Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy

Abstract

Purpose: To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM). Design: Retrospective, observational, multicenter case series review. Participants: Consecutive patients diagnosed with idiopathic AEPVM. Methods: Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature. Main Outcome Measures: Clinical features of idiopathic AEPVM and differential diagnosis. Results: Eighteen patients (age range, 21e74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations. Conclusions: Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis.

Imaging features and functional outcome in a case of idiopathic acute exudative polymorphous vitelliform maculopathy

“… 2 Although AEPVM shares similarities with Best's disease and pattern dystrophies, BEST1 and peripherin/RDS genes are normal. 3 The prognosis is excellent with most patients regaining excellent VA as serous detachments resolve over a course of several months. 3 Minor vitelliform residues might persist for over a year ( Fig.…”

“… 3 The prognosis is excellent with most patients regaining excellent VA as serous detachments resolve over a course of several months. 3 Minor vitelliform residues might persist for over a year ( Fig. 2 , Fig.…”

Acute bilateral serous retinal detachments with spontaneous resolution in a 6-year-old boy

“…AEPVM is a rare disorder, with symptoms of (sub)acute bilateral vision loss, frequently – but not always – preceded by headache [ 1 ], [ 2 ], [ 3 ]. Usually there are no or very few signs of intraocular inflammation.…”

“…The fundoscopic findings are remarkable with bilateral multifocal central serous retinal detachments and round to oval white-yellowish lesions in a peculiar honeycomb-like pattern at the level of the retinal pigment epithelium, between and around the vascular arcades. These lesions do not occur in every patient, and they usually present after several months [ 2 ]. Fluorescein angiography shows early hyperfluorescence of the multifocal yellow lesions and staining in the late phase.…”

Vitelliform changes in the central retina occurring in adults

“…У пациентов не выявляются мутации в генах бестрофина и периферина, заболевание развивается билатерально, электроокулография в половине случаев остается неизменной [47]. В ряде исследований описаны случаи, когда триггером для развития острой экссудативной полиморфной макулопатии становятся паранеопластические процессы [48].…”