Ocular findings in Brazilian identical twins with Cohen syndrome: case report

A case of identical male twins with Cohen syndrome who present multiple ophthalmic findings is reported. The patients were identical 16 year-old twin boys who showed down slanting eyelids, mild ptosis, high-grade myopia, small cortical lens opacities, posterior subcapsular cataracts, myotic and corectopic pupils with poor dilation due to focal iris atrophy and retinochoroidal dystrophy. Ophthalmologists must be aware of the ocular and systemic findings of Cohen syndrome in the evaluation of young patients with mental retardation and visual impairment.

Ocular masquerade syndrome associated with extranodal nasal natural killer/T-cell lymphoma: case report

A 33-year-old woman complained of unilateral eyelid edema and blurred vision. Initial ophthalmic examination disclosed anterior chamber reaction with keratic precipitates on the cornea, without posterior abnormalities. Anterior uveitis was treated. Despite that, patient showed rapidly progressive unilateral vision loss with optic nerve swelling. Systemic workup was inconclusive, as well as cranial magnetic resonance imaging and cerebrospinal fluid examination. Based on the hypothesis of optic neuritis, intravenous methylprednisolone pulse was performed with no success. During the following days, the patient presented pericardial effusion and cardiac tamponade, progressing to death. Necropsy was performed and diagnosis of extranodal natural killers/T-cell lymphoma, nasal type with ocular involvement was confirmed by immunohistochemistry.

Monocular vision and increased distance reducing the effects of visual manipulation on body sway

The goal of this study was to examine the coupling between visual information and body sway with binocular and monocular vision at two distances from the front wall of a moving room. Ten participants stood as still as possible inside of a moving room facing the front wall in conditions that combined room movement with monocular/binocular vision and distance from the front wall (75 and 150cm). Visual information effect on body sway decreased with monocular vision and with increased distance from the front wall. In addition, the combination of monocular vision with the farther distance resulted in the smallest body sway response to the driving stimulus provided by the moving room. These results suggest that binocularvision near the front wall provides visual information of a better quality than the monocular vision far from the front wall. We discuss the results with respect to two modes of visual detection of body sway: ocular and extraocular. (C) 2009 Elsevier Ireland Ltd. All rights reserved.

Vascular leakage stimulates phenotype alteration in ocular cells, contributing to the pathology of proliferative vitreoretinopathy

Plasma leaking from damaged retinal blood vessels can have a significant impact on the pathologies of the posterior segment of the eye. Inflammation in the eye and metabolic change resulting from diabetes mellitus causes vascular leakage with alteration of the phenotype of retinal pigment epithelial (RPE) cells and fibrocytes, resulting in changes in cell function. Phenotypically altered cells then significantly contribute to the pathogenesis of retinopathies by being incorporated into tractional membranes in the vitreous, where they secrete matrix molecules, such as fibronectin, and express altered cell surface antigens. We hypothesize that there is a direct relationship between the leaking of plasma and the proliferation and phenotypic change of RPE cells and fibroblasts, thus exacerbating the pathology of retinal disease. If the hypothesis is correct, control of vascular leakage becomes an important target of therapy in proliferative vitreoretinopathy.

Transmission of ocular media in labrid fishes

Wrasses (Labridae) are the second largest family of fishes on the: Great Barrier Reef (after the Gobiidae) and, in terms of morphology and lifestyle, one of the most diverse. They occupy all zones of the reef from the very shadow reef flats to deep slopes, feeding on a variety of fauna. Many wrasses also have elaborately patterned bodies and reflect a range of colours from ultraviolet (UV) to far red. As a first step to investigating the visual system of these fishes we measured the transmission properties of the ocular media of 36 species from the Great Barrier Reef, Australia, and Hawaii, California and the Florida Keys, USA. Transmission measurements were made of whole eyes with a window cut into the back, and also of isolated lenses and corneas. Based on the transmission properties of the corneas the species could be split into two distinct groups within which the exact wavelength of the cut-off was variable. One group had visibly yellow corneas, while the corneas of the other group appeared clear to human observers. Five species had ocular media that transmitted wavelengths below 400 nm, making a perception of UV wavelengths for those species possible. Possible functional roles for the different filler types are discussed.

