Retinal Pigment epithelium-Bruch's Membrane Research Articles

Temporal Changes in the Retinal Pigment Epithelium-Bruch's Membrane Complex Thickness After Autologous Retinal Transplantation in Myopic Eyes.

To investigate the association between the thickness of the retinal pigment epithelium (RPE)-Bruch's membrane (BM) complex and the development of retinal autograft edema as a postoperative complication following autologous retinal transplantation (ART). This retrospective study examined data from 28 eyes of 28 patients (14 males, 14 females; mean age, 61.5 ± 19.8 years) who underwent ART and were followed for 1 year. The RPE-BM complex thickness was measured 2000 µm from the fovea using Image J software. Additionally, the graft blood flow was also evaluated by optical coherence tomography angiography and fluorescein angiography. Macular hole (MH) diameters ranged from 711.2 ± 251.9 µm to 1299.9 ± 333.0 µm, with MH closure achieved in all patients. RPE-BM complex thickness decreased by 4.17 µm at 6 months and 4.34 µm at 1 year, showing significant differences from preoperative measurements (29.88 ± 4.99 µm; 6 months: 95% confidence interval [CI], 1.62-6.71, P = 0.0018; 1 year: 95% CI, 2.03-6.65 µm, P = 0.00044). The decrease was significantly greater in the edema-positive group (95% CI, -8.33 to -0.82, P = 0.020). Furthermore, the rates of ellipsoid zone (EZ) recovery, alignment of neurosensory layers (ANL), and graft reperfusion were lower in the edema-positive group (EZ, P = 0.017; ANL, P = 0.0098; reperfusion, P = 0.039). After ART, RPE-BM complex thickness decreases, particularly in cases with postoperative edema, suggesting a potential relationship between RPE function and postoperative outcomes, highlighting the importance of monitoring RPE-BM complex thickness after surgery.

Outer Retinal Thickness Is Associated With Cognitive Function in Normal Aging to Intermediate Age-Related Macular Degeneration.

Research on Alzheimer's disease (AD) and precursor states demonstrates a thinner retinal nerve fiber layer (NFL) compared to age-similar controls. Because AD and age-related macular degeneration (AMD) both impact older adults and share risk factors, we asked if retinal layer thicknesses, including NFL, are associated with cognition in AMD. Adults ≥ 70 years with normal retinal aging, early AMD, or intermediate AMD per Age-Related Eye Disease Study (AREDS) nine-step grading of color fundus photography were enrolled in a cross-sectional study. Optical coherence tomography (OCT) volumes underwent 11-line segmentation and adjustments by a trained operator. Evaluated thicknesses reflect the vertical organization of retinal neurons and two vascular watersheds: NFL, ganglion cell layer-inner plexiform layer complex (GCL-IPL), inner retina, outer retina (including retinal pigment epithelium-Bruch's membrane), and total retina. Thicknesses were area weighted to achieve mean thickness across the 6-mm-diameter Early Treatment of Diabetic Retinopathy Study (ETDRS) grid. Cognitive status was assessed by the National Institutes of Health Toolbox cognitive battery for fluid and crystallized cognition. Correlations estimated associations between cognition and thicknesses, adjusting for age. Based on 63 subjects (21 per group), thinning of the outer retina was significantly correlated with lower cognition scores (P < 0.05). No other retinal thickness variables were associated with cognition. Only the outer retina (photoreceptors, supporting glia, retinal pigment epithelium, Bruch's membrane) is associated with cognition in aging to intermediate AMD; NFL was not associated with cognition, contrary to AD-associated condition reports. Early and intermediate AMD constitute a retinal disease whose earliest, primary impact is in the outer retina. Our findings hint at a unique impact on the brain from the outer retina in persons with AMD.

Quantification of choroidal hyperreflective layer: A swept-source optical coherence tomography study.

