SUCCESSFUL CLOSURE OF A LARGE FULL-THICKNESS MACULAR HOLE SECONDARY TO AN IDIOPATHIC RETINAL VASOPROLIFERATIVE TUMOR USING THE INVERTED INTERNAL LIMITING MEMBRANE FLAP TECHNIQUE.

PURPOSE: To report a case of large full thickness macular hole (MH) as an uncommon complication of an idiopathic retinal vasoproliferative tumor (RPVT) and the successful closure of the full-thickness MH by the inverted internal limiting membrane Flap technique. METHODS: A case report. A 40-year-old woman was referred for a progressive and profound visual loss on the right eye. The fundus examination showed a full-thickness MH and the presence of an idiopathic RPVT surrounding by numerous exudates in the inferior temporal of the peripheral retina. In addition, the spectral domain optical coherence tomography imaging confirmed the large size of the full-thickness MH (740 µ m-diameter). First, we treated the RPVT using the transconjunctival cryotherapy. Then, we performed the macular surgery including 25 Gauge vitrectomy, inverted internal limiting membrane flap technique, and gas tamponade. RESULTS: In the postoperative period, the RPVT remained inactive with no recurrence of the MH or excessive gliosis proliferation on the internal limiting membrane flap area. Moreover, visual acuity rose up from 20/100 (0.2) at 1-month follow-up to 20/40 (0.5) at 6-month follow-up. CONCLUSION: In our patient, the full-thickness MH was precisely large and secondary to an RPVT. The treatment of the tumor by transconjunctival cryotherapy had been efficient and the postoperative follow-up after the macular procedure involving the inverted internal limiting membrane flap technique showed the anatomical and functional recovery of the macula.

Pentosan Polysulfate Maculopathy: Prevalence, Spectrum of Disease, and Choroidal Imaging Analysis Based on Prospective Screening.

PURPOSE: To describe the prevalence and spectrum of disease of pentosan polysulfate (PPS) maculopathy in a large multimodal retinal imaging study and to report the results of choroidal vascularity index (CVI) analysis. DESIGN: Prospective cohort study Methods: Of 741 patients prescribed PPS within a large university database, 100 (13.4%) with any consumption agreed to participate in a prospective screening investigation. Multimodal retinal imaging including near-infrared reflectance (NIR), fundus autofluorescence (FAF), and spectral domain optical coherence tomography (SD-OCT) was performed in all patients. Characteristic findings of affected patients were identified, and affected and unaffected cohorts were compared. CVI, defined as stromal choroidal area (SCA) divided by the total choroidal area, was analyzed. RESULTS: The prevalence of PPS maculopathy was 16%. NIR illustrated punctate hyperreflective lesions with early presentation. FAF illustrated a speckled macular network of hypo- and hyperautofluorescence colocalized with multifocal hyperreflective retinal pigment epithelial lesions on SD-OCT. Advanced cases demonstrated varying degrees of atrophy. The affected cohort exhibited significantly greater mean PPS therapy duration, mean daily dosage, and mean cumulative dosage (19.5±5.5 years, 433.9±137.6 mg, 3,103.1±1,402.2 g) compared with the unaffected cohort (7.1±6.6 years, 291.6±177.6 mg, 768.4±754.8 g). SCA was significantly lower and CVI was significantly greater in the affected vs the unaffected group. CONCLUSIONS: This prospective cohort study identified a prevalence of PPS maculopathy of 15%-20% among PPS users who agreed to participate. A spectrum of findings may be observed with multimodal retinal imaging. Significant choroidal abnormalities associated with this characteristic maculopathy may provide surrogate markers of macular toxicity.

Identification and Characterization of Epivascular Glia Using En Face Optical Coherence Tomography.