Vision in the peafowl (Aves : Pavo cristatus)

The visual sense of the Indian blue-shouldered peafowl Pavo cristatus was investigated with respect to the spectral absorption characteristics of the retinal photoreceptors, the spectral transmittance of the ocular media and the topographic distribution of cells in the retinal ganglion cell layer. Microspectrophotometry revealed a single class of rod, four spectrally distinct types of single cone and a single class of double cone. In the case of the single cone types, which contained visual pigments with wavelengths of maximum absorbance (lambda(max)) at 424, 458, 505 and 567 nm, spectral filtering by the ocular media and the different cone oil droplets with which each visual pigment is associated gives predicted peak spectral sensitivities of 432, 477, 537 and 605 nm, respectively. Topographic analysis of retinal ganglion cell distribution revealed a large central area of increased cell density (at peak, 35,609 cells mm(-2)) with a poorly defined visual streak extending nasally. The peafowl has a calculated maximum spatial resolution (visual acuity) in the lateral visual field of 20.6 cycles degrees(-1). These properties of the peafowl eye are discussed with respect to its visual ecology and are compared with those of other closely related species.

Visual Biology of Hawaiian Coral Reef Fishes. III. Environmental Light and an Integrated Approach to the Ecology of Reef Fish Vision

In the previous two papers in this three-part series, we have examined visual pigments, ocular media transmission, and colors of the coral reef fish of Hawaii. This paper first details aspects of the light field and background colors at the microhabitat level on Hawaiian reefs and does so from the perspective and scale of fish living on the reef. Second, information from all three papers is combined in an attempt to examine trends in the visual ecology of reef inhabitants. Our goal is to begin to see fish the way they appear to other fish. Observations resulting from the combination of results in all three papers include the following. Yellow and blue colors on their own are strikingly well matched to backgrounds on the reef such as coral and bodies of horizontally viewed water. These colors, therefore, depending on context, may be important in camouflage as well as conspicuousness. The spectral characteristics of fish colors are correlated to the known spectral sensitivities in reef fish single cones and are tuned for maximum signal reliability when viewed against known backgrounds. The optimal positions of spectral sensitivity in a modeled dichromatic visual system are generally close to the sensitivities known for reef fish. Models also predict that both UV-sensitive and red-sensitive cone types are advantageous for a variety of tasks. UV-sensitive cones are known in some reef fish, red-sensitive cones have yet to be found. Labroid colors, which appear green or blue to us, may he matched to the far-red component of chlorophyll reflectance for camouflage. Red cave/hole dwelling reef fish are relatively poorly matched to the background they are often viewed against but this may be visually irrelevant. The model predicts that the task of distinguishing green algae from coral is optimized with a relatively long wavelength visual pigment pair. Herbivorous grazers whose visual pigments are known possess the longest sensitivities so far found. Labroid complex colors are highly contrasting complementary colors close up but combine, because of the spatial addition, which results from low visual resolution, at distance, to match background water colors remarkably well. Therefore, they are effective for simultaneous communication and camouflage.

Visual Biology of Hawaiian Coral Reef Fishes. I. Ocular Transmission and Visual Pigments

The visual biology of Hawaiian reef fishes was explored by examining their eyes for spectral sensitivity of their visual pigments and for transmission of light through the ocular media to the retina. The spectral absorption curves for the visual pigments of 38 species of Hawaiian fish were recorded using microspectrophotometry. The peak absorption wavelength (lambda(max)) of the rods varied from 477-502 nm and the lambda(max) of individual species conformed closely to values for the same species previously reported using a whole retina extraction procedure. The visual pigments of single cone photoreceptors were categorized, dependent on their lambda(max)-values, as ultraviolet (347-376 nm), violet (398-431 nm) or blue (439-498 nm) sensitive cones. Eight species possessed ultraviolet-sensitive cones and 14 species violet-sensitive cones. Thus, 47% of the species examined displayed photosensitivity to the short-wavelength region of the spectrum. Both identical and nonidentical paired and double cones were found with blue sensitivity or green absorption peaks (> 500 nm). Spectrophotometry of the lens, cornea, and humors for 195 species from 49 families found that the spectral composition of the light transmitted to the retina was most often limited by the lens (73% of species examined). Except for two unusual species with humor-limited eyes, Acanthocybium solandri (Scombridae) and the priacanthid fish, Heteropriacanthus cruentatus, the remainder had corneal-limited eyes. The wavelength at which 50% of the light was blocked (T50) was classified according to a system modified from Douglas and McGuigan (1989) as Type I, T50 < = 355 nm, (32 species); Type IIa, 355 < T50 < = 380 nm (30 species); Type IIb, 380 < T50 405 nm (84 species). Possession of UV-transmitting ocular media follows both taxonomic and functional lines and, if the ecology of the species is considered, is correlated with the short-wavelength visual pigments found in the species. Three types of short-wavelength vision in fishes are hypothesized: UV-sensitive, UV-specialized, and violet-specialized. UV-sensitive eyes lack UV blockers (Type I and IIa) and can sense UV light with the secondary absorption peak or beta peak of their longer wavelength visual pigments but do not possess specialized UV receptor cells and, therefore, probably lack UV hue discrimination. UV-specialized eyes allow transmission of UV light to the retina (Type I and IIa) and also possess UV-sensitive cone receptors with peak absorption between 300 and 400 nm. Given the appropriate perceptual mechanisms, these species could possess true UV-color vision and hue discrimination. Violet-specialized eyes extend into Type IIb eyes and possess violet-sensitive cone cells. UV-sensitive eyes are found throughout the fishes from at least two species of sharks to modern bony fishes. Eyes with specialized short-wavelength sensitivity are common in tropical reef fishes and must be taken into consideration when performing research involving the visual perception systems of these fishes. Because most glass and plastics are UV-opaque, great care must be taken to ensure that aquarium dividers, specimen holding containers, etc., are UV-transparent or at least to report the types of materials in use.

