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Biomedical subjects

J D Gass

Publications and source records attributed to J D Gass.

At least 55 records · Page 3Linked to original sources
PubMed1995

Pattern of retinal dysfunction in acute zonal occult outer retinopathy.

PURPOSE: To elucidate the pathophysiology of the diseases recently grouped under the name of acute zonal occult outer retinopathy (AZOOR). METHODS: Twenty-four patients with the diagnosis of multiple evanescent white dot syndrome, acute idiopathic blind-spot enlargement syndrome, acute macular neuroretinopathy, multifocal choroiditis, or pseudo-presumed ocular histoplasmosis syndrome, or a combination of these diagnoses, were evaluated on one or more visits with full-field electroretinograms (ERGs) and kinetic and static perimetry. Sera and IgG fractions were tested using immunofluorescence on cryostat sections of fixed and unfixed human and rat retina. RESULTS: Patients with AZOOR as a group showed abnormal results for all eight ERGs and two visual field parameters measured. Interocular asymmetry was a prominent feature of the patients' test results. Logistic regression indicated that interocular differences in ERG parameters significantly increased the efficacy of identifying patients with AZOOR beyond that of the parameter values alone. Visual field area correlated well with ERG a-wave amplitude. Serial visual function tests in a subset of patients showed that there could be short-term partial recovery or progressive loss of function. Autoantibodies to a specific retinal cell type were not detected. CONCLUSIONS: All patients with AZOOR showed a pattern of visual dysfunction that was photoreceptor in origin, patchy in its distribution across the retina, and asymmetric in the two eyes. There was considerable variability in the severity and the natural history of retinal dysfunction in the patients studied. The full-field ERG was found to be a valuable adjunct in diagnosing AZOOR, especially when the expected interocular variation in measured parameters was known for control subjects. No evidence was obtained for autoantibodies to any retinal cell type in the patients with AZOOR who were tested.

Acute Disease↗
PubMed1995

Idiopathic retinal vasculitis, aneurysms, and neuro-retinitis. Retinal Vasculitis Study.

PURPOSE: The authors describe the clinical feature of ten patients with a new syndrome characterized by the presence of retinal vasculitis, multiple macroaneurysms, neuro-retinitis, and peripheral capillary nonperfusion. METHODS: The authors evaluated ten patients identified to have clinical features compatible with the syndrome of idiopathic retinal vasculitis, aneurysms and neuroretinits (IRVAN). Clinical examination findings, sequential funds photographs (when available), fluorescein angiograms, systemic investigations, response to therapy, and visual outcomes were reviewed. RESULTS: Seven eyes of four patients sustained a marked decrease in visual acuity of 20/200 or worse. Visual loss was due to a combination of an exudative maculopathy and sequelae of retinal ischemia. Capillary nonperfusion was seen in all ten patients and was severe enough to warrant panretinal laser photocoagulation in six patients. Systemic investigations were uniformly noncontributory. Oral prednisone appears to have little beneficial effects on patients with this disorder. CONCLUSIONS: Patients with IRVAN have characteristic retinal features that readily identify this syndrome. An increased awareness of this rare syndrome may help to identify sight-threatening complications at an earlier stage. The authors caution against extensive medical investigations.

Adult↗
PubMed1995

Bilateral bullous exudative retinal detachment complicating idiopathic central serous chorioretinopathy during systemic corticosteroid therapy.

