Disciform Macular Degeneration Research Articles

Recurrent retinal artery ischemia as a presenting symptom of ophthalmic artery aneurysm

An 81 years old woman presented with sudden painless greying of her vision in the right eye. This resolved within l-2 hours without any residual visual loss. She experienced similar symptoms twice in 4 months. The attacks lasted forty to sixty minutes. She had no history of hypertension, diabetes mellitus, migraine, ischemic heart disease and atrial fibrillation. Further neurological and systemic enquiry was negative On ocular examination, best visual acuity was 6/9 right eye and 6/60 in left eye. Slit lamp biomicroscopy, tonometry and discs appearances were normal. Right macula showed multiple drusens and dry type of ARMD.There was left disciform macular degeneration. The vascular pattern of both fundi was normal and did not reveal any embolus. Extraocular movements were normal. Cardiovascular examination, Carotid artery palpation and auscultation were all normal. Serum cholesterol level was normal. Thrombophilic screen was negative. Magnetic Resonance Angiogram showed aneurysm of right ophthalmic artery near the junction of carotid-ophthalmic artery. After neurosurgical consultation, she was advised to take Tab. Aspirin 75mg daily.

Aqueous flare in eyes with senile disciform macular degeneration: correlation with clinical stage and area of neovascular membrane.

The aim of the present study was to investigate the aqueous flare in eyes with senile disciform macular degeneration (SDMD), divided into different clinical stages, and the correlation between the aqueous flare and the area of the neovascular membrane. Eighty-six eyes of 44 patients with SDMD were examined using a laser flare meter. The area of the neovascular membrane was measured by means of a digitizer in images obtained using indocyanine green videoangiography. The mean value of the aqueous flare was 5.91 +/- 2.51 (photon count/ms) in 7 predisposing stage eyes, 5.68 +/- 1.64 in 15 initial stage eyes, 9.09 +/- 7.65 in 24 advanced form eyes, 5.40 +/- 1.42 in 11 disciform scar eyes, and 5.36 +/- 1.72 in 29 fellow eyes. The aqueous flare value was significantly (P < 0.01) higher in eyes with the advanced form of SDMD than in the fellow eyes. There were no significant differences in aqueous flare values between eyes with predisposing stage, initial stage, and disciform scar and fellow eyes. The aqueous flare value increased significantly with increasing area of neovascular membrane (R = 0.68, P < 0.01). The present results suggest that the aqueous flare increases with increasing neovascular membrane area in eyes with SDMD, and decreases with scarization of the neovascular membrane.

A second independent Tyr168Cys mutation in the tissue inhibitor of metalloproteinases-3 (TIMP3) in Sorsby's fundus dystrophy.

Sorsby's fundus dystrophy (SFD) is a rare autosomal dominant macular disorder with age of onset usually in the fourth decade. It is characterised by loss of central vision owing to subretinal neovascularisation and disciform macular degeneration. In an effort to identify the SFD gene, the disease locus was first mapped to chromosome 22q13-qter by genetic linkage analysis, the same chromosomal region as the gene encoding the tissue inhibitor of metalloproteinases-3 (TIMP3). Subsequently, two separate mutations in TIMP3 were found in affected members of two unrelated SFD pedigrees (Tyr168Cys and Ser181Cys). More recently, two additional SFD related mutations, Ser156Cys and Gly167Cys, have provided further confirmation that heterozygous mutations in TIMP3 are causally responsible for the SFD phenotype. We now report the occurrence of the Tyr168Cys mutation in an SFD patient of Austrian descent and show that this mutation found earlier in an American SFD family arose independently. The new findings add to an emerging pattern of SFD mutations which all seem to affect the C-terminal region of the mature TIMP3 protein. In addition, all known mutations cause a change of an amino acid to a cysteine residue. This suggests a critical role for the additional C-terminal free thiol group in SFD pathogenesis.

Vision testing in atrophic macular degeneration.