Massive ocular hemorrhage resulting in blindness in a patient with the sickle cell trait who developed leptospirosis. Case report

This case report describes the findings of a 18 year-old black male from Bahia, a Northeastern state in Brazil, with the sickle cell trait, who developed bilateral hyphema and vitreous hemorrhage with blindness in the course of leptospirosis. The patient started to complain of blurred vision four days after the start of fever and muscular pain and approximately twelve hours after the introduction of penicillin. The severity of the leptospirosis in conjunction with sickle cell trait was considered to be the most likely explanation for this ocular complication.

OCULAR SYPHILIS IN A KIDNEY TRANSPLANT RECIPIENT

We present a case of ocular syphilis after a renal transplantation involving progressive vision loss without clinically identifiable ocular disease. Electroretinography showed signs of ischemia, especially in the internal retina. A serological test was positive for syphilis. Lumbar puncture revealed lymphocytic meningitis and a positive serologic test for syphilis in the cerebrospinal fluid. The patient was treated with penicillin, and had a quick vision improvement. In the case of transplant recipients, clinicians should always consider the diagnosis of ocular syphilis in cases with unexplained visual acuity decrement, as this condition may cause serious complications if not treated.

Mineralocorticoid receptor is involved in rat and human ocular chorioretinopathy.

Central serous chorioretinopathy (CSCR) is a vision-threatening eye disease with no validated treatment and unknown pathogeny. In CSCR, dilation and leakage of choroid vessels underneath the retina cause subretinal fluid accumulation and retinal detachment. Because glucocorticoids induce and aggravate CSCR and are known to bind to the mineralocorticoid receptor (MR), CSCR may be related to inappropriate MR activation. Our aim was to assess the effect of MR activation on rat choroidal vasculature and translate the results to CSCR patients. Intravitreous injection of the glucocorticoid corticosterone in rat eyes induced choroidal enlargement. Aldosterone, a specific MR activator, elicited the same effect, producing choroid vessel dilation -and leakage. We identified an underlying mechanism of this effect: aldosterone upregulated the endothelial vasodilatory K channel KCa2.3. Its blockade prevented aldosterone-induced thickening. To translate these findings, we treated 2 patients with chronic nonresolved CSCR with oral eplerenone, a specific MR antagonist, for 5 weeks, and observed impressive and rapid resolution of retinal detachment and choroidal vasodilation as well as improved visual acuity. The benefit was maintained 5 months after eplerenone withdrawal. Our results identify MR signaling as a pathway controlling choroidal vascular bed relaxation and provide a pathogenic link with human CSCR, which suggests that blockade of MR could be used therapeutically to reverse choroid vasculopathy.

Anti-vascular endothelial growth factor acts on retinal microglia/macrophage activation in a rat model of ocular inflammation.