To investigate variation in reflectivity of choroidal layers in normal eyes. From the swept-source optical coherence tomography database, we retrospectively included eyes with a normal fundus. Choroidal reflectivity was measured on the horizontal and vertical B-scan optical coherence tomography images. The optical barrier of the choroid was defined as the first hill in the middle of the reflectance graph from the retinal pigment epithelium-Bruch's membrane complex to the chorioscleral junction. The optical barrier of the choroid was identified in 91 eyes of 91 individuals. The amplitude of peak reflectivity of the optical barrier of the choroid at macular center (142.85 ± 15.04) was greater than those in superior (136.12 ± 14.08) or inferior macula (135.30 ± 16.13) (P = 0.028, P = 0.008, respectively). Latency between the peak of the retinal pigment epithelium-Bruch's membrane complex and the optical barrier of the choroid at macular center (48.11 ± 13.78 μm) was shorter than those in nasal macula (55.58 ± 19.21 μm) (P = 0.021). The amplitude of the peak reflectivity of the optical barrier of the choroid in the center negatively correlated with the latency between the retinal pigment epithelium-Bruch's membrane complex and the optical barrier of the choroid (P < 0.001). An optical barrier exists in the inner choroid of the normal eye. Its depth depends on the location within the macula. Further studies are mandatory to evaluate variations in the barrier in the eyes with chorioretinal disease.

Retinal pigment epithelium-Bruch's membrane volume in grading of age-related macular degeneration.

To assess the agreement of optical coherence tomography (OCT) algorithm-based retinal pigment epithelium -Bruch's membrane complex volume (RBV) with fundus photograph-based age-related macular degeneration (AMD) grading. Digital color fundus photographs (CFPs) and spectral domain OCT images were acquired from 96 elderly subjects. CFPs were graded according to Age-Related Eye Disease Study (AREDS) classification. OCT image segmentation and RBV data calculation were done with Orion™ software. Univariate and multivariate analyses were performed to find out whether AMD lesion features associated with higher RBVs. RBV correlated with AMD grading (rs=0.338, P=0.001), the correlation was slightly stronger in early AMD (n=52; rs=0.432, P=0.001). RBV was higher in subjects with early AMD compared with those with no AMD lesions evident in fundus photographs (1.05±0.20 vs 0.96±0.13 mm3, P=0.023). In multivariate analysis higher RBVs were associated significantly with higher total drusen (β=0.388, P=0.027) and pigmentation areas (β=0.319, P=0.020) in fundus photographs, whereas depigmentation area (β=-0.295, P=0.015) associated with lower RBV. RBV correlate with AMD grading status, with a stronger association in patients with moderate, non-late AMD grades. This effect is driven mostly by lesions with drusen or pigmentation. Lesions with depigmentation tend to have lower values. RBV is more comprehensive measurement of the key area of AMD pathogenesis, compared to sole drusen volume analysis. RBV measurements are independent on grader variations and offer a possibility to quantify early and middle grade AMD lesions in a research setting, but may not substitute fundus photograph-based grading in the whole range of AMD spectrum.

A Novel Surgical Approach for Big Sheet Allogenic Retinal Pigment Epithelium-Bruch Membrane Complex Transplantation Into the Subretinal Space.

Allogenic transplantation of retinal pigmented epithelium monolayer sheet has experienced bottlenecks due to imperfect surgical techniques. In this study, we developed a novel approach for allogenic transplantation of big sheets of retinal pigment epithelium (RPE)-Bruch membrane complex. RPE-Bruch membrane complex sheets of 5 × 6 mm2 to 10 × 10 mm2 were taken from donated eyes. Through a novel approach, the sheets of RPE-Bruch membrane complex were transplanted into the subretinal space of eight eyes (8 patients) with late-stage retinitis pigmentosa. The patients were followed up for 5 ± 2 months. All RPE-Bruch membrane complexes were successfully inserted into the subretinal space during the surgery. Follow-up examinations also showed that the grafts attached well to the transplantation site. No rejection or retinal detachment was found. Through our technique, big sheets of allogenic RPE-Bruch membrane complexes could be implanted into the subretinal space smoothly. This novel approach may be useful for big sheet of allogenic RPE-derived or stem cells-derived RPE transplantation in the treatment of RP and other retinal dystrophic diseases.

GENETIC FACTORS AND CHARACTERISTICS ON SPECTRAL-DOMAIN OPTICAL COHERENCE TOMOGRAPHY ARE ASSOCIATED WITH CHOROIDAL THICKNESS IN ABCA4 -RELATED RETINOPATHY.

