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PubMed2005

In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa.

Genetic and environmental factors modify the severity of human neurodegenerations. Retinal degenerations caused by rhodopsin gene mutations show severity differences within and between families and even within regions of the same eye. Environmental light is thought to contribute to this variation. In the naturally occurring dog model of the human disorder, we found that modest light levels, as used in routine clinical practice, dramatically accelerated the neurodegeneration. Dynamics of acute retinal injury (consisting of abnormal intraretinal light scattering) were visualized in vivo in real time with high-resolution optical imaging. Long term consequences included fast or slow retinal degeneration or repair of injury depending on the dose of light exposure. These experiments provide a platform to study mechanisms of neuronal injury, repair, compensation, and degeneration. The data also argue for a gene-specific clinical trial of light reduction in human rhodopsin disease.

Animals↗

PubMed2004

Theoretical estimation of retinal oxygenation during retinal artery occlusion.

The aim of the present work was to simulate the oxygenation of the whole retina under normal conditions as well as during retinal ischemia. A differential equation describing how oxygen is transported from blood to tissue, diffuses through the tissue and is consumed according to Michaelis-Menten kinetics was constructed. The outer retina was divided into three regions of which one was set to have consumption. The inner retina was considered as one uniform region with respect to maximal rate of oxygen consumption and blood flow. The results suggest that extreme hyperoxia would be needed to make the choroid capable of supplying the whole retina during total retinal artery occlusion and moreover confirm that light might to some extent be beneficial. As supplying 100% oxygen by nose cannula or common oxygen mask can hardly increase the arterial oxygen tension to the levels needed to rescue the whole retina, the effects of oxygen treatment of total retinal artery occlusion are expected to be modest, both in darkness and light, unless a non-rebreather face mask system is used.

Animals↗

PubMedDate not supplied

Atypical retinitis proliferans, retinal telangiectasis, and vitreous hemorrhage in a patient with tuberous sclerosis.

This report describes an unusual case of recurrent vitreous hemorrhage and atypical retinal neovascularization in a patient with tuberous sclerosis. During three years of observation, the patient also developed retinal telangiectasis with macular edema and lipid exudation. Although the patient did not have an obvious astrocytic hamartoma, a diffuse, flat retinal hamartoma within the nerve fiber layer was suspected.

Adult↗

PubMed1993

Trauma-induced retinal detachment associated with giant retinal tears.

Giant retinal tears may arise spontaneously, but approximately 25% occur in association with ocular trauma. The clinical findings and results of surgical management in 38 cases of traumatic giant retinal tear seen at Moorfields Eye Hospital in London during a 10-year period are presented. Patients were young (mean age = 29 years) and mostly men (n = 36; 95%). Trauma was penetrating in 14 eyes (37%) and nonpenetrating in 24 (63%). Initial surgical management consisted of pars plana vitrectomy and fluid-silicone oil exchange in the majority of cases. Lensectomy was performed for opacity or dislocation in 23 (61%) eyes. Reattachment was achieved in 34 (89%) eyes 12 months after surgery. Most of the surgical failures occurred in eyes with penetrating trauma. Raised intraocular pressure was an associated problem that required treatment in 12 (32%) eyes. Visual acuity at final follow-up examination ranged from 6/6 to no perception of light (NPL; mean = 6/36). These results compare favorably with published figures for the treatment of spontaneous giant retinal tears.

Adolescent↗

PubMed1999

Removal of silicone oil with vision improvement after rhegmatogenous retinal detachment following CMV retinitis in patients with AIDS.

PURPOSE: To report that silicone oil may be safely removed from immuno-recovered patients with acquired immunodeficiency syndrome (AIDS) after instillation for cytomegalovirus (CMV)-related rhegmatogenous retinal detachment. METHOD: We report two patients with CMV-related retinal detachment who had previously been treated with vitrectomy and silicone oil. RESULTS: Six months after removal of silicone oil, the retina remained attached in both patients. Without specific anti-CMV therapy, there was no relapse of CMV retinitis while patients were undergoing highly active antiretroviral therapy. Best-corrected visual acuity improved in both patients. CONCLUSION: It appears to be possible to remove silicone oil safely from patients with AIDS who show immune recovery, thus avoiding side effects of long-standing silicone oil and increasing quality of life. After silicone oil removal, visual acuity was improved.

AIDS-Related Opportunistic Infections↗

PubMed2006

Assessment of retinal function in patients with HIV without infectious retinitis by multifocal electroretinogram and automated perimetry.

