Hyperreflective Band Research Articles

Hyperreflective ganglion cell layer band in a large cohort of non-syndromic retinitis pigmentosa: Frequency and clinical correlations.

Recently, an 'hyperreflective ganglion cell layer band' (HGB) has been described on spectral-domain optical coherence tomography (SD-OCT) in a subset of patients with retinitis pigmentosa (RP). This study aims to validate and describe the frequency of HGB in a large cohort of Portuguese patients with RP. This single-centre, cross-sectional cohort study included consecutive patients with a genetic diagnosis of RP. SD-OCT images were reviewed to identify the presence of the HGB and other retinal comorbidities. The HGB was defined as a continuous hyperreflective band within the thickness of the ganglion cell layer (GCL). We built mixed-effects regression models, accounting for inter-eye correlations, to investigate features predictive of visual acuity. Subsequently, a reduced model was fitted. A total of 398 eyes from 201 patients were included. HGB was identified in 69 (17.3%) eyes from 39 (19.4%) patients. Patients presenting with the HGB were significantly younger at diagnosis and at symptom onset. Median BCVA [ETDRS (IQR)] was 65 (29) letters in eyes with the HGB and 70 (21) letters in eyes without HGB (p < 0.001). In both the full and reduced mixed-effects models, the presence of HGB and macular hole (MH) was significantly associated with worse BCVA. This study validates the recent description of HGB within the GCL in a subset of patients with RP. Eyes with HGB demonstrated significantly worse BCVA compared to those without HGB, suggesting that the presence of HGB may serve as an SD-OCT biomarker of worse visual prognosis in these patients.

Photoreceptor outer segment reflectivity with ultrahigh resolution visible light optical coherence tomography in systemic hydroxychloroquine use.

To evaluate outer retinal organization in normal subjects and those using hydroxychloroquine (HCQ) with ultrahigh resolution visible light optical coherence tomography (VIS-OCT). Forty eyes of 22 adult subjects were recruited from a tertiary care retina practice including controls (20 eyes, 12 subjects, mean age 40±22yrs, mean logMAR BCVA 0.19, 90% female) and subjects with a history of HCQ use (20 eyes, 10 subjects, mean age 62±17yrs, mean logMAR BCVA 0.03, 67% female). Each subject was imaged using a custom-built VIS-OCT device (axial resolution 1.3μm) and FDA-approved OCT devices. Using VIS-OCT, control subjects demonstrate 5 and 6 hyperreflective bands in the foveal and parafoveal regions, respectively, between the outer nuclear layer and Bruch's membrane. These bands demonstrate intensity profiles complementary to the known histopathologic distribution of rods and cones. In comparison to controls, subjects taking HCQ demonstrate blunting of all bands, particularly bands 2-4. In all cases of suspected or known toxicity, VIS-OCT demonstrated attenuation of band 3i and in no cases was there attenuation of other bands that was more severe than band 3i, suggesting that changes in the reflectivity of Band 3i may be the earliest identifiable sign of HCQ toxicity. VIS-OCT of the outer retina demonstrates a unique outer retinal banding pattern corresponding to photoreceptor density profiles. There is a notable attenuation of the photoreceptor outer segment reflectivity profile associated with early HCQ toxicity. This finding may be an early, and possibly reversible, sign of HCQ toxicity, primarily impacting the cones.

The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease.

Stargardt disease (STGD1), associated with biallelic variants in the ABCA4 gene, is the most common heritable macular dystrophy and is currently untreatable. To identify potential treatment targets, we characterized surviving STGD1 photoreceptors. We used clinical data to identify macular regions with surviving STGD1 photoreceptors. We compared the hyperreflective bands in the optical coherence tomographic (OCT) images that correspond to structures in the STGD1 photoreceptor inner segments to those in controls. We used adaptive optics scanning light ophthalmoscopy (AO-SLO) to study the distribution of cones and AO-OCT to evaluate the interface of photoreceptors and retinal pigment epithelium (RPE). We found that the profile of the hyperreflective bands differed dramatically between patients with STGD1 and controls. AO-SLOs showed patches in which cone densities were similar to those in healthy retinas and others in which the cone population was sparse. In regions replete with cones, there was no debris at the photoreceptor-RPE interface. In regions with sparse cones, there was abundant debris. Our results raise the possibility that pharmaceutical means may protect surviving photoreceptors and so mitigate vision loss in patients with STGD1.

