1/43. persistent hyperplastic primary vitreous with retinal tumor in tuberous sclerosis: report of a case including tumoral immunohistochemistry and cytogenetic analyses.OBJECTIVE: The authors describe an ocular lesion combining the characteristics of persistent hyperplastic primary vitreous (PHPV) and a retinal tumor in an infant with tuberous sclerosis complex (TSC). STUDY DESIGN: Case report. methods: immunohistochemistry and cytogenetic studies were performed on TSC cells from an intraocular tumor in a 6-week-old infant. RESULTS: Histopathologic examination showed a thick fibrovascular membrane between the aspect of the lens and the astrocytic component of the mass. glial fibrillary acidic protein (GFAP) showed a variable intracytoplasmic reaction in the astrocytic proliferation, involving approximately 50% of the cells. Tissue culture studies showed a fairly rapid proliferation of fusiform cells, consistent with bipolar astrocytic cells. Cytogenetic studies showed one abnormal clone consisting of three hyperdiploid cells with a loss of chromosome 9 and a gain of chromosomes 6 and 12. CONCLUSION: The atypical localization of the retinal tumor could be explained by the fact that it was trapped during its proliferation by the retinal detachment associated with the PHPV.- - - - - - - - - - ranking = 1keywords = retinal detachment, detachment (Clic here for more details about this article) |
2/43. Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.- - - - - - - - - - ranking = 1keywords = retinal detachment, detachment (Clic here for more details about this article) |
3/43. retinoblastoma with acute lymphoblastic leukemia, polyposis coli, and multiple hamartomas.It has long been recognized that compared with their age- and sex-matched controls, survivors of hereditary retinoblastoma have a considerably higher risk of the development of second malignancies (10% at 20 years and 15% at 30 years of follow-up), including osteosarcoma, leiomyosarcoma, melanoma, fibrosarcoma, and other rare spindle cell sarcomas. patients with the nongenetic variety of retinoblastoma do not particularly seem to have an increased incidence of other malignancies than the general population. However, it should be noted that a child with unilateral disease carries a 15% chance of having germline mutation. The cumulative mortality rate from second malignancies was 1.5% at 40 years after unilateral retinoblastoma diagnosis and 26% for bilateral cases in a large survey of 1458 patients. A child with unilateral retinoblastoma, cafe au lait spots, hairy nevus, and grouped pigmentation of retina in the fellow eye is described who furthermore developed acute leukemia and polyposis coli.- - - - - - - - - - ranking = 0.062016467266662keywords = pigment (Clic here for more details about this article) |
4/43. Therapeutic options for capillary papillary hemangiomas.PURPOSE: To describe the results and therapeutic complications of treatment of papillary capillary hemangiomas over the last 13 years. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Five cases of unilateral capillary papillary hemangiomas were reviewed. Three patients had von Hippel-Lindau disease, and two had no associated familial or systemic disease. methods: Two patients were treated with argon green laser, one patient received argon green and diode photocoagulation, and two patients underwent argon green photocoagulation and diode transpupillary thermotherapy (TTT); two eyes also underwent vitreoretinal surgery. Follow-up ranged from 1 to 13 years. MAIN OUTCOME MEASURES: The presence of exudative retinal detachment associated with the activity of the hemangioma and the visual acuity were the two main parameters. RESULTS: Prompt argon green laser treatment was the most effective therapy; -II was ineffective. vitreoretinal surgery, transscleral drainage, and argon endolaser photocoagulation in one case resolved bullous retinal detachment secondary to tumor exudation. Pretreatment visual acuity (VA) levels ranged from 20/25 to counting fingers; posttreatment VA levels ranged from 20/25 to light perception. CONCLUSIONS: If left untreated, papillary hemangiomas may evolve to exudative retinal detachment and marked VA decreases. Although we have not established an ideal therapy, we recommend appropriate treatment on diagnosis. Close follow-up and multiple treatments with argon laser are likely the best therapeutic course.- - - - - - - - - - ranking = 3keywords = retinal detachment, detachment (Clic here for more details about this article) |
5/43. Presumed rhegmatogenous retinal detachment in patients with retinoblastoma.PURPOSE: To review the characteristics, treatment, and outcome of presumed rhegmatogenous retinal detachment (RD) in patients with retinoblastoma. methods: Descriptive consecutive case series study from 1970 to 1996. RESULTS: Of 45 eyes with retinoblastoma that received various modalities of eye-sparing treatment and adequate follow-up, five developed presumed rhegmatogenous RD after treatment. All five eyes had previous treatment with external beam radiation, four of which also had been treated with cryotherapy. To minimize the potential spread of the malignancy intraoperatively, long disease-free intervals were ensured before scleral buckling surgeries were performed, and special care was taken during subretinal fluid drainage. The RDs were totally reattached in three eyes and partially reattached in the other two. CONCLUSION: Presumed rhegmatogenous RD may occur after external beam radiation or cryotherapy for retinoblastoma. It poses special challenges in management.- - - - - - - - - - ranking = 5keywords = retinal detachment, detachment (Clic here for