Incidence and clinical significance of fovea plana in the French population with age-related cataract.

BACKGROUND: Fovea plana is defined as an immature macula diagnosed by OCT, showing the unusual shunt of the inner retinal layers into the fovea. The incidence of fovea plana in the adult population remains to be determined. The aim of this study was to determine the incidence of fovea plana in the French population with age-related cataract. METHODS: Consecutive patients who underwent cataract surgery in Rothschild Foundation Hospital, France, between January and March 2021, with preoperative analyzable OCT scans available, were retrospectively screened in order to determine the incidence of fovea plana in these population. Ophthalmological characteristics of patients were reported, and detailed. RESULT: Fovea plana was encountered in 20 out of 204 patients during the 3 months corresponding to an incidence of 9.8%. One of those patients had stage 2 fovea plana. CONCLUSION: Although fovea plana is defined as an immature macula, it is not rare in preoperative population. This macular aspect was not associated with poor visual acuity in our cohort.

Foveal hypoplasia in parents of patients with albinism.

BACKGROUND: Albinism is a group of genetic disorders characterized by general skin and retinal hypopigmentation. It is in most cases an autosomal recessive condition. Foveal hypoplasia (FH) is one of the main criteria for the diagnosis of albinism. The aim of this study was to analyze the macular profile of the parents of patients with albinism. METHODS: This study included a case series of 27 patients with albinism seen in Rothschild Foundation between April 2017 and February 2020. Spectral-domain optical coherence tomography (SD-OCT) and OCT angiography (OCT-A) were performed in every patient when possible and in every available parents. FH was graded according to Thomas' classification based on OCT. Next generation sequencing-based gene panel testing was performed in parents and children when a FH was detected on OCT in a parent. RESULTS: Twenty-seven patients with albinism were examined. Nine parents had FH based on the OCT B-scan (33%). In parents without FH based on the SD-OCT B-scan (67%), OCT-A showed a reduced avascular zone in the deep vascular plexus in 4 parents. Six parents carried variants that could explain their phenotype, including TYR R402Q hypomorphic alleles. CONCLUSION: This study showed the presence of FH in parents of patients with albinism, and aimed to genetically explain this phenotype.

Simultaneous perception of prosthetic and natural vision in AMD patients.

Loss of photoreceptors in atrophic age-related macular degeneration (AMD) results in severe visual impairment. Since the low-resolution peripheral vision is retained in such conditions, restoration of central vision should not jeopardize the surrounding healthy retina and allow for simultaneous use of the natural and prosthetic sight. This interim report, prespecified in the study protocol, presents the first clinical results with a photovoltaic substitute of the photoreceptors providing simultaneous use of the central prosthetic and peripheral natural vision in atrophic AMD. In this open-label single group feasibility trial (NCT03333954, recruitment completed), five patients with geographic atrophy have been implanted with a wireless 2 x 2 mm-wide 30 µm-thick device, having 378 pixels of 100 µm in size. All 5 patients achieved the primary outcome of the study by demonstrating the prosthetic visual perception in the former scotoma. The four patients with a subretinal placement of the chip demonstrated the secondary outcome: Landolt acuity of 1.17 ± 0.13 pixels, corresponding to the Snellen range of 20/460-20/565. With electronic magnification of up to a factor of 8, patients demonstrated prosthetic acuity in the range of 20/63-20/98. Under room lighting conditions, patients could simultaneously use prosthetic central vision and their remaining peripheral vision in the implanted eye and in the fellow eye.

[Efficacy and tolerance of Latanoprost given as a first intention in the treatment of primitive open angle glaucoma in African melanoderm]. / Efficacité et tolérance du Latanoprost donné en première intention dans le traitement du glaucome primitif a angle ouvert chez le mélanoderme africain.

