Sara Touhami

Visual Acuity in Birdshot Retinochoroidopathy Evaluation

PURPOSE To determine the statistical correlation between visual acuity (VA) and various quantitative parameters relevant to birdshot retinochoroidopathy (BRC) evaluation. DESIGN Hospital-based retrospective observational study. METHODS setting: Institutional. STUDY POPULATION Consecutive HLA29+ BRC patients were included between May and August 2013 at a single tertiary center (Pitié-Salpétrière Hospital, Paris). OBSERVATION PROCEDURES Demographic data and quantitative parameters relevant to BRC at baseline were collected: VA, degree of anterior and posterior inflammatory reaction, foveal thickness measured by optical coherence tomography (OCT), Arden ratio, and electrooculography (EOG) light peak. MAIN OUTCOME MEASURES Correlation between VA and the other parameters of the ipsilateral and fellow eye was performed using Spearman rank correlation coefficients. RESULTS Fifty-five patients were included. Mean VA was 6/9.5 in the right eye (OD) and 6/12 in the left eye (OS). Mean foveal thickness was 240 μm OD (range: 112-606) and 251 μm OS (range: 85-662). Mean Arden ratio was 159% OD and 160% OS. EOG light peak was 714 mV OD (range: 316-1379) and 746 mV OS (range: 272-1652). VA of a given eye was moderately correlated with VA of the contralateral eye (r = 0.4). On the contrary, all other parameters showed a strong correlation between both eyes (all r > 0.7, P < .01). Overall, none of the studied parameters was correlated with its VA (all r < 0.5). CONCLUSION In BRC, visual acuity alone does not seem to fully reflect the disease severity in terms of clinical or ancillary quantitative findings at baseline.

Activated monocytes resist elimination by retinal pigment epithelium and downregulate their OTX2 expression via TNF‐α

Orthodenticle homeobox 2 (OTX2) controls essential, homeostatic retinal pigment epithelial (RPE) genes in the adult. Using cocultures of human CD14+ blood monocytes (Mos) and primary porcine RPE cells and a fully humanized system using human‐induced pluripotent stem cell‐derived RPE cells, we show that activated Mos markedly inhibit RPEOTX2 expression and resist elimination in contact with the immunosuppressive RPE. Mechanistically, we demonstrate that TNF‐α, secreted from activated Mos, mediates the downregulation of OTX2 and essential RPE genes of the visual cycle among others. Our data show how subretinal, chronic inflammation and in particular TNF‐α can affect RPE function, which might contribute to the visual dysfunctions in diseases such as age‐related macular degeneration (AMD) where subretinal macrophages are observed. Our findings provide important mechanistic insights into the regulation of OTX2 under inflammatory conditions. Therapeutic restoration of OTX2 expression might help revive RPE and visual function in retinal diseases such as AMD.

Everolimus-induced posterior reversible encephalopathy syndrome and bilateral optic neuropathy after kidney transplantation.

Transplant 2011; 30: 1320. 10. Manez R, Kelly RH, Kobayashi M, et al. Immunoglobulin G lymphocytotoxic antibodies in clinical liver transplantation: studies toward further defining their significance. Hepatology 1995; 21: 1345. 11. Leca N, Warner P, Bakthavatsalam R, et al. Outcomes of simultaneous liver and kidney transplantation in relation to a high level of preformed donor-specific antibodies. Transplantation 2013; 96: 914. 12. Fung J, Griffin M, Duquesnoy R, et al. Successful sequential liver-kidney transplantation in a patient with performed lymphocytotoxic antibodies. Transplant Proc 1987; 19(1 Pt 1): 767. 13. lye MW, Duffy BF, Phelan DL, et al. Protective effects of liver transplantation on a simultaneously transplanted kidney in a highly sensitized patient. Transplantation 1990; 50: 1051. 14. Olausson M, Mjornstedt L, Norden G, et al. Successful combined partial auxiliary liver and kidney transplantation in highly sensitized cross-match positive recipients. Am J Transplant 2007; 7: 130. 15. Topilsky Y, Raichlin E, Hasin T, et al. Combined heart and liver transplant attenuates cardiac allograft vasculopathy compared with isolated heart transplantation. Transplantation 2013; 95: 859. 16. Daly RC, Topilsky Y, Joyce L, et al. Combined heart and liver transplantation: protection of the cardiac graft from antibody rejection by initial liver implantation. Transplantation 2013; 95: e2. 17. Couetil JP, Houssin DP, Soubrane O, et al. Combined lung and liver transplantation in patients with cystic fibrosis. A 4 1/2-year experience. J Thorac Cardiovasc Surg 1995; 110: 1415.

