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1.
Ophthalmology ; 130(9): 947-957, 2023 09.
Article in English | MEDLINE | ID: mdl-37088447

ABSTRACT

PURPOSE: To compare the efficacy and the safety of submacular hemorrhage (SMH) management using either surgical pars plana vitrectomy (PPV) or pneumatic displacement (PD) with tissue plasminogen activator (tPA) and vascular endothelial growth factor (VEGF) inhibitor added to each arm. DESIGN: Randomized, open-label, multicenter superiority study. PARTICIPANTS: Ninety patients with neovascular age-related macular degeneration (nAMD) 50 years of age or older with recent SMH (≤ 14 days) of more than 2 optic disc areas and predominantly overlying the retinal pigment epithelium. METHODS: Patients were assigned randomly to surgery (PPV, subretinal tPA [maximum, 0.5 ml/50 µg], and 20% sulfur hexafluoride [SF6] tamponade) or PD (0.05 ml intravitreal tPA [50 µg] and 0.3 ml intravitreal pure SF6). Both groups were asked to maintain a head upright position with the face forward at 45° for 3 days after intervention and received 0.5 mg intravitreal ranibizumab at the end of the intervention, at months 1 and 2, as the loading phase, and then on a pro re nata regimen during a 6-month follow-up. MAIN OUTCOME MEASURES: The primary efficacy endpoint was mean best-corrected visual acuity (VA) change at month 3. The secondary endpoints were mean VA change at month 6, 25-item National Eye Institute Visual Function Questionnaire composite score value at months 3 and 6, number of anti-VEGF injections, and complications during the 6-month follow-up. RESULTS: Of the 90 patients randomized, 78 patients (86.7%) completed the 3-month efficacy endpoint visit. The mean VA change from baseline to month 3 in the surgery group (+16.8 letters [95% confidence interval (CI), 8.7-24.9 letters]) was not significantly superior to that in the PD group (+16.4 letters [95% CI, 7.1-25.7 letters]; adjusted difference ß, 1.9 [-11.0; 14.9]; P = 0.767). Both groups achieved similar secondary outcomes at month 6. No unexpected ocular safety concerns were observed in either group. CONCLUSIONS: Surgery did not yield superior visual gain nor additional benefit for SMH secondary to nAMD compared with PD at 3 months, with intravitreal anti-VEGF added to each arm. Both treatment strategies lead to a clinical improvement of VA without safety concerns for SMH over 6 months. Both design and results of the trial cannot be used to establish equivalence between treatments. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.


Subject(s)
Macular Degeneration , Tissue Plasminogen Activator , Humans , Middle Aged , Infant, Newborn , Tissue Plasminogen Activator/therapeutic use , Angiogenesis Inhibitors/therapeutic use , Fibrinolytic Agents/therapeutic use , Vascular Endothelial Growth Factor A , Ranibizumab/therapeutic use , Retinal Hemorrhage/drug therapy , Retinal Hemorrhage/etiology , Retinal Hemorrhage/surgery , Macular Degeneration/complications , Macular Degeneration/drug therapy , Retinal Pigment Epithelium , Intravitreal Injections
2.
Int J Mol Sci ; 22(23)2021 Nov 23.
Article in English | MEDLINE | ID: mdl-34884448

ABSTRACT

Pathogenic variants in CRB1 lead to diverse recessive retinal disorders from severe Leber congenital amaurosis to isolated macular dystrophy. Until recently, no clear phenotype-genotype correlation and no appropriate mouse models existed. Herein, we reappraise the phenotype-genotype correlation of 50 patients with regards to the recently identified CRB1 isoforms: a canonical long isoform A localized in Müller cells (12 exons) and a short isoform B predominant in photoreceptors (7 exons). Twenty-eight patients with early onset retinal dystrophy (EORD) consistently had a severe Müller impairment, with variable impact on the photoreceptors, regardless of isoform B expression. Among them, two patients expressing wild type isoform B carried one variant in exon 12, which specifically damaged intracellular protein interactions in Müller cells. Thirteen retinitis pigmentosa patients had mainly missense variants in laminin G-like domains and expressed at least 50% of isoform A. Eight patients with the c.498_506del variant had macular dystrophy. In one family homozygous for the c.1562C>T variant, the brother had EORD and the sister macular dystrophy. In contrast with the mouse model, these data highlight the key role of Müller cells in the severity of CRB1-related dystrophies in humans, which should be taken into consideration for future clinical trials.