To determine the correlation between a group of vision tests in atrophic macular degeneration (AMD) in an office setting. Patients with documented vision loss from atrophic macular degeneration in one eye were invited to attend an eye clinic every three months for a series of six vision tests for their good eye followed by fundus photography. Modified contrast sensitivity, blue/yellow anomaloscopy, flicker fusion frequency, Amsler grid, and photostress recovery time were correlated with Snellen acuity using the Pearson correlation coefficient. The regression of the Snellen acuity on sex, age and the presence of disciform macular degeneration in the other eye was obtained using a general linear model. The correlation with Snellen acuity result was low for all tests. It was highest for Amsler grid abnormality (r = -0.33345) and blue/yellow anomaloscopy matching range (r = -0.20742), where r denotes the correlation coefficient. Patient age was strongly correlated with Snellen results (P = 0.0001), but it was not significantly related to sex (P = 0.1187) or the presence of disciform macular degeneration in the other eye (P = 0.9989). The photostress recovery time showed enormous inter-visit variations and poor correlation with Snellen acuity (P = 0.0526). The course of AMD is routinely assessed by Snellen acuity and any of several additional tests. When employing a test battery in an office setting, a clinician needs to know the relative utility and correlation between the tests at his disposal. Of the tests used in this study, the Amsler grid was the most useful addition to the Snellen acuity at all stages of atrophic macular degeneration, and blue/yellow anomaloscopy was useful only in mild macular degeneration where Snellen acuity was 6/12 or better.

Increased prevalence of disciform macular degeneration after cataract extraction with implantation of an intraocular lens.

After cataract extraction with implantation of an intraocular lens the increased transmission of ultraviolet and blue light may accelerate the development of age-related macular degeneration by producing free radicals in the retina. The maculae of 82 randomly selected postmortem human pseudophakic eyes and 16 fellow phakic eyes were examined by light microscopy. The presence of a basal laminar deposit, hard and soft drusen, thickening and calcification of Bruch's membrane, geographic atrophy, subretinal neovascularisation, and disciform scars was assessed in a standardised way. An age-matched series of 126 postmortem phakic eyes was used as control group. There was no difference between the two groups, except for a higher prevalence of hard drusen (exact trend test, p = 0.038) and disciform scars for the pseudophakic eyes (Fisher's exact test, p = 0.007). There was no significant correlation between either age-related changes in the macula or disciform degeneration and the length of time between cataract surgery and death. No significant difference was found between pseudophakic eyes with or without ultraviolet filter. These findings do not confirm that disciform scar formation is caused by an increase in ultraviolet or blue light.

Clinically important ocular reactions to systemic drug therapy.

Many systemically administered drugs produce ocular adverse effects. Fortunately, relatively few are capable of causing significant, irreversible visual impairment. It is the responsibility of every clinician when prescribing systemic therapeutic agents to be aware of potential adverse ocular reactions, to appreciate their significance, and to inform the patient of the potential risks of treatment. In instances where serious adverse reactions relate to the cumulative effects of prolonged treatment, it is the responsibility of the prescribing physician to institute appropriate methods of visual screening. In this respect, it is most important to obtain the necessary individual baseline measurements before treatment is commenced. Chloroquine retinopathy is probably the most feared of all adverse ocular reactions to systemic drug therapy. However, it occurs only rarely if the daily dosage of chloroquine does not exceed 250mg. Regular screening using automated perimetry is mandatory if prolonged therapy is contemplated. Amiodarone almost inevitably produces corneal deposits. These rarely produce symptoms, and resolve upon withdrawal of the drug. Optic neuropathy has recently been described with amiodarone. Systemic anticoagulant therapy may be associated with intraocular hemorrhage in patients with pre-existing disciform macular degeneration, and such agents should be used with caution in affected individuals. Systemic corticosteroids produce posterior subcapsular cataracts in susceptible individuals which may profoundly affect visual acuity. Although elevated intraocular pressure may also result from systemic therapy, the relationship between the pressure rise and development of glaucomatous changes remains unclear. Ethambutol may produce optic neuropathy if the daily dosage exceeds 15 mg/kg. The changes are usually reversible within a few weeks of stopping treatment. High doses of tamoxifen may produce a maculopathy with loss of visual acuity, if given for prolonged periods. The risk must be weighed against the benefits of treatment. Patients receiving more than 800 mg/day of thioridazine have developed retinopathy, which is usually reversible if detected early enough. Tricyclic antidepressants and other agents with anticholinergic properties may cause disturbances of accommodation and pupillary dilatation. The latter may rarely precipitate acute angle closure glaucoma in susceptible individuals.

Evolution of Angioid Streaks

Angioid streaks of the fundus are not apparent at birth. In order to study their evolution, we examined in a retrospective manner the fundus pictures of 111 subjects with angioid streaks. The earliest form of angioid streaks became apparent at age 8 with findings of narrow short radial discontinuous hypopigmented streaks. Thereafter angioid streaks enlarged in length and width. The end-stage was disciform macular degeneration, helicoid peripapillary atrophy, or diffuse choroidal sclerosis with obscuration of the angioid streaks. We conclude that angioid streaks represent a dynamic manifestation of an underlying retinochoroidal degenerative process.