PURPOSE: The purpose of this study was to describe the clinical features of epivascular glia (EVG) using en face optical coherence tomography (OCT). DESIGN: Retrospective cross-sectional study. METHODS: Single-institution en face OCT images were reviewed. Eyes displaying EVG were captured with manual internal limiting membrane (ILM) segmentation and analyzed with customized segmentation . A random age- and sex-matched control group was selected to determine relative epiretinal membrane (ERM) prevalence. RESULTS: Characteristic hyper-reflective ILM plaques with dendrite-like radiations were identified using en face OCT and displayed vascular predilection. A total of 161 eyes with EVG (the EVG group) and 2,315 eyes without EVG (control group) were identified from a total cohort of 1,298 patients (or 2,476 eyes). The prevalence of EVG was 161 of 2,476 eyes (6.5%) and 119 of 1,298 patients (9.2%) in the cohort. Mean age was 79.3 ± 10.7 years old in the EVG group and 55.9 ± 24.6 years old in the control group (P <.001). An advanced posterior vitreous detachment (PVD) stage was more common in the EVG group (grade 3: 41.7%; grade 4: 48.6%) than in the control group (grade 3: 18.5%; grade 4: 26.9%; P <.001). Contractile ERM was present in 71 of 161 eyes (44.1%) with EVG compared to 30 of 161 eyes (18.6%) in a random age- and sex-matched control cohort without EVG (P <.001). CONCLUSIONS: EVG previously described with histopathology and scanning electron microscopy can be identified using en face OCT. In this study, these lesions were associated with older age, pseudophakia, and advanced PVD, supporting the role of Müller cell activation through ILM breaks triggered by PVD, a pathogenic mechanism proposed by previous studies.

Choriocapillaris Flow Deficits and Treatment-Naïve Macular Neovascularization Secondary to Age-Related Macular Degeneration.

Purpose: To evaluate choriocapillaris (CC) flow deficits (FD) in eyes with treatment-naïve macular neovascularization (MNV) and to compare CC FD around exudative versus nonexudative MNV. Methods: Treatment-naïve eyes with a diagnosis of either exudative or nonexudative AMD and type 1 MNV were included. Normal control eyes were age-matched to each AMD eye one to one. En face optical coherence tomography angiograms were analyzed for percentage of CC FD (FD%) in two concentric 500 µm rings, ring 1 and ring 2, surrounding the dark halo around MNV. The mean CC FD% in ring 1 and ring 2 was evaluated for each eye. A secondary analysis was similarly carried out to investigate the differences in CC FD% in exudative versus nonexudative treatment-naïve MNV. Results: Twenty-three eyes with treatment-naïve MNV were age matched with 23 normal controls. The mean CC FD% was significantly greater in both rings in the MNV versus the normal control group (P < 0.05) and was significantly greater in the inner ring, closer to the lesion, than the outer ring. The mean FD% was also greater in both rings in the exudative versus the nonexudative MNV group, but this difference did not reach statistical significance. Conclusions: The CC FD% was greater in the area surrounding MNV versus age-matched normal controls and in the ring closer to the MNV lesion. Further, CC FD was greater in eyes with exudative versus nonexudative MNV in both rings surrounding the associated dark halo, although this difference was not statistically significant.

Relationship Between Nerve Fiber Layer Hemorrhages and Outcomes in Central Retinal Vein Occlusion.

Purpose: To evaluate the depth and pattern of retinal hemorrhage in acute central retinal vein occlusion (CRVO) and to correlate these with visual and anatomic outcomes. Methods: Retinal hemorrhages were evaluated with color fundus photography and fluorescein angiography at baseline and follow-up. Snellen visual acuity (VA), central foveal thickness (CFT), extent of retinal ischemia, and development of neovascularization were analyzed. Results: 108 eyes from 108 patients were evaluated. Mean age was 63.6 ± 16.1 years with a predilection for the right eye (73.1%). Average follow-up was 17.2 ± 19.2 months. Mean VA at baseline was 20/126 and 20/80 at final follow-up. Baseline (P = 0.005) and final VA (P = 0.02) in eyes with perivascular nerve fiber layer (NFL) hemorrhages were significantly worse than in eyes with deep hemorrhages alone. Baseline CFT was greater in the group with perivascular hemorrhages (826 ± 394 µm) compared to the group with deep hemorrhages alone (455 ± 273 µm, P < 0.001). The 10 disc areas of retinal ischemia was more common in patients with perivascular (80.0%) and peripapillary (31.3%) versus deep hemorrhages alone (16.1%, P < 0.001). Neovascularization of the iris was more common, although this differrence was not significant, in the groups with peripapillary (14.3%) and perivascular (2.0%) NFL versus deep hemorrhages alone (0.0%). Conclusions: NFL retinal hemorrhages at baseline correlate with more severe forms of CRVO, with greater macular edema, poorer visual outcomes, and greater risk of ischemia and neovascularization. This may be related to the organization of the retinal capillary plexus. The depth and pattern of distribution of retinal hemorrhages in CRVO may provide an easily identifiable early biomarker of CRVO prognosis.