Subject(s)
Ependymoglial Cells/pathology , Eye Proteins/genetics , Eye Proteins/metabolism , Macular Degeneration/pathology , Membrane Proteins/genetics , Membrane Proteins/metabolism , Mutation , Nerve Tissue Proteins/genetics , Nerve Tissue Proteins/metabolism , Retinal Dystrophies/pathology , Retinitis Pigmentosa/pathology , Adolescent , Age of Onset , Alternative Splicing , Child , Child, Preschool , Ependymoglial Cells/metabolism , Eye Proteins/chemistry , Female , Genetic Association Studies , Humans , Infant , Macular Degeneration/genetics , Macular Degeneration/metabolism , Male , Membrane Proteins/chemistry , Models, Molecular , Mutation, Missense , Nerve Tissue Proteins/chemistry , Point Mutation , Retinal Dystrophies/genetics , Retinal Dystrophies/metabolism , Retinitis Pigmentosa/genetics , Retinitis Pigmentosa/metabolism , Retrospective Studies , Sequence Deletion , Young Adult
3.
Retina ; 40(11): 2140-2147, 2020 Nov.
Article in English | MEDLINE | ID: mdl-31876891

ABSTRACT

PURPOSE: To compare the effect of intravitreal injections of air with gas on vitreomacular traction (VMT) release and attempt to analyze predictive factors for success. METHODS: The medical records of patients with symptomatic VMT undergoing intravitreal injections (0.3 mL) of either octafluoropropane (C3F8) or air were retrospectively reviewed. The VMT release (primary end point) and the best-corrected visual acuity (secondary end point) were noted 1 month after injection. At baseline and 1 month after the injection, a macular optical coherence tomography was performed. RESULTS: Twenty-four eyes of 22 patients were included. Vitreomacular traction was released in 10 cases, 7 among 11 C3F8-injected eyes (63%) and 3 among 13 air-injected eyes (23%) (P = 0.045). In eyes with released VMT, ETDRS improved from 61 ± 35 (0-100) to 65 ± 37 (0-100) 1 month after the injection (P = 0.03). All patients with VMT release had a horizontal vitreomacular adhesion of less than 600 µm. Five eyes (23%) underwent vitrectomy after the injection of gas or air. CONCLUSION: Posterior vitreous detachment in VMT can be observed with both air and gas injection with a low complication rate. The occurrence of VMT release observed with air seemed to be less frequent than that observed with gas.


Subject(s)
Air , Endotamponade/methods , Fluorocarbons/administration & dosage , Retinal Diseases/surgery , Vitrectomy , Vitreous Detachment/surgery , Aged , Female , Follow-Up Studies , Humans , Intravitreal Injections , Male , Middle Aged , Retinal Diseases/pathology , Retrospective Studies , Tissue Adhesions , Tomography, Optical Coherence , Visual Acuity , Vitreous Detachment/pathology
4.
BMC Ophthalmol ; 20(1): 468, 2020 Dec 01.
Article in English | MEDLINE | ID: mdl-33256663

ABSTRACT

BACKGROUND: To report the first case of belatacept-associated multidrug-resistant Cytomegalovirus retinitis in a kidney transplant recipient. CASE PRESENTATION: A 76-year-old African male renal allograft recipient was admitted for acute visual loss of the right eye. Ophthalmological examination of the right eye showed anterior uveitis and vitritis associated with large paravascular haemorrhages and yellow necrotic borders, involving the posterior pole but not the fovea. Both Cytomegalovirus DNA in plasma and aqueous humor were positive. The patient had had several episodes of Cytomegalovirus reactivation subsequent to the introduction of belatacept. His cytomegalovirus was multi-drug resistant, and was treated with maribarir, intravitreal and systemic injections of foscarnet, and anti-Cytomegalovirus human immunoglobulin. In parallel, belatacept was stopped and switched to tacrolimus. Cytomegalovirus DNA became undetectable and there was partial improvement of visual acuity at the last ophthalmologic examination, 18 months after the initial diagnosis of Cytomegalovirus retinitis. CONCLUSION: Cytomegalovirus retinitis is an uncommon opportunistic infection in kidney transplant recipients. Cytomegalovirus retinitis is a serious infection because of the risk of blindness and the occurrence of associated life-threatening opportunistic infections. In view of the recent literature, kidney transplant recipients treated by belatacept immunosuppression may be at increased risk for Cytomegalovirus disease, notably Cytomegalovirus retinitis. The occurrence of Cytomegalovirus retinitis may help improve the selection of patients converted to belatacept.


Subject(s)
Cytomegalovirus Retinitis , Kidney Transplantation , Abatacept/therapeutic use , Aged , Antiviral Agents/therapeutic use , Cytomegalovirus Retinitis/diagnosis , Cytomegalovirus Retinitis/drug therapy , Ganciclovir/therapeutic use , Humans , Kidney Transplantation/adverse effects , Male
5.
Ophthalmologica ; 243(5): 347-354, 2020.
Article in English | MEDLINE | ID: mdl-32160616

