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1.
BMJ Case Rep ; 17(5)2024 May 09.
Article in English | MEDLINE | ID: mdl-38724217

ABSTRACT

Acute macular neuroretinopathy (AMN) affects the outer retina and is most likely induced by non-inflammatory ischaemia of the retinal deep capillary plexus and choriocapillaris. A man in his early 20s developed Valsalva retinopathy following weightlifting at the gym and presented with blurring of vision in the left eye 1 month after the initial retinal haemorrhages had resolved. A diffuse, purplish, donut-shaped, perifoveal lesion was seen on funduscopy and was well defined by an optical coherence tomography angiography (OCTA) en face image in the left eye. Outer retinal changes on optical coherence tomography (OCT) and a dense co-localised scotoma on a visual field (VF) examination confirmed the diagnosis of AMN, and the patient was started on a tapering dose of oral steroids. Improvement was seen in OCT, OCTA and VF during the 6-month follow-up visit. The use of OCTA en face imaging enabled the accurate identification of the lesion in the affected layers of the retina.


Subject(s)
Retinal Diseases , Tomography, Optical Coherence , Valsalva Maneuver , Humans , Male , Tomography, Optical Coherence/methods , Retinal Diseases/etiology , Retinal Diseases/physiopathology , Retinal Diseases/diagnosis , Retinal Diseases/diagnostic imaging , Fluorescein Angiography/methods , Adult , Macula Lutea/diagnostic imaging , Macula Lutea/pathology , Acute Disease , Scotoma/etiology , Scotoma/physiopathology , Visual Acuity
2.
Ophthalmol Retina ; 8(4): 331-339, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38752998

ABSTRACT

OBJECTIVE: To describe and quantify the structural and functional consequences of retinal vasculopathy with cerebral leukoencephalopathy (RVCL) on the neurosensory retina. DESIGN: Cross sectional descriptive study from December 2021 to December 2022. PARTICIPANTS: Retinal vasculopathy with cerebral leukoencephalopathy patients (n = 9, 18 eyes) recruited from the RVCL Research Center at Washington University in St. Louis. METHODS: Retinal vasculopathy with cerebral leukoencephalopathy patients underwent comprehensive ophthalmological evaluation including OCT, OCT angiography (OCTA), ultrawidefield fundus imaging, retinal autofluorescence, dark adaptation, electroretinography (ERG), Goldmann kinetic perimetry, and fluorescein angiography (FA). MAIN OUTCOME MEASURES: Comprehensive characterization from various modalities including best-corrected visual acuity, central subfield thickness (µm) from OCT, foveal avascular zone (mm2) from OCTA, dark adaptation rod intercept (seconds), cone response in ERG, and presence or absence of vascular abnormalities, leakage, neovascularization, and nonperfusion on FA. RESULTS: A total of 18 eyes from 9 individuals were included in this study. The best-corrected visual acuity ranged from 20/15 to 20/70. The mean central subfield thickness from OCT was 275.8 µm (range, 217-488 µm). The mean foveal avascular zone (FAZ) from OCTA was 0.65 (range, 0.18-1.76) mm2. On dark adaptometry, the mean time was 5.02 (range, 2.9-6.5) minutes, and 1 individual had impaired dark adaptation. Electroretinography demonstrated mild cone response impairment in 4 eyes. On FA, there was evidence of macular and peripheral capillary nonperfusion in 16 of 18 eyes and notable areas of vascular leakage and retinal edema in 5 of the 18 eyes. CONCLUSIONS: This study illustrates the phenotypic spectrum of disease and may be clinically valuable for aiding diagnosis, monitoring disease progression, and further elucidating the pathophysiology of RVCL to aid in the development of therapies. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.


Subject(s)
Electroretinography , Fluorescein Angiography , Leukoencephalopathies , Multimodal Imaging , Tomography, Optical Coherence , Visual Acuity , Humans , Male , Female , Cross-Sectional Studies , Tomography, Optical Coherence/methods , Adult , Fluorescein Angiography/methods , Electroretinography/methods , Middle Aged , Leukoencephalopathies/diagnosis , Leukoencephalopathies/physiopathology , Visual Fields/physiology , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology , Retinal Diseases/etiology , Retinal Vessels/diagnostic imaging , Retinal Vessels/physiopathology , Retinal Vessels/pathology , Young Adult , Fundus Oculi , Adolescent
3.
J Biomed Sci ; 31(1): 48, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38730462