To investigate the possible correlation factors of choroidal thickness in ABCA4 -related retinopathy. A total of 66 patients were included in the cohort. It is a retrospective, cross-sectional laboratory investigation. The patients were tested using whole-exon sequencing and ophthalmic examinations, including slit-lamp examinations, best-corrected visual acuity, spectral-domain optical coherence tomography, fundus photograph, and fundus autofluorescence. Besides demographic characteristics (age, onset age, duration), we selected genetic factors and ocular characteristics on spectral-domain optical coherence tomography as the candidates related to choroidal thickness. Mutation type (inframe mutation or premature termination codon), epiretinal membrane, retinal pigment epithelium- Bruch membrane integrity, and macular curvature changes were identified as related factors to choroidal thickness in ABCA4 -related retinopathy after the adjustment of Logistic LASSO regression. Mutation type, epiretinal membrane, retinal pigment epithelium-Bruch membrane integrity, and macular curvature changes are related factors to choroidal thinning. These findings could provide us a further understanding for the pathological process and clinical features of ABCA4 mutation.

Review of Retinal Imaging Modalities for Hydroxychloroquine Retinopathy.

This review provides an overview of conventional and novel retinal imaging modalities for hydroxychloroquine (HCQ) retinopathy. HCQ retinopathy is a form of toxic retinopathy resulting from HCQ use for a variety of autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus. Each imaging modality detects a different aspect of HCQ retinopathy and shows a unique complement of structural changes. Conventionally, spectral-domain optical coherence tomography (SD-OCT), which shows loss or attenuation of the outer retina and/or retinal pigment epithelium-Bruch's membrane complex, and fundus autofluorescence (FAF), which shows parafoveal or pericentral abnormalities, are used to assess HCQ retinopathy. Additionally, several variations of OCT (retinal and choroidal thickness measurements, choroidal vascularity index, widefield OCT, en face imaging, minimum intensity analysis, and artificial intelligence techniques) and FAF techniques (quantitative FAF, near-infrared FAF, fluorescence lifetime imaging ophthalmoscopy, and widefield FAF) have been applied to assess HCQ retinopathy. Other novel retinal imaging techniques that are being studied for early detection of HCQ retinopathy include OCT angiography, multicolour imaging, adaptive optics, and retromode imaging, although further testing is required for validation.

Arterial hypertension and retinal layer thickness: the Beijing Eye Study

PurposeTo investigate relationships between blood pressure and the thickness of single retinal layers in the macula.MethodsParticipants of the population-based Beijing Eye Study, free of retinal or optic nerve disease, underwent...

New Prospects for Retinal Pigment Epithelium Transplantation.

Retinal pigment epithelium (RPE) transplants rescue photoreceptors in selected animal models of retinal degenerative disease. Early clinical studies of RPE transplants as treatment for age-related macular degeneration (AMD) included autologous and allogeneic transplants of RPE suspensions and RPE sheets for atrophic and neovascular complications of AMD. Subsequent studies explored autologous RPE-Bruch membrane-choroid transplants in patients with neovascular AMD with occasional marked visual benefit, which establishes a rationale for RPE transplants in late-stage AMD. More recent work has involved transplantation of autologous and allogeneic stem cell-derived RPE for patients with AMD and those with Stargardt disease. These early-stage clinical trials have employed RPE suspensions and RPE monolayers on biocompatible scaffolds. Safety has been well documented, but evidence of efficacy is variable. Current research involves development of better scaffolds, improved modulation of immune surveillance, and modification of the extracellular milieu to improve RPE survival and integration with host retina.

Effect of aging and lifestyle on healthy macular photoreceptors and retinal pigment epithelium-Bruch membrane complex thickness.