ABSTRACT

BACKGROUND: Three-dimensional (3D) visualization systems, also known as heads-up systems, are now available for eye surgery and as with every new device there is need for a specific evaluation. OBJECTIVES: The aim of this study was to compare the efficiency, surgical comfort, and safety of a 3D visualization system to a standard binocular microscope (BM) in routine ophthalmologic procedures. METHOD: After a 4-week training period, a 3D visualization system (Ngenuity, Alcon®) available in one of the Robert Debré Hospital Ophthalmology Departments' operating rooms was compared to a standard BM (OPMI LUMIRA 700, Zeiss®), in the process of a call for new device evaluation. From December 2017 to March 2018, 5 surgeons and their respective residents were asked to fill in a questionnaire for all procedures. Before the surgery, the surgeon recorded: (i) the type of surgery (cataract [PK], retinal detachment [RD], epiretinal membrane peeling [ERM], macular hole, vitreous haemorrhage [VH]), (ii) the type of visualization system chosen (3D or BM), and (iii) the estimated surgical risk (low, intermediate, or high grade). At the end of the procedure, the primary surgeon recorded the remaining parameters, including: (i) surgery duration, (ii) intraoperative complications, (iii) percentage of endoillumination for posterior segment surgeries, (iv) status of the operator (senior or resident) and operator switch if necessary (senior only, resident only, or resident with help of the senior), and rated: (i) the visual comfort (low, normal, excellent), (ii) the operative fluency (low, normal, excellent), (iii) backaches (none, low, moderate, important), and (iv) headaches (range from 0 to 10). Age and sex were collected retrospectively. The procedures performed with 3D and BM were subsequently compared using univariate (χ2, Fisher, Wilcoxon) and multivariate analysis (generalized linear model), allowing us to identify parameters independently associated with PK surgery duration. RESULTS: A total of 102 valid questionnaires, relative to 73 PK and 29 vitreoretinal procedures, respectively, were analysed. As regards PK (3D, n = 25 vs. BM, n = 48), the mean age, sex ratio, surgical risk, intraoperative complications (1/25 vs. 4/48), visual comfort, backaches, and headaches were similar between the two systems. The use of 3D allowed faster PK surgeries (16.44 ± 4.36 vs. 21.44 ± 7.50 min; p = 0.007) and slightly enhanced the operative fluency. In vitreoretinal surgeries (3D, n = 14 vs. BM, n = 15), no obvious differences between the two visualization systems were observed, although the use of the 3D system was found to slightly decrease the operative fluency. Parameters independently associated with PK surgery duration were 3D visualization (ß = -4.4 ± 1.4; p = 0.002), high preoperative surgical risk (ß = 6.2 ± 2.4; p = 0.012), intraoperative complications (ß = 8.7 ± 2.6; p = 0.001), and surgeon status (ß = -4.4 ± 1.3; p = 0.001) in univariate and multivariate analysis. CONCLUSIONS: 3D visualization can be safely used in routine practice. It slightly improves the operative fluency, allowing faster PK surgery.


Subject(s)
Anterior Eye Segment/diagnostic imaging , Eye Diseases/diagnosis , Imaging, Three-Dimensional/instrumentation , Microscopy/instrumentation , Posterior Eye Segment/diagnostic imaging , Aged , Equipment Design , Female , Follow-Up Studies , Humans , Male , Retrospective Studies
6.
Hum Mol Genet ; 26(22): 4367-4374, 2017 11 15.
Article in English | MEDLINE | ID: mdl-28973654

ABSTRACT

In this study, we report a novel duplication causing North Carolina macular dystrophy (NCMD) identified applying whole genome sequencing performed on eight affected members of two presumed unrelated families mapping to the MCDR1 locus. In our families, the NCMD phenotype was associated with a 98.4 kb tandem duplication encompassing the entire CCNC and PRDM13 genes and a common DNase 1 hypersensitivity site. To study the impact of PRDM13 or CCNC dysregulation, we used the Drosophila eye development as a model. Knock-down and overexpression of CycC and CG13296, Drosophila orthologues of CCNC and PRDM13, respectively, were induced separately during eye development. In flies, eye development was not affected, while knocking down either CycC or CG13296 mutant models. Overexpression of CycC also had no effect. Strikingly, overexpression of CG13296 in Drosophila leads to a severe loss of the imaginal eye-antennal disc. This study demonstrated for the first time in an animal model that overexpression of PRDM13 alone causes a severe abnormal retinal development. It is noteworthy that mutations associated with this autosomal dominant foveal developmental disorder are frequently duplications always including an entire copy of PRDM13, or variants in one DNase 1 hypersensitivity site at this locus.


Subject(s)
Corneal Dystrophies, Hereditary/genetics , Cyclin C/genetics , Histone-Lysine N-Methyltransferase/genetics , Adult , Animals , Chromosome Mapping , Chromosomes, Human, Pair 6 , Corneal Dystrophies, Hereditary/metabolism , Cyclin C/metabolism , Drosophila melanogaster , Eye Proteins/genetics , Female , Genetic Linkage , Haplotypes , Histone-Lysine N-Methyltransferase/metabolism , Humans , Male , PR-SET Domains , Pedigree , Whole Genome Sequencing
7.
Doc Ophthalmol ; 136(2): 97-111, 2018 04.
Article in English | MEDLINE | ID: mdl-29536324

