Symposium on age-related macular degeneration.
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Biomedical subjects
Publications and source records attributed to J D Gass.
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The postmortem histopathologic findings of eyes from two patients with acquired immunodeficiency syndrome (AIDS) are presented. Both patients had succumbed to multiple opportunistic infections. All of the eyes demonstrated calcified cotton-wool patches of the retina. To date, there have been no prior descriptions of calcification of cotton-wool spots (CWS). The pathophysiologic mechanisms unique to AIDS that allow some of these CWSs to calcify have yet to be determined.
Two apparently healthy patients experienced visual loss and had a peculiar flecked retina simulating fundus flavimaculatus in one eye some months before developing signs and symptoms of the systemic form of non-Hodgkin's large-cell lymphoma. Both experienced spontaneous improvement in visual symptoms and partial resolution of the fundus changes.
It is widely held that ocular toxoplasmosis (1) involves inner retinal layers and (2) shows marked vitreous cellular reaction. This article reports on punctate outer retinal layer toxoplasmosis, a subset of ocular toxoplasmosis characterized by grey-white lesions of deep retina and retinal pigment epithelium, and associated with little or no overlying vitreous reaction. Acute lesions may resolve and become fine punctate white dots. Recognition of this uncommon presentation of toxoplasmosis is important, since this may allow for potentially efficacious therapy.
Recurrent multiple branch retinal arterial occlusions of unknown cause occurred in nine apparently healthy individuals. With the exception of three patients who developed associated auditory symptoms, none developed other signs or symptoms of systemic vascular disease. Ophthalmoscopic and fluorescein angiographic findings suggested focal arteritis and arteriolitis as the cause of the obstructions. Most patients retained good visual acuity.
Electron microscopic findings in an eye removed from a woman who had biomicroscopic and angiographic signs of cystoid macular edema occurring in association with a peripheral choroidal melanoma disclosed evidence that the anatomic basis for the biomicroscopic and angiographic changes was the polycystic expansion of the extravascular compartment of the retina by serous exudate and not, as previously reported, edema of the Mueller's cells.
We studied 41 young and middle-aged adults who developed innumerable, small, round, uniformly sized, yellow, subretinal nodules that were often arranged in clusters throughout the posterior fundus. Angiographically, they fluoresced discretely during the early arteriovenous phase. In middle or later life 17 of these patients developed visual loss caused by vitelliform retinal detachment in the macula of one or both eyes. Spontaneous resolution of the detachment and retention of good visual acuity occurred frequently. We found evidence indicating that the multiple, small, subretinal nodules are probably focal areas of pigment epithelial attenuation overlying nodular thickenings of the basement membrane of the pigment epithelium. The term basal laminar drusen would distinguish these nodules from those of typical drusen, which are caused by focal detachments of attenuated pigment epithelium and its usually normal-thickness basement membrane from the inner collagenous zone of Bruch's membrane.
Two eyes were enucleated for angle-closure associated with familial exudative vitreoretinopathy. Characteristic features included total retinal detachment, peripheral retinal vascular proliferation, and a fibrovascular preretinal membrane. Angle-closure glaucoma developed in one case when sudden massive subretinal hemorrhage, associated with abnormal intraretinal vessels, produced anterior displacement of the lens-iris diaphragm; in the second case, angle-closure was associated with the development of rubeosis iridis. Intraretinal inflammatory cells and exudates were also seen in the enucleated eyes.
Between December 1962 and May 1979, growth of choroidal and ciliary body melanomas was documented in 34 patients prior to enucleation, and in one patient whose eye was obtained at autopsy. Melanoma growth rates in 33 of these 35 eyes were calculated and compared with mitotic index, tumor cytology, tumor size, and mortality. Each melanoma appeared to grow at a constant rate that varied widely in different melanomas. Growth rate correlated with mitotic index. Most large tumors and epithelioid and mixed cell melanomas demonstrated fast growth rates. Most melanomas causing death contained epithelioid cells and were growing rapidly. These findings do not support the conclusion of Zimmerman and colleagues that enucleation is responsible for most metastatic deaths. Rather, it argues against one of the fundamental assumptions on which their conclusion is based, namely, that all melanomas have a relatively uniform slow growth rate prior to enucleation.