PURPOSE: To present evidence that systemic corticosteroid therapy may cause bilateral bullous serofibrinous exudative retinal detachment in some patients with idiopathic central serous chorioretinopathy. BACKGROUND: Idiopathic central serous chorioretinopathy usually causes mild, transient loss of central vision, usually in otherwise healthy men with a type A personality. A few patients have permanent visual loss because of chronic and recurrent retinal detachment. The clinical findings in these patients may lead to incorrect diagnoses and use of corticosteroid therapy. METHODS: The clinical and photographic records of three patients in whom bilateral bullous serofibrinous exudative retinal detachment associated with idiopathic central serous chorioretinopathy developed after treatment with systemic corticosteroids were reviewed. RESULTS: Systemic corticosteroid treatment was instituted (1) as a prophylaxis to prevent exacerbation of the disease while undergoing surgery in the fellow eye, and (2) as the result of misdiagnoses of multifocal choroiditis and retinal vasculitis (Eales disease). Two of the patients had a history of chronic recurrent retinal detachments before institution of corticosteroid treatment. In one of these patients, bilateral chronic inferior retinal detachment developed, causing peripheral retinal vascular nonperfusion, retinal neovascularization, and vitreous hemorrhage. All three patients had severe permanent visual loss in one or both eyes. CONCLUSION: The findings in these patients provide further evidence that systemic corticosteroid treatment may cause severe exacerbation of retinal detachment and lasting visual loss in some patients with idiopathic central serous retinopathy. Recognition of the atypical presentations of this disorder is important to avoid incorrect diagnoses and treatment.

Adrenal Cortex Hormones↗
PubMed1995

A peripherin/retinal degeneration slow mutation (Pro-210-Arg) associated with macular and peripheral retinal degeneration.

BACKGROUND: Mutations in the peripherin/retinal degeneration slow (RDS) gene have been identified in patients with retinitis pigmentosa and pattern macular dystrophy. The authors initially examined a large family affected with both peripheral and macular degeneration, inherited as an autosomal dominant trait. Screening for peripherin/RDS mutations identified a previously unreported nucleotide alteration in all of the affected individuals. Two additional families later were found to have this same mutation. METHODS: DNA samples from the members of three unrelated families were screened for peripherin/RDS mutations by denaturing gradient gel electrophoresis of the polymerase chain reaction-amplified peripherin/RDS coding sequences. The sequence change that was detected was further characterized by DNA sequencing. Family members were examined and evaluated with psychophysical and electrophysiologic methods. RESULTS: A proline to arginine mutation in codon 210 of peripherin/RDS was found in all clinically affected individuals. Macular changes included extensive geographic atrophy, pigment epithelial changes, and/or drusen. The proline to arginine mutation was not found among 100 healthy individuals, making it unlikely to be a nondisease-causing polymorphism. CONCLUSIONS: The authors identified a novel peripherin/RDS gene mutation associated with autosomal dominant retinal degeneration in patients from three different families. The largest family showed a broad variability in the expressivity of the mutation. The overlap of clinical features with those of age-related maculopathy highlights the need to consider photoreceptor-specific genes as potential factors in the etiology of the latter condition.

Adolescent↗
PubMed1995

[Age-dependent idiopathic macular foramen. Current concepts of the pathogenesis, diagnosis, and treatment].

Senile macular hole usually affects only one eye. It is caused by tangential contraction of the premacular vitreous cortex, particularly that in the foveolar area. Its early stages produce characteristic biomicroscopic findings. In approximately 50% of cases, spontaneous vitreofoveal separation and recovery of vision occurs. Differentiation between a stage 1 impending hole and a stage 2 hole may be difficult. A stage 2 hole may begin as either a central foveolar dehiscence or as a foveolar tear. In both cases, the holes may be accompanied by a similar appearing prehole opacity (pseudo-operculum in the former, and an operculum in the latter cases) in 75 to 80% of the cases. The visual prognosis is probably better in patients whose hole begin in the center. Histopathological examination of the prehole opacity for evidence of retinal tissue at the time of surgery should provide us with important information concerning the relative frequency of the two types of hole development. Meanwhile, there is accumulating circumstantial evidence to suggest that the majority of macular holes begin as a central dehiscence with little loss of retinal tissue and enlarge as a result of centrifugal displacement and concentration of the retinal receptors around the hole, and that visual improvement following surgery occurs not only because of reattachment of the retina, but also centripetal movement of the retinal receptors. Results of a small randomized study of treatment of stage 1 impending holes, while inconclusive, suggest that surgery is of little benefit. Although uncontrolled pilot studies of surgical treatment of full-thickness holes are encouraging, we must await results of the randomized trials to know its value.

