Faculty Bibliography

PURPOSE:: To compare the functional and morphologic characteristics and evolution of lamellar macular holes (LMHs) with and without lamellar hole-associated epiretinal proliferation (LHEP). METHODS:: This was a retrospective observational case review of 145 eyes of 136 patients with LMH seen in a vitreoretinal clinical practice, and the eyes were subdivided into 2 groups based on the presence or absence of LHEP. Main outcome measures were logarithm of minimal angle of resolution (logMAR) visual acuity and morphologic characteristics as seen with spectral domain optical coherence tomography over retrospective follow-up. RESULTS:: In 62 eyes (42.7%), LHEP was detected, while 83 eyes (57.3%) had the presence of epiretinal membrane without LHEP. The mean logMAR visual acuity in eyes with LHEP was 0.51 (20/65 Snellen equivalent), which was significantly poorer than that in the eyes without LHEP at 0.33 (20/43 Snellen equivalent, P = 0.002). Multivariate analysis showed that the presence of LHEP was significantly associated with larger LMH diameter at the middle retinal level (P = 0.01) and thinner retinal thickness at the base of the LMH (P < 0.001). A higher proportion of eyes with LHEP (88%) had ellipsoid disruption compared with eyes without LHEP (24%, P = 0.001). Over the mean retrospective follow-up of 26 months, 5% of eyes with LHEP had functional decline of 0.3 logMAR visual acuity compared with 4% of eyes without LHEP (P = 0.99), whereas 18% of eyes with LHEP had morphologic progression compared with 13% of eyes without LHEP (P = 0.49). CONCLUSION:: Eyes with LMH and LHEP were associated with poorer visual acuity, larger LMH diameters, thinner retinal thickness, and higher incidence of ellipsoid disruption compared with eyes without LHEP, suggesting a process involving more severe retinal tissue loss and injury. Both LMH with and without LHEP seemed to be stable configurations over time.

PURPOSE:: The aim of this study was to characterize peau d'orange and angioid streaks, characteristic findings in eyes of patients with pseudoxanthoma elasticum, by examining fundus photography and optical coherence tomography imaging. METHODS:: Color photographs were evaluated directly as were the component red and green channels. Optical coherence tomography images were evaluated for reflectivity pattern of the band corresponding to the retinal pigment epithelium-Bruch membrane complex. RESULTS:: Eighteen eyes of 9 patients with a mean age of 48.7 years (range, 31-61 years) were examined; 7 of them were women. Color photographs showed areas of yellowish opacification that obscured visualization of the underlying choroid. At the outer edges of this confluent area, opacification were nonconfluent changes with similar appearance and these regions were typical peau d'orange. Angioid streaks occurred within and extended up to the outer border of the confluent opacification. Underlying choroidal details could be seen through the regions of peau d'orange and through the gaps in angioid streaks. The red channel image showed increased reflectivity from the confluent deposit and improved visualization of the choroidal vasculature, except where the confluent opacification was located. Optical coherence tomography imaging showed increased reflectivity from the outer border of the retinal pigment epithelium-Bruch membrane complex. CONCLUSION:: The findings suggest that the confluent region is the relevant lesion, not the subconfluent zone known as peau d'orange. Imaging characteristics of the confluent area of opacity are consistent with diffuse infiltration with calcium, a chief histologic abnormality of pseudoxanthoma elasticum. The name coquille d'oeuf was suggested for the confluent area of opacity as a consequence.

Importance: Macular telangiectasia type 2 (MacTel 2) is a rare disease in which abnormalities of the retinal vasculature play a key role. The vascular abnormalities are typically evaluated using fluorescein angiography, a modality with known defects in imaging the deeper layers of the retinal vasculature. Angiography based on optical coherence tomography can image vessels based on flow characteristics without dye injection and may provide improved information concerning the pathophysiology of MacTel 2. Objective: To investigate MacTel 2 using optical coherence tomographic angiography. Design, Setting, and Participants: Fourteen eyes of 7 patients with MacTel 2 were analyzed in a community-based retina practice. The flow imaging was based on split-spectrum amplitude decorrelation angiography, which can dissect layers of vessels in the retina. The inner retinal vascular plexus, the outer plexus, and deeper vascular invasion into the outer and subretinal spaces were optically dissected in en face images based on flow. Main Outcomes and Measures: Visualization and qualitative evaluation of the vascular layers of the retina as they may be affected by MacTel 2, both in terms of depth and topographic characteristics. Results: A consistent set of retinal vascular changes were seen in the eyes with MacTel 2. There was some loss of capillary density in the inner retinal vascular plexus but many more prominent alterations in the deep retinal vascular plexus. In milder forms of the disease, the deep plexus showed dilation and telangiectasis and, in more advanced cases, thinning and loss. The remaining vessels were elongated and widely spaced capillary segments. Invasion by new vessels into the outer and subretinal spaces occurred subjacent to the regions showing greatest flow imaging abnormalities in the inner and deep retinal vascular layers. Conclusions and Relevance: As evidenced by the patients in this study, important retinal vascular changes in MacTel 2 occur in the deep capillary plexus of the retina, a layer poorly visualized by fluorescein angiography and, to a lesser extent, in the inner vascular plexus. The proliferation of vessels in the outer and subretinal spaces may be in part compensatory for poor retinal perfusion by established vascular layers in the retina.