ABSTRACT

Retinal degenerative diseases, including diabetic retinopathy (DR) and age-related macular degeneration (AMD), loom as threats to vision, causing detrimental effects on the structure and function of the retina. Central to understanding these diseases, is the compromised state of the blood-retinal barrier (BRB), an effective barrier that regulates the influx of immune and inflammatory components. Whether BRB breakdown initiates retinal distress, or is a consequence of disease progression, remains enigmatic. Nevertheless, it is an indication of retinal dysfunction and potential vision loss.The intricate intercellular dialogues among retinal cell populations remain unintelligible in the complex retinal milieu, under conditions of inflammation and oxidative stress. The retina, a specialized neural tissue, sustains a ceaseless demand for oxygen and nutrients from two vascular networks. The BRB orchestrates the exchange of molecules and fluids within this specialized region, comprising the inner BRB (iBRB) and the outer BRB (oBRB). Extracellular vesicles (EVs) are small membranous structures, and act as messengers facilitating intercellular communication in this milieu.EVs, both from retinal and peripheral immune cells, increase complexity to BRB dysfunction in DR and AMD. Laden with bioactive cargoes, these EVs can modulate the retinal microenvironment, influencing disease progression. Our review delves into the multifaceted role of EVs in retinal degenerative diseases, elucidating the molecular crosstalk they orchestrate, and their microRNA (miRNA) content. By shedding light on these nanoscale messengers, from their biogenesis, release, to interaction and uptake by target cells, we aim to deepen the comprehension of BRB dysfunction and explore their therapeutic potential, therefore increasing our understanding of DR and AMD pathophysiology.


Subject(s)
Blood-Retinal Barrier , Extracellular Vesicles , Blood-Retinal Barrier/metabolism , Blood-Retinal Barrier/physiopathology , Extracellular Vesicles/metabolism , Humans , Diabetic Retinopathy/physiopathology , Diabetic Retinopathy/metabolism , Retinal Diseases/physiopathology , Retinal Diseases/metabolism , Macular Degeneration/physiopathology , Macular Degeneration/metabolism , Animals
4.
Exp Eye Res ; 242: 109885, 2024 May.
Article in English | MEDLINE | ID: mdl-38574944

ABSTRACT

The retinal microcirculation system constitutes a unique terminal vessel bed of the systemic circulation, and its perfusion status is directly associated with the neural function of the retina. This vascular network, essential for nourishing various layers of the retina, comprises two primary microcirculation systems: the retinal microcirculation and the choroidal microcirculation, with each system supplying blood to distinct retinal layers and maintaining the associated neural function. The blood flow of those capillaries is regulated via different mechanisms. However, a range of internal and external factors can disrupt the normal architecture and blood flow within the retinal microcirculation, leading to several retinal pathologies, including diabetic retinopathy, macular edema, and vascular occlusions. Metabolic disturbances such as hyperglycemia, hypertension, and dyslipidemia are known to modify retinal microcirculation through various pathways. These alterations are observable in chronic metabolic conditions like diabetes, coronary artery disease, and cerebral microvascular disease due to advances in non-invasive or minimally invasive retinal imaging techniques. Thus, examination of the retinal microcirculation can provide insights into the progression of numerous chronic metabolic disorders. This review discusses the anatomy, physiology and pathophysiology of the retinal microvascular system, with a particular emphasis on the connections between retinal microcirculation and systemic circulation in both healthy states and in the context of prevalent chronic metabolic diseases.


Subject(s)
Metabolic Diseases , Microcirculation , Retinal Vessels , Humans , Microcirculation/physiology , Retinal Vessels/physiopathology , Metabolic Diseases/physiopathology , Retinal Diseases/physiopathology , Regional Blood Flow/physiology
7.
Ophthalmic Surg Lasers Imaging Retina ; 55(5): 255-262, 2024 May.
Article in English | MEDLINE | ID: mdl-38408221

ABSTRACT

BACKGROUND AND OBJECTIVE: Our objective was to monitor variables via spectral-domain optical coherence tomography (SD-OCT) and identify the most relevant biomarkers related to best-corrected visual acuity (BCVA) in radiation retinopathy (RR). PATIENTS AND METHODS: A post-hoc analysis of the two-year Ranibizumab for Radiation Retinopathy (RRR) trial analyzed vision and OCT parameters including intraretinal fluid, ellipsoid zone (EZ) disruption, retinal pigment epithelium atrophy, hard exudates, retinal hemorrhage, retinal neovascularization, and subfoveal fluid. BCVA and SD-OCT parameters were evaluated by univariate analysis and a mixed-effects model. RESULTS: Forty eyes from the RRR trial were included. Intraretinal cyst vertical size (week 24: P = 0.032; week 48: P = 0.021), neovascularization (week 48: P = 0.028; week 72: P = 0.025), and EZ disruption (week 72: P = 0.029; week 104: P = 0.019) were the clinical parameters most relevant to BCVA by univariate analysis in at least two time points. The mixed-effects model confirmed the relevance of intraretinal cyst vertical size (P = 0.001) and neovascularization (P = 0.001) but not EZ disruption (P = 0.119) over the course of the study. CONCLUSIONS: This study characterizes the course of visual loss in RR by identifying intraretinal cyst vertical size, neovascularization, and EZ disruption as biomarkers of poor BCVA over a span of two years. Larger multicenter studies are needed to confirm these findings. [Ophthalmic Surg Lasers Imaging Retina 2024;55:255-262.].