Aged↗
PubMed1994

Biomicroscopic and histopathologic considerations regarding the feasibility of surgical excision of subfoveal neovascular membranes.

Surgical excision of subfoveal neovascular membranes may result in recovery of excellent visual acuity in patients with presumed ocular histoplasmosis but not in patients with age-related macular degeneration. To provide an explanation for this discrepancy, I analyzed the clinical and histopathologic findings in five patients with presumed ocular histoplasmosis. These findings provide evidence that the new vessels arising in the choroid in these patients usually grow within the subsensory retinal space and not in the subpigment epithelial space, as occurs in patients with age-related macular degeneration. In presumed ocular histoplasmosis, the new vessels are partly engulfed by a monolayer of proliferating retinal pigment epithelium. Surgical excision of this membrane permits reapproximation of the retinal receptors and native pigment epithelium and may be associated with remarkable return of visual acuity.

Adult↗
PubMed1994

Differentiating macular holes from macular pseudoholes.

Surgical treatments of macular holes have become increasingly effective in inducing resolution of the cuff of surrounding subretinal fluid, resulting in increased vision in many patients. However, for many conditions that mimic a macular hole, either surgery is not indicated or different surgical manipulations are necessary. Differentiating macular holes from some forms of macular pseudoholes can be difficult or impossible based solely on clinical examination. Adjunctive tests that may enhance the accuracy of diagnosis are either not feasible or not available to most clinical practices. We evaluated three clinic-based tests for their value in allowing the differentiation between macular holes and macular pseudoholes: Amsler grid testing, Watzke-Allen sign, and laser aiming beam test. These tests were evaluated in three groups of clinically defined patients: those with full-thickness macular holes, those with macular pseudoholes, and those who had previously undergone successful macular hole treatment. Although the Amsler grid testing was sensitive in correlating with clinically defined macular holes, it was not specific. The Watzke-Allen sign and, to a greater extent, the laser aiming beam test were extremely sensitive and specific in correlating clinically defined full-thickness macular holes and pseudoholes. These tests improve the accuracy of diagnosis of full-thickness macular holes.

Aged↗
PubMed1994

Follow-up and ultrasonographic examination of patients with macular pseudo-operculum.

Twenty-nine patients (30 eyes) with pseudo-opercula were followed up for six to 65 months (mean, 24 months). Visual acuity was 20/30 or better in all but one eye that was amblyopic. Only one eye developed a decrease in visual acuity and changes interpreted as a stage 1 (foveal detachment induced by tangential traction of the cortical vitreous) impending hole. After vitrectomy visual acuity returned to 20/20. Ultrasonographic examination confirmed the presence of pseudo-opercula in all 22 eyes examined. We concluded that the a pseudo-operculum is a favorable prognostic sign and that its presence is demonstrable ultrasonographically.

Adult↗
PubMed1994

Melanotic schwannoma of the choroid. Immunohistochemistry and electron microscopic observations.

PURPOSE: The purpose of this article is to describe what is probably the first reported case of a melanotic schwannoma of the choroid and to discuss the clinical and histopathologic features that serve to differentiate this rare intraocular tumor from a malignant melanoma of the choroid. PATIENT AND METHODS: A 21-year-old woman underwent enucleation of the right eye for a slowly enlarging pigmented choroidal mass that was associated with ipsilateral blindness. The tumor was studied by light microscopy, immunohistochemistry, and electron microscopy. FINDINGS: The tumor was composed of fascicles and whorls of pigmented and nonpigmented plump spindle cells that had light microscopic features of a schwannoma. The tumor cells showed immunoreactivity to S-100 protein and HMB-45. Electron microscopy showed cytoplasmic processes surrounded by a continuous basement membrane and abundant extracellular collagen. The pigmented spindle-shaped cells contained melanosomes in different stages of melanogenesis. The findings were compatible with a melanotic schwannoma. CONCLUSION: Melanotic schwannoma has been reported in soft tissues, peripheral nerves, and in the orbit. To the authors' knowledge, this is the first report of a melanotic schwannoma of the choroid. This extremely rare pigmented choroidal tumor may be very difficult to differentiate clinically from choroidal melanoma.

