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Biomedical subjects

J D Gass

Publications and source records attributed to J D Gass.

At least 37 records · Page 2Linked to original sources

Clinicopathologic correlations of surgically excised type 1 and type 2 submacular choroidal neovascular membranes.

PURPOSE: To correlate the histologic and clinical classification of type 1 (subretinal pigment epithelium) and type 2 (subsensory retina) choroidal neovascularization. METHODS: Ten eyes of 10 patients with surgically excised choroidal neovascularization that were routinely processed and could be histologically classified as having a type 1 or type 2 configuration were studied. The patients were clinically classified as having type 1 or type 2 choroidal neovascularization according to preoperative fundus appearance of both eyes and patient age. The histologic and clinical classifications were made in a masked fashion, and the results were compared. RESULTS: The histologic classification was type 1 and type 2 for three and seven specimens, respectively. The clinical classification matched the histologic classification in nine of 10 cases. Clinical fundus characteristics that distinguished type 2 choroidal neovascularization included a subretinal pigmented halo or pigmented plaque in the area of the choroidal neovascularization and sharply defined borders. Patients with type 1 membranes were on average older (76 years) than patients with type 2 membranes (53 years). CONCLUSIONS: It is generally possible to clinically ascertain by clinical fundus appearance and age of a patient whether subfoveal choroidal neovascularization represents a type 1 or type 2 configuration.

Adult↗

Idiopathic central serous chorioretinopathy.

Idiopathic central serous chorioretinopathy (ICSC) is usually seen in young males with Type A personality. Clinical evaluation of the macula with fundoscopy and biomicroscopy, coupled with fluorescein angiography establishes the diagnosis. Indocyanine green angiographic studies have reinformed that the basic pathology lies in choriocapillaries and retinal pigment epithelium. Most of the ICSC resolve completely in four months, and some of them could resolve early with direct photocoagulation of the leaking site. Oral steroids have no role, and could even cause an adverse reaction.

Adult↗

Familial internal limiting membrane dystrophy. A new sheen retinal dystrophy.

OBJECTIVE: To describe the clinicopathologic features of a previously unreported retinal dystrophy. METHODS: Fourteen members of a single family were examined. The medical records of 2 additional family members were reviewed. Pathologic examination was performed on 2 eyes of 1 affected patient. RESULTS: Five individuals were identified with a retinal dystrophy characterized by a glistening inner retinal surface throughout the posterior pole. Visual loss occurred in 3 affected patients in later life owing to superficial polycystic retinal edema and retinal folds. Electroretinographic testing revealed a selective diminution of the b wave. Pathologic examination revealed an abnormal internal limiting membrane with schisis cavities in the inner retina. Endothelial cell swelling, pericyte degeneration, and basement membrane thickening were present in retinal capillaries. CONCLUSIONS: A previously unreported sheen retinal dystrophy is described. Pedigree analysis suggests an autosomal dominant mode of inheritance. A primary defect in Müller cells is the suspected, but unproved, cause. No effective treatment for the associated visual loss is known. The term familial internal limiting membrane dystrophy is proposed to describe this condition.

Adult↗

Echographic findings in benign reactive lymphoid hyperplasia of the choroid.

BACKGROUND: Benign reactive lymphoid hyperplasia is an uncommon uveal tumor. Misdiagnosis may lead to unnecessary enucleations. Clinical evaluation alone is usually not sufficient to diagnose this tumor. OBJECTIVE: To evaluate the utility of standardized echography in the recognition of this disease. METHODS: Retrospective review of the medical and echographic records of 6 patients with choroidal involvement of benign reactive lymphoid hyperplasia. RESULTS: Extraocular episcleral lymphoid nodules were present in 5 of the 6 patients and were usually seen as a late manifestation of the disease. Two patients had repeated echography after treatment of the tumor and showed marked regression of the episcleral nodules. CONCLUSIONS: A combination of clinical and echographic findings can assist in the accurate diagnosis of this rare uveal tumor. The presence of episcleral nodules on standardized echography supports the diagnosis.

Aged↗

Bilaterality of idiopathic macular holes.

BACKGROUND: There has been wide variation in estimates of the incidence of bilateral idiopathic macular holes in the literature. This report of a large series of patients with macular holes provides further information regarding incidence of bilaterality, interval between onset in the first and the second eye, and visual outcome. METHODS: A retrospective chart review was done of 550 patients with idiopathic macular holes examined at the Bascom Palmer Eye Institute between 1968 and 1994. The incidence of bilaterality was estimated from 365 patients in whom the fellow eye was normal at the initial examination. The rate of onset in the fellow eye was evaluated by survival analysis. Mean follow-up was 31 months (median 17 months). RESULTS: Patients with incomplete macular holes (stage 1, aborted stage 1, lamellar) or full-thickness holes had a 19% incidence of bilaterality at 48 months follow-up. In the subset of 32 patients with full-thickness macular holes in the first eye, 13% developed full-thickness holes in the fellow eye within 48 months. The median interval between the onset in the first and in the second eye was 17.5 months. Visual acuity was excellent and stable in eyes with aborted stage 1 and lamellar holes. The visual acuity in the first eye with full-thickness macular hole decreased to 20/200 or worse in 79% of cases within 36 months' follow-up. CONCLUSION: The incidence of bilaterality and poor visual function in the majority of full-thickness idiopathic macular holes by 3 years' duration should be considered when advising patients and planning management.

