Eight-year multimodal follow-up of recurrent idiopathic acute exudative polymorphous vitelliform maculopathy

Abstract

Purpose: To describe and analyze clinical findings in a patient with recurrent idiopathic acute exudative polymorphous vitelliform maculopathy (AEPVM), followed in detail, and to propose the diagnostic and follow-up algorithm.

Design: Retrospective observational analysis.

Patient: A young adult male patient diagnosed with idiopathic AEPVM who developed two relapses in a 12-month period eight years after the initial onset.

Methods: Review of clinical charts, multimodal imaging, and electrophysiology findings. The patient repeatedly underwent complete ophthalmic examinations, including best-corrected visual acuity testing (BCVA), slit-lamp and fundus examinations; digital fundus photography, time-domain optical coherence tomography (OCT) in 2009 (Stratus OCT, Carl Zeiss Meditec, USA) and spectral-domain OCT in 2017-2018 (Spectralis-OCT, Heidelberg Engineering, Germany), together with fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICGA), all with HRA2 (Heidelberg Engineering, Germany); microperimetry (MP-1 microperimeter, Nidek, Japan). Laser flare photometry (Kowa FM-600, Japan) and electrophysiology testing were also performed.

Main outcome measures: Clinical features of long-lasting recurrent idiopathic AEPVM, and diagnostic and follow-up algorithm in such rare cases.

Results: Case report of a 25-year-old male Caucasian patient with typical features of AEPVM, including serous neuroepithelial detachment with irregular retinal elevations, ophthalmoscopically resembling retinal folds, with subsequent subretinal accumulation of characteristic yellow-white vitelliform deposits. Features in this case rarely described, or even not yet reported, include indocyanine- and fluorescein-negative intraretinal cystic changes, optic disc hyperfluorescence on FA, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and very slow anatomical improvement of the relapses. Bimonthly fundus autofluorescence evaluation together with SD-OCT were the most informative diagnostic methods, demonstrating the evolution of pathological signs.

Conclusion: AEPVM may be a recurrent or even chronic condition with uncertain long-term visual outcomes. It may have variable clinical presentations depending on the stage of the disease, and both clinical manifestations and imaging features of different stages of the pathologic process may overlap. Patients should be made aware that visual improvement occurs very slowly, if at all. Bimonthly fundus autofluorescence evaluation together with SD-OCT should be recommended in such cases.

Keywords: Acute; Exudative; Fluorescein angiography; Fundus autofluorescence; Idiopathic; Imaging; Indocyanine green angiography; Intraretinal fluid; Laser flare photometry; Lipofuscin; Long-term evaluation; Maculopathy; Microperimetry; Multimodal imaging; Polymorphous; Recurrent; Subretinal deposits; Subretinal fluid; Vitelliform.