Adult↗
PubMed1994

Idiopathic recurrent branch retinal arterial occlusion. Natural history and laboratory evaluation.

PURPOSE: To investigate the long-term visual and systemic prognosis of patients with idiopathic recurrent branch retinal artery occlusions, and to test recent hypotheses regarding possible causes of this syndrome. METHODS: The authors retrospectively reviewed the medical and photographic records of 16 eligible patients. Each of 15 living patients was interviewed by one of the authors, then underwent follow-up ophthalmic examination, formal visual field testing, and a battery of clinical laboratory tests. RESULTS: Over a mean follow-up of 9 years, only three eyes (9%) lost visual acuity from foveal ischemia, although nine eyes (28%) had central and/or extensive peripheral visual field loss at final examination. Ocular neovascular complications developed in eight eyes (25%). Eight patients (50%) had associated vestibuloauditory and/or transient sensorimotor symptoms, but serious permanent neurologic deficits or recurrent systemic thromboembolic events did not develop. Although most patients had one or more vaso-occlusive risk factors, extensive laboratory testing failed to define the etiology of the arterial occlusions. CONCLUSIONS: On long-term follow-up, the visual, neurologic, and systemic prognosis for most patients with idiopathic recurrent branch retinal arterial occlusions remains favorable. Although it is probable that such patients are etiologically heterogeneous, the authors theorize that many have mild or partial manifestations of the microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.

Adult↗
PubMed1994

Biomicroscopic and histopathologic considerations regarding the feasibility of surgical excision of subfoveal neovascular membranes.

An understanding of the difference between the growth pattern of choroidal neovascularization within the subsensory retinal space, usually encountered in children and young adults, and that within the subpigment epithelial space, typically in older patients with age-related macular degeneration, is of fundamental importance in considering whether to surgically remove these subfoveal neovascular membranes. Their removal in young patients has some chance of restoring useful central visual function. Their removal in older patients will be associated with removal of the native pigment epithelium and little likelihood of restoration of function in the area of the membrane, unless some means are found to resurface the area with pigment epithelium.

Adult↗
PubMed1994

Macular hole syndromes. Echographic findings with clinical correlation.

BACKGROUND: Anatomic characteristics of macular holes may be difficult to define, even with contact lens biomicroscopy. With the advent of successful macular hole surgical techniques, accurate diagnosis has become increasingly important to avoid unnecessary or incorrect surgery. Echography may provide additional diagnostic information in some cases. The purpose of this study is to correlate echographic and intraoperative clinical features of macular holes. METHODS: The echographic features and intraoperative findings in 25 patients were compared. The spectrum of identifiable echographic features included (1) a thin, smooth, membrane-like surface minimally elevated over the macula (limited posterior vitreous face separation); (2) macular thickening; (3) an operculum; and (4) a complete posterior vitreous face separation. Intraoperative clinical findings included the presence or absence of (1) a thin, limited posterior vitreous face separation, (2) a subretinal fluid cuff, (3) an operculum, and (4) a complete posterior vitreous face separation. RESULTS: Echographic and intraoperative findings correlated regarding the limited posterior face separation in 23 of 25 patients, a surrounding subretinal fluid cuff (macular thickening) in 24 of 25 patients, an operculum in 19 of 25 patients, and complete posterior vitreous face separation in 24 of 25 patients. Thus, echography was very effective in detecting the position of the posterior vitreous face. Overall, echography correlated accurately with 90 (90%) of 100 of these four features. CONCLUSION: Echographic features correlate accurately with clinical features.

