Ophthalmic Center

San Siro, Italy

Ophthalmic Center

San Siro, Italy

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Ebran J.-M.,University of Angers | Mimoun G.,Ecole Militaire Ophthalmic Center | Donati A.,Ophthalmic Center | Jean-Pastor M.-J.,University Hospital of Sainte Marguerite | And 2 more authors.
Journal Francais d'Ophtalmologie | Year: 2016

Introduction: Pseudoxanthoma elasticum (PXE), a rare hereditary connective tissue disorder,may be complicated by angioid streaks (AS) and choroidal neovascularization (CNV), whichmay lead to irreversible loss of visual acuity (VA). Here we describe the safety and efficacy ofranibizumab in patients with CNV secondary to PXE.Methods: A multicenter (n = 23), observational study of a retrospective/prospective cohort,performed under real world conditions in France in all patients with CNV secondary to PXEwho received at least one ranibizumab injection as of October 2011. The study objectives wereto describe the mean annual number and reason for ranibizumab injections since initiation,evolution of best-corrected visual acuity (BCVA by Early Treatment Diabetic Retinopathy Study[ETDRS] letters), and safety.Results: Patients (n = 72; 98 eyes) had a mean age of 59.6 ± 8.3 years and consisted of 54.2%men. The criterion for retreatment was based mainly on loss of VA, progression of CNV andangiographic leakage. CNV was primarily subfoveal or juxtafoveal (73.4%), and the initial meanVA was 64.6 ETDRS letters. On average, visual acuity of all eyes analyzed was relatively stableduring the 2-year follow-up (62.3 letters vs 64.6 letters at the first injection), and 88.6% ofeyes maintained VA between -15 and +15 letters or gained over 15 letters. No deaths or newintolerances were described. Conclusions: These results showed that ranibizumab was able to maintain stable VA in cli-nical practice for at least 2 years in patients with CNV secondary to PXE, and to significantlyreduce the frequency of neovascularization relapses, with a limited number of injections. Thetreatment was well tolerated by the patients. © 2015 Elsevier Masson SAS. All rights reserved.


PubMed | Novartis, University of Angers, Ecole Militaire Ophthalmic Center, Ophthalmic Center and University Hospital of Sainte Marguerite
Type: Journal Article | Journal: Journal francais d'ophtalmologie | Year: 2016

Pseudoxanthoma elasticum (PXE), a rare hereditary connective tissue disorder, may be complicated by angioid streaks (AS) and choroidal neovascularization (CNV), which may lead to irreversible loss of visual acuity (VA). Here we describe the safety and efficacy of ranibizumab in patients with CNV secondary to PXE.A multicenter (n=23), observational study of a retrospective/prospective cohort, performed under real world conditions in France in all patients with CNV secondary to PXE who received at least one ranibizumab injection as of October 2011. The study objectives were to describe the mean annual number and reason for ranibizumab injections since initiation, evolution of best-corrected visual acuity (BCVA by Early Treatment Diabetic Retinopathy Study [ETDRS] letters), and safety.Patients (n=72; 98 eyes) had a mean age of 59.68.3years and consisted of 54.2% men. The criterion for retreatment was based mainly on loss of VA, progression of CNV and angiographic leakage. CNV was primarily subfoveal or juxtafoveal (73.4%), and the initial mean VA was 64.6 ETDRS letters. On average, visual acuity of all eyes analyzed was relatively stable during the 2-year follow-up (62.3 letters vs 64.6 letters at the first injection), and 88.6% of eyes maintained VA between -15 and +15 letters or gained over 15 letters. No deaths or new intolerances were described.These results showed that ranibizumab was able to maintain stable VA in clinical practice for at least 2years in patients with CNV secondary to PXE, and to significantly reduce the frequency of neovascularization relapses, with a limited number of injections. The treatment was well tolerated by the patients.


Grenier J.,Maladies Sensorielles Genetiques | Meunier I.,Maladies Sensorielles Genetiques | Meunier I.,French Institute of Health and Medical Research | Meunier I.,Montpellier University | And 16 more authors.
Ophthalmology | Year: 2016

Purpose To search for WFS1 mutations in patients with optic atrophy (OA) and assess visual impairment. Design Retrospective molecular genetic and clinical study. Participants Patients with OA followed at a national referral center specialized in genetic sensory diseases. Methods Mutation screening in WFS1 was performed by Sanger sequencing. WFS1-positive patients were evaluated on visual acuity (VA) and retinal nerve fiber layer (RNFL) thickness using time-domain (TD) or spectral-domain (SD) optical coherence tomography (OCT). Statistical analysis was performed. Main Outcome Measures Mutation identification, VA values, and RNFL thickness in sectors. Results Biallelic WFS1 mutations were found in 3 of 24 unrelated patients (15%) with autosomal recessive nonsyndromic optic atrophy (arNSOA) and in 8 patients with autosomal recessive Wolfram syndrome (arWS) associated with diabetes mellitus and OA. Heterozygous mutations were found in 4 of 20 unrelated patients (20%) with autosomal dominant OA. The 4 WFS1-mutated patients of this latter group with hearing loss were diagnosed with autosomal dominant Wolfram-like syndrome (adWLS). Most patients had VA decrease, with logarithm of the minimum angle of resolution (logMAR) values lower in arWS than in arNSOA (1.530 vs. 0.440; P = 0.026) or adWLS (0.240; P = 0.006) but not differing between arNSOA and adWLS (P = 0.879). All patients had decreased RNFL thickness that was worse in arWS than in arNSOA (SD OCT, 35.50 vs. 53.80 μm; P = 0.018) or adWLS (TD-OCT, 45.84 vs. 59.33 μm; P = 0.049). The greatest difference was found in the inferior bundle. Visual acuity was negatively correlated with RNFL thickness (r = −0.89; P = 0.003 in SD OCT and r = −0.75; P = 0.01 in TD-OCT). Conclusions WFS1 is a gene causing arNSOA. Patients with this condition had significantly less visual impairment than those with arWS. Thus systematic screening of WFS1 must be performed in isolated, sporadic, or familial optic atrophies. © 2016 American Academy of Ophthalmology