OBJECTIVE: To assess the reduction in IOP and ocular symptoms in patients newly diagnosed with POAG and treated with latanoprost as monotherapy. PATIENTS AND METHOD: A multicentric, cross-sectional, descriptive study was conducted. We included adults newly diagnosed with POAG. All patients received one drop of preserved latanoprost 0.005% in each eye every night for 12 weeks. Changes in IOP and ophthalmic signs and symptoms were assessed during and at the end of treatment. RESULTS: A total of 524 patients were included, with a participation rate of 93% at 12 weeks. The mean age was 52.79±17.33 years, and the sex ratio M/F was 1.39. At inclusion, the mean IOP was 21.68±9.72mmHg. After 2 weeks of treatment, the mean IOP was 15.49±5.81mmHg, for a reduction of 28.55%. After 12 weeks of treatment, the mean IOP was 13.16±3.54mmHg, for a reduction of 39.30%. The main symptom recorded was a gritty foreign body sensation, the frequency of which was 4.72% at W2 and 2.45% at W12. The main sign was hyperemia (4.33% at W2 and 1.84% at W12). CONCLUSION: Latanoprost given as first-line monotherapy in POAG in blacks considerably reduces IOP. The incidence of side effects remains low; it is higher at the start of treatment.

[Variability of chloroquine and hydroxychloroquine retinopathy among various ethnicities]. / Différences ethniques parmi les patients souffrant de toxicité maculaire aux antipaludéens de synthèse.

INTRODUCTION: Current screening recommendations for chloroquine (CQ) and hydroxychloroquine (HCQ) retinopathy are based on central 10°C static perimetry and a high-resolution SD-OCT with a special attention to the inferior part of the macula where the toxicity usually starts by ellipsoid zone disruption. However, Melles and Marmor, have recently shown a great variability in the topography of the initial toxicity observed among various ethnicities, which is important to keep in mind so as not to miss early toxicity in certain subgroups of patients. METHODS: Review of the literature. RESULTS: Ethnic differences have been shown regarding the topography of the initial retinal toxicity of CQ and HCQ, particularly between Caucasian and Asian subjects. In Caucasians, the first signs of toxicity are more often localized in the inferior para-foveal area associated with a decrease in retinal sensitivity in the upper 10°C visual field. However, in Asian subjects, the first signs of toxicity appear more pericentral (still inferior) with an extramacular pattern that could be missed by the usual 10°C visual field screening. DISCUSSION/CONCLUSION: The pathophysiology of these ethnic differences is unknown and may be due to distinct genetic predisposition to CQ and HCQ toxicity. Screening strategies should be adjusted to the ethnicity and performed in Asian subjects with larger visual fields (30°C), along with SD-OCT, looking for ellipsoid disruption≥8°C from the fovea. The recognition of this pericentral topography and an adjusted screening protocol should avoid late diagnosis in Asians treated with CQ and HCQ.

[Update on recommendations for screening for hydroxychloroquine retinopathy]. / Mise à jour des recommandations sur la toxicité rétinienne des antipaludéens de synthèse.

INTRODUCTION: Recommendations for screening for chloroquine (CQ) and hydroxychloroquine (HCQ) retinopathy have recently been changed by the American Academy of Ophthalmology, taking into account new published data on toxicity prevalence, risk factors, location of onset in the retina and the efficacy of screening tests. METHODS: Literature review. RESULTS AND DISCUSSION: The risk of developing CQ or HCQ retinopathy depends on the daily dose and duration of treatment. At recommended doses, the risk is<1 % at 5 years, <2 % at 10years but increases to about 20 % after 20years of treatment. The maximum recommended daily dose is 5.0mg/kg for HCQ and 2.3mg/kg for CQ. The two main risk factors are the daily dose and duration of treatment. The presence of kidney failure and treatment with tamoxifen are also significant risk factors. A baseline examination should be performed at the initiation of treatment to rule out pre-existing maculopathy. The screening is then annual and starts from the 5th year of treatment. The two tests recommended for screening are the automated visual field and spectral domain OCT. Multifocal ERG and autofluorescence fundus imaging are only carried out secondarily to confirm the pathology.

[Classification of vitreomacular adhesion and macular holes]. / Classification des adhérences vitréomaculaires et trous maculaires.

