Search

Purpose: To correlate in vivo human retina optical coherence tomography (OCT)3 images with histology., Design: Case series., Methods: Linear OCT3 scans through the macula and optic nerve were obtained in three eyes of three patients who then underwent exenteration surgery for orbital cancers. OCT3 images were then correlated with histology., Results: On histology, two eyes were normal, and one eye had dry macular degeneration. The plexiform layers on histology correlated with the green/yellow areas on the OCT3 scans, and the nuclear layers correlated with the black areas on the OCT3 scans., Conclusions: The authors are unaware of previous reports correlating histology to in vivo human retina OCT3 images. Our findings using human eyes are not different from previous animal studies, in that the plexiform layers are optically highly backscattering and the nuclear layers are not.

Purpose: To report a patient who presented with photopsias, night blindness, exudative retinal detachments, and melanoma-associated retinopathy in her right eye 23 years after the left eye was enucleated for a choroidal melanoma., Methods: Assessment of fundus findings, fluorescein angiograms, and electroretinograms., Results: The patient had recurrent exudative detachments of the macula in her right eye and electroretinogram responses consistent with the diagnosis of melanoma-associated retinopathy. The abdominal computed tomography (CT) scan was negative, but 13 months later, CT scanning revealed many masses in her liver. Fine-needle biopsy confirmed the diagnosis of metastatic melanoma., Conclusion: To our knowledge, this is the first report of melanoma-associated retinopathy in a patient with a previous choroidal melanoma.

Age-related macular degeneration is the leading cause of blindness in developing countries. Irradiating the exudative form, in which a choroidal neovascular membrane develops in the subfoveal area, is presently a treatment under investigation. In 1995, Massachusetts General Hospital, collaborating with Massachusetts Eye and Ear Infirmary, initiated a protocol to treat SCNV membranes using the proton beam at the Harvard Cyclotron Laboratory and the EYEPLAN program with a light-field setup. EYEPLAN requires the axial eye length, membrane dimensions, and manipulation of the eye to include a 4.0-mm radial margin around the membrane so that the aperture margin (50% isodose line on the posterior retina) abuts the inferior aspect of the limbus. Review of 100 individually prepared plans showed that 95% of the fabricated apertures were circular (aspect ratio < 1.095) with diameters 9.5 to 15.0 mm. This information was used to develop an automated standard plan. Thirty-nine plans were developed for axial lengths ranging from 21.0 to 25.0 mm and membrane sizes from 1.5 to 6.75 mm in the usual way as the reviewed ones. Circular targets were outlined centered on the fovea. Distal and proximal 90% ranges (modulation) to the target, and doses to macula, optic disc, lens, ciliary body, retina, and globe were calculated. An automated standard plan requiring the same input data, but avoiding the need for individual plans, was developed. The program outputs the aperture diameter, fixation angle for the light-field setup, range and modulation, and calculates dose to the macula and optic nerve and percentage of retina receiving > or = 50% and > or = 90% of the prescribed dose. Individual plans require approximately 1.5 hours; the standard plan, 5 minutes. The standard plan could have treated 86% of the reviewed plans. The automated plan provides accurate and efficient treatment parameters for the majority of patients.

Purpose: To evaluate peripheral blood lymphocyte subpopulations in patients with choroidal melanoma., Methods: In this prospective study, peripheral blood lymphocytes of 226 patients afflicted with choroidal melanoma were analyzed by flow cytometry and compared with those of 49 age-matched and gender-matched control subjects. Subpopulations of peripheral blood lymphocytes were further identified by monoclonal antibodies specific to cell-surface markers. Statistical analysis was performed by a Student t test., Results: There was no overall difference between the patients with choroidal melanoma and the control subjects with regard to peripheral blood lymphocyte subpopulations. However, when the patients were divided into subgroups based on their clinical characteristics, differences in natural killer (NK) cell population and activated T cells were noted in two subgroups. Patients with ciliary body involvement showed a statistically significant reduction in NK cells (194 +/- 101 vs 260 +/- 178 per mm3; P = .01). The number of activated T cells in this subgroup of patients was increased but not statistically significantly (7.32 +/- 4.79 vs 6.09 +/- 4.34 per mm3; P = .08). In patients with extrascleral extension, a statistically significant increase in activated T cells was noted (9.84 +/- 7.41 vs 6.25 +/- 4.3 per mm3; P = .02). The NK cells in this subgroup of patients were also reduced, but the reduction did not achieve statistical significance (178 +/- 123 vs 248 +/- 167 per mm3; P = .24)., Conclusions: We noted statistically significant differences in peripheral blood lymphocytes in two subgroups of patients with clinically less favorable choroidal melanoma.