Importance: The retinal vasculature is involved in many ocular diseases that cause visual loss. Although fluorescein angiography is the criterion standard for evaluating the retina vasculature, it has risks of adverse effects and known defects in imaging all the layers of the retinal vasculature. Optical coherence tomography (OCT) angiography can image vessels based on flow characteristics and may provide improved information. Objective: To investigate the ability of OCT angiography to image the vascular layers within the retina compared with conventional fluorescein angiography. Design, Setting, and Participants: In this study, performed from March 14, 2014, through June 24, 2014, a total of 5 consecutive, overlapping B-scan OCT angiography images composed of 216 A-scans were obtained at 216 discrete positions within a region of interest, typically a 2 x 2-mm area of the retina. The flow imaging was based on split-spectrum amplitude decorrelation angiography (SSADA), which can dissect layers of vessels in the retina. These distinct layers were compared with the fluorescein angiograms in 12 healthy eyes from patients at a private practice retina clinic to evaluate the ability to visualize the radial peripapillary capillary network. The proportion of the inner vs outer retinal vascular layers was estimated by 3 masked readers and compared with conventional fluorescein angiograms of the same eyes. Main Outcomes and Measures: Outcome measures were visualization of the radial peripapillary capillary network in the fluorescein and SSADA scans and the proportion of the inner retinal vascular plexus vs the outer retinal capillary plexus as seen in SSADA scans that would match the fluorescein angiogram. Results: In none of the 12 eyes could the radial peripapillary capillary network be visualized completely around the nerve head by fluorescein angiography, whereas the network was readily visualized in the SSADA scans. The fluorescein angiograms were matched, with a mean proportion of the inner vascular plexus being 95.3% (95% CI, 92.2%-97.8%) vs 4.7% (95% CI, 2.6%-5.7%) for the outer capillary plexus from the SSADA scans. Conclusions and Relevance: Fluorescein angiography does not image the radial peripapillary or the deep capillary networks well. However, OCT angiography can image all layers of the retinal vasculature without dye injection. Therefore, OCT angiography, and the findings generated, have the potential to affect clinical evaluation of the retina in healthy patients and patients with disease.

Two eyes with neovascular age-related macular degeneration and a suboptimal response to intravitreal ranibizumab and bevacizumab developed tears after being switched to intravitreal aflibercept, a drug with enhanced binding characteristics to vascular endothelial growth factor. Both eyes had sub-retinal pigment epithelium (RPE) choroidal neovascularization adherent to the back surface of the RPE in the fibrovascular RPE detachment that showed increased contracture of the fibrovascular tissue following the use of aflibercept. The driving force to develop the tears may be related to the recently described angiofibrotic switch, which is governed by the ration of connective tissue growth factor to vascular endothelial growth factor. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:87-90.].

PURPOSE: To survey the anatomic structures seen in the posterior vitreous using a newly developed technique, dynamic focusing and windowed averaging swept source optical coherence tomography. DESIGN: A cross-sectional study of subjects without a history of eye disease or posterior vitreous detachment. METHODS: A focused illumination beam was swept through the scan depth during 96 successive B-scans and the corresponding most highly resolved portion of each scan was used to make an averaged composite image. The main outcome measures were the frequency and interconnectedness of anatomic features visualized. RESULTS: There were 44 eyes of 25 subjects, who ranged in age from 23 to 62. An optically empty space was seen above the macula in all eyes, and corresponded to the premacular bursa. Above the optic nerve head was a conical space corresponding to the area of Martegiani. The two areas were interconnected in 25 (56.8%) of cases. Anterior to the premacular bursa was another lacuna, that was named the supramacular bursa, which was separate from the premacular bursa in horizontal scans centered on the fovea and found in 38 (86.4%) of eyes. Both the supra- and pre-macular bursae coursed anteriorly and in 21 (55.3%) were seen to interconnect. CONCLUSIONS: The anatomic arrangement of the vitreous is consistent in living eyes with no posterior vitreous detachment, and does not correspond precisely to that described from dissection studies of autopsy specimens. The constancy of the specific findings suggests there may be some beneficial effect from the architectural structure of the vitreous that enhances evolutionary fitness.

PURPOSE:: To determine if pseudodrusen seen in fundus photography, particularly infrared scanning laser ophthalmoscopy, colocalize with subretinal drusenoid deposits imaged by optical coherence tomography. METHODS:: The patients were scanned with spectral domain optical coherence tomography having an A-scan spacing of 5.9 mum and a B-scan spacing of 11 mum. En face slabs were derived from this data set at distances 50 mum to 90 mum above the Bruch membrane reference plane to image the subretinal drusenoid deposit and also 6 mum below Bruch membrane to image the level of the choriocapillaris. The corresponding infrared scanning laser ophthalmoscopy image was registered to the optical coherence tomography data by aligning the retinal blood vessels in each imaging modality through elastic warping. RESULTS:: All ten eyes of nine consecutively imaged patients showed a concordance between the pseudodrusen and the subretinal drusenoid deposit in every case. At their more internal aspects, subretinal drusenoid deposits were generally isolated foci of reflectivity and with decreasing distances above the reference plane appeared to become broader, reaching confluence with neighboring deposits analogous to a topographical map of mountains. In contrast to previous reports based on optical coherence tomography, and in keeping with histologic evaluation, no patient was seen to have widespread abnormalities in choriocapillaris imaging. CONCLUSION:: This study, using an unprecedented scan density, showed that pseudodrusen appearance can be attributed to subretinal drusenoid deposits. The results of this study have widespread applicability in the understanding of age-related macular degeneration and the associations of the lesions with other structures in the outer retinal neurovascular unit.