Subject(s)
Angiogenesis Inhibitors , Biomarkers , Intravitreal Injections , Radiation Injuries , Ranibizumab , Retinal Diseases , Tomography, Optical Coherence , Visual Acuity , Humans , Ranibizumab/administration & dosage , Tomography, Optical Coherence/methods , Angiogenesis Inhibitors/administration & dosage , Angiogenesis Inhibitors/therapeutic use , Male , Female , Radiation Injuries/diagnosis , Radiation Injuries/drug therapy , Radiation Injuries/etiology , Middle Aged , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology , Retinal Diseases/drug therapy , Retinal Diseases/etiology , Aged , Vascular Endothelial Growth Factor A/antagonists & inhibitors , Retina/radiation effects , Retina/pathology , Retina/diagnostic imaging
8.
Doc Ophthalmol ; 148(3): 167-171, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38363513

ABSTRACT

PURPOSE: Multiple myeloma (MM) is a plasma cell dyscrasia leading to proliferation of monoclonal plasma cells. Ocular involvement in multiple myeloma is uncommon but can occur. The ocular manifestations of MM may include the cornea, uvea, and retinal vasculature. We present a rare case of autoimmune retinopathy associated with smoldering MM. CASE: A 76-year-old female with no significant past medical or ocular history presented with four months of worsening vision, difficulty with night driving, and loss of peripheral vision. Examination was notable for pallor of the optic nerves and vascular attenuation. Visual field testing demonstrated significant and progressive field loss in both eyes. An electroretinogram was extinguished under all conditions. Serum protein electrophoresis showed a significant elevation of IgG with an M-spike, and a subsequent bone marrow biopsy was performed showing 12.5% plasma cells, consistent with the diagnosis of MM. CAR antibody testing was positive for anti-enolase, anti-GAPDH, and anti-Rab6 antibodies, consistent with autoimmune retinopathy. DISCUSSION: Autoimmune retinopathy associated with MM is exceedingly rare. Management of this condition is challenging, as treatment of the underlying disease does not often lead to improvement in visual symptoms. Ultimately, visual prognosis is very poor, and both patients and clinicians should be aware of the guarded visual potential. CONCLUSION: The association of autoimmune retinopathy with multiple myeloma is rare. It is crucial for physicians to be aware of such manifestations to ensure timely and appropriate diagnosis and management for patients.


Subject(s)
Autoimmune Diseases , Electroretinography , Retinal Diseases , Smoldering Multiple Myeloma , Humans , Aged , Female , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Smoldering Multiple Myeloma/diagnosis , Retinal Diseases/etiology , Retinal Diseases/diagnosis , Retinal Diseases/immunology , Retinal Diseases/physiopathology , Visual Fields/physiology , Visual Acuity/physiology , Multiple Myeloma/immunology , Multiple Myeloma/complications , Multiple Myeloma/diagnosis
9.
Prog Retin Eye Res ; 100: 101244, 2024 May.
Article in English | MEDLINE | ID: mdl-38278208

ABSTRACT

Inherited retinal diseases (IRD) are a leading cause of blindness in the working age population and in children. The scope of this review is to familiarise clinicians and scientists with the current landscape of molecular genetics, clinical phenotype, retinal imaging and therapeutic prospects/completed trials in IRD. Herein we present in a comprehensive and concise manner: (i) macular dystrophies (Stargardt disease (ABCA4), X-linked retinoschisis (RS1), Best disease (BEST1), PRPH2-associated pattern dystrophy, Sorsby fundus dystrophy (TIMP3), and autosomal dominant drusen (EFEMP1)), (ii) cone and cone-rod dystrophies (GUCA1A, PRPH2, ABCA4, KCNV2 and RPGR), (iii) predominant rod or rod-cone dystrophies (retinitis pigmentosa, enhanced S-Cone syndrome (NR2E3), Bietti crystalline corneoretinal dystrophy (CYP4V2)), (iv) Leber congenital amaurosis/early-onset severe retinal dystrophy (GUCY2D, CEP290, CRB1, RDH12, RPE65, TULP1, AIPL1 and NMNAT1), (v) cone dysfunction syndromes (achromatopsia (CNGA3, CNGB3, PDE6C, PDE6H, GNAT2, ATF6), X-linked cone dysfunction with myopia and dichromacy (Bornholm Eye disease; OPN1LW/OPN1MW array), oligocone trichromacy, and blue-cone monochromatism (OPN1LW/OPN1MW array)). Whilst we use the aforementioned classical phenotypic groupings, a key feature of IRD is that it is characterised by tremendous heterogeneity and variable expressivity, with several of the above genes associated with a range of phenotypes.