ABSTRACT

PURPOSE: Pericentral visual field changes and disruption of the ellipsoid layer on spectral domain optical coherence tomography (SD-OCT) are the main features of antimalarial retinal toxicity. C-Scan OCT or "en face" enables a topographic frontal view of the changes observed within the different retinal layers in particular the ellipsoid layer. The aim of this prospective study was to compare multifocal ERG (mfERG) responses with the results of C-Scan OCT ("en face" OCT) in patients with abnormal visual field and to analyze relationships between the structural and functional abnormalities. METHODS: In 354 consecutive patients screened for antimalarial toxicity between January 1, 2014 and December 31, 2016, central visual field, mfERG recording, C-Scan OCT and short-wavelength fundus autofluorescent imaging were performed. RESULTS: Among the 17/354 patients with abnormal central visual field results, all presented with abnormalities on the mfERG at least in one eye. In 16/33 eyes, there was a good concordance between focal loss of the mfERG response and the disruption of the ellipsoid layer on C-Scan OCT. In one eye with characteristic changes in the ellipsoid layer on the C-Scan OCT, the mfERG was normal, whereas in three eyes the mfERG was abnormal in eyes with a normal C-Scan OCT. CONCLUSIONS: The contribution of the C-Scan OCT changes remains difficult to establish as there is no strict concordance with the local ERG responses. Although C-Scan OCT technology provides a new approach in analyzing focal abnormalities in the photoreceptor-retinal pigment epithelium interface, the sensitivity of this method compared with mfERG and other tests (central visual field, B-Scan OCT) needs to be evaluated. This study is still ongoing on a larger cohort.


Subject(s)
Antimalarials/toxicity , Electroretinography/methods , Hydroxychloroquine/toxicity , Retina/drug effects , Retinal Diseases/diagnosis , Tomography, Optical Coherence/methods , Adult , Aged , Female , Fundus Oculi , Humans , Male , Middle Aged , Prospective Studies , Retina/pathology , Retinal Diseases/chemically induced , Retinal Pigment Epithelium/drug effects , Visual Fields/drug effects
8.
Ophthalmology ; 123(9): 1865-73, 2016 09.
Article in English | MEDLINE | ID: mdl-27320518

ABSTRACT

PURPOSE: To assess the association of clinical and biological factors with extensive macular atrophy with pseudodrusen (EMAP) characterized by bilateral macular atrophy occurring in patients aged 50 to 60 years and a rapid progression to legal blindness within 5 to 10 years. DESIGN: A national matched case-control study. PARTICIPANTS: Participants were recruited in 10 French Departments of Ophthalmology and their associated clinical investigation centers. All 115 patients with EMAP had symptoms before the age of 55 years due to bilateral extensive macular atrophy with a larger vertical axis and diffuse pseudodrusen. Three controls without age-related macular degeneration (AMD) or retinal disease at fundus examination were matched for each patient with EMAP by gender, age, and geographic area (in total 415). METHODS: Subjects and controls underwent an eye examination including color, red-free autofluorescent fundus photographs and spectral-domain optical coherence tomography with macular analysis. The interviews collected demographic, lifestyle, family and personal medical history, medications, and biological data. Associations of risk factors were estimated using conditional logistic regression. MAIN OUTCOME MEASURES: Extensive macular atrophy with pseudodrusen status (cases vs. controls). RESULTS: Extensive macular atrophy with pseudodrusen most frequently affected women (70 women, 45 men). After multivariate adjustment, family history of glaucoma or AMD was strongly associated with EMAP (odds ratio [OR], 2.3, P = 0.008 and OR, 1.5, P = 0.01, respectively). No association was found with cardiac diseases or their risk factors. Mild and moderate kidney disease and higher neutrophil rate were associated with a reduced risk of EMAP (OR, 0.58, P = 0.04; OR, 0.34, P = 0.01; and OR, 0.59, P = 0.003, respectively). On the contrary, eosinophilia (OR, 1.6; P = 0.0002), lymphocytosis (OR, 1.84; P = 0.0002), increased erythrocyte sedimentation rate (OR, 6.5; P = 0.0005), decreased CH50 (P = 0.001), and high plasma C3 level (P = 0.023) were significantly associated with a higher risk of EMAP. CONCLUSIONS: This study documents an association between EMAP and family history of AMD and glaucoma, a clear female predominance, and a systemic inflammatory profile. The reduced CH50 and increased C3 plasma values could reflect a more severe complement pathway dysfunction than in AMD, leading to early pseudodrusen and rapid development of geographic atrophy. There is no association of EMAP with AMD cardiac diseases or cardiac risks, including cigarette smoking.


Subject(s)
Geographic Atrophy/epidemiology , Macular Degeneration/epidemiology , Retinal Drusen/epidemiology , Adult , Aged , Aged, 80 and over , Blindness , Case-Control Studies , Choroidal Neovascularization/epidemiology , Diagnostic Techniques, Ophthalmological , Disease Progression , Female , France/epidemiology , Geographic Atrophy/etiology , Humans , Macular Degeneration/etiology , Male , Middle Aged , Odds Ratio , Photography , Retinal Drusen/etiology , Risk Factors , Sex Distribution , Tomography, Optical Coherence , Visual Acuity
9.
Graefes Arch Clin Exp Ophthalmol ; 254(11): 2175-2181, 2016 Nov.
Article in English | MEDLINE | ID: mdl-27207466