PURPOSE: To determine if multifocal electroretinogram (mfERG) testing shows abnormalities that correspond to perimetric defects in HIV positive patients without infectious retinitis. METHODS: We studied three groups of patients: HIV negative controls, HIV high CD4 nadir patients (lowest CD4 T cell count is over 100) and low CD4 nadir patients (below 100 for over 6 months). Twenty-six HIV positive eyes and 16 HIV negative control eyes were studied by mfERG. A subset of 10 eyes also underwent computerized perimetry for comparison. We analyzed mfERG by hexagons as well as by quadrants and rings. RESULTS: Of 103 hexagon locations there was no significant difference in the amplitudes P1 and N1 (nV/degree) between the three studied groups (p>0.05), similarly, the latencies were not different (p>0.05). All eyes with significant visual field defects at the 0.01 and 0.005 level (Humphrey pattern deviation; 24-2) were compared to mfERG amplitudes and latencies at those locations-there were no corresponding defects in mfERG data (p>0.2). CONCLUSION: In the era of HAART there are still demonstrable visual field defects and other evidence of damage to the retinal nerve fiber layer in HIV patients. Our mfERG studies show that the damage appears to affect the inner retina, the outer retina is spared. Further studies of inner retinal structure and function are indicated to elucidate this process.

Adult↗

PubMed1994

Diffuse chronic retinal pigment epitheliopathy and exudative retinal detachment.

Two patients with bilateral chronic retinal pigment epitheliopathy are presented. Both patients had large areas of pigment epithelial decompensation and small pigment epithelial detachments at the posterior pole. In the macula subretinal fluid was scanty, but the first patient developed an extensive bullous retinal detachment with shifting of subretinal fluid with changes in posture in both eyes, the second patient had similar detachment in one eye. The etiology of the pigment epithelial disorder remained unknown. No inflammatory cells were found in the vitreous specimen or subretinal fluid in the first patient. Treatment with peroral corticosteroids alone or in combination with azathioprine and cyclosporin A, as well as surgery for retinal detachment in one eye, proved unsuccessful. Argon laser coagulation of the decompensated areas in the macula resulted in resorption of subretinal fluid and reattachment of the exudative detachment.

Adult↗

PubMed1978

Retinitis pigmentosa and retinal oedema.

Twenty-five patients with retinitis pigmentosa and retinal leakage were investigated. Oedema was present in dominant and X-linked inherited disease and is likely to be present in recessive disease as well. We suggest that this might be a general response seen in many types of tapeto-retinal degeneration to actively degenerating photoreceptors or pigment epithelium.

Adult↗

PubMed1998

Retinal astrocytoma associated with hypertrophy of the retinal pigment epithelium: clinical report and follow-up.

Retinal astrocytoma is a rare and benign tumor of the retina most frequently seen in patients with tuberous sclerosis. We describe the case of a 12-year-old boy with solitary retinal astrocytoma in the right eye associated with hypertrophy of the retinal pigment epithelium in the left eye. In this work the instrumental examinations, the differential diagnosis and the follow-up of 7 years are described.

Astrocytoma↗

PubMed1976

Heterogeneity of congenital retinal non-attachment, falciform folds and retinal dysplasia. A guide to genetic counselling.

The heritable falciform folds and congenital retinal non-attachment reported in the literature are here divided into monosymptomatic and complex types. Of the former, autosomal dominant and recessive disorders are known. The complex types comprise several syndromes, both autosomal recessive and X-linked. Retinal dysplasia is sometimes seen in histological sections from these affections, but it is an unspecific histological reaction, and not a disease sui generis. Attention is drawn to the syndrome with microcephaly, microphthalmia and falciform detachment which, though previously described, has not so far been delineated. It is hypothesized that hydrocephaly and congenital retinal non-attachment with or without microphthalmia and persistent hyperplastic primary vitreous constitute yet another 'new' syndrome, mainly seen in females.

Female↗

PubMed1979

Acute severe uveitis with retinal vasculitis and retinal detachment.

5 adult patients (7 eyes) with acute severe uveitis with retinal vasculitis and retinal detachment of unknown etiology were studied. Yellowish exudations which appeared in the peripheral fundus rapidly extended posteriorly, and there was a patchy perivasculitis, white sheathing and obliteration of the retinal vessels. The absorption of these yellowish exudations was followed by atrophy of the retina and the choroid. Secondary detachment of the retina then developed, and the visual acuity remained poor in most cases.

Acute Disease↗

PubMed1990

Ultracytochemical studies on blood-retinal barriers: enzymatic polarity in retinal capillaries.

The ultracytochemical localization of Mg2(+)-ATPase and Ca2(+)-ATPase activities was determined in rat retinal capillary endothelial cells. The predominant localization of both enzymatic activities was on the antiluminal cell membranes in all retinal capillaries. The two enzymes investigated showed a positive reaction on the pinocytic vesicles of capillary endothelial cells. Differences in the intensity of enzyme activities of the luminal and antiluminal plasma membranes may reflect the polarity in the capillary endothelial cells and relate to blood-retinal barrier mechanisms.

Animals↗

PubMedDate not supplied

Congenital retinal macrovessel causes reduced retinal sensitivity at the macula.