PubMed | Maladies Sensorielles Genetiques, French Institute of Health and Medical Research, Montpellier University and Ophthalmic Center
Type: Journal Article | Journal: Ophthalmology | Year: 2016

To search for WFS1 mutations in patients with optic atrophy (OA) and assess visual impairment.Retrospective molecular genetic and clinical study.Patients with OA followed at a national referral center specialized in genetic sensory diseases.Mutation screening in WFS1 was performed by Sanger sequencing. WFS1-positive patients were evaluated on visual acuity (VA) and retinal nerve fiber layer (RNFL) thickness using time-domain (TD) or spectral-domain (SD) optical coherence tomography (OCT). Statistical analysis was performed.Mutation identification, VA values, and RNFL thickness in sectors.Biallelic WFS1 mutations were found in 3 of 24 unrelated patients (15%) with autosomal recessive nonsyndromic optic atrophy (arNSOA) and in 8 patients with autosomal recessive Wolfram syndrome (arWS) associated with diabetes mellitus and OA. Heterozygous mutations were found in 4 of 20 unrelated patients (20%) with autosomal dominant OA. The 4 WFS1-mutated patients of this latter group with hearing loss were diagnosed with autosomal dominant Wolfram-like syndrome (adWLS). Most patients had VA decrease, with logarithm of the minimum angle of resolution (logMAR) values lower in arWS than in arNSOA (1.530 vs. 0.440; P= 0.026) or adWLS (0.240; P= 0.006) but not differing between arNSOA and adWLS (P= 0.879). All patients had decreased RNFL thickness that was worse in arWS than in arNSOA (SD OCT, 35.50 vs. 53.80 m; P= 0.018) or adWLS (TD-OCT, 45.84 vs. 59.33 m; P= 0.049). The greatest difference was found in the inferior bundle. Visual acuity was negatively correlated with RNFL thickness (r=-0.89; P= 0.003 in SD OCT and r=-0.75; P= 0.01 in TD-OCT).WFS1 is a gene causing arNSOA. Patients with this condition had significantly less visual impairment than those with arWS. Thus systematic screening of WFS1 must be performed in isolated, sporadic, or familial optic atrophies.


Heiner P.,Vision Eye Institute | Ligabue E.,Ophthalmic Center | Fan A.,Chinese University of Hong Kong | Lam D.,Chinese University of Hong Kong
Clinical Ophthalmology | Year: 2014

Purpose: To evaluate the safety and effectiveness of a single-piece hydrophobic acrylic intraocular lens (IOL) (enVista ® MX60; Bausch and Lomb Incorporated, Rochester, NY, USA) following implantation to correct aphakia subsequent to extracapsular cataract extraction in adults. Subjects and methods: This was an open-label, non-interventional, observational study conducted in 19 university and private-practice settings in Europe and the Asia-Pacific region to investigate clinical outcomes of the MX60 IOL in standard practice. Eligible subjects were at least 18 years of age and had undergone standard phacoemulsification and extracapsular cataract extraction with implantation of the MX60 IOL. The primary safety endpoint was the occurrence of adverse events, and the primary effectiveness endpoints included visual and refractive outcomes and stability, with data collected up to 2 years post-procedure. Results: In this multicenter study, pooled data of 255 eyes were collected and analyzed. Excellent visual and refractive outcomes and stability were demonstrated. At postoperative visit 4 (61-180 days postoperative), 62.2% of subjects achieved a Snellen best-corrected distance visual acuity (CDVA) of 20/20 (decimal 1.00), and 97.8% of subjects achieved a CDVA of 20/40 (decimal 0.50) or better. One eye (1.0%) underwent neodymium:yttrium aluminum garnet capsulotomy at 12 months post-procedure. No glistenings of any grade were reported for any subject at any visit. Adverse events were infrequent and were consistent with incidences generally reported with cataract surgery. Conclusion: This study, which enrolled all comers, provided evidence of the excellent safety and effectiveness of the MX60 IOL in standard practice. Favorable clinical outcomes included outstanding visual and refractive outcomes and stability. No glistenings were reported at any postoperative visit. © 2014 Arora et al.

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