Aged↗

Progressive subretinal fibrosis and blindness in patients with multifocal granulomatous chorioretinitis.

PURPOSE: To describe the clinical and histopathologic findings in four eyes of three patients who became blind because of multifocal choroiditis and massive subretinal fibrosis. METHODS: Clinicopathologic correlative study. RESULTS: During a period of several years, three healthy elderly patients developed severe visual loss only partly explained by multifocal chorioretinitis and massive subretinal fibrosis. Histopathologic examination of four eyes from these patients disclosed widespread destruction of the outer retina and retinal pigment epithelium, massive areas of subretinal fibrous tissue proliferation, granulomatous inflammation centered around degenerated and fragmented Bruch's membrane, and chronic uveitis. No infectious organisms were identified by special stains or electron microscopy in one eye. CONCLUSIONS: Clinical and histopathologic findings in these three patients were consistent with an autoimmune disease process directed at the retina, retinal pigment epithelium, inner choroid, or all three. Cellular injury in this location can result in massive subretinal fibrosis. Subretinal fibroplasia, however, is probably a nonspecific reparative response to injury. The pathogenesis of this blinding disorder in elderly patients may be similar to the less severe disease usually occurring in younger patients with multifocal choroiditis, panuveitis, and punctate inner choroiditis.

Aged↗

Spontaneous regression of neovascularization at the disk and elsewhere in diabetic retinopathy.

PURPOSE: To report three cases of spontaneous regression of new vessels of the disk and elsewhere in proliferative diabetic retinopathy. METHODS: We retrospectively examined the clinical records of three young women (27, 26, and 19 years old) with insulin-dependent diabetes mellitus (for 16, eight, and 17 years, respectively) associated with other systemic diseases and referred to us for treatment of retinopathy. Complete general and ophthalmologic findings, visual field, and angiographic and electroretinographic examinations were available for each patient. RESULTS: All the patients showed marked, spontaneous improvement of their proliferative retinopathy, with regression of new vessels. Assessment of metabolic control did not bring to light any noteworthy change during the period when the new vessels disappeared. Regression of new vessels was associated with a marked improvement in blood-retinal barrier breakdown as detected on fluorescein angiograms. CONCLUSIONS: Although very unusual, spontaneous regression of neovascularizations in proliferative diabetic retinopathy can happen. We can offer no explanation for the regression of the new vessels and retinovascular hyperpermeability.

Adult↗

Paraproteinemias associated with serous detachments of the retinal pigment epithelium and neurosensory retina.

BACKGROUND: Serous retinal and retinal pigment epithelial detachments resembling central serous chorioretinopathy developed in three patients who had paraproteinemias. METHODS: The patients in this study were examined at two institutions, and their charts, photographs, and fluorescein angiograms were reviewed by the authors. Two patients had cryoglobulinemia and one had lgA, lgM benign gammopathy. RESULTS: These patients had decreased central visual acuity and metamorphopsia at the initial examination. All three patients were women. Two patients were taking systemic corticosteroids in tapering doses at the time of the initial examination. The multiple serous retinal pigment epithelial and retinal detachments were unilateral in one patient and bilateral in two patients. Two patients were treated with laser photocoagulation because of chronic visual loss and foveal detachments. At the most recent examination, each patient had visual acuity of 20/30 or better in both eyes and complete resolution of subretinal fluid. CONCLUSIONS: Patients with paraproteinemia may develop multiple serous detachments of the retinal pigment epithelium and of the neurosensory retina. The chorioretinopathy in these patients may have been related to their paraproteinemia, type A personality, corticosteroid use, or a combination of these factors. The final visual acuity in these three patients was good.

Adult↗

Analysis of phosducin as a candidate gene for retinopathies.