ABSTRACT

BACKGROUND: This present retrospective case control study was designed to evaluate circadian disturbance in patients with chronic idiopathic central serous chorioretinopathy (ICSC). METHODS: Between January 1st, 2012, and November 30th, 2014, 29 consecutive patients with chronic ICSC examined in a referral setting were compared with a gender-matched and age-matched control group of 29 patients. A history of pharmacologic medication (including corticosteroid treatment), sleep disturbance, irregular working hours, cardiovascular risk factors, and depressive anxiety disorders was noted. RESULTS: The median age of the patients was 52, and in the control subjects it was 50. The male-female ratio for both groups was 4.8:1. Patients with chronic ISCS were more likely to be exposed to irregular working hours (p < 0.01, OR 9.3 [2.29-37.6]) and to present with overweight than the control subjects (p = 0.016). No significant differences were found for sleeping disturbances, pharmacological medication, cardiovascular risk factors, or depressive anxiety disorders. CONCLUSIONS: In this preliminary study, the exposition of irregular working hours as a risk factor for chronic ICSC was identified, which had not been previously reported. If further studies confirm these findings, then employment with regular working hours could be recommended for chronic ICSC patients.


Subject(s)
Central Serous Chorioretinopathy/physiopathology , Chronobiology Disorders/complications , Visual Acuity , Adult , Aged , Central Serous Chorioretinopathy/epidemiology , Central Serous Chorioretinopathy/etiology , Chronobiology Disorders/physiopathology , Female , Follow-Up Studies , France/epidemiology , Humans , Incidence , Male , Middle Aged , Retrospective Studies , Risk Factors , Young Adult
11.
Ophthalmology ; 121(12): 2406-14, 2014 Dec.
Article in English | MEDLINE | ID: mdl-25085631

ABSTRACT

PURPOSE: To assess the frequency of and to characterize the clinical spectrum and optical coherence tomography findings of vitelliform macular dystrophy linked to IMPG1 and IMPG2, 2 new causal genes expressed in the interphotoreceptor matrix. DESIGN: Retrospective epidemiologic, clinical, electrophysiologic, and molecular genetic study. PARTICIPANTS: The database of a national referral center specialized in genetic sensory diseases was screened for patients with a macular vitelliform dystrophy without identified mutation or small deletion or large rearrangement in BEST1 and PRPH2 genes. Forty-nine families were included. METHODS: Clinical, imaging, and electro-oculogram findings were reviewed. Mutation screening of IMPG1 and IMPG2 genes were performed systematically. MAIN OUTCOME MEASURES: Frequency, inheritance, and clinical pattern of vitelliform dystrophy associated with IMPG1 and IMPG2 mutations were characterized. RESULTS: IMPG1 was the causal gene in 3 families (IMPG1 1-3, 11 patients) and IMPG2 in a fourth family (2 patients). With an autosomal dominant transmission, families 1 and 2 had the c.713T→G (p.Leu238Arg) mutation in IMPG1 and family 4 had the c.3230G→T (p.Cys1077Phe) mutation in IMPG2. Patients with IMPG1 or IMPG2 mutations had a late onset and moderate visual impairment (mean visual acuity, 20/40; mean age of onset, 42 years), even in the sporadic case of family 3 with a presumed recessive transmission (age at onset, 38 years; mean visual acuity, 20/50). Drusen-like lesions adjacent to the vitelliform deposits were observed in 9 of 13 patients. The vitelliform material was above the retinal pigment epithelium (RPE) at any stage of the macular dystrophy, and this epithelium was well preserved and maintained its classical reflectivity on spectral-domain optical coherence tomography (SD-OCT). Electro-oculogram results were normal or borderline in 9 cases. CONCLUSIONS: IMPG1 and IMPG2 are new causal genes in 8% of families negative for BEST1 and PRPH2 mutations. These genes should be screened in adult-onset vitelliform dystrophy with (1) moderate visual impairment, (2) drusen-like lesions, (3) normal reflectivity of the RPE line on SD-OCT, and (4) vitelliform deposits located between ellipsoid and interdigitation lines on SD-OCT. These clinical characteristics are not observed in the classical forms of BEST1 or PRPH2 vitelliform dystrophies.


Subject(s)
Extracellular Matrix Proteins/genetics , Eye Proteins/genetics , Genetic Predisposition to Disease , Mutation , Proteoglycans/genetics , Vitelliform Macular Dystrophy , Adult , Aged , Case-Control Studies , Electrooculography , Female , Humans , Male , Middle Aged , Phenotype , Retinal Pigment Epithelium/pathology , Retrospective Studies , Tomography, Optical Coherence , Visual Acuity , Vitelliform Macular Dystrophy/genetics , Vitelliform Macular Dystrophy/pathology , Vitelliform Macular Dystrophy/physiopathology
12.
Mult Scler ; 20(7): 848-53, 2014 06.
Article in English | MEDLINE | ID: mdl-24177207