Hyperreflective Stress Lines and Macular Holes.

Purpose: To determine the prevalence of a central hyperreflective line in eyes with full-thickness macular holes (FTMH) and lamellar macular holes (LMH) and to elucidate the pathoanatomic importance of this optical coherence tomography (OCT) sign. Methods: This retrospective analysis evaluated patients with FTMH and LMH at the Stein Eye Institute. Clinical data was collected and SD-OCT volume scans were analyzed for the presence of a central vertical hyperreflective line in 3 separate cohorts: patients with SD-OCT preceding FTMH development, patients with SD-OCT after pars plana vitrectomy (PPVT) treatment for FTMH, and patients with SD-OCT of LMH. Results: In total, 93 eyes with FTMH and 88 eyes with LMH were identified. Of the 93 FTMH eyes, SD-OCT volume scans were available before development of the FTMH in 12 eyes. Of these, 6 (50%) displayed a vertical hyperreflective line preceding the development of the FTMH. Fifty-one eyes underwent PPVT with resolution of the FTMH, and 26 displayed a hyperreflective line after resolution (51%). Of the 88 eyes with LMH, 22 displayed a hyperreflective line (25%). All hyperreflective lines were noted at the central fovea. Conclusions: SD-OCT illustrated the presence of a central vertical hyperreflective line preceding FTMH and after resolution of FTMH after PPVT in approximately one-half of cases, and concurrent with LMH in 25% of cases. This vertical hyperreflective line may represent an early SD-OCT marker for the development of FTMH, and may be a sign of central foveal dehiscence owing to disruption of the Muller cell cone.

Distinctive Mechanisms and Patterns of Exudative Versus Tractional Intraretinal Cystoid Spaces as Seen With Multimodal Imaging.

PURPOSE: To determine clear-cut distinctions between tractional and exudative intraretinal cystoid spaces subtypes. DESIGN: Retrospective, multicenter, observational case series. METHODS: A cohort of patients diagnosed with intraretinal cystoid spaces and imaged with optical coherence tomography (OCT), fluorescein angiography (FA), blue fundus autofluorescence (BFAF), en face OCT, and OCT angiography (OCT-A) was included in the study. All images were qualitatively and quantitatively evaluated. RESULTS: In this study were included 72 eyes of 69 patients. Exudative intraretinal cystoid spaces (36/72 eyes, 50%) displayed a "petaloid" morphology as seen with en face OCT, FA, and BFAF. Tractional intraretinal cystoid spaces (24/72 eyes, 33.3%), displayed a radial "spoke-wheel" en face OCT pattern. There was no leakage with FA and BFAF did not reveal specific patterns. Eyes with full-thickness macular hole (FTMH, 12/72 eyes, 16.7%) displayed a "sunflower" en face OCT appearance. FTMH showed OCT, OCT-A, and BFAF features of both exudative and tractional cystoid spaces, but without any FA leakage. Inner nuclear layer (INL) thickness was significantly lower in tractional cystoid spaces (P < .001). There were a greater number of INL cystoid spaces in both the exudative and FTMH subgroups (P = .001). The surface area of INL cystoid spaces was significantly lower in the tractional subgroup (P < .001). There was a significant reduction of the microvascular density in eyes with exudative vs tractional (P = .002) and FTMH (P < .001) subgroups. CONCLUSIONS: Exudative and tractional intraretinal cystoid spaces displayed characteristic multimodal imaging features and they may represent 2 different pathologic conditions with equally different clinical implications.