Natural Course of Adult-Onset Foveomacular Vitelliform Dystrophy: A Spectral-Domain Optical Coherence Tomography Analysis

We read with great interest the article by Querques and associates describing progressive stages in patients with presumed adult-onset foveomacular vitelliform dystrophy (AOFVD): vitelliform, pseudohypopyon, vitelliruptive, and atrophic. The authors also noted a significant decrease in visual acuity in eyes showing progression through these stages.

Based on our spectral-domain optical coherence tomography findings in patients diagnosed with AOFVD, we respectfully disagree with Querques and associates with regard to the existence of 4 stages in this entity. In our series of 25 eyes of 14 patients followed up for a median of 23 months (range, 21 to 29 months), we observed only 2 stages: vitelliform and atrophic. We also found an important vision loss coincident with the disappearance of the vitelliform deposit, leading to atrophy of the retinal pigment epithelium and the photoreceptor layer—the atrophic stage—in 3 eyes (12%).

However, multiple causes may produce acquired vitelliform lesions. These share common imaging features in the fundus appearance and the spectral-domain optical coherence tomography findings (AOFVD, cuticular drusen, nonneovascular age-related macular degeneration, angioid streaks, acute exudative polymorphous vitelliform maculopathy, vitreomacular traction, subretinal drusenoid deposits, and central serous corioretinopathy ). Multimethod imaging analysis is necessary to assess the origin of the vitelliform lesion, including color fundus photography, fundus autofluorescence, fluorescein angiography, and spectral-domain optical coherence tomography. Furthermore, electrophysiologic tests may be included altogether with a familial history of macular diseases to perform an accurate differential diagnosis.

In our opinion, judging by the images presented in article by Querques and associates, the authors failed to rule out all causes of acquired vitelliform lesions, thus leading to their description of progressive stages in the natural course of misdiagnosed AOFVD. Their exclusion criteria included presence at baseline of geographic atrophy, signs of choroidal neovascularization or subretinal fibrosis, or any other retinal disease in the study eye. However, they did not specifically mention the presence of cuticular drusen, subretinal drusenoid deposits, or central serous chorioretinopathy.

In summary, the macular analysis by multimethod imaging must be performed to establish the diagnosis of AOFVD, because the acquired vitelliform lesions are the result of several pathologic characteristics that share common clinical features. Grouping these pathologic features under the name of AOFVD may lead to inexact classification of stages in the natural course of this disease.

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Jan 12, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Natural Course of Adult-Onset Foveomacular Vitelliform Dystrophy: A Spectral-Domain Optical Coherence Tomography Analysis

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