Hyperreflective Ganglion Cell Layer Band in Gyrate Atrophy.

We have described the hyperreflective ganglion cell layer band (HGCB) in a series of cases of gyrate atrophy. Clinical fundus examination and multimodal imaging which included optical coherence tomography (OCT) was done in all cases. Four patients (one male, three female) were studied. In all four cases, a hyperreflective band was noted in the ganglion cell layer. In three patients, the band was continuous, and in one patient, the band was patchy. To conclude, HGCB is a novel OCT sign in gyrate atrophy and can be valuable in prognostication of disease. [Ophthalmic Surg Lasers Imaging Retina 2024;55:598-602.].

Biomarker evidence of early vision and rod energy-linked pathophysiology benefits from very low dose DMSO in 5xFAD mice

Here, we test whether early visual and OCT rod energy-linked biomarkers indicating pathophysiology in nicotinamide nucleotide transhydrogenase (Nnt)-null 5xFAD mice also occur in Nnt-intact 5xFAD mice and whether these biomarkers can be pharmacologically treated. Four-month-old wild-type or 5xFAD C57BL/6 substrains with either a null (B6J) Nnt or intact Nnt gene (B6NTac) and 5xFAD B6J mice treated for one month with either R-carvedilol + vehicle or only vehicle (0.01% DMSO) were studied. The contrast sensitivity (CS), external limiting membrane-retinal pigment epithelium (ELM-RPE) thickness (a proxy for low pH-triggered water removal), profile shape of the hyperreflective band just posterior to the ELM (i.e., the mitochondrial configuration within photoreceptors per aspect ratio [MCP/AR]), and retinal laminar thickness were measured. Both wild-type substrains showed similar visual performance indices and dark-evoked ELM-RPE contraction. The lack of a light–dark change in B6NTac MCP/AR, unlike in B6J mice, is consistent with relatively greater mitochondrial efficiency. 5xFAD B6J mice, but not 5xFAD B6NTac mice, showed lower-than-WT CS. Light-adapted 5xFAD substrains both showed abnormal ELM-RPE contraction and greater-than-WT MCP/AR contraction. The inner retina and superior outer retina were thinner. Treating 5xFAD B6J mice with R-carvedilol + DMSO or DMSO alone corrected CS and ELM-RPE contraction but not supernormal MCP/AR contraction or laminar thinning. These results provide biomarker evidence for prodromal photoreceptor mitochondrial dysfunction/oxidative stress/oxidative damage, which is unrelated to visual performance, as well as the presence of the Nnt gene. This pathophysiology is druggable in 5xFAD mice.

Presumed macular toxicity of high-dose intravitreal ganciclovir

Abstract: A 56-year-old male with bilateral cytomegalovirus retinitis was initiated on oral valganciclovir and intravitreal ganciclovir injection twice a week in first 2 weeks and once a week in subsequent weeks. An intravitreal dose of 2 mg/0.08 mL was administered in the 1st week. Considering the suboptimal response, the dose of intravitreal ganciclovir was increased to 4 mg/0.08 mL. After 4 weeks of therapy (four doses of 4-mg ganciclovir), the patient complained of worsening of vision. Fundus examination showed resolving retinitis, but the optical coherence tomography (OCT) showed neurosensory detachment (NSD) at the fovea in both eyes with inner retinal hyperreflectivity in the right eye. Considering the possibility of retinitis involving fovea, a higher dose of intravitreal ganciclovir (6 mg/0.08 mL) was administered. The patient reported further worsening of vision immediately after removing the eye patch 2 hr following the injection. OCT showed a full-thickness hyperreflective vertical band with ellipsoid zone disruption at the fovea and inner retinal hyperreflectivity in the right eye. Hyperreflective vertical band with NSD was seen in the left eye. A possibility of macular toxicity due to intravitreal ganciclovir was considered. Intravitreal therapy was stopped and oral valganciclovir was continued. At 1-month follow-up, the patient reported improvement in vision. The hyperreflectivity and NSD were resolved at month follow-up. Although higher doses of ganciclovir up to 6 mg have been used in the past, it would be better to avoid higher doses considering the development of presumed drug toxicity in our case following the use of 4 mg and 6 mg.