Investigate how aging and some lifestyle factors correlate with changes in macular photoreceptor and retinal pigment epithelium-Bruch membrane complex layers thickness in a sample of healthy population by using OCT segmentation technique. A cross-sectional study conducted at the ophthalmology department in the Medical City in Baghdad. All participants underwent an interview of medical history and lifestyle habits. Maculae of all participants were scanned using spectral domain optical coherence tomography, followed by manual segmentation of retinal layers. The study included 152 healthy participants (152 eyes), their mean age was 57.3 ± 6.7 years, gender distribution was 82(53.9%) males and 70(46.1%) females. There was a decrement in retinal pigment epithelium-Bruch membrane layer thickness at central subfield (500 µm radius from fovea minimum) by - 0.178 µm/year (p = 0.019), inner macula (500-1500 µm radius from fovea minimum) by - 0.263 µm/year (p = 0.002), outer macula (1500-3000 µm radius from fovea minimum) by - 0.225 µm/year (p = 0.015) with no statistically significant effect on photoreceptor thickness. Physical activity and smoking had statistically no significant effect on these layers thickness, however smokers had higher photoreceptor thickness at fovea minimum and central subfield. Body mass index increase by one kg/m2 correlated with a decrement in photoreceptor layer thickness by - 0.108 µm (p = 0.007). Aging process affected the retinal pigment epithelium-Bruch membrane complex and photoreceptor layer thickness in a healthy population, and this process can be slowed down by avoiding some lifestyles which aggravate these changes like obesity.

Cross-sectional assessment of the ellipsoid zone and the retinal pigment epithelium-Bruch membrane complex after systemic isotretinotin use

Purpose To evaluate the cross-sectional areas of the retinal pigment epithelium-Bruch membrane complex (RPE-B) and ellipsoid zon (EZ) and the thickness of the macula, retinal nerve fibre layer (RNFL), and ganglion cell-inner plexiform layer (GC-IPL) in patients using short-term systemic isotretinoin. Method A total of 43 right eyes of 43 patients treated with systemic isotretinoin for acne vulgaris were included in this prospective study. Macula, GC-IPL, RNFL thicknesses and central cross-sectional EZ and RPE-B areas were evaluated with optical coherence tomography (Zeiss, Cirrus HD OCT 5000) prior to treatment and in first, second and third months after the onset of isotretinoin treatment. For the measurement of EZ and RPE-B area, foveal EDI-OCT scans were binarized by using the public domain software ImageJ 1.51 s. Results Mean duration of isotretinoin treatment was 77 ± 15 days and mean dose was 2228 ± 574 milligrams. There was a statistically significant increment in central cross-sectional EZ and RPE-B areas in each follow-up examination, when analysed by repeated measurement analysis (p:0.002 and p:0.006, respectively). There was no correlation between total isotretinoin dose and the difference between final and basal EZ and RPE-B areas (p > 0.05, for both). When repeated measurements in follow-up examinations were compared, GC-IPL thicknesses except the superotemporal region (p:0.040) and RNFL thicknesses did not show a significant difference (p > 0.05). There was not any significant relation between total isotretinoin dose and 3rd month and basal measurement differences in macula, GC-IPL and RNFL thicknesses in any area (p > 0.05, for all). Conclusion There has been an increase in the area of RPE-B and EZ with short-term use of isotretinoin therapy. Future studies examining the relationship between functional tests and the RPE-B and EZ areas may provide more in-depth information on the effects of isotretinoin in the eye.

Clinical Evaluation of an Instrument-Integrated OCT-Based Distance Sensor for Robotic Vitreoretinal Surgery

PurposeTo assess the efficacy of an instrument-integrated OCT (iiOCT)-based distance sensor during robotic vitreoretinal surgery using the Preceyes Surgical System (PSS; Preceyes B.V.).DesignSingle-center interventional study.ParticipantsPatients requiring vitreoretinal surgery.MethodsFive patients were enrolled. Standard preoperative OCT images were obtained. After vitrectomy, a predefined set of actions was performed using the iiOCT-based sensor. Images then were processed to assess the signal-to-noise ratio (SNR) at various angles to the retina and at different distances between the instrument tip and the retinal surface. Preoperative and intraoperative OCT images were compared qualitatively and quantitatively.Main Outcomes MeasuresThe feasibility in performing surgical tasks using the iiOCT-based sensor during vitreoretinal surgery, the SNR when imaging the retina, differences among intraoperative and preoperative OCT images, and characteristics of intraoperative retinal movements detected with the iiOCT-based probe.ResultsSurgeons were able to perform all the tasks but one. The PSS was able to maintain a fixed distance. The SNR of the iiOCT-based sensor signal was adequate to determine the distance to the retina and to control the PSS. Analysis of iiOCT-based sensor A-scans identified 3 clearly distinguishable retinal layers, including the inner retinal boundary and the interface at the retinal pigment epithelium–Bruch’s membrane. Thickness values differed by less than 5% from that measured by preoperative OCT, indicating its accuracy. The Fourier analysis of iiOCT-based sensor recordings identified anteroposterior retinal movements attributed to heartbeat and respiration.ConclusionsThis iiOCT-based sensor was tested successfully and promises reliable use during robot-assisted surgery. An iiOCT-based sensor is a promising step toward OCT-guided robotic retinal surgery.