Subject(s)
Cone-Rod Dystrophies , Leber Congenital Amaurosis , Phenotype , Humans , Leber Congenital Amaurosis/genetics , Leber Congenital Amaurosis/therapy , Leber Congenital Amaurosis/physiopathology , Cone-Rod Dystrophies/genetics , Cone-Rod Dystrophies/physiopathology , Genotype , Molecular Biology , Retinal Diseases/genetics , Retinal Diseases/physiopathology , Retinal Diseases/therapy , Eye Diseases, Hereditary/genetics , Eye Diseases, Hereditary/physiopathology
10.
Am J Ophthalmol ; 262: 73-85, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38280677

ABSTRACT

PURPOSE: This study aimed to ascertain the occurrence of foveal hypoplasia (FH) in individuals diagnosed with familial exudative vitreoretinopathy (FEVR). DESIGN: Retrospective cohort study. METHODS: In this study, FEVR families and sporadic cases were diagnosed at the Eye and ENT Hospital, Fudan University, between 2017 and 2023. All patients attended routine ophthalmologic examinations and genetic screenings. The classification of FH was determined using optical coherence tomography (OCT) scans. The FH condition was classified into 2 subgroups: group A (FH being limited to the inner layers) and group B (FH affecting the outer layers). A total of 102 eyes from 58 patients were suitable for analysis. RESULTS: Forty-nine mutations in LRP5, FZD4, NDP, TSPAN12, KIF11, CTNNB1, and ZNF408 were examined and detected, with 26 of them being novel. Forty-seven eyes (46.1%) revealed FH. The majority (53.2%) were due to the typical grade 1 FH. Patients with mutations in LRP5 and KIF11 were found to exhibit a higher prevalence of FH (P = .0088). Group B displayed the lowest visual acuity compared with group A (P = .048) and the group without FH (P < .001). The retinal arteriolar angle in group B was significantly smaller than in group A (P = .001) and those without FH (P < .001). CONCLUSIONS: This study offers a new diagnostic approach and expands the spectrum of FEVR mutations. LRP5 and KIF11 were found to be more susceptible to causing FH in patients with FEVR. FEVR eyes with FH exhibited both greater visual impairment and reduced retinal arteriolar angles. The assessment of foveal status in patients with FEVR should be valued.


Subject(s)
Eye Diseases, Hereditary , Eye Proteins , Familial Exudative Vitreoretinopathies , Fovea Centralis , Frizzled Receptors , Kinesins , Low Density Lipoprotein Receptor-Related Protein-5 , Mutation , Tetraspanins , Tomography, Optical Coherence , Visual Acuity , Humans , Male , Familial Exudative Vitreoretinopathies/diagnosis , Female , Retrospective Studies , Fovea Centralis/abnormalities , Kinesins/genetics , Low Density Lipoprotein Receptor-Related Protein-5/genetics , Adult , Eye Proteins/genetics , Visual Acuity/physiology , Child , Frizzled Receptors/genetics , Adolescent , Tetraspanins/genetics , Eye Diseases, Hereditary/diagnosis , Eye Diseases, Hereditary/genetics , Eye Diseases, Hereditary/physiopathology , Young Adult , Retinal Diseases/genetics , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology , DNA Mutational Analysis , Pedigree , Fluorescein Angiography/methods , Child, Preschool , Middle Aged , Eye Abnormalities/genetics , Eye Abnormalities/diagnosis , DNA-Binding Proteins , Nerve Tissue Proteins , Transcription Factors
11.
Retina ; 44(5): 868-877, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38170761

ABSTRACT

PURPOSE: To explore characteristic imaging features of nonparaneoplastic autoimmune retinopathy (npAIR) to augment diagnostic criteria. METHODS: This is a retrospective cohort study of patients with npAIR evaluated at the Emory Eye Center between 2013 and 2019. Multimodal fundus images were evaluated to characterize the evolution of the disease. RESULTS: Twenty-one eyes of 12 patients were classified as having npAIR. Five patients (42%) were female, with median (range) age of 59 years (45-85 years). Median baseline visual acuity was 20/30 (20/20 to hand motions). Disease was asymmetric in 11 patients (92%). Common imaging findings included absence of bone spicules (86% of affected eyes), presence of attenuated vessels (86%), and speckled hypoautofluorescence in perimacular and perivenular regions. Three eyes were noted to present early with subtle splotchy fundus autofluorescence abnormality, ultimately developing characteristic speckled perimacular hypoautofluorescence. On optical coherence tomography, 18 eyes (86%) had loss of outer retinal bands with relative foveal sparing and a tapered transition zone. CONCLUSION: Many eyes with npAIR exhibit a subacute, asymmetric, generalized photoreceptor degeneration featuring outer retinal atrophy with relative foveal sparing, retinal vascular attenuation, absence of bone spicules, and speckled hypoautofluorescence often in a perimacular and perivenular distribution. Findings of this study augment diagnostic criteria to improve specificity and accessibility of testing for npAIR.


