Progression of Atrophy and Visual Outcomes in Extensive Macular Atrophy with Pseudodrusen-like Appearance

Abstract

Purpose: To report visual outcomes and rate of retinal pigment epithelium (RPE) atrophy progression in patients with extensive macular atrophy with pseudodrusen-like appearance (EMAP).Design: Retrospective, observational study.Participants: Patients with EMAP and symptom onset before 55 years of age, at least 12 months of followup using Spectralis blue-light fundus autofluorescence (BAF) and OCT and with no other ocular or systemic conditions.Methods: Best-corrected visual acuity (BCVA), BAF, and OCT images were reviewed at baseline and at each annual visit until the last available follow-up. Atrophy was measured by 2 graders using the region finder software on Heidelberg Explorer and confirmed using OCT scans covering the entire atrophic lesion. The following imaging biomarkers were analyzed at each visit: foveal atrophy, vitreomacular traction, outer retinal tubulations, choroidal caverns and subfoveal choroidal thickness, border autofluorescence pattern (hyper-autofluorescent or isoautofluorescent), and border irregularity as expressed by circularity index (CI).Main Outcome Measures: Primary outcomes were annual rate of atrophy enlargement and BCVA loss in EMAP patients. Secondary outcomes included the assessment of potential factors able to predict disease progression.Results: Thirty-six eyes from 18 patients with EMAP (6 men [33%]; mean age at symptom onset, 48.1 AE 1.7 years) were included. Mean follow-up lasted 32.8 AE 14.3 months. RPE atrophy increased from 10.8 AE 6.3 mm 2 at baseline to 18.1 AE 8.3 mm 2 at the end of follow-up, with a rate of 2.91 AE 1.09 mm 2 /year. Faster progression was associated with smaller CI at baseline (P ¼ 0.02) and with iso-autofluorescent lesion borders (P ¼ 0.01). Visual acuity declined progressively at a rate of 7.4 AE 5.8 letters per year, with 57% of eyes showing vision of 20/ 200 Snellen or worse at the 4-year follow-up. Worse visual outcomes were observed in patients with early foveal involvement at baseline (P ¼ 0.02).Conclusions: Patients affected by EMAP present a rapid expansion of RPE atrophy that is comparable with the diffuse-trickling form of geographic atrophy. More irregular and iso-autofluorescent lesion borders seem to predict faster progression. Our findings may provide relevant information for patient counseling and future interventional approaches to select the best candidates and proper clinical outcomes. Ophthalmology

Quantitative multimodal imaging of extensive macular atrophy with pseudodrusen and geographic atrophy with diffuse trickling pattern

“…Additionally, SCR correlates with atrophy progression rates and circularity. The latter is another useful parameter in distinguishing EMAP and DTGA from nDTGA, and its correlation with the progression rate has already been described in the literature 5 . These findings suggest that the simultaneous presence of low circularity and SCR supports the diagnosis of EMAP or DTGA.…”

“…On the other hand, nDTGA does not always exhibit the Bruch-RPE splitting. Fleckenstein M. et al have already described this feature in DTGA, and recently Romano F. et al have also documented this OCT characteristic in EMAP 3 , 5 . We surmise that this phenomenon might be compatible with the presence of basal laminar deposits, which could in turn explain the grayish appearance of the atrophy on FAF images of EMAP and DTGA.…”

“…A diffuse Bruch-RPE splitting has been described in both EMAP and DTGA 3 , 5 , therefore we designed a surrogate quantitative parameter for this phenomenon, and called it “EZ disruption distance”. On horizontal OCT scans, we checked the presence of a Bruch-RPE splitting at the edges of the atrophic lesion (Fig.…”

“…Despite these distinctive characteristics, it may be difficult to distinguish macular atrophy secondary to EMAP from the diffuse-trickling geographic atrophy (DTGA) pattern, which is considered the most aggressive form of GA secondary to AMD, since both conditions share many overlapping features: a relatively young age at presentation, bilaterality, fast progression, lobular and grayish aspect of the atrophic area on fundus autofluorescence (FAF), and predominance of pseudodrusen 3 , 4 . Furthermore, a diffuse splitting of the Bruch-RPE complex has been reported in both EMAP and DTGA 3 , 5 .…”

Characteristics and Classification of Choroidal Caverns in Patients with Various Retinal and Chorioretinal Diseases

“…Several studies have shown a dotted or linear area of high reflectivity in the caverns [ 1 ], whereas others denied the presence of such areas [ 7 ]. Although choroidal caverns usually occur in patients with GA [ 8 , 9 , 10 ], pachychoroid spectrum disease [ 11 , 12 , 13 , 14 , 15 ], retinal pigment epithelium (RPE) atrophy diseases such as Stargardt disease [ 16 ], best vitelliform dystrophy [ 17 ] and rod–cone dystrophy [ 7 , 18 ], they are also seen in normal eyes or choroidal osteoma [ 19 ]. Although previous studies have described the shape and size of choroidal caverns, they have not systematically analyzed the related characteristics of choroidal caverns.…”