BACKGROUND: Optical coherence tomography has significantly changed the approach to vitreomacular diseases, including macular holes (MH). OCT provides information on differential diagnoses (lamellar hole, pseudo-hole), the MH size, the status of the vitreous, and the status of the various retinal layers. The evolution of diagnostic tools and treatment justifies the need for an update of the current classification of vitreomacular diseases. METHOD: A group of retina specialists met several times to discuss the international classification, recently published by The International Vitreomacular Traction Study Group, focusing on vitreomacular adhesion (VMA), vitreomacular traction (VMT) and MH. It was compared to the classification currently used in France, based on the ophthalmoscopic system of Gass, then further delineated by Gaudric's OCT studies, in order to suggest a French adaptation to the international classification. RESULTS: An adapted classification for clinical use in France and in connection with the international classification is proposed. VMA are classified as an "associated" or "isolated" VMA respectively with or without macular disease. MH are distinguished as "primary" or "secondary" MH depending on whether the etiology is known or not, and classified as "small", "medium" and "large" depending on the size of the hole, and according to the presence or absence of VMT. Finally, VMT are described according to presence or absence of an epimacular membrane and according to the width of the adhesion.

[Intravitreal ranibizumab for management of choroidal neovascularization secondary to angioid streaks: a case report]. / Traitement par injection intravitréenne de ranibizumab de néovaisseaux choroïdiens compliquant des stries angioïdes : à propos d'un cas.

Angioid streaks are biomicroscopically observable manifestations that frequently lead to choroidal neovascularization. Traditional treatments used to include laser photocoagulation or photodynamic therapy. Over the past few years, anti-VEGF therapies have been used as an alternative treatment. The case of a 54-year-old patient who received anti-VEGF therapy (ranibizumab) for the treatment of choroidal neovascularization secondary to angioid streaks is reported. The patient received two injections that led to complete resolution of intraretinal fluid and reduction in lesion size. After 1 year of follow-up, the patient has presented no recurrence. This case illustrates the efficacy of intravitreal anti-VEGF therapy for choroidal neovascularization in angioid streaks. Further prospective studies on a larger number of patients should help establish the best treatment and follow-up strategies.

[Has surgery of the vitreous evolved?]. / La chirurgie du vitré évolue-t-elle ?

Three ports pars plana vitrectomy has been described 50 years ago. From indications to salvage the peripheral retina function, it has progressively shifted towards functional indications in macular pathologies, trying initially to stabilize and now to improve visual acuity. This has been achieved by improvement, not only of surgical material but also of microscopes, intraoperative rools such as perfluorocarbone liquids and more recently the use of vital dyes.

[Intravitreal bevacizumab treatment for choroidal neovascularization in Best's disease]. / Traitement par injection intravitréenne de bevacizumab de néovaisseaux choroïdiens compliquant une maladie de Best.

Best's disease is an autosomal-dominant macular dystrophy, which might lead to choroidal neovascularization. The case of a 9-year-old boy followed up for a Best's disease is hereby reported. The boy was referred to our service department for a decrease in visual acuity in his right eye. Ophthalmoscopic examination revealed a foveolar vitelliform lesion, complicated by a choroidal neovascularization. The patient received a single intravitreal bevacizumab injection, which led to a complete resolution of intraretinal fluid and a significant reduction in neovascularization size. Visual acuity also improved. This case underlines the efficiency efficacy of intravitreal anti-VEGF therapy for choroidal neovascularization in Best's disease.

[Surgical management of sub-retinal haemorrhage secondary to polypoidal choroidal vasculopathy]. / Prise en charge chirurgicale des hémorragies sous-rétiniennes secondaires à une vasculopathie polypoïdale.