Shortcomings and pitfalls of ocular MRI

MRI was performed in 41 patients with ocular lesions: 27 cases of malignant melanoma, 5 of haemorrhage, 3 of choroidal metastasis, 3 of senile disciform macular degeneration, 2 retinoblastomas and 1 hamartoma. On MRI 5 small lesions (less than 2 mm thick): 1 melanoma, the 3 metastases and the hamartoma, were not seen. All the malignant melanomas visualised were hyperintense compared to the vitreous on T1-weighted images. On T2-weighted images 24 of 26 lesions were hypointense compared to the vitreous. The remaining two lesions were almost isointense, corresponding to amelanotic lesions. These MRI features did not differ significantly from those of retinoblastomas, senile disciform macular degeneration or subacute choroidal haemorrhage. Major shortcomings of MRI in lesions of the globe lie in a lack of spatial resolution and poor specificity of the findings.

Klinik, Differentialdiagnose und Histopathologie der spontanen subretinalen Massenblutung

This report describes the clinico-histopathologic correlation of three eyes (of three patients aged 80, 82 and 86 years) with massive subretinal hemorrhage. Two blind eyes were enucleated because of a painful therapy-resistant angle-closure glaucoma, one eye was suspected to have a melanoma of the choroid. Bleeding was predisposed by anticoagulant therapy for systemic problems in one patient and by thrombocytopenia in another patient. Histopathologically a disciform macular degeneration was found in all three eyes as the suspected source of bleeding. The differentiation of subretinal bleeding is from malignant melanoma of the choroid which is made possible by ultrasonic examination of the eye.

Juvenile disciform macular degeneration

Abstract A retrospective study of ten Icelandic cases of juvenile disciform macular degeneration, 1981 – 1988 was undertaken in the only referral clinic of a country of 250000 people. Some of these cases were associated with punctate inner choroidopathy or recurrent multifocal choroiditis and the disciform lesions occurred primarily in young, otherwise healthy, moderately myopic women.

Sorsby's Fundus Dystrophy

Ever since Sorsby described his pseudoinflammatory dystrophy in five families, its characteristics have been unclear. The findings in ten affected members of a seven-generation pedigree are discussed and the literature is reviewed. Patients with this dominantly inherited fundus dystrophy lose central vision between the second and fourth decade of life. Three variations in the fundus appearances were distinguished: in the first and most common, white to yellow fundus spots (which are not drusen) accompany a disciform macular degeneration; in the second, the fundus spots are absent; in the third, the yellow deposits are associated with atrophic macular degeneration. Atrophy of the retina, pigment epithelium, and choroid then slowly progresses toward the periphery. Treatment does not halt the progress of the disease. Although variations in this dystrophy may be examples of genetic heterogeneity, Sorsby's fundus dystrophy is a distinct clinical disorder.

Analysis of monochromatic green argon-laser treatment for age-related macular degeneration

A retrospective evaluation of 61 consecutive age-related disciform macular degeneration patients who received green argon-laser photocoagulation was done to evaluate the visual results and to search for characteristics of the presenting lesion that correlated with prognosis. For subretinal new vessels within 200 micron of the foveal avascular center, average pretreatment visual acuity was 20/80 and post-treatment 20/200. Twenty-eight percent improved, 36% stabilized, and 36% had worse acuity at last follow-up. Where new vessels were more than 200 micron from the foveal center, 36% improved, 20% stabilized, and 44% had worse acuity. The average pre- and post-treatment visual acuity for this group was 20/80. Overall, 36% required more than one treatment, and associated retinal vascular obliteration was noted in 31%. Hemorrhage over part of the neovascularization, turbid pigment epithelial detachment, and pigment epithelial detachment in the foveal avascular zone were signs that yielded the worst visual prognosis.

Senile Macular Degeneration – The Effects and Management: The Pathological Processes

This paper, the second in the series on the effects and management of senile macular degeneration (SMD), presents an overview of the pathological processes in the evolution of SMD. The normal macula is first defined and the ageing and degenerative changes which can occur are then described. The histological changes are related to the clinical observations of pigmentary disturbance in the development of either geographic atrophy or disciform macular degeneration. The importance of the appearance of drusen as an indicator of the stage of degeneration is detailed.