ABSTRACT

BACKGROUND: Optic neuritis (ON) may be the first symptom of a central nervous system demyelinating, systemic or infectious disease but few patients experience recurrent episodes and have a negative workup. OBJECTIVE: This disorder, named relapsing optic neuritis (RON), is poorly described in the literature and still presents a particular challenge in diagnosis and management. METHODS: We describe the clinical, laboratory, magnetic resonance imaging (MRI) and disability course of RON in a French cohort of 62 patients, based on a multicentre, retrospective, observational study. RESULTS: In our cohort, we identified two distinct groups of RON patients. The first is characterised by relapsing inflammatory optic neuritis (RION, 68%), which is non-progressive, whereas the second presented as a chronic relapsing inflammatory optic neuritis (CRION, 32%), which is progressive. We have noted more cases with steroid dependence in the CRION group than the RION group (42% vs 10%). The long-term visual prognosis was more severe in CRION patients and neuromyelitis optica-immunoglobulin G (NMO-IgG)-positive patients. CONCLUSION: RON is likely a separate entity corresponding to an autoimmune disease that differs from multiple sclerosis (MS), NMO and vasculitis. We provide a new classification system based on a better understanding of RON which could allow an improved management by early treatment of poor prognosis forms.


Subject(s)
Optic Neuritis/diagnosis , Adolescent , Adult , Biomarkers/blood , Chronic Disease , Disability Evaluation , Disease Progression , Female , France , Humans , Immunoglobulin G/blood , Kaplan-Meier Estimate , Magnetic Resonance Imaging , Male , Middle Aged , Optic Neuritis/classification , Optic Neuritis/immunology , Optic Neuritis/physiopathology , Predictive Value of Tests , Recurrence , Retrospective Studies , Terminology as Topic , Time Factors , Visual Acuity , Young Adult
13.
Graefes Arch Clin Exp Ophthalmol ; 252(2): 339-45, 2014 Feb.
Article in English | MEDLINE | ID: mdl-24178807

ABSTRACT

BACKGROUND: In non-arteritic anterior ischemic optic neuropathy (NA-AION), no treatments have demonstrated to be effective in recovering visual loss in randomized clinical trials. Oral steroids have been evaluated, and small series of intravitreal triamcinolone acetonide (IVTA) injection in NA-AION have been reported. The purpose of our study was to report the visual outcome and morphological changes in response to a single IVTA injection as a treatment for patients with NA-AION. PATIENTS AND METHODS: The charts of 36 patients with visual symptoms and optic disc swelling caused by NA-AION were evaluated. Twenty-one patients had received 4 mg IVTA and were compared with 15 non-treated patients. Visual acuity (VA), retinal nerve fiber layer thickness and static visual field were evaluated after 6 months. RESULTS: VA improvement at 6 months is statistically better in the treated group than in the non-treated group (p = 0.0035). In the treated group, there was a significant inverse correlation between the delay of the injection and the visual acuity achieved at 6 months (p < 0.0083**, r = -0.56). A significant improvement of the visual field was noted in the injected group when compared with the non-treated group at 6 months (p < 0.0028). DISCUSSION: In this retrospective study, patients receiving IVTA in the acute phase of NA-AION have better improvement of VA and visual field during the follow-up period of 6 months. However, only a large randomized controlled trial may enable to evaluate the benefits of IVTA Injections on visual outcome in NA-AION.


Subject(s)
Glucocorticoids/therapeutic use , Optic Neuropathy, Ischemic/drug therapy , Triamcinolone Acetonide/therapeutic use , Aged , Aged, 80 and over , Arteritis/drug therapy , Arteritis/physiopathology , Female , Humans , Intraocular Pressure/physiology , Intravitreal Injections , Male , Middle Aged , Nerve Fibers/pathology , Optic Neuropathy, Ischemic/physiopathology , Retinal Ganglion Cells/pathology , Retrospective Studies , Treatment Outcome , Visual Acuity/physiology , Visual Fields/physiology
14.
Semin Ophthalmol ; : 1-9, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38661124

ABSTRACT

PURPOSE: To report new indications for deep temporalis fascia (DTF) grafts in the ophthalmic field. METHODS: Monocentric retrospective interventional case series study. All the patients who underwent a DTF graft in an unpublished new indication over the study period (May 2020-October 2023) were included. For each patient, gender, age, graft indication, outcomes, complications, and follow-up duration were collected. In most cases, the DTF graft was covered by a vascularized flap. RESULTS: Eight patients underwent a DTF graft over the study period. The indications were: radiotherapy-induced scleral necrosis in three cases, tendinoplasty to replace the inferior rectus muscle tendon invaded by a locally advanced conjunctival carcinoma in one case, Ahmed glaucoma valve tube exposure in one case, intraocular lens with scleral fixation exposure in one case, orbital cerebrospinal fluid fistula (orbitorrhea) in one case, and post-traumatic complete corneal graft loss in one case. The DTF graft was successful in 87.5% of cases after a mean follow-up of 11.4 months. No complications were observed. CONCLUSIONS: DTF graft is a highly versatile graft that can be easily harvested. New indications for DTF grafts may include the repair of radiotherapy-induced scleral necrosis, the creation of oculomotor tendon and the temporary packing of large ocular tissue loss in an emergency context. Further studies with a longer follow-up are needed to confirm our preliminary results.

