year Follow-Up of Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy: Case Report and Literature
Khaled El Matri1,2,3, Mohamed Foued Rmili1,2, Ahmed Arfaoui1,2
1Department of Ophthalmology B, Hedi Rais Institute of Ophthalmology of Tunis, Tunis, Tunisia.
Purpose:
To report a case of idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM), assessed with comprehensive multimodal imaging, with a 2-year follow-up.
Methods:
Observational case report and literature review.
Results:
A 43-year-old female presented with acute bilateral vision decrease. Funduscopy revealed multiple bilateral round yellowish lesions with a foveal vitelliform-like deposit. Lesions were both hyper- and hypoautofluorescent. They were hyperfluorescent on fluorescein angiography and hypofluorescent on indocyanine green angiography. Swept-source optical coherence tomography demonstrated bilateral macular thickening with subfoveal fluid and hyperreflective bleb-like deposits associated with photoreceptor outer segment shedding and ellipsoid thickening. Electrooculogram revealed an impaired retinal pigment epithelium function. An extensive workup excluded autoimmune, infectious, and malignant causes, supporting the diagnosis of idiopathic AEPVM. The patient was monitored closely without treatment and showed significant anatomical and visual improvement over 2 years.
Conclusions:
AEPVM is a rare acute disease with characteristic imaging findings. Despite the similarities with vitelliform macular dystrophies, family history and genetic workup are typically negative. Most cases resolve spontaneously, but recurrences may occur.