Ophthalmologic impairment during adulthood in central congenital hypoventilation syndrome: a longitudinal cohort analysis of nine patients.

Abstract Background: Congenital central hypoventilation syndrome (CCHS) is a rare dysgenetic form of neurocristopathy associated with dysfunction of the autonomic nervous system. Ophthalmologic abnormalities are reported in CCHS children, and range from pupillary and iris abnormalities to ptosis, strabismus and convergence deficiency. Since earlier CCHS diagnosis and multidisciplinary management, combined with improved ventilatory support techniques, the lifespan of children with CCHS has been considerably lengthened. The oldest of them have now reached adult age and we report in this study the results of their ophthalmologic examination. Materials and methods: Nine CCHS adult patients were prospectively included during a 14-month period. Each patient underwent complete ophthalmologic examination, static pupillometry with scotopic and photopic pupillary diameter (PD) measures, Humphrey 24-2 visual field analysis, macular OCT and complete orthoptic assessment including a Hess-Lancaster test. Results: Ophthalmologic abnormalities were found in six of the nine patients (66%). The main features were strabismus in six patients (66%). Four patients (44%) displayed abnormal pupillary function, with a decrease in average scotopic PD (3.8 ± 1.4 mm), average photopic PD (3.5 ± 1.2 mm), and average percentage of pupillary constriction (7.6 ± 8.5%). Three patients (33%) exhibited iris abnormalities such as iris atrophy, smooth iris surface and atrophic sphincter. Conclusion: This study allowed the description of ophthalmologic abnormalities occurring in CCHS in a series of adult patients, thus improving current knowledge of the disease. The prevalence of pupillary and iris lesions were lower than those observed in a series of children, suggesting that they could be considered as systemic disease severity markers.

Optical coherence tomography and confocal microscopy aspects of a Schnyder's corneal dystrophy case

Schnyder’s corneal dystrophy (SCD) is a rare autosomal dominant bilateral condition that results in the deposition of symmetric cholesterol and phopholipids in the anterior corneal stroma in an annular, discoid fashion [1]. SCD is a corneal disease; however systemic associations have been reported such as hypercholesterolemia. Although known as Schnyder’s crystalline corneal dystrophy, crystalline deposits occur in only 54% of the cases [2] and the dystrophic material was shown to be mainly made of phospholipids [3,4]. On slit-lamp, although the clinical presentation appears to be varied [1,2], the typical aspect is

Apports du ranibizumab dans le traitement des télangiectasies maculaires de type 2 non néovascularisées

Les télangiectasies maculaires idiopathiques (TMI) se définissent comme des altérations uniou bilatérales des capillaires juxtafovéolaires, qui deviennent dilatés, tortueux et anormalement perméables. Elles affectent le plus souvent des sujets âgés de 40 à 60 ans et peuvent se compliquer d’œdème maculaire cystoïde, de néovascularisation choroïdienne et d’atrophie maculaire. Plusieurs classifications ont été proposées [1] ; le traitement des formes les plus classiques est bien codifié : injection d’inhibiteurs du facteur de croissance vasculaire (anti-VEGF) [2], cependant, il n’existe pas de consensus thérapeutique pour les formes non compliquées de TMI de type 2. En particulier, la place des anti-VEGF reste à préciser. Nous rapportons un cas de TMI de type 2 non néovascularisées traitées avec succès par injection intra-vitréenne d’anti-VEGF.