Purpose: To examine benzoporphyrin derivative angiography as a modality for studying photosensitizer biodistribution in experimental choroidal melanomas., Methods: A liposomal preparation of benzoporphyrin derivative was used in this study. Digital benzoporphyrin derivative angiograms were performed in 10 rabbits (six for experimental choroidal melanomas, two for normal choroids, and two for irides) using a Topcon ImageNet H1024 digital imaging system, a Kodak Megaplus video camera, and a Topcon TRC-50-VT fundus camera. Only one eye from each rabbit was used. Filters specifically designed for benzoporphyrin derivative (peak absorption at 580 nm and peak emission at 695 nm) were used. Benzoporphyrin derivative (1 mg/kg) was injected into an ear vein while images of tumor, normal choroid, or iris were being obtained. Follow-up images were obtained during the first 3 hours and at 24 hours after injection. Fluorescence microscopy was performed in all 10 rabbits using 1 mg/kg of benzoporphyrin derivative. Tumor-bearing eyes were enucleated at the same time points that angiograms were performed, and the two sets of results were compared for maximum dye accumulation., Results: Digital angiography demonstrated that maximal benzoporphyrin derivative fluorescence occurred in tumors 15 to 45 minutes after injection. Fluorescence photometry corroborated these results., Conclusion: Photosensitizer angiography is a valid modality for determining the optimum treatment time for photodynamic therapy.

We report three cases of uveal lymphoid neoplasia that were diagnosed early in their course. One case exhibited a posterior form, presenting with progressive hyperopia from a serous-macular detachment and choroidal involvement along with retrobulbar involvement. This patient was treated with proton beam irradiation. Two cases displayed an anterior form, with fixed fleshy epibulbar masses resembling salmon patches, and choroidal involvement. The histologic findings from biopsy of these anterior masses are presented. One of these patients was treated with complete excision of the mass and double freeze-thaw cryotherapy of the scleral bed, and the other patient was treated with conventional photon beam irradiation. The clinical features of uveal lymphoid neoplasia in its early form are discussed. Evaluation and treatment strategies for these early forms of uveal lymphoid neoplasia are reviewed.

Purpose: Because limited data exist on the incidence of lens changes after therapeutic intraocular irradiation, we studied a group of patients who underwent proton irradiation for uveal melanoma, in order to estimate cumulative rates of radiation-induced posterior subcapsular opacities and age-related lens changes at specific time points. after irradiation., Methods: Cumulative rates for each type of opacity were estimated from among the 383 patients, treated between 1987 and 1989, who had clear lenses or minimal lens changes before irradiation. Cox's proportional hazards model was used to evaluate the independent effects of tumor and treatment-related characteristics on the development of posterior subcapsular opacities in these patients. Risk factors for posterior subcapsular opacities were evaluated., Results: By three years after irradiation, posterior subcapsular opacities had developed in 42% of the patients, and rates increased significantly with lens dose and with tumor height. The risk of posterior subcapsular opacities was over three times higher when the lens received a substantial dose of irradiation (adjusted relative risk, 3.25; 95% confidence interval, 1.60-6.59), as compared with minimal dose, and was three times higher when the tumor was highly elevated (adjusted relative risk, 3.05; 95% confidence interval, 1.45-6.40) as compared with minimally elevated lesions. Opacities in the other segments of the lens were age related., Conclusions: These data show that patients receiving therapeutic intraocular irradiation have a high risk of developing posterior subcapsular opacities. As expected, the leading risk determinant of posterior subcapsular change is the amount of irradiation received by the lens.

Because familial occurrences of uveal melanoma are rare, there have only been 15 cases of familial melanoma reported in the literature. We studied 11 additional families with a total of 24 members, in which two or more members were afflicted with uveal melanoma. The median age at diagnosis was 56.5 years, similar to the median age at diagnosis of the remaining patients treated in our institution. Thirteen were male and 11 were female, which is consistent with the slight predominance of males found in many large surveys of patients with uveal melanoma. There is a possibility of a heritable component present in these families.

Fifty eyes with rhegmatogenous retinal detachment associated with proliferative diabetic retinopathy were treated surgically. Follow-up examination was from six months to 14 years with an average of 33 months. Successful reattachment of the retina was achieved in 74% of the cases, and visual acuity was preserved or improved in 60%.

A 30-year-old black woman with biopsy-proven sarcoidosis and sickle cell anemia had peripheral retinal neovascularization. Elevated fibrovascular proliferations and distal retinal ischemia with nonperfused ghost vessels passing through the area of neovascularization were found at the temporal periphery. No signs of intraocular inflammation were present. Both diseases occur in black patients and should always be considered in the presence of peripheral retinal neovascularization.

We studied nine cases of single, small, intraretinal foveal hemorrhages in one eye each of nine young adults during the last five years. The patients ranged in age from 18 to 47 years and were otherwise healthy. Four patients gave a history of strenuous exercise, most often weight lifting. The visual acuities ranged from 6/6 (20/20) to 6/60 (20/200) and most patients complained of a small central or paracentral scotoma. Fluorescein angiography did not show any retinal or choroidal vascular abnormalities and recovery of visual acuity was excellent in all patients available for follow-up examination during a period of two weeks to six months.