AICAR upregulates ABCA1/ABCG1 expression in the retinal pigment epithelium and reduces Bruch's membrane lipid deposit in ApoE deficient mice

The etiology of age-related macular degeneration (AMD) is diverse; however, recent evidence suggests that the lipid metabolism-cholesterol pathway might be associated with the pathophysiology of AMD. The ATP-binding cassette (ABC) transporters, ABCA1 and ABCG1, are essential for the formation of high-density lipoprotein (HDL) and the regulation of macrophage cholesterol efflux. The failure of retinal or retinal pigment epithelium (RPE) cholesterol efflux to remove excess intracellular lipids causes morphological and functional damage to the retina. In this study, we investigated whether treatment with 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), an AMP-activated protein kinase (AMPK) activator, improves RPE cholesterol efflux and Bruch's membrane (BM) lipid deposits. The protein and mRNA levels of ABCA1 and ABCG1 in ARPE-19 cells and retinal and RPE/choroid tissue from apolipoprotein E-deficient (ApoE-/-) mice were evaluated after 24 weeks of AICAR treatment. The cholesterol efflux capacity of ARPE-19 cells and the cholesterol-accepting capacity of apoB-depleted serum from mice were measured. The thickness of the BM and the degree of lipid deposition were evaluated using electron microscopy. AICAR treatment increased the phosphorylation of AMPK and the protein and mRNA expression of ABCA1 and ABCG1 in vitro. It promoted cholesterol efflux from ARPE-19 cells and upregulated the protein and mRNA levels of ABCA1 and ABCG1 in the retina and RPE in vivo. ApoB-depleted serum from the AICAR-treated group showed enhanced cholesterol-accepting capacity. Long-term treatment with AICAR reduced BM thickening and lipid deposition in ApoE-/- mice. In conclusion, AICAR treatment increased the expression of lipid transporters in the retina and RPE in vivo, facilitated intracellular cholesterol efflux from the RPE in vitro, and improved the functionality of HDL to accept cholesterol effluxed from the cell, possibly via AMPK activation. Collectively, these effects might contribute to the improvement of early age-related pathologic changes in the BM. Pharmacological improvement of RPE cholesterol efflux via AMPK activation may be a potential treatment strategy for AMD.

Inflammatory retinal and choroidal neovascularization in a case of post-fever immune retinitis

The development of retinal and choroidal neovascularization in posterior uveitis is attributed to the angio-inflammatory drive with/without ruptures in the retinal pigment epithelium-Bruch's membrane complex. We report a unique case of a 15-year-old Asian-Indian female who developed immune retinitis post-typhoid fever along with simultaneous retinal and choroidal neovascularization. After ruling out infectious etiologies, she was initiated on a course of systemic steroids considering that the retinitis had immune-mediated pathogenesis. Subsequently, the retinitis lesion and choroidal neovascular membrane healed with scarring while the retinal neovascular complex showed fibrotic regression.

PERIPHERAL EXUDATIVE HEMORRHAGIC CHORIORETINOPATHY-A NEW ADDITION TO THE SPECTRUM OF PACHYCHOROID DISEASE?