Acetazolamide Challenge Changes Outer Retina Bioenergy-Linked and Anatomical OCT Biomarkers Depending on Mouse Strain.

The purpose of this study was to test the hypothesis that optical coherence tomography (OCT) bioenergy-linked and anatomical biomarkers are responsive to an acetazolamide (ACZ) provocation. C57BL/6J mice (B6J, a strain with relatively inefficient mitochondria) and 129S6/ev mice (S6, a strain with relatively efficient mitochondria) were given a single IP injection of ACZ (carbonic anhydrase inhibitor) or vehicle. In each mouse, the Mitochondrial Configuration within Photoreceptors based on the profile shape Aspect Ratio (MCP/AR) index was determined from the hyper-reflective band immediately posterior to the external limiting membrane (ELM). In addition, we tested for ACZ-induced acidification by measuring contraction of the external limiting membrane-retinal pigment epithelium (ELM-RPE) thickness; the hyporeflective band (HB) signal intensity at the photoreceptor tips was also examined. Finally, the nuclear layer thickness was measured. In response to ACZ, MCP/AR was greater-than-vehicle in B6J mice and lower-than-vehicle in S6 mice. ACZ-treated B6J and S6 mice both showed ELM-RPE contraction compared to vehicle-treated mice, consistent with dehydration in response to subretinal space acidification. The HB intensity at the photoreceptor tips and the outer nuclear layer thickness (B6J and S6), as well as the inner nuclear layer thickness of B6J mice, were all lower than vehicle following ACZ. Photoreceptor respiratory efficacy can be evaluated in vivo based on distinct rod mitochondria responses to subretinal space acidification measured with OCT biomarkers and an ACZ challenge, supporting and extending our previous findings measured with light-dark conditions.

Paracentral Acute Middle Maculopathy: A Review

Paracentral acute middle maculopathy (PAMM) is identified by the occurrence of an inner nuclear layer (INL) hyperreflective band on optical coherence tomography (OCT), which was subsequently accompanied by INL atrophy. A multitude of systemic diseases and vasculopathy risk factors have been linked to PAMM. PAMM, which has been associated with infection with Coronavirus disease 2019(COVID 19), may indicate post-infectious complications. By influencing vascular endothelial cells, COVID-19 infection can induce an inflammatory and procoagulatory state, resulting in vascular thromboembolic complications, although the precise mechanisms underlying these complications remain unknown. Thus, the present review explores and discusses about paracentral acute middle maculopathy, its diagnosis and treatment and adds a note on its implications after the outbreak of COVID 19. Bangladesh Journal of Medical Science Vol.23 (Special Issue) 2024 p.S115-S121

Phenotypic characteristics of Danish patients with achromatopsia.

To describe the phenotype of Danish patients with genetically verified achromatopsia (ACHM) with special focus on signs of progression on structural or functional parameters, and possible genotype-phenotype correlations. Forty-eight patients were identified, with disease-causing variants in five different genes: CNGA3, CNGB3, GNAT2, PDE6C and PDE6H. Longitudinal evaluation was possible for 11 patients and 27 patients participated in a renewed in-depth phenotyping consisting of visual acuity assessment, optical coherence tomography (OCT), fundus autofluorescence, colour vision evaluation, contrast sensitivity, mesopic microperimetry and full-field electroretinography. Foveal morphology was evaluated based on OCT images for all 48 patients using a grading system based on the integrity of the hyperreflective photoreceptor band, the inner segment ellipsoid zone (ISe). Signs of progression were evaluated based on longitudinal data and correlation with age. We found a statistically significant positive correlation between OCT grade and age (Spearman ρ = 0.62, p < 0.0001) and we observed changes in the foveal morphology in 2 of 11 patients with ≥5 years of follow-up. We did not find any convincing correlation between age and functional parameters (visual acuity, retinal sensitivity and contrast sensitivity) nor did we find correlation between structural and functional parameters, or any clear genotype-phenotype correlation. Some patients with ACHM demonstrate signs of progressive foveal changes in OCT characteristics with increasing age. This is relevant in terms of possible new treatments. However, functional characteristics, such as visual acuity, remained stable despite changing foveal structure. Thus, seen from a patient perspective, ACHM can still be considered a non-progressive condition.