15.
Trials ; 25(1): 273, 2024 Apr 22.
Article in English | MEDLINE | ID: mdl-38649937

ABSTRACT

BACKGROUND: Macular edema (ME) results from hyperpermeability of retinal vessels, leading to chronic extravasation of plasma components into the retina and hence potentially severe visual acuity loss. Current standard of care consists in using intravitreal injections (IVI), which results in a significant medical and economic burden. During diabetic retinopathy (DR) or retinal vein occlusion (RVO), it has recently been shown that focal vascular anomalies (capillary macro-aneurysms, also termed TelCaps) for telangiectatic capillaries may play a central role in the onset, early recurrence, and/or persistence of ME. Since targeted photocoagulation of TelCaps may improve vision, identification, and photocoagulation of TelCaps, it may represent a way to improve management of ME. OBJECTIVE: The Targeted Laser in (Diabetic) Macular Edema (TalaDME) study aims to evaluate whether ICG-guided targeted laser (IGTL), in association with standard of care by IVI, allows reducing the number of injections during the first year of treatment compared with IVI only, while remaining non-inferior for visual acuity. METHODS: TalaDME is a French, multicentric, two-arms, randomized, sham laser-controlled, double-masked trial evaluating the effect of photocoagulation of TelCaps combined to IVI in patients with ME associated with TelCaps. Patients with vision loss related to center involved ME secondary to RVO or DR and presenting TelCaps are eligible. Two hundred and seventy eyes of 270 patients are randomized in a 1:1 ratio to standard care, i.e., IVI of anti-VEGF solely (control group) or combined with IGTL therapy (experimental group). Stratification is done on the cause of ME (i.e., RVO versus diabetes). Anti-VEGF IVI are administered to both groups monthly for 3 months (loading dose) and then with a pro re nata regimen with a monthly follow-up for 12 months. The primary endpoint will be the number of IVI and the change in visual acuity from baseline to 12 months. Secondary endpoints will be the changes in central macular thickness, impact on quality of life, cost of treatment, and incremental cost-utility ratio in each groups. KEY SAFETY: Rare but severe AE linked to the use of IVI and laser, and previously described, are expected. In the sham group, rescue laser photocoagulation may be administered by the unmasked investigator if deemed necessary at month 3. DISCUSSION: The best management of ME associated with TelCaps is debated, and there have been no randomized study designed to answer this question. Given the fact that TelCaps may affect 30 to 60% of patients with chronic ME due to DR or RVO, a large number of patients could benefit from a specific management of TelCaps. TalaDME aims to establish the clinical and medico-economic benefits of additional targeted laser. The results of TalaDME may raise new recommendations for managing ME and impact healthcare costs. TRIAL REGISTRATION: EudraCT: 2018-A00800-55/ NCT03751501. Registration date: Nov. 23, 2018.


Subject(s)
Angiogenesis Inhibitors , Diabetic Retinopathy , Laser Coagulation , Macular Edema , Retinal Vein Occlusion , Vascular Endothelial Growth Factor A , Visual Acuity , Humans , Macular Edema/etiology , Macular Edema/drug therapy , Macular Edema/surgery , Retinal Vein Occlusion/drug therapy , Retinal Vein Occlusion/complications , Diabetic Retinopathy/drug therapy , Laser Coagulation/methods , Vascular Endothelial Growth Factor A/antagonists & inhibitors , Angiogenesis Inhibitors/administration & dosage , Angiogenesis Inhibitors/adverse effects , Angiogenesis Inhibitors/therapeutic use , France , Treatment Outcome , Multicenter Studies as Topic , Intravitreal Injections , Time Factors , Equivalence Trials as Topic , Combined Modality Therapy
16.
Article in English | MEDLINE | ID: mdl-37639637

ABSTRACT

PURPOSE: To describe the molecular diagnosis and atypical ocular presentation of a patient who suffered for a Rendu-Osler-Weber syndrome associated with juvenile polyposis (JP) syndrome. METHODS: This is a case report of a patient that underwent fundus examen, brain Magnetic Resonance Imaging (MRI) and arteriography. Genetic testing was performed by next-generation-sequencing (NGS). RESULTS: A 35-year-old woman presented with right hemiplegia with right homonymous lateral hemianopia and homolateral complete sensory deficit. She also had Roth spots in her left fundus. Genetic testing revealed a pathogenic variation in the heterozygous state in the SMAD-4 gene (c.1245_1248del). CONCLUSION: Hereditary Hemorrhagic Telangiectasia (HTT) also known as Rendu-Osler-Weber syndrome is a rare autosomal dominant disease which reveals mostly with epistaxis and cutaneous telangiectasias. Our clinical case reports Roth spots in the context of HTT associated with juvenile polyposis syndrome. SMAD-4 mutation may explain the presence of a carotid-ophthalmic aneurysm which is not a lesion usually found in HTT.