Subject(s)
Autoimmune Diseases , Fluorescein Angiography , Retinal Diseases , Tomography, Optical Coherence , Visual Acuity , Humans , Female , Middle Aged , Retrospective Studies , Male , Aged , Tomography, Optical Coherence/methods , Autoimmune Diseases/diagnosis , Fluorescein Angiography/methods , Aged, 80 and over , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology , Fundus Oculi
12.
Retina ; 44(5): 861-867, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38109708

ABSTRACT

PURPOSE: To investigate the structure and blood flow of the retina and choroid in Cushing syndrome and their relationship with cortisol levels. METHODS: A consecutive series of patients with Cushing syndrome with adrenocortical carcinoma were included in this study. Cortisol levels gradually returned to normal after adrenalectomy. Optical coherence tomography and optical coherence tomography angiography were used to assess patients with Cushing syndrome before and after the surgery for retina and choroid. Correlation analysis was performed between cortisol level and fundus changes. RESULTS: Compared with normal cortisol levels, patients with Cushing syndrome had significantly lower central macular thickness with increased cortisol level (220.82 ± 16.59 µ m and 223.68 ± 15.78 µ m, P = 0.019). However, the central choroidal thickness was higher with increased cortisol level (255.18 ± 105.89 µ m and 205.94 ± 87.04 µ m, P < 0.001). The choriocapillaris flow area was higher with increased cortisol level (2.05 ± 0.14 mm 2 and 2.00 ± 0.13 mm 2 , P = 0.02). The change of choriocapillaris flow area was correlated with the score of Huaxi Emotional-distress Index and 24-hour urine-free cortisol (24h-UFC). CONCLUSION: The increased cortisol level was correlated with lesser central macular thickness and thicker central choroidal thickness. The decrease of choriocapillaris flow area was correlated with 24h-UFC, indicating the effect of increased cortisol level on choroidal vessels.


Subject(s)
Choroid , Cushing Syndrome , Fluorescein Angiography , Hydrocortisone , Tomography, Optical Coherence , Humans , Tomography, Optical Coherence/methods , Hydrocortisone/blood , Male , Female , Cushing Syndrome/diagnosis , Cushing Syndrome/complications , Cushing Syndrome/physiopathology , Choroid/pathology , Adult , Fluorescein Angiography/methods , Middle Aged , Retinal Vessels/diagnostic imaging , Retinal Vessels/pathology , Regional Blood Flow/physiology , Retina/pathology , Retinal Diseases/etiology , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology
13.
Transl Vis Sci Technol ; 12(6): 14, 2023 Jun 01.
Article in English | MEDLINE | ID: mdl-38752575

ABSTRACT

Purpose: To establish an inducible model of retinal ischemia/reperfusion injury (RI/RI) in nonhuman primates (NHPs) to improve our understanding of the disease conditions and evaluate treatment interventions in humans. Methods: We cannulated the right eye of rhesus macaques with a needle attached to a normal saline solution reservoir at up to 1.9 m above the eye level that resulted in high intraocular pressure of over 100 mm Hg for 90 minutes. Retinal morphology and function were monitored before and after RI/RI over two months by fundus photography, optical coherence tomography, electroretinography, and visual evoked potential. Terminal experiments involved immunostaining for retinal ganglion cell marker Brn3a, glial fibrillary acidic protein, and quantitative polymerase chain reaction to assess retinal inflammatory biomarkers. Results: We observed significant and progressive declines in retinal and retinal nerve fiber layer thickness in the affected eye after RI/RI. We noted significant reductions in amplitudes of electroretinography a-wave, b-wave, and visual evoked potential N2-P2, with minimal recovery at 63 days after injury. Terminal experiments conducted two months after injury revealed ∼73% loss of retinal ganglion cells and a fivefold increase in glial fibrillary acid protein immunofluorescence intensity compared to the uninjured eyes. We observed marked increases in tumor necrosis factor-alpha, interferon-gamma, interleukin-1beta, and inducible nitric oxide synthase in the injured retinas. Conclusions: The results demonstrated that the pathophysiology observed in the NHP model of RI/RI is comparable to that of human diseases and suggest that the NHP model may serve as a valuable tool for translating interventions into viable treatment approaches. Translational Relevance: The model serves as a useful platform to study potential interventions and treatments for RI/RI or blinding retinal diseases.