We report the case of a 34-year-old black woman with acute and severe unilateral loss of sight related to idiopathic polypoidal choroidal vasculopathy responsible for a sub macular haemorrhage (1/10 on the Monoyer scale). The patient underwent a pars plana vitrectomy associated with a sub retinal administration of tissue plasminogen activator (100 µg) and a pneumatic displacement by gas (C2F6) with facedown positioning for 5 days. There were no intraoperative complications and the clot was lysed and totally displaced from the macula. There was no recurrence of the disease and the retinal epithelium detachment decreased progressively. The final visual acuity was 7/10. This case report illustrates the capacity and efficacy of this surgical procedure in the management of sub macular haemorrhage related to polypoidal choroidal vasculopathy. It provides effective displacement of the clot, limiting retinal damage induced by sub macular haemorrhage. Furthermore, it allows early treatment of the polypoidal aneurysm by laser or dynamic phototherapy and increases final visual acuity. Randomised studies are expected to determine the indication for this surgical procedure in the management of polypoidal choroidal vasculopathy and the possible association of laser, dynamic phototherapy, or anti-VEGF treatments.

[Susac syndrome: study of five cases]. / Syndrome de Susac: étude de cinq cas.

INTRODUCTION: Susac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss. METHODS: We report a series of five patients with Susac syndrome followed in our department from 1997 to 2007. RESULTS: There were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n=1), ophthalmological (n=1), auditory (n=1) and clinical triad (n=2). Neurologic symptoms included encephalopathy (n=2), headache (n=5), transient ischemic attacks (n=1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n=4], cylophosphamid [n=3], intravenous immunoglobulins [n=5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n=2), led to dramatic clinical improvement (n=5). CONCLUSION: Due to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.

[Brain hypoperfusion revealed by an ocular ischemic syndrome]. / Bas débit cérébral révélé par une ischémie oculaire.

We report the case of a 59-year-old female presenting an ocular ischemic syndrome caused by occlusion of the internal carotid artery. MRI showed multiple previous episodes of stroke. Therapy consisted in stopping the antihypertensive medication in order to stabilize the residual cerebral perfusion pressure and initiating statin and antiaggregant therapy.

[Acute posterior multifocal placoid pigment epitheliopathy, serpiginous and multifocal choroiditis: etiological and therapeutic management]. / Epithéliopathies en plaques, choroïdites serpigineuses et multifocales: analyse étiologique et prise en charge thérapeutique.

PURPOSE: To highlight the importance of an extensive medical work-up in serpiginous and multifocal choroiditis, and acute posterior multifocal placoid pigment epitheliopathy before therapeutic management. PATIENTS AND METHODS: Records of patients referred to our department, between January 2000 and January 2002, for the diagnostic and therapeutic management of choroiditis or acute posterior multifocal placoid pigment epitheliopathy were retrospectively reviewed. All patients had a complete ophthalmologic examination, fluorescein and infrared angiographies. An extensive work-up was performed in order to exclude an infectious etiology. RESULTS: Fourteen patients were included (six cases of serpiginous choroiditis, four cases of multifocal choroiditis, and four cases of APMPPE). The mean age was 42.1 years and the sex ratio was 9: 5. Six patients presented with a history of tuberculosis in the family or with a tuberculosis primary infection. Toxoplasmic retinochoroiditis was confirmed in one case by a positive PCR applied to the aqueous humor. In the serpiginous choroiditis group, two patients have been treated with antituberculous drugs, one of whom was initially resistant to immunosuppressive regimens. In the acute posterior multifocal placoid pigment epitheliopathy group, one patient was treated with antituberculous drugs and another received antibiotics. CONCLUSION: An infectious agent may be associated with this group of clinical presentations. All patients presenting with severe forms of ocular inflammation, who resist to corticosteroids or immunosuppressive regimens, must undergo an extensive infectious work-up in order to propose a specific treatment.

[Extensive toxoplasmic retinochoroiditis. Diagnostic and therapeutic management]. / Toxoplasmose oculaire extensive. Conduite diagnostique et thérapeutique.