PURPOSE: To evaluate whether anti-vascular endothelial growth factor (VEGF) neutralizing antibodies injected in the vitreous of rat eyes influence retinal microglia and macrophage activation. To dissociate the effect of anti-VEGF on microglia and macrophages subsequent to its antiangiogenic effect, we chose a model of acute intraocular inflammation. METHODS: Lewis rats were challenged with systemic lipopolysaccharide (LPS) injection and concomitantly received 5 µl of rat anti-VEGF-neutralizing antibody (1.5 mg/ml) in the vitreous. Rat immunoglobulin G (IgG) isotype was used as the control. The effect of anti-VEGF was evaluated at 24 and 48 h clinically (uveitis scores), biologically (cytokine multiplex analysis in ocular media), and histologically (inflammatory cell counts on eye sections). Microglia and macrophages were immunodetected with ionized calcium-binding adaptor molecule 1 (IBA1) staining and counted based on their differential shapes (round amoeboid or ramified dendritiform) on sections and flatmounted retinas using confocal imaging and automatic quantification. Activation of microglia was also evaluated with inducible nitric oxide synthase (iNOS) and IBA1 coimmunostaining. Coimmunolocalization of VEGF receptor 1 and 2 (VEGF-R1 and R2) with IBA1 was performed on eye sections with or without anti-VEGF treatment. RESULTS: Neutralizing rat anti-VEGF antibodies significantly decreased ocular VEGF levels but did not decrease the endotoxin-induced uveitis (EIU) clinical score or the number of infiltrating cells and cytokines in ocular media (interleukin [IL]-1β, IL-6, tumor necrosis factor [TNF]-α, and monocyte chemoattractant protein [MCP]-1). Eyes treated with anti-VEGF showed a significantly decreased number of activated microglia and macrophages in the retina and the choroid and decreased iNOS-positive microglia. IBA1-positive cells expressed VEGF-R1 and R2 in the inflamed retina. CONCLUSIONS: Microglia and macrophages expressed VEGF receptors, and intravitreous anti-VEGF influenced the microglia and macrophage activation state. Taking into account that anti-VEGF drugs are repeatedly injected in the vitreous of patients with retinal diseases, part of their effects could result from unsuspected modulation of the microglia activation state. This should be further studied in other ocular pathogenic conditions and human pathology.

Rôle des cellules souches épithéliales de la cornée et de leur micro-environnement dans le traitement des destructions de la surface oculaire [Darwin or Lamarck? Understanding the ocular surface and its normal or abnormal differentiation in order to cure ocular surface destruction with corneal opacification].

According to the World Health Organization, 5.1% of blindnesses or visual impairments are related to corneal opacification. Cornea is a transparent tissue placed in front of the color of the eye. Its transparency is mandatory for vision. The ocular surface is a functional unit including the cornea and all the elements involved in maintaining its transparency i.e., the eyelids, the conjunctiva, the lymphoid tissue of the conjunctiva, the limbus, the lacrymal glands and the tear film. The destruction of the ocular surface is a disease caused by : traumatisms, infections, chronic inflammations, cancers, toxics, unknown causes or congenital abnormalities. The treatment of the ocular surface destruction requires a global strategy including all the elements that are involved in its physiology. The microenvironnement of the ocular surface must first be restored, i.e., the lids, the conjunctiva, the limbus and the structures that secrete the different layers of the tear film. In a second step, the transparency of the cornea can be reconstructed. A corneal graft performed in a healthy ocular surface microenvironnement will have a better survival rate. To achieve these goals, a thorough understanding of the renewal of the epitheliums and the role of the epithelial stem cells are mandatory.

Analysis of significant factors influencing visual acuity in ocular syphilis.

BACKGROUND: The aim of this study is to determine whether statistical associations can be demonstrated in ocular syphilis between baseline clinical and laboratory parameters with visual acuity at presentation and with any change in visual acuity after treatment. METHODS: Charts of 26 patients (42 eyes) with ocular syphilis presenting to the Uveitis clinic of the Jules-Gonin Eye Hospital were reviewed. A baseline cross-sectional analysis was performed in order to identify any association between visual acuity at presentation and demographic, clinical or laboratory parameters. After treatment, any analogy between these parameters and a change in visual acuity was subsequently assessed in a series of univariate comparisons. RESULTS: The following factors were associated with worse initial visual acuity: severity of visual field impairment at presentation (p=0.012), macular oedema (p=0.004) and optic neuropathy (p=0.031). There was a borderline association with the presence of vasculitis on fluroangiography (p=0.072). Improvement in best corrected visual acuity after treatment was significantly associated with the presence of vasculitis on fluroangiography (p=0.005), neurosyphilis, according to lumbar puncture findings (p=0.037) and marginally with anterior uveitis (p=0.070). Inflammation relapse was associated with the coexistence of pain as presenting sign (p<0.001) and with a longer duration of symptoms prior to the initial visit (p=0.023). CONCLUSIONS: Severe ocular inflammation associated with vasculitis, vitritis or anterior uveitis in ocular syphilis would appear to be a reversible phenomenon that responds well to appropriate antibiotic treatment, resulting in improvement in visual acuity. Prompt treatment enables a good visual prognosis, while any delay in therapy increases the risk of subsequent relapse.