Subject(s)
Disease Models, Animal , Electroretinography , Evoked Potentials, Visual , Macaca mulatta , Reperfusion Injury , Retinal Ganglion Cells , Tomography, Optical Coherence , Animals , Reperfusion Injury/metabolism , Reperfusion Injury/pathology , Reperfusion Injury/physiopathology , Evoked Potentials, Visual/physiology , Retinal Ganglion Cells/pathology , Retinal Ganglion Cells/metabolism , Glial Fibrillary Acidic Protein/metabolism , Retinal Diseases/metabolism , Retinal Diseases/pathology , Retinal Diseases/physiopathology , Retina/pathology , Retina/metabolism , Retina/physiopathology , Male , Transcription Factor Brn-3A/metabolism , Female
14.
Biomed Environ Sci ; 35(2): 107-114, 2022 Feb 20.
Article in English | MEDLINE | ID: mdl-35197175

ABSTRACT

OBJECTIVE: We wanted to investigate the radial peripapillary capillary (RPC) network in patients with Bietti crystalline dystrophy (BCD). METHODS: We compared RPC densities in the disk and different peripapillary regions, obtained using optical coherence tomography angiography in 22 patients with BCD (37 eyes) and 22 healthy subjects (37 eyes). The BCD group was then divided into Stage 2 and Stage 3 subgroups based on Yuzawa staging, comparing the RPC densities of the two. RESULTS: The disk area RPC density was 38.8% ± 6.3% in the BCD group and 49.2% ± 6.1% in the control group ( P < 0.001), and peripapillary region RPC density was significantly lower in the BCD group than in the control group (49.1% ± 4.7% and 54.1% ± 3.0%, respectively, P < 0.001). There were no significant RPC density differences between the tempo quadrant and inside disk of Stages 2 and 3 subgroups; the other areas showed a significantly lower RPC density in Stage 3 than in Stage 2 BCD. CONCLUSION: The BCD group RPC density was significantly lower than the control group. The reduction of RPC density in the tempo quadrant occurred mainly in the Stage 1 BCD. In contrast, the reduction of RPC density in superior, inferior, and nasal quadrants occurred mainly in Stage 2.


Subject(s)
Corneal Dystrophies, Hereditary/diagnostic imaging , Corneal Dystrophies, Hereditary/physiopathology , Retinal Diseases/diagnostic imaging , Retinal Diseases/physiopathology , Adult , Aged , Angiography , Female , Humans , Male , Microvascular Density , Microvessels/diagnostic imaging , Microvessels/physiopathology , Middle Aged , Retinal Vessels/diagnostic imaging , Retinal Vessels/physiopathology , Tomography, Optical Coherence
15.
Retina ; 42(3): 540-547, 2022 03 01.
Article in English | MEDLINE | ID: mdl-35188492

ABSTRACT

PURPOSE: To explore the characteristics and underlying mechanisms of myopic traction maculopathy (MTM) with axial length less than 26.5 mm and to assess the effectiveness of macular buckling for the treatment of MTM. METHODS: Thirty-eight MTM eyes with axial length less than 26.5 mm were prospectively enrolled. Thirty-one eyes received surgery, and they were followed up for at least 6 months. Characteristics of MTM and surgical outcomes were evaluated. RESULTS: Of the MTM eyes, 92.11% (35/38) showed posterior staphyloma. Narrow macular staphyloma was the most common type (54.29%, 19/35), followed by peripapillary (37.14%, 13/35). Three cases (8.57%) had wide macular staphyloma, and 44.74% of cases (17/38) had vitreoretinal traction. Twenty-two MTM eyes of type T3 underwent macular buckling surgery, and all the cases achieved foveal reattachment after the surgery. The mean best-corrected visual acuity improved significantly at the 6-month follow-up (P < 0.001). Nine MTM eyes of type T4 or T5 received combined surgery, all macular holes recovered, and the best-corrected visual acuity also improved postoperatively (P = 0.008) as of the 6-month visit. CONCLUSION: Posterior staphyloma might serve as the initial force of the pathogenesis of MTM in eyes with axial length ˂26.5 mm. Macular buckling is a productive way to improve the MTM.