A 23-year-old man had bilateral multiple choroidal lesions eight years after removal of a bronchial carcinoid tumor. Choroidal biopsy confirmed that the clinical diagnosis and conventional radiotherapy to one eye resulted in rubeosis iridis, neovascular glaucoma, cataract, and vitreous hemorrhage. We were able to destroy the tumors in the other eye by photocoagulation and proton beam irradiation without any adverse effects. The patient is in good health 1 1/2 years after therapy and he has 6/6 (20/20) vision in the eye that was treated.

Five patients with choroidal malignant melanomas were treated with proton irradiation with a cyclotron. We developed an accurate method of aiming the proton beam within the eye. Four to five tantalum rings, 2 mm in diameter, were sutured to the sclera at the edges of the tumor, which is localized by indirect ophthalmoscopy and transillumination. The rings were used as markers for stereotactic radiography to align precisely the tumors with the proton beam. The patients were given a total tumor dose of 4,730 to 6,670 rads, delivered in five equal fractions, over a period of eight to nine days. All patients tolerated the treatments well without any adverse effects. The tumor response to therapy could not be evaluated at the completion of treatment since there was no immediate observable reaction of the tumor or of the surrounding retina. Although there has been no definite regression in any patient, we observed a change in the "color" of the tumor in the first two patients and resolution of two serous retinal detachments.

Unlabelled: We compared survival experiences for three groups with uveal melanoma: (1) 120 patients treated by proton beam irradiation from 1975 to 1981; (2) 235 patients treated by enucleation from 1953 to 1973 (enucleation group 1); and (3) 161 patients treated by enucleation from 1975 to 1981 (enucleation group 2). The following variables were predictive of melanoma-related deaths., Treatment: rate ratio (ratio of the rate of death for one category of the variable relative to the rate of death for another category) of 6.32 (95% confidence interval 1.70 to 23.51) for enucleation group 1 vs proton beam, 3.06 (0.81 to 11.54) for enucleation group 2 vs proton beam, and 2.07 (1.05 to 4.07) for enucleation group 1 vs enucleation group 2. Size: rate ratio of 3.61 (1.51 to 8.64) for large (16+ mm) vs small (less than 11 mm) tumors., Location: rate ratio of 3.19 (1.56 to 6.53) for tumors anterior vs posterior to the equator. When death from all causes was evaluated as the outcome, the estimated rate ratios were similar. When melanoma metastasis was evaluated as the outcome, the estimated rate ratios were reduced in magnitude.

A 32-year-old man with atypical retinitis pigmentosa developed bilateral neovascularization of the optic disk. One year after the diagnosis, vitreous hemorrhage occurred in one eye. Panretinal photocoagulation of this eye resulted in some regression of the neovascularization.

Although the underlying cause or causes of uveal melanoma have yet to be elucidated, important insights may be gained by examining the epidemiologic features of the disease. Uveal melanoma is an uncommon cancer with an incidence of only six cases per million population per year. It is most often diagnosed in the sixth decade and is somewhat more common in males. Apart from sporadic reports of family clusters, uveal melanoma is not considered an inherited disease. Whether some environmental exposure triggers the development of uveal melanoma remains an open question. Sunlight has been proposed as an environmental risk factor because sunlight is known to cause melanoma of the skin and both diseases are rare in nonwhite races. Unlike cutaneous melanoma, however, rates have not been increasing over time and do not vary by latitude. This paper evaluates the available evidence for sunlight and other potential risk factors for uveal melanoma, highlighting areas requiring further research.

We successfully treated a patient with uveal effusion syndrome and abnormal sclera with a partial-thickness sclerectomy. Part of the sclera was immediately cultured, and the excised sclera and the cultured cells were examined by electron microscopy. The sclera demonstrated increased glycosaminoglycan-like deposits between the scleral fibers. The cultured scleral cells showed large intracellular glycogen-like deposits, which were not seen in cells cultured from two control scleras. These findings may be the result of a metabolic defect, which causes a thick, impermeable sclera in some cases of uveal effusion.

Proton magnetic resonance imaging (1H MRI) has emerged as a clinically useful tool for the diagnosis of intraocular tumors. During the last four years 1H MRI characteristics, including spin-lattice relaxation times (T1) and spin-spin relaxation times (T2), have been established for several types of tumors. The introduction of surface coils to the imaging process has significantly improved the quality of intraocular MR images, leading some clinicians to suggest that 1H MR images are preferable to CT scans. Another MRI technique, in which sodium-23 (23Na) is imaged rather than protons, is now under development as tool for intraocular diagnosis. The potential of 23Na MRI depends upon the high concentration and "visibility" of sodium in the vitreous body, and upon the apparent differences in sodium behavior in normal cells vs. tumor cells. The metabolism of normal ocular tissues and intraocular tumors may be probed noninvasively with phosphorus-31 MR spectroscopy (31P MRS). Much progress has been made during the last few years in understanding the appearance of 31P MR spectra of many types of healthy and diseased cells and tissues. Clinical application of this technique to the diagnosis and monitoring of intraocular tumors following conservative treatment will be dependent upon the development of spectroscopy techniques that collect information from the volume of interest (tumor) only.