17.
Front Med (Lausanne) ; 10: 1120283, 2023.
Article in English | MEDLINE | ID: mdl-36968840

ABSTRACT

Introduction: Descemet membrane endothelial keratoplasty (DMEK) is the main treatment for Fuchs' dystrophy (FECD). The outcomes are excellent, but the final visual recovery may vary from patient to patient with sometimes no obvious reason of such a spread. Methods: We conducted a clinical prospective multicentric study to identify the predictive factors for the visual result 1 year after surgery. Eighty three patients (83 eyes) were included. Results: Postoperative BCVA after 1 year was 0.20 ± 0.18 logMAR. Logistic regression revealed that good visual recovery correlated negatively with preoperative central macular thickness (p < 0.001) and the need for rebubbling (p = 0.05), and positively with preoperative visual acuity (p = 0.009). Multivariate formula to predict the 1-year BCVA has been suggested. Discussion: Preoperative retinal status seems to be the main predictive factor for long-term visual result after DMEK. Our predictive multivariate model could assist in better informing the patient about the prognosis of the surgery, and in adjusting the therapeutic strategy also, further highlighting the essential collaboration between both cornea and retina subspecialists.

18.
Eye (Lond) ; 37(4): 638-643, 2023 03.
Article in English | MEDLINE | ID: mdl-35273350

ABSTRACT

PURPOSE: Altered glucose metabolism, along with low-grade inflammation, has been proposed to be involved in retinal detachment (RD)-induced cone loss. Here, we assessed intravitreal glucose and cytological profile in patients with macula-off RD. METHODS: Glucose concentration was analysed in vitreous samples from 137 non-diabetic patients undergoing vitrectomy for either primary macula-off RD (n = 73) or epiretinal membrane (ERM; n = 64). Cellularity was assessed in vitreous cytospin preparations by a semi-quantitative immunostaining approach. RESULTS: Intravitreal glucose concentration was higher in the RD group (2.28 mmol.L-1 n =73 vs 1.6 mmol.L-1 n = 64; p < 0.0001). Overall cellularity and density of macrophages were significantly higher in the vitreous of RD patients (respectively p = 0.003 and p < 0.0001). Among the RD patients, intravitreal glucose concentration correlated with macrophages density (p = 0.002): its levels remained significantly higher in eyes in which macrophages were innumerable compared to lower macrophages densities RD eyes (p = 0.0095). CONCLUSIONS: We observed a strong relationship between intravitreal glucose concentration and vitreous macrophage density. Additional indicators for vitreous glycation and low-grade inflammation should be further studied.


Subject(s)
Epiretinal Membrane , Retinal Detachment , Humans , Vitrectomy , Epiretinal Membrane/surgery , Inflammation , Glucose
19.
Am J Ophthalmol Case Rep ; 27: 101601, 2022 Sep.
Article in English | MEDLINE | ID: mdl-35668738

ABSTRACT

Purpose: We report the use of a rapid multiplex polymerase chain reaction (PCR) system in the microbiological diagnosis and the therapeutic management of a severe bacterial keratitis case. Observations: During the management of a severe bacterial keratitis case, standard microbiological diagnostic methods were performed. At the same time, an additional ocular swab sampling from the cornea was performed and analyzed using two rapid multiplex PCR assays allowing the simultaneous detection of 29 different virus, yeast and bacteria genomes. Using combination of two rapid multiplex PCR systems, the microbiological diagnosis of a severe Pseudomonas aeruginosa induced keratitis was performed within 90 minutes after an ocular sampling. A rapid subsequent adaptation of local antibiotic treatment was performed allowing to the young patient to regain 6 months after her hospital admission a final visual acuity of 20/20 in her right eye. Conclusions and importance: The present case report suggests that the use of a rapid multiplex PCR strategy may result in a decrease of the mean hospital stage duration for severe infectious keratitis and in an improvement of the clinical outcome of severe keratitis infections. Nevertheless, additional prospective studies are needed to evaluate whether this innovative strategy may replace the current standard approach and optimize the therapeutic management of severe corneal infections.

20.
Article in English | MEDLINE | ID: mdl-35055748

ABSTRACT

While exposure to pesticides is a known risk factor for neurodegenerative brain diseases, little is known about the influence of environment on glaucoma neuropathy. We aimed to determine whether farmers are at higher risk of developing severe primary open-angle glaucoma (POAG). This retrospective cohort study (tertiary referral center, Reims University Hospital, France) included patients diagnosed with POAG in the last two years. Univariate analysis and adjusted multivariate logistic regression were performed to evaluate the association between agricultural profession and all recorded data. Glaucoma severity (primary outcome) and the number of patients who underwent filtering surgery (secondary outcome) were analyzed. In total, 2065 records were screened, and 772 patients were included (66 in the farmer group and 706 in the nonfarmer group). The risk of severe glaucoma was higher in the farmer group (adjusted odds ratio (aOR) 1.87, p = 0.03). More patients underwent filtering surgery in the farmer group in univariate analysis (p = 0.02) but with no statistical significance after adjustment (p = 0.08). These results suggest pesticide exposure may be a factor accelerating the neurodegeneration in POAG, although a direct link between the agricultural profession and the disease requires further extended studies to be demonstrated.


Subject(s)
Glaucoma, Open-Angle , Pesticides , Glaucoma, Open-Angle/complications , Glaucoma, Open-Angle/etiology , Humans , Logistic Models , Pesticides/adverse effects , Retrospective Studies , Risk Factors
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