THE OUTCOME OF INTRAVITREAL BROLUCIZUMAB FOR NEOVASCULAR AGE-RELATED MACULAR DEGENERATION TREATMENT

Introduction : Age-related macular degeneration (AMD) is a disease that results in irreversible severe loss of central vision in people over 50 years old. Hence, anti-VEGF therapy has become a standard treatment for neovascular AMD. Brolucizumab is a single-chain antibody fragment that inhibits VEGF-A binding to VEGF receptors. It has a higher molar concentration that allows a greater reduction in the central subfield thickness (CST)&#x0D; Case Illustration : A 59-year-old male with a chief complaint of gradually blurred vision in the left eye for 2 years. BCVA of the left eye was 0.3, OCT showed a hyperreflective band in the sub-neurosensory space with subretinal fluid in the left eye (Figure 1), and OCT-A revealed choroidal neovascularization (Figure 2). The patient was diagnosed with AMD AREDS IV in the left eye. He had undergone three monthly injections of brolucizumab. BCVA of the left eye after injection improved to 1.0 and OCT imaging showed no subretinal fluid, no intraretinal fluid, and no pigment epithelial detachment.&#x0D; Discussion : Brolucizumab has a greater reduction in CST because of its high molecular concentration. Results of HAWK and HARRIER concluded that brolucizumab was non-inferior towards aflibercept in BCVA change at week 48. According to the HAWK trial, a greater reduction of CST was seen in week 16 among eyes treated with brolucizumab. Brolucizumab was also well tolerated and adverse event rates were similar to aflibercept.&#x0D; Conclusion : Brolucizumab could be an alternative treatment option considering its efficacy in improving visual acuity and reducing CST.

Immunohistochemical, functional, and anatomical evaluation of patients with idiopathic epiretinal membrane

PurposeThe main purpose of this study was to perform an immunohistochemical, functional, and anatomical evaluation of patients with idiopathic epiretinal membrane (ERM).MethodsTwenty-four specimens of idiopathic ERM from 24 consecutive patients who underwent 23 G pars plana vitrectomy for ERM and internal limiting membrane (ILM) peeling at the San Juan University Hospital in Alicante (Spain) in 2019 were analyzed. All patients underwent a complete ophthalmological examination including measurement of best corrected visual acuity (BCVA) and macular analysis by spectral-domain optical coherence tomography (SD-OCT) at the time of diagnosis and 3 months after surgery. Specific glial fibrillar acid protein antibodies (GFAP) and S100 calcium-binding protein β (S100β) immunostaining markers were used to identify the macroglial component of the ERM, Müller cells, and astrocytes. Ionized calcium-binding adapter molecule 1 protein (Iba1) antibodies were used as specific markers for inflammatory cells, such as microglia and macrophages.ResultsMean preoperative BCVA measured with Snellen chart was 0.3 and 0.6 preoperatively and at 3 months after surgery, respectively. SD-OCT identified 15 patients (62.5%) with a disruption of the outer retinal hyperreflective bands. The immunohistochemical study showed the presence of Müller cells in almost all cases (91.6%), as well of abundant microglia and macrophages. Microglia and macrophages were more frequently present in earlier stages of ERM. Microglia were present in ERM independently of the outer retinal hyperreflective bands integrity as measured by SD-OCT. A greater presence of macrophages was found in those ERMs with no outer retinal hyperreflective band disruption.ConclusionsMüller cells seem to be the most frequent cell group in ERMs, with also presence of microglia cells and macrophages. Astrocytes were more frequently found in early stages of ERMs. Microglia and macrophages were most frequent in ERMs with early stage (1, 2, or 3) than in advanced stages (4).

Paracentral acute middle maculopathy as the first sign of internal carotid artery dissection