INTRODUCTION: To assess the diagnostic and therapeutic management of extensive toxoplasmic retinochoroiditis. PATIENTS AND METHODS: The files of all patients referred between December 1999 and December 2001 for the management of a severe, potentially sight-threatening toxoplasmic retinochoroiditis were retrospectively analyzed. The therapeutic strategy and the progression of intraocular inflammation are reported. RESULTS: Thirteen eyes of seven patients were finally included in the study. The sex ratio (F/M) and the mean age were respectively 4/3 and 44.5 years. Most of the patients were immunocompromised. Both eyes were initially affected in five cases. The diagnosis was confirmed by polymerase chain reaction (PCR) after anterior chamber paracentesis in six cases. Retinal detachment was observed in three cases, initially or during follow-up. All patients were treated with a combination of sulfadiazine and pyrimethamine, but azithromycin was necessary in two cases. Clindamycin was used in two cases of allergy to sulfadiazine. Corticosteroids were associated in five cases. For all patients, infection and inflammation were finally controlled. The visual acuity improved more than two lines in four eyes and remained stable in seven other eyes. DISCUSSION: Clinical diagnosis is still a challenge in severe cases of extensive toxoplasmic retinochoroiditis. PCR is helpful in identifying Toxoplasma gondii DNA. A systemic immunosuppression is frequently associated with a positive PCR. Treatment is based on a standard antiparasitic association and steroids must be discussed for each case according to the intensity of inflammation and the degree of immunosuppression. CONCLUSION: Extensive ocular toxoplasmosis is a serious condition. The final prognosis depends on the location of the necrotic lesions, rapid diagnosis, and efficient treatment.

Surgical removal of choroidal neovascular membranes after laser photocoagulation for diabetic maculopathy.

PURPOSE: Choroidal neovascularisation (CNV) occurs rarely following laser photocoagulation for macular oedema in diabetic retinopathy, and its management is not well established. We report the clinical course and visual outcomes in a series of patients who underwent surgical extraction of the CNV membrane. METHODS: A retrospective review of 4 cases was carried out. RESULTS: Two women and 2 men, mean age 59.5 (range 58-62) years, were reviewed. The CNV developed 2-24 (mean 11) months after laser coagulation and resulted in decreased visual acuity to between 6/60 and HM. All underwent pars plana vitrectomy, extraction of the CNV membrane and fluid-air exchange. Follow-up ranged between 9 and 48 months. In 2 patients, the vision improved by 4 and 1 Snellen lines respectively and remained stable, in 1 patient it improved by 1 line initially but then regressed to CF, and in 1 patient it remained unchanged. Recurrence of CNV occurred in only 1 patient. Histological characteristics were those of CNV without evidence of photoreceptors. CONCLUSIONS: This study shows that surgical removal of post-laser CNV is technically feasible despite the previous laser scars and may have beneficial outcome. This surgical approach may provide a therapeutic option in such patients.

TPA-assisted vitrectomy for proliferative diabetic retinopathy: results of a double-masked, multicenter trial.

OBJECTIVE: Some complications of vitrectomy are related to adherence of the vitreous body to the retina. We studied whether these complications could be decreased by injecting a proteolytic enzyme, tissue plasminogen activator (TPA), at the beginning of surgery to aid separation of the vitreous from the retina. METHODS: Fifty-six patients receiving surgery for complications of proliferative diabetic retinopathy were divided into two groups in this prospective, randomized, double-blind study. Group I patients received 25 microg of intravitreal TPA in buffered salt solution (BSS) 15 minutes before vitrectomy. Group II received BSS alone. Postoperative follow-up lasted up to 3 months. The major criteria for comparison were the number of perioperative iatrogenic tears, the gain in visual acuity, and the reattachment rate of tractional retinal detachments. RESULTS: No difference was found between the two groups for the principal indicators or for complications. CONCLUSION: In proliferative diabetic retinopathy, the use of 25 microg of TPA by intravitreal injection 15 minutes before vitrectomy does not improve the results. No specific complications of the method were noted. The failure can be attributed to a too-short delay between TPA injection and beginning of surgery, an insufficient dose, or an insufficient quantity of plasminogen in the vitreous at the beginning of the intervention.