PURPOSE: To report a case of reduced macular sensitivity at the macula due to congenital retinal macrovessel as measured by liquid crystal display (LCD) microperimetry. METHODS: Case report. RESULTS: We present a patient with normal visual acuity OU, with fundus OS revealing a congenital retinal macrovessel. LCD microperimetry of the macular area OS revealed reduced sensitivity of the superior retina (median sensitivity, 13.0 dB) compared to the inferior retina (median sensitivity, 15.0 dB) based on numerical values of the sensitivity. Such a difference of sensitivity was not found in OD which had a normal fundus ezamination. CONCLUSIONS: The patient's congenital macrovessel may be causing a relative angioscotoma with reduced retinal sensitivity in the superior half of the macula as compared to the inferior half.

Aged↗

PubMed1993

In vivo response of the rat's retinal pigment epithelium to azide at advanced stages of hereditary retinal dystrophy.

Electrophysiological properties of the retinal pigment epithelium (RPE) were studied in the rat with hereditary retinal dystrophy (rdy). Transocular potential changes evoked by intravenous bolus injections of azide and thiocyanate (SCN-) are the only available indication of RPE state when degeneration of rods is in progress. Also determined were age-dependent decrease in retinal DNA content and in counts of cones that survive after degeneration of rods. The azide response in the pigmented and albino rdy rat was already reduced at the earliest age tested (60 d) and continued to decrease till the age of 2 years. The SCN-response was similarly affected but seemed to decline faster than the azide response. The azide/SCN- response ratio was significantly increased in albino mutants, especially around the age of 400 d. At the age of 10 months and later, the azide and SCN- responses became slower than those of normals. A prolonged exposure of 1,200 1x light to dystrophic rats older than 110 did not affect the azide and SCN- responses whereas the same exposure abolishes the responses of normal rats and of the dystrophic rats at early stages. In rdy rats, the electrophysiological changes were considered to correlate with structural changes of the junctional RPE complex and with abnormal membrane enzyme distribution discovered by others. These RPE changes may contribute to the decreasing cone cell number after rod cell disappearance.

Aging↗

PubMed1999

Combined central retinal artery and central retinal vein occlusion following pars plana vitrectomy.

Combined occlusion of the central retinal artery and central retinal vein is an infrequently encountered clinical entity. Although there are reports in the literature of a combined occlusion of the central retinal artery and vein, its occurrence following pars plana vitrectomy has not been described. We report the case of an elderly patient who developed this unusual occurrence following an uneventful pars plana vitrectomy for a posteriorly dislocated IOL.

Aged↗

PubMed2000

[An update on the treatment of central retinal vein thrombosis (retinal vein occlusion)].

The paper reviews the actual therapeutical means in a severe invalid eye disease. The treatment is not able to improve the retinal blood flow, being more effective on the main symptoms and complications. The isovolumetric hemodilution method as an up-to-date method in the treatment of central retinal vein occlusion, appears to bring better recuperation hopes, by a higher improvement of the local anatomical and hemodynamical factors. That is the reason why we shall expose it widely in our paper. Unfortunately, despite its complexity, the treatment of central retinal vein occlusion is not satisfactory from the point of view of the high number of patients that remain with a low visual acuity. The main goal of the therapy is the recovery of visual function, but a few aspects and especially the late presentation for ophthalmological examination, reduce the objective of treatment to prevent complications.

Humans↗

PubMed1976

[Traumatic retinal angiopathy. Apropos of 2 cases of Purtscher's retinitis].

Two cases of traumatic retinal angiopathy (Purtscher's retinitis are described. Both have a different evolution and cause different damage, but conform to the characteristic retinal image caused by contusion. One case was due to thoracic compression without evident bone fracture, and eventually recovered normal vision, while the other has multiple bone fractures and ended in bilateral optic atrophy whith a marked visual deficit.

Adult↗

PubMed2004

Retinal selectivity of gene expression in rat retinal versus brain capillary endothelial cell lines by differential display analysis.

PURPOSE: The retina is a neural tissue especially differentiated for vision and, thus, the inner blood-retinal barrier (inner BRB) specific molecules may play an essential role in maintaining neural functions in the retina. The purpose of the present study was to identify selectively expressed genes at the inner blood-retinal barrier compared with the blood-brain barrier (BBB). METHODS: A comparison of expressed genes between conditionally immortalized rat retinal (TR-iBRB) cell lines and brain capillary endothelial (TR-BBB) cell lines was performed using mRNA differential display analysis and quantitative real time PCR analysis. The rat M-cadherin gene was cloned by performing 5' RACE, and its protein expression was detected by immunoblot analysis. RESULTS: Eight clones were identified as highly expressed genes in TR-iBRB cells including GATA-binding protein-3 (GATA-3), cytosolic branched chain amino transferase (BCATc), and M-cadherin (cadherin-15). The rat M-cadherin gene was cloned from TR-iBRB cells, for the first time, and has >86% amino acid sequence identity to the previously cloned mammalian M-cadherins. Rat M-cadherin expression in TR-iBRB cells was much greater than that in TR-BBB cells as far as mRNA and protein levels were concerned. CONCLUSIONS: M-cadherin, GATA-3, and BCATc are highly expressed in TR-iBRB cells compared with TR-BBB cells and may indeed be involved in unique functions at the inner BRB.

Amino Acid Sequence↗