Phosducin, a retina-expressed gene mapped to chromosome 1q25-32.1, was analyzed as a candidate gene for retinopathies. The phosducin gene was cloned and characterized, and PCR primers were designed. Eighty-three patients with various retinopathies and 45 control subjects (24 American, 21 Japanese) were analyzed for mutations in the phosducin gene by PCR, denaturing gradient gel electrophoresis (DGGE), and sequencing. A heterozygous sequence variant changing a glycine to arginine at codon 178 was found in one Usher syndrome type II (USH2) patient, while the other USH2 patients did not show any coding sequence variant. A heterozygous sequence variant changing an asparagine to lysine at codon 174 was found in a patient with a severe retinal degeneration in the category of diseases known as acute zonal occult outer retinopathy (AZOOR). Three non-coding sequence variants were found. Two of these were always present together and found in 20.8% of American and 2.4% of Japanese control subjects, reflecting a difference in population pools. In conclusion, the phosducin gene did not show mutations consistent with it being the causative gene for USH2, but its possible pathogenicity in AZOOR or other retinopathies remains an open question which may be answered by further analysis.

Base Sequence↗

Ipsilateral facial and uveal arteriovenous and capillary angioma, microphthalmos, heterochromia of the iris, and hypotony: an oculocutaneous syndrome simulating Sturge-Weber syndrome.

Sturge-Weber syndrome is a disorder characterized by ipsilateral cavernous hemangioma of the face, uvea, and brain in patients who may present with an enlarged eye, exudative retinal detachment, glaucoma, and seizures. This report presents the clinicopathologic findings of an otherwise healthy infant with ipsilateral arteriovenous and capillary hemangiomas of the face and uveal tract, microphthalmos, iris heterochromia, hypotony, and absence of central nervous system involvement. The association of an arteriovenous-capillary angioma of the ocular adnexa and ipsilateral uveal tract is a syndrome that is distinct from Sturge-Weber syndrome.

Arteriovenous Malformations↗

Acute annular outer retinopathy as a variant of acute zonal occult outer retinopathy.

PURPOSE: A 23-year-old man developed a rapid-onset, large, dense scotoma that was associated with a peculiar gray intraretinal ring corresponding to the edge of the scotoma and normal fluorescein angiographic findings. This disorder may represent a variant of acute zonal occult outer retinopathy. METHODS: We studied one patient by ophthalmoscopic examination and fluorescein angiography. RESULTS: The patient experienced a short period of concentric enlargement of the scotoma and narrowing of the retinal vessels within the ring, followed by stabilization of the scotoma and slow progressive depigmentation and intraretinal migration of the retinal pigment epithelium within the zone of visual loss. The cause of this disorder, which affects primarily the outer retina, was not determined. CONCLUSIONS: We speculate that this disorder is part of the spectrum of acute zonal occult outer retinopathy and that the gray border, which separates the normal from the abnormal retina, represents an immune ring phenomenon.

Acute Disease↗

Reappraisal of biomicroscopic classification of stages of development of a macular hole.

PURPOSE: To update the biomicroscopic classification and anatomic interpretations of the stages of development of age-related macular hole and provide explanations for the remarkable recovery of visual acuity that occurs in some patients after vitreous surgery. METHODS: Recent biomicroscopic observations of various stages of macular holes are used to postulate new anatomic explanations for these stages. RESULTS: Biomicroscopic observations include the following: (1) the change from a yellow spot (stage 1-A) to a yellow ring (stage 1-B) during the early stages of foveal detachment is unique to patients at risk of macular hole; (2) the prehole opacity with a small stage 2 hole may be larger than the hole diameter; and (3) the opacity resembling an operculum that accompanies macular holes is indistinguishable from a pseudo-operculum found in otherwise normal fellow eyes. CONCLUSIONS: The change from a yellow spot (stage 1-A) to a yellow ring (stage 1-B) is caused primarily by centrifugal displacement of retinal receptors after a dehiscence at the umbo. The hole may be hidden by semiopaque contracted prefoveolar vitreous cortex bridging the yellow ring (stage 1-B occult hole). Stage 1-B occult holes become manifest (stage 2 holes) either after early separation of the contracted prefoveolar vitreous cortex from the retina surrounding a small hole or as an eccentric can-opener-like tear in the contracted prefoveolar vitreous cortex, at the edge of larger stage 2 holes. Most prehole opacities probably contain no retinal receptors (pseudo-opercula). Surgical reattachment of the retina surrounding the hole and centripetal movement of the foveolar retina induced by gliosis may restore foveal anatomy and function to near normal.

Aging↗

Asymmetric canthaxanthin retinopathy.

PURPOSE/METHODS: We studied a case of crystalline retinopathy occurring after prolonged use of oral canthaxanthin. The patient was followed up for 38 months during which time she sustained a branch retinal vein occlusion in her left eye. RESULTS/CONCLUSIONS: An asymmetric appearance to the crystalline deposition was noted with increased sedimentation seen in the left eye. Vascular alterations after a branch retinal vein occlusion may lead to local stasis that may exacerbate the development of canthaxanthin retinopathy.

Canthaxanthin↗