Purpose: To demonstrate choroidal vascular changes and report a novel choroidal thickness contour in eyes with peripheral exudative hemorrhagic chorioretinopathy (PEHCR). Methods: Retrospective, observational, comparative case series. Fourteen eyes of nine patients with PEHCR and 14 eyes of 14 age-matched and sex-matched controls underwent swept-source optical coherence tomography. Choroidal thickness was measured from posterior edge of the retinal pigment epithelium–Bruch membrane to choroidoscleral interface at 11 points 1,000 µm apart. Large choroidal vessel thickness was also measured. Results: In PEHCR group, the choroid was thinnest at 3 mm nasal to fovea (mean 95.3 ± 33.5 µm) and thickest at 7 mm temporal to fovea (mean 272.7 ± 80.2 µm), with gradual increase in choroidal thickness from nasal to temporal periphery. The choroid was thickest subfoveally (259.7 ± 63.8 µm) in the control group. The choroid was significantly thicker in temporal periphery in PEHCR eyes as compared to controls (P = 0.0002). The mean large choroidal vessel thickness was 202.4 ± 50.8 µm in the PEHCR group and 160.6 ± 40.5 µm in the control group (P = 0.0235). Conclusion: Peripheral exudative hemorrhagic chorioretinopathy eyes showed progressively increasing choroidal thickness toward the temporal periphery, compared with age-matched and sex-matched controls. This gave rise to a club-shaped choroidal contour compared with the bowl-shaped contour seen in control eyes. Thicker choroid and pachyvessels favor inclusion of PEHCR in the pachychoroid disease spectrum.

Presumed Natural History of Combined Hamartoma of the Retina and Retinal Pigment Epithelium

To correlate structural changes of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) with patient age. Retrospective study. Fifty eyes of 49 patients (age range, 1-74 years) with CHRRPE studied at 9 tertiary vitreoretinal institutions. We analyzed the clinical findings with respect to lesion topography and pigmentation as well as investigated the OCT findings regarding the thickness, vitreoretinal interface, outer plexiform layer distortion, ellipsoid zone disruption, and retinal pigment epithelium-Bruch's membrane complex involvement of CHRRPE. Clinical and imaging findings of CHRRPE at different ages. Analysis of 50 CHRRPE patients revealed that younger patients were more likely to demonstrate partial thickness involvement of the retina (P= 0.009) with predominantly inner retinal layer involvement (P=0.04). The inverse was true for older patients with CHRRPE. In addition, older patients more commonly showed pigmentary changes. Eyes with CHRRPE were more likely to show an increase in central macular thickness independently of tumor location. Based on these findings, we believe that CHRRPE typically begins in the inner retina and continues toward the outer retina over time, with increase in central macular thickness, despite the location of the tumor.

Genetic LAMP2 deficiency accelerates the age-associated formation of basal laminar deposits in the retina

The early stages of age-related macular degeneration (AMD) are characterized by the accumulation of basal laminar deposits (BLamDs). The mechanism for BLamDs accumulating between the retinal pigment epithelium (RPE) and its basal lamina remains elusive. Here we examined the role in AMD of lysosome-associated membrane protein-2 (LAMP2), a glycoprotein that plays a critical role in lysosomal biogenesis and maturation of autophagosomes/phagosomes. LAMP2 was preferentially expressed by RPE cells, and its expression declined with age. Deletion of the Lamp2 gene in mice resulted in age-dependent autofluorescence abnormalities of the fundus, thickening of Bruch's membrane, and the formation of BLamDs, resembling histopathological changes occurring in AMD. Moreover, LAMP2-deficient mice developed molecular signatures similar to those found in human AMD-namely, the accumulation of APOE, APOA1, clusterin, and vitronectin-adjacent to BLamDs. In contrast, collagen 4, laminin, and fibronectin, which are extracellular matrix proteins constituting RPE basal lamina and Bruch's membrane were reduced in Lamp2 knockout (KO) mice. Mechanistically, retarded phagocytic degradation of photoreceptor outer segments compromised lysosomal degradation and increased exocytosis in LAMP2-deficient RPE cells. The accumulation of BLamDs observed in LAMP2-deficient mice was eventually followed by loss of the RPE and photoreceptors. Finally, we observed loss of LAMP2 expression along with ultramicroscopic features of abnormal phagocytosis and exocytosis in eyes from AMD patients but not from control individuals. Taken together, these results indicate an important role for LAMP2 in RPE function in health and disease, suggesting that LAMP2 reduction may contribute to the formation of BLamDs in AMD.

En Face Optical Coherence Tomography Imaging of the Photoreceptor Layers in Hydroxychloroquine Retinopathy

To investigate the application of en face optical coherence tomography (OCT) imaging in eyes with hydroxychloroquine (HCQ) retinopathy. Retrospective case series. Setting: Institutional. Sixty-two eyes of 31 Asian patients with HCQ retinopathy. Macular volume scanning using swept-source OCT was performed in 6× 6-mm and 9× 9-mm areas centered on the fovea. Segmentation of the photoreceptor layers was automatically performed between the inner border of the ellipsoid zone and that of the retinal pigment epithelium-Bruch membrane complex to obtain en face OCT images. Findings from the en face images were qualitatively and quantitatively evaluated; they were analyzed and correlated with the fundus autofluorescence and visual field findings. En face OCT findings. All eyes with HCQ retinopathy had a beaten-bronze appearance in the areas with photoreceptor defects, whereas those with intact photoreceptors had areas with smooth surfaces, which were occasionally demarcated by hyporeflective margins, on the en face OCT images. The presence and extent of the retinopathy could be quickly determined using the images. They also provided quantitative information on the progression based on the areas of intact photoreceptors compared over several visits. Furthermore, the area of central intact photoreceptors significantly correlated with the mean deviation, pattern standard deviation, and visual field index of 30-2 visual field examinations (all P < .001). En face OCT may be useful for visualizing the presence and extent of HCQ retinopathy and its progression. The area of central intact photoreceptors measured on en face OCT images showed a significant association with functional visual field defects. This imaging may be a helpful adjunct for screening and follow-up examinations of HCQ retinopathy.

Pigmented paravenous retinochoroidal atrophy: a case report

BackgroundPigmented paravenous retinochoroidal atrophy (PPRCA) is an unusual retinal degeneration, and its performance on optical coherence tomography angiography (OCTA) is unclear. We report a Chinese female case of PPRCA and her OCTA features.Case presentationA 66-year-old female patient was referred to the author’s center for gradual progressive loss of vision in both eyes and photophobia of 2 years duration. She reported having no family history of inherited ocular diseases. The funduscopic examination revealed bone-spicule pigmentation and retinochoroidal atrophy along the retinal veins. This patient was diagnosed with PPRCA which is a rare disease, uncommon in females, more commonly affecting the paravascular fundus. Noninvasive imaging techniques features of this patient was described, including ultra-wide field fundus autofluorescence, spectral domain optical coherence tomography (SD-OCT), OCTA (SSADA), etc. The en face OCTA images demonstrated areas of flow void beneath the retinal pigment epithelium-Bruch membrane layer suggestive of choriocapillaris hypoperfusion that corresponded with indocyanine green angiography (ICGA). Further studies should be conducted to clarify the relationship between choriocapillaris hypoperfusion and the development of PPRCA.ConclusionsThe OCTA features in patients with PPRCA has not been described previously in the literature. This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease.

Multimodal Imaging for Differential Diagnosis of Bietti Crystalline Dystrophy

To evaluate the diagnostic usefulness of multimodal imaging in patients with Bietti crystalline dystrophy (BCD). Retrospective cross-sectional study. Patients with chorioretinal dystrophy accompanied by crystalline-like deposits. The right eyes of the patients were analyzed. Fundus photograph, near-infrared reflectance (NIR), fundus autofluorescence (FAF), and OCT images were evaluated. Presence of hyperreflectivity on NIR, well-demarcated areas of decreased FAF, hyperreflective material at or on the retinal pigment epithelium-Bruch's membrane complex, and outer retinal tubulation were graded for each patient. All exons and franking introns of CYP4V2 were screened using Sanger sequencing. Sensitivity and specificity of the findings to discriminate patients with and without CYP4V2 mutation. In total, 33 patients were included in the study. Sanger sequencing revealed homozygous or compound heterozygous CYP4V2 mutations in 20 patients and heterozygous mutations in 2 patients. Among the investigated factors, hyperreflective appearance on NIR imaging yielded 100% sensitivity and 100% specificity in this cohort. The presence of outer retinal tubulation also was sensitive (95%), but specificity was moderate (45%). The revised diagnoses of patients without CYP4V2 mutations included retinitis pigmentosa, late-onset macular dystrophy, and central areolar choroidal dystrophy. Multimodal imaging, especially NIR imaging, is useful to differentiate BCD patients with CYP4V2 mutations from patients with other chorioretinal dystrophies accompanied by crystalline-like retinal deposits.