How to help patients with senile macular degeneration

Senile macular degeneration is the most common cause of blindness in England in people over 65 years of age.1 Its cause is not known, but the clinical picture results from changes in Bruch’s membrane and the pigment epithelium of the retina. In disciform macular degeneration, severe visual loss results from new vessels growing from the choroid into the subretinal space, producing serous detachment, exudate, haemorrhage, and finally macular scarring. In the less severe ‘dry’ form, atrophy of the pigment epithelium is followed by loss of retinal receptors.

Experimental Subretinal Hemorrhage in Rabbits

In order to simulate the hemorrhagic detachment stage of disciform macular degeneration, we injected fresh autologous blood into the subretinal space of albino rabbits and studied these hemorrhages clinically with the ophthalmoscope and by light and electron microscopy. One hour after injection, retinal changes were minimal and limited to occasional photoreceptor edema. At one day, there was marked damage of the photoreceptor cells characterized by edema and disintegration of the photoreceptors and pyknosis of the outer nuclear layer. Some photoreceptors had been pulled off the retina by contraction or movement of the blood clot, or both. By seven days, the photoreceptor cells were almost absent. Subretinal hemorrhage in rabbits led to irreversible retinal destruction within 24 hours. We think that the mechanism of the destruction of the overlying retina consists of a combination of a diffusion barrier by the clot, mechanical damage to the outer segments by contraction of the clot, and iron toxicity.

Macular Photocoagulation: Optimal Wavelength Selection

Relative merits of different laser wavelengths for macular photocoagulation are analyzed in terms of light scattering and li-ght absorption in hemoglobin, melanin, and macular xanthophyll. Disadvantages of inner retinal damage in the macula caused by the blue-green argon photocoagulators in standard clinical use are discussed, along with advantages of argon green and krypton yellow light. Results of treatment of subretinal neovascularization in disciform macular degeneration are presented. A repetitive treatment, close follow-up protocol was employed with the objectives of closing neovasculature and preserving central visual field. In 74 successive patients treated with monochromatic argon green radiation, neovasculature was closed in 93%, central visual field loss was reduced or stabilized in 76%, and visual acuity was improved or stabilized in 70%. Results were analyzed in terms of location and size of the neovascular membrane, and It was found that best results were obtained with neovascularization farther than 600 microns from the foveola and smaller than 2 mm2. In 17 patients with neovascular membranes more than 600 microns from the foveola, neovasculature was closed in 100%, central visual field loss was reduced or stabilized in 94%, and visual acuity was improved or stabilized in 88%. Similar results were obtained with monochromatic krypton yellow radiation.

Giant cells in disciform macular degeneration of the human eye

Giant cells in disciform macular degeneration of the human eye

Sorsby's pseudoinflammatory macular dystrophy.

Forty-three additional members of a family described originally by Sorsby and Mason have been examined in order to typify pseudoinflammatory macular dystrophy more accurately. Six new affected members were identified by history alone and a further 7 were examined. Vision is lost from disciform macular degeneration generally during the 5th decade of life. Thereafter peripheral degeneration occurs in some affected members. Before loss of vision the fundus changes include fine drusen-like deposits at the level of Bruch's membrane, angioid streaks, and plaque-like deposits of yellow subretinal material in the macular region. These changes are different from those seen in dominant drusen.

Survey of ophthalmic conditions in rural Lesotho.

The first survey of ocular problems in rural Lesotho was conducted by a visiting eye team and consisted of an examination of 1266 ophthalmic clinic patients at 15 representative sites throughout the country. The most common ocular conditions encountered were: conjunctivitis, refractive error, vernalis, senile cataract, glaucoma, corneal opacities and trauma. This disease pattern differs from most developing African nations for reasons of climate, geography and diet. The absence of disciform macular degeneration, narrow-angle glaucoma, and rarity of retinal detachment was noted. The educational impact of the survey, and its importance for future planning are suggested.

Senile disciform macular degeneration: features indicating suitability for photocoagulation.

During a 1-year period 398 new patients were seen with disciform macular degeneration (530 eyes). The lesions were studied retrospectively, and those in which the neovascular tissue did not underlie the fovea, and therefore were treatable, were identified. The following conclusions were drawn: (1) Treatment is possible in most patients with good acuity and few with poor acuity. (2) Treatment is possible in a large proportion of patients with a short history and few with a long history of visual loss. (3) As many as 50% of all patients with senile disciform macular degeneration may be amenable to treatment if seen early enough in the course of their disease. (4) Over one-third of eyes with lesions that are untreated have a visual acuity of 6/60 or better 3 years after the onset of symptoms. (5) If a controlled trial proves that treatment is beneficial, these results emphasise the need for rapid referral and show that these patients will generate a large additional clinical load.