We thank Dolz-Marco and associates for their interest in and comments on our recent article on the natural course of adult-onset foveomacular vitelliform dystrophy (AOFVD) by spectral-domain optical coherence tomography. The clinical features of AOFVD were described first by Gass in 1974. The disease then was characterized by a late-onset (between 30 and 50 years of age) solitary, round or oval, elevated, yellow, subretinal lesion of the fovea, ranging from one-third to 1 disk diameter in size and often accompanied by a central pigmented spot. Since 1974, several cases of AOFVD have been reported in multiple clinical series. These series revealed high variability in the appearance and progression of AOFVD, and unfortunately, have generated a number of different terms and abbreviations for this disease and have supported the idea that it may represent a heterogeneous group of disorders with variable clinical features. These undefined features may have led Dolz-Marco and associates to misdiagnose as AOFVD the series of 25 eyes investigated by spectral-domain optical coherence tomography to whom they refer. In fact, it should be noted that, as recently reported by Meunier and associates, age of onset is the only major criterion to distinguish juvenile vitelliform macular dystrophy from AOFVD (2 diseases otherwise clinically indistinguishable). Thus, it is not surprising that a similar progression, through different stages, may account for both vitelliform macular dystrophy and AOFVD, as described in our article.

In our series, as clearly stated in the Methods, we excluded eyes with a central spot of hyperpigmentation surrounded by a depigmentation halo at presentation. These eyes may share similarities with, but may not actually not represent, AOFVD. It seems likely that Dolz-Marco and associates included such cases in their series, and thus, differently from us, they did not evidence the different stages in the disease progression. Moreover, as stated in the Methods in our series, at least 3 horizontal line scans were placed into the lesion area (superior, middle, and inferior), and this may have helped us to distinguish the different stages.

We agree with Dolz-Marco and associates that multimethod imaging must be performed to establish the diagnosis of AOFVD, as we actually did in our series. We also agree that grouping acquired vitelliform lesions under the name of AOFVD may lead to inexact classification and evaluation of the natural course of these pathologic features. This is the reason why we included only eyes diagnosed with AOFVD. It is disappointing that Dolz-Marco and associates did not note that we excluded eyes with any other retinal disease, including cuticular drusen (as clearly stated in the Discussion on page 312, line 35); in that case, the disease should have been grouped under the name of acquired vitelliform lesions, rather than AOFVD. We believe that the study Dolz-Marco and associates refer to would be interesting in showing how acquired vitelliform lesions other than AOFVD progress over time.

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Jan 12, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Reply

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