The biochemical status of whole blood stored in containers fabricated of ethylene ethylacrylate (EEA) film was monitored at several times during 4 weeks of storage at 4 degrees C. Fifteen biochemical indicators were studied to reflect on erythrocyte integrity, cellular metabolism, plasma protein stability, and microaggregate formation. Comparison to storage in polyvinyl chloride (PVC) containers was made by distributing aliquots from each unit of blood among the containers being compared. Whole blood in EEA developed significantly higher levels of plasma hemoglobin, erythrocyte osmotic fragility, and D-glycerate-2,3-diphosphate (2,3-DPG), and somewhat greater glucose utilization, lactate production, and pH. These biochemical differences were not of great magnitude and the data suggest that EEA containers are compatible with the storage of whole blood.
The red blood cells of blood stored in containers made of polyvinylchloride (PVC) film are osmotically more stable and lose on average about 1/3 less hemoglobin than when blood is stored in another plastic [poly-(ethylene-co-ethyl acrylate); EEA]. The stability of uniform volumes of stored red blood cells varies directly with PVC surface area, whereas changes in EEA surface area have comparatively little or no effect. PVC contains high concentrations of the plasticizer, diethylhexylphthalate (DEHP), known to migrate into blood and to have a high potential for toxicity. To determine if DEHP could be the red cell stabilizing agent in PVC, whole blood was stored in containers made from EEA into which was incorporated varying amounts of DEHP. Incorporation of DEHP into EEA significantly reduced erythrocyte osmotic fragility (p = 0.01). The degree of reduced fragility correlated with the level of DEHP in the cell phase implicating DEHP in PVC containers as the stabilizing agent for red cells.
Between December 1962 and May 1979, growth of choroidal and ciliary body melanomas was documented in 34 patients prior to enucleation. Twenty-seven of the 34 patients underwent enucleation during the same time period (1967 through 1976), when primary treatment with a cobalt 60 episcleral plaque was employed in a nonrandomized fashion in 21 patients with similar-sized melanomas. Comparison of these two groups of patients, none of whom was unavailable for follow-up and all of whom were followed up for a minimum of 66 months, showed that the probability of dying of metastatic melanoma within five years after cobalt 60 therapy was 50% compared with 16% after enucleation. Factors of potential importance in explaining this difference included nonrandomization, small number of patients in both groups, and greater frequency of anterior location of tumors in the cobalt 60 group (76% vs 22%). A randomized, controlled clinical trial is required to determine the relative safety of treatment for uveal melanomas.
In three patients (two women, 73 and 74 years old, and one man, 74 years old), a pigment epithelial rip occurred near the edge of a hypopigmented, partly organized choroidal neovascular membrane at the time of its treatment with krypton red photocoagulation. In all three cases, final visual acuity was 20/200.
Twenty-nine eyes of 27 patients demonstrated the typical features of neuroretinitis with a mascular star. Historical and fluorescein angiographic features of this condition implicate a bloodborne infectious agent. It is likely that multiple agents can produce this condition, including the agent of cat-scratch fever and leptospirosis. A small subgroup may exist in whom this condition resembles anterior ischemic optic neuropathy and has a less favorable clinical course than is usual. There is evidence that this condition is not related to demyelinating disease. It is emphasized that this condition is not a maculopathy since vascular leakage in the optic nerve causes the star. Therefore, we suggest that this condition be renamed Leber's idiopathic stellate neuroretinitis.
This report confirms a previous report that elderly patients with serous detachments of the pigment epithelium prior to and after developing a pigment epithelial tear at one border present a characteristic ophthalmoscopic and fluorescein angiographic appearance. Evidence is presented that subpigment epithelial choroidal neovascularisation and not irregular separation of the basement membrane from its pigment epithelium is the primary cause of the detachment and tear in the pigment epithelium.
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