Subject(s)
Axial Length, Eye/pathology , Myopia, Degenerative/complications , Retinal Diseases/diagnosis , Retinal Diseases/surgery , Vitreous Body/pathology , Adult , Aged , Female , Humans , Male , Middle Aged , Prospective Studies , Retinal Diseases/physiopathology , Retrospective Studies , Scleral Buckling , Tissue Adhesions/diagnosis , Tomography, Optical Coherence , Visual Acuity/physiology , Vitrectomy , Young Adult
16.
Invest Ophthalmol Vis Sci ; 63(1): 15, 2022 01 03.
Article in English | MEDLINE | ID: mdl-35015028

ABSTRACT

Purpose: This study investigates the association between local retina structure and visual function in a cohort with long-term hydroxychloroquine (HCQ) use. Methods: The study included 84 participants (54 participants without toxicity and 30 participants with toxicity) with history of chronic HCQ use (14.5 ± 7.4 years) who had testing with spectral-domain optical coherence tomography (SD-OCT) imaging and Humphrey 10-2 visual fields. Optical coherence tomography (OCT) metrics (total and outer retina thickness [TRT and ORT], minimum intensity [MinI], and ellipsoid zone [EZ] loss) were sampled in regions corresponding to visual field test locations. Univariate linear correlations were investigated and a multivariate random forest regression using a combination of OCT metrics was used to predict visual field sensitivity by locus using a leave-one-out cross-validation strategy. Results: In univariate linear regression, EZ loss demonstrated the strongest relationship with visual field sensitivities in the parafoveal ring with R2 = 0.58. TRT and ORT revealed positive correlations with visual field sensitivity (R2 = 0.57 and 0.40, respectively), whereas total and outer retinal MinI yielded negative correlations (R2 = 0.10 and 0.22). The multivariate model improved correlations (R2 = 0.66) yielding a root mean squared error of 3.8 decibel (dB). Feature importance analysis identified EZ loss as the most relevant predictor of function. Conclusions: Multiple OCT-derived quantitative metrics used in combination can provide information to predict local sensitivities. The results indicate a strong relationship between retinal function and OCT measures, which contribute to the understanding of the retinal toxicity caused by HCQ as well as being applicable to outcome development for other degenerative diseases of the outer retina.


Subject(s)
Hydroxychloroquine/adverse effects , Retinal Diseases/chemically induced , Tomography, Optical Coherence/methods , Visual Acuity , Visual Fields/drug effects , Aged , Antirheumatic Agents/adverse effects , Electroretinography , Female , Fluorescein Angiography , Follow-Up Studies , Fundus Oculi , Humans , Male , Middle Aged , Prospective Studies , Retinal Diseases/diagnosis , Retinal Diseases/physiopathology , Visual Field Tests
17.
Retin Cases Brief Rep ; 16(1): 59-66, 2022 Jan 01.
Article in English | MEDLINE | ID: mdl-31764886

ABSTRACT

PURPOSE: To report new multimodal imaging features that enhance our understanding of the inflammatory and ischemic nature of acute idiopathic maculopathy (AIM) and to correlate structural and functional changes due to a reversible initial choroidal ischemia over a clinical course of 3.5 months. METHODS: A 31-year-old man presented with acute central vision loss in his right eye due to coxsackievirus-associated AIM. Serial multimodal retinal imaging including confocal true color fundus photography, blue-light fundus autofluorescence (BAF), near-infrared reflectance (NIR), spectral domain optical coherence tomography and swept-source optical coherence tomography (SD-OCT + SS-OCT), and en face SS-OCT angiography (SS-OCTA) were performed over a 3.5-month follow-up. Eidon true color confocal scanner camera (Centervue, Padova, Italy) was used for color and BAF imaging. Near-infrared reflectance and SD-OCT images were obtained with the Heidelberg Spectralis OCT (HRA2 + OCT; Heidelberg Engineering, Heidelberg, Germany). For SS-OCT and en face SS-OCT and SS-OCTA images, the PLEX Elite 9,000 (Carl Zeiss Meditec, Inc, Dublin, CA) was used. Central alterations in choriocapillaris flow were analyzed with SS-OCTA using the University of Washington choriocapillaris (CC) flow deficit quantification algorithm available through the ARI Network. Flow deficit area and density values were analyzed and compared between the first and last examinations. Corresponding en face OCT imaging was used to distinguish true flow defects from artifacts secondary to shadowing. RESULTS: In the acute stage of AIM, a bacillary layer detachment appearing as a yellow-grayish foveal elevation surrounded by a hypopigmented parafoveal ring was evident in a Bull's eye configuration, corresponding to a hyperreflective ring upon NIR and a hyperautofluorescent ring with BAF. SD + SS-OCT showed mostly intraretinal fluid consistent with a bacillary layer detachment in conjunction with a thickened inner choroid. At presentation, SS-OCTA demonstrated a marked reduction of choriocapillaris flow signal. At 1 week, early resolution of retinal fluid was followed by restoration of the ellipsoid zone at 5 weeks, while restoration of the interdigitation zone and reduction in retinal pigment epithelium/Bruch membrane complex thickening occurred more slowly. Swept-source OCT angiography showed a gradual, but incomplete, recovery of inner choroidal flow signal at 3.5-month follow-up. CONCLUSION: Acutely, AIM may present with a photoreceptor splitting foveal bacillary layer detachment associated with a marked reduction in inner choroidal flow signal on SS-OCTA. Thereafter, restoration of the outer retinal layers and gradual normalization of choroidal flow signal appear to support the often-benign nature of the disease.