Fundus Oculi↗
PubMed1993

Choroidal neovascularization associated with choroidal nevi.

OBJECTIVE: We undertook a retrospective study of all choroidal nevi with overlying neovascularization seen at Bascom Palmer Eye Institute, Miami, Fla, to determine long-term effects on vision and whether the presence of neovascularization represented increased malignant potential of the lesion. DESIGN: A computer search of patients with a coded diagnosis of both a choroidal nevus and choroidal neovascularization was performed. Cases in which the neovascularization was directly overlying the nevus were used for the study. RESULTS: The records of 23 patients followed up for a mean of 6.5 years were reviewed for visual acuity, effect of treatment, and change in the size of the choroidal lesion. Fifteen of the 23 patients had a final visual acuity in the affected eye of 20/200 or better. Five of six patients treated with laser had visual improvement of 2 or more lines. Only one of these lesions showed any growth, and this was after 17 years of no growth. CONCLUSIONS: Choroidal neovascularization associated with choroidal nevi can have profound effects on vision. Laser treatment, when indicated, is effective and may be safely performed. The clinical course of these lesions, to date, does not indicate any clinically significant malignant transformation.

Adult↗
PubMed1993

Fluorescein angiography in the diagnosis of giant cell arteritis.

Clinical data and fundus fluorescein angiograms were analyzed from 35 patients with acute (onset less than four weeks) anterior ischemic optic neuropathy. Nineteen of the 35 patients (54%) had nonarteritic disease, and 16 patients (46%) had giant cell arteritis confirmed by biopsy. Patients with arteritis had higher erythrocyte sedimentation rates, larger cup/disk ratios, and delayed fluorescein dye appearance and choroidal filling times. Three additional patients with cranial arteritis confirmed by biopsy, but without visual loss, had angiographic characteristics similar to patients with arteritic ischemic neuropathy. We consider fluorescein angiography a valuable diagnostic adjunct in identifying patients with giant cell arteritis.

Adrenal Cortex Hormones↗
PubMed1993

Acute zonal occult outer retinopathy. Donders Lecture: The Netherlands Ophthalmological Society, Maastricht, Holland, June 19, 1992.

PURPOSE: This report describes 13 patients, predominantly young women, with a syndrome characterized by rapid loss of one or more large zones of outer retinal function, photopsia, minimal funduscopic changes, and electroretinographic abnormalities affecting one or both eyes. All patients on follow-up examination had persistent visual field defects, and most had chronic photopsia and zones of pigment epithelial atrophy. Evidence is presented that these patients probably represent part of the spectrum of a single disorder that includes the multiple evanescent white-dot syndrome (MEWDS), acute idiopathic blind-spot-enlargement syndrome (AIBSES), acute macular neuroretinopathy (AMN), and the pseudo-presumed ocular histoplasmosis syndrome (P-POHS). METHODS: The medical records of these 13 patients and 2 additional patients, who developed, in addition to the features of this syndrome, funduscopic changes typical of MEWDS, AMN, and P-POHS, were reviewed and follow-up obtained. RESULTS: These patients had extensive unrewarding medical and neurological investigations because of suspected diagnoses, including central nervous system disorders, cancer-associated retinopathy, retinal vasculitis, diffuse unilateral subacute neuroretinitis, and tapetoretinal degenerations. Although most patients retained good visual acuity, all had permanent visual field loss that in some cases was severe. The cause of the disorder was not determined. No effective treatment was found. CONCLUSIONS: Acute visual loss and photopsia in these patients is probably caused by damage to large zones of the outer retina that appears unaffected ophthalmoscopically. Electroretinography is important in early diagnosis. Future investigations probably will reveal further evidence linking this disorder to MEWDS, AIBSES, AMN, and P-POHS.

Acute Disease↗