Purpose: It is proposed to present a clinical case of a patient with a paracentral acute middle maculopathy (PAMM) as the first sign of presentation of severe internal carotid artery (ICA) dissection.Methods: An initial basic ophthalmologic examination of the patient was performed, followed by an optical coherence tomography (OCT) and an OCT‐angiography (OCT‐A) of each eye. Finally, the patient was evaluated by a neurologist, performing a cerebral and supra‐aortic trunks (SAT) computed tomography (CT) angiography.Results: We present a 54‐year‐old patient who came to the emergency room complaining of central blurred vision in his left eye (OS) during 24 h. On ophthalmological examination, it is remarkable a visual acuity (VA) of counting fingers at 1 m in OS. Eye fundus (EF) of OS showed small oval whitish macular lesions. Macular OCT of OS revealed hyperreflective bands in the internal nuclear layer (INL) and OCT‐A showed areas with low flow at deep capillary plexus (DCP) and macular vascular density (MVD) parameters decreased compared to the right eye (OD). During the examination, the patient began to feel a slight loss of strength in his right hemibody with dizziness, so he was explored by a neurologist and a cerebral and SAT CT angiography was performed, which reported severe left ICA dissection with an area of hypoperfusion in the territory of the left middle cerebral artery, but without intracranial vascular occlusion. Since there was no image of occlusion and given the mild neurological symptoms, endovascular treatment was rejected and anticoagulation with enoxaparin was established. Two months later, VA of OS improved to 1, with no significant lesions in the EF. The OCT of OS showed a slight thinning of the INL and the OCT‐A revealed a greater flow in the DCP with better MVD values.Conclusions: This entity should be considered in patients with visual disturbances. PAMM may be the first sign of ICA pathology.

Hyperreflective material boundary remodelling: A new morphological biomarker of good functional outcomes in neovascular age‐related macular degeneration

Aims/Purpose: To characterize the hyperreflective material (HRM) change over 9‐month follow‐up in treatment‐naïve neovascular age‐related macular degeneration (nAMD) eyes who underwent antiangiogenic treatment.Methods: Spectral‐domain optical coherence tomography images of 207 study eyes enrolled in AVENUE trial were included to analyse HRM features including location, evolvement and boundaries. We looked for the development of a highly hyperreflective band with continuity to the adjacent unaffected retinal pigment epithelium on the inner aspect of persistent HRM, which we termed complete HRM‐BR, if more than 90% of HRM was covered by this feature. Other HRM evolution patterns included complete resolution of subretinal HRM (SHRM) and partial/no remodelling of persistent SHRM. We analysed associations of HRM morphological patterns with vision and intralesional hypertransmission into the choroid (HTC), a proxy for HRM‐associated macular atrophy, at Month 9. Predictive features for complete HRM‐BR were explored.Results: At Month 9, persistent SHRM was present in 57% of the 207 included eyes. Of these, complete HRM‐BR developed in 44.9%, which was associated with greater visual outcome (+4.2 letters vs. −6.1 letter) and less intralesional HTC (20.8% vs. 69.2%) compared to partial/no HRM‐BR at Month 9. These eyes with complete HRM‐BR had similar visual outcome and intralesional HTC development to eyes with resolved SHRM (+2.2 letters; 26.8%). Presence of intralesional HTC (baseline OR 0.06, month 1 OR 0.18, p ≤ 0.01) was associated with lower odds of complete HRM‐BR development by month 9.Conclusions: In nAMD eyes undergoing antiangiogenic therapy, complete HRM‐BR developed frequently when SHRM persisted. It was associated with better visual outcome and less MA compared to eyes with partial/no HRM‐BR, and with similar outcomes compared to those with resolved SHRM. This morphological pattern can be predicted at an early stage by RPE‐related HRM features.

Uncommon case of basal linear deposits and its tomographic and indocyanine green angiography findings

Purpose: To report a rare and uncommon case of patient presenting drusen associated to basal linear deposit (BlinD) and macular vitelliform material (VM).Methods: Patient was assessed with multimodal imaging: fundus autofluorescence (FAF), indocyanine green angiography (ICGA) and enhanced depth imaging optical coherence tomography (EDI‐OCT). Electroretinogram (ERG) and visually evoked potentials (VEP) along with systemic evaluation were also performed.Results: A 52‐year‐old woman consulted for ophthalmological check‐up, without any complaint. Past history revealed breast cancer treated with chemotherapy and hormone therapy, currently in remission. Visual acuity was 10/10 in the right eye (RE) and 8/10 in the left eye (LE). Slit‐lamp anterior examination was unremarkable. Fundus examination revealed multiple interlaced round yellowish lesions mainly in the posterior pole associated to a pigmented macular lesion on the LE. FAF showed bilateral macular hypoautofluorescent lesions. ICGA highlighted bilateral scattered hypofluorescent spots (SHS) mainly noted in the late phase, distributed throughout the posterior pole, with partial confluence. EDI‐OCT featured the presence of drusenoid retinal pigment epithelium (RPE) elevation along with a thick and distinctive hyperreflective band between the RPE and Bruch's membrane (BM). Associated bilateral hyperreflective VM were also detected. ERG and VPE were unremarkable. Patient underwent systemic evaluation insuring absence of cancer recurrence, choroidal metastases, paraneoplastic syndrome or any systemic disease.Conclusions: BlinD is defined as a deposit located between the RPE and the BM. Recently, this entity has been correlated with SHS seen in late stages on ICGA and might be a precursor sign of age‐related macular degeneration. Associations between drusen and BlinD and between drusen and VM have been previously reported; however, the concomitance of these three features has, to our knowledge, never been reported.

Do multiple physiological OCT biomarkers indicate age-related decline in rod mitochondrial function in C57BL/6J mice?

To test the hypothesis that rod photoreceptor mitochondria function in vivo progressively declines over time. 2, 12, and 24 month-old dark- and light-adapted C57BL/6J (B6J) mice were examined by OCT. We measured (i) an index of mitochondrial configuration within photoreceptors measured from the profile shape aspect ratio (MCP/AR) of the hyperreflective band posterior to the external limiting membrane (ELM), (ii) a proxy for energy-dependent pH-triggered water removal, the thickness of the ELM-retinal pigment epithelium (ELM-RPE), and its correlate (iii) the hyporeflective band (HB) signal intensity at the photoreceptor tips. Visual performance was assessed by optokinetic tracking. In 2 and 24 month-old mice, MCP/AR in both inferior and superior retina was smaller in light than in dark; no dark-light differences were noted in 12 month-old mice. Dark-adapted inferior and superior, and light-adapted superior, ELM-RPE thickness increased with age. The dark-light difference in ELM-RPE thickness remained constant across all ages. All ages showed a decreased HB signal intensity magnitude in dark relative to light. In 12 month-old mice, the dark-light difference in HB magnitude was greater than in younger and older mice. Anatomically, outer nuclear layer thickness decreased with age. Visual performance indices were reduced at 24 month-old compared to 2 month-old mice. While the working hypothesis was not supported herein, the results raise the possibility of a mid-life adaptation in rod mitochondrial function during healthy aging in B6J mice based on OCT biomarkers, a plasticity that occurred prior to declines in visual performance.

Purtscher-like retinopathy and paracentral acute middle maculopathy following breast filler injection

BackgroundTo report a rare case of pulmonary and ocular complications with visual loss due to bilateral Purtscher-like retinopathy and paracentral acute middle maculopathy (PAMM) following a hyaluronic acid (HA) filler injection to the breast. Systemic and visual recovery was attained following corticosteroid therapy.Case presentationA 27-year-old lady presented with painless blurring of vision in both eyes for 2 weeks following hyaluronic acid breast filler injections by a non-medical practitioner. She was initially admitted to the medical ward for diffuse alveolar haemorrhage and altered sensorium. The presenting visual acuity was counting fingers in both eyes. Bilateral dilated fundus examination showed hyperaemic discs, concentric rim of retinal whitening around macula with patches of polygonal-shaped retinal whitening, generalised cotton-wool spots, tortuous veins, and flame-shaped haemorrhages. Spectral-domain optical coherence tomography (SD-OCT) macula revealed hyper-reflective bands at the inner nuclear layer (INL). Fluorescein angiography demonstrated hot discs, delayed arm-to-retina time, arterial filling, and arterio-venous transit time with staining of the vessels at the posterior pole. She was managed with a tapering dose of systemic corticosteroids. The visual acuity improved to 6/12 over 8 weeks with significant anatomical and functional improvement. Dilated fundus examination showed resolution of initial funduscopy findings. The hyper-reflective bands on the OCT had resolved with subsequent thinning of the INL and disorganisation of retinal inner layers.ConclusionFiller injections are in increasing demand and are frequently being performed by non-medical practitioners. Visual loss from non-facial HA fillers is rare. Inadvertent entry of HA into a blood vessel may potentially cause systemic and sight-threatening ocular complications. Good anatomical knowledge and proper injection technique are vital in preventing this unfortunate sequela. There are limited reports on successful visual recovery following various treatment approaches and we hope this case provides valuable insights.

Retinal Pigment Epithelial Detachments Devoid of Retinal Pigment Epithelium.

To describe two patients with chronic central serous chorioretinopathy (CSC) showing what appeared to be retinal pigment epithelium detachments (PED) lacking imaging findings consistent with retinal pigment epithelium (RPE) over the elevation. The patients underwent comprehensive ophthalmic examination, including multicolor fundus photography, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography (SD-OCT). A 70-year-old male and a 58-year-old male, both diagnosed with chronic CSC, showed PED-like lesions that were hypoautofluorescent, suggesting an absence of RPE. SD-OCT B-scans showed serous, dome-shaped elevations composed of a narrow, mildly hyperreflective band (9-10 μm thick) that demonstrated hypertransmission of light. The material that constituted the elevation was contiguous with the outer portion of the RPE band at the lesion borders. Based on the multimodal imaging findings we hypothesize that these elevations of the retina have lost their overlying RPE. A thin layer of material that could represent a residual layer of basal laminar deposit produced by the RPE remains overlying the detachments, possibly accounting for their dome shape and structural stability.

Detection of Paracentral Acute Middle Maculopathy Can Prevent Blindness and Death.

Depth resolved imaging tools such as optical coherence tomography (OCT) are at the forefront of a new era of retinal diagnostics. These advancements have remarkably broadened our understanding of macular ischemia and the structure of the retinal capillary plexus (RCP). Because of the predominant vertical organization of the RCP, the middle retinal layer is at greater risk of ischemia and infarction. Paracentral acute middle maculopathy (PAMM) is a manifestation of this risk and is the result of infarction of the inner nuclear layer (INL), detectable on OCT as a hyperreflective band within the INL. On OCT angiography (OCTA), PAMM is the result of hypoperfusion of the deep retinal vascular complex i.e the intermediate retinal capillary plexus and the deep retinal capillary plexus.

RPGR: Deep Phenotyping and Genetic Characterization With Findings Specific to the 3'-end of ORF15.

To describe a group of patients with retinitis pigmentosa GTPase regulator (RPGR)-related retinopathy with a tapetal-like retinal sheen and corresponding changes in the reflectivity of the ellipsoid zone on optical coherence tomography (OCT) imaging. A retrospective case series of 66 patients with a disease-causing variant in RPGR was performed. An expert examiner, masked to patient demographics, clinical evaluations, and specific RPGR variant, analyzed color fundus photographs for the presence of a tapetal-like retinal sheen and assessed OCT images for the presence of an abnormally broad hyper-reflective band in the outer retina. Longitudinal reflectivity profiles were generated and compared with healthy controls. Twelve patients (18.2%) had a retinal sheen on color images that cosegregated with an abnormally broad hyper-reflective ellipsoid zone band on OCT imaging. Three-fourths of these patients were male, had a cone-rod dystrophy, and had pathogenic RPGR variants located toward the 3'-end of ORF15. This group had a different longitudinal reflectivity profile signature compared with controls. After a period of prolonged dark adaptation, the abnormal hyper-reflective band on OCT became less apparent, and the outer retinal layers adopted a more normal appearance. RPGR-related retinopathy should be considered for males presenting with retinal sheen, abnormal ellipsoid zone hyper-reflectivity, and cone or cone-rod dysfunction on ERG, and pursued with molecular testing. Our results have implications for understanding the role of the C-terminal domain encoded by RPGR ORF15 in the phototransduction cascade. Further, the findings may be important to incorporate into both inclusion criteria and outcome measure developments in future RPGR-related cone or cone-rod dystrophy clinical trials.

Optical coherence tomography features of lightning-induced macular injury

A 15-year-old boy with history of lightning exposure presented with sudden onset diminution of vision in left eye. The best-corrected visual acuity (BCVA) was 6/6 in right eye and 6/18 in left eye. Anterior segment examination was normal. A yellow dot was seen at the fovea in the left eye. Optical coherence tomography (OCT) showed a vertical hyperreflective band at fovea in right eye and low-reflective dome-shaped elevation, horizontal hyperreflective band, and disruption of ellipsoid zone (EZ) in left eye. Patient developed an episode of acute anterior uveitis in the left eye during the follow-up. At 6-month follow-up visit, BCVA in the right eye was 6/6 and in left eye was 6/36. Left eye developed grade 2 posterior subcapsular cataract during the follow-up. Extensive EZ loss and hyporeflective spaces were noted on OCT in the left eye.