Subject(s)
Choroid , Macular Degeneration , Retinal Diseases , Adult , Choroid/diagnostic imaging , Choroid/physiopathology , Humans , Macular Degeneration/diagnostic imaging , Macular Degeneration/physiopathology , Male , Multimodal Imaging , Retinal Diseases/diagnostic imaging , Retinal Diseases/physiopathology , Tomography, Optical Coherence
18.
J Atheroscler Thromb ; 29(1): 11-23, 2022 Jan 01.
Article in English | MEDLINE | ID: mdl-33239480

ABSTRACT

AIMS: Small arteries can be visualized in the ocular fundus, and findings of retinopathy based on Scheie classification are often applied to evaluate the impact of hypertension and atherosclerosis. However, the relationship between damage in the large and small arteries has not been investigated sufficiently, especially in the early stages. The present study investigated possible associations between large artery atherosclerosis and small artery retinopathy in untreated middle-aged individuals. METHODS: Untreated middle-aged workers undergoing periodic health check-ups (n=7,730, 45±8 years) were enrolled in this study. The absence or presence and extent of retinopathy were characterized by ophthalmologists as hypertensive (H0-4) and atherosclerotic grades (S0-4) based on Scheie classification. Large artery atherosclerosis was examined based on functional assessment of the cardio-ankle vascular index (CAVI) and morphological assessment of the carotid intima-media thickness (IMT) by ultrasound. RESULTS: We found significant differences in CAVI and carotid IMT between individuals with and without hypertensive or atherosclerotic retinopathy. Multivariable regression analysis showed that the presence of hypertensive and atherosclerotic retinopathy was significantly associated with CAVI and carotid IMT. Logistic regression analysis with the endpoint of a hypertensive or atherosclerotic lesion revealed that CAVI and carotid IMT are independent determinants of retinopathy. CONCLUSIONS: CAVI and carotid IMT were significantly associated with the presence of retinopathy based on Scheie classification in untreated middle-aged subjects, implying that atherosclerotic examination in large arteries could reveal early-stage small artery retinopathy.


Subject(s)
Atherosclerosis/complications , Atherosclerosis/diagnosis , Hypertension/complications , Hypertension/diagnosis , Retinal Diseases/complications , Retinal Diseases/diagnosis , Adult , Age Factors , Ankle Brachial Index , Atherosclerosis/physiopathology , Carotid Intima-Media Thickness , Cohort Studies , Female , Humans , Hypertension/physiopathology , Male , Middle Aged , Regression Analysis , Retinal Diseases/physiopathology
19.
Retina ; 42(1): 152-158, 2022 01 01.
Article in English | MEDLINE | ID: mdl-34369439

ABSTRACT

PURPOSE: To define the effect of age-related macular degeneration (AMD) and diabetic retinopathy (DR) on the ocular thermographic profile. METHODS: This retrospective cross-sectional study included subjects diagnosed with DR or AMD between January and April 2019. Individuals without ocular disease served as controls. A thermal imaging camera was used for ocular surface temperature (OST) acquisition. The mean temperatures of the medial cantus, lateral cantus, and cornea were calculated. RESULTS: Thermographic images were obtained from 133 subjects (260 eyes, 97 DR and 163 AMD) and 48 controls (55 eyes). Ocular surface temperature was higher among patients with AMD and lowest among patients with DR (P < 0.001). A subgroup analysis revealed that eyes with diabetic macular edema had significantly higher OSTs than DR eyes without diabetic macular edema. Moreover, the OST in eyes with diabetic macular edema was similar to the measurements of the AMD group. There were no differences in OSTs between neovascular and nonneovascular AMD eyes. CONCLUSION: Although AMD and DR are considered posterior segment conditions, their effect on OST implies that the entire globe is involved. Although both conditions result from similar multifactorial pathophysiologic changes, the differences in OST between DR and AMD might be due to dissimilarity in the balance of pathologic processes involved in each condition. Further research is required to better understand the pathophysiology of these diseases and their effect on OST as well as to determine the effect of vasculature, circulation, and tissue metabolism on ocular temperature.


Subject(s)
Body Temperature/physiology , Eye/physiopathology , Retinal Diseases/physiopathology , Retinal Vessels/diagnostic imaging , Thermography/methods , Aged , Aged, 80 and over , Cross-Sectional Studies , Female , Humans , Male , Retina/diagnostic imaging , Retina/physiopathology , Retinal Diseases/diagnosis , Retinal Vessels/physiopathology , Retrospective Studies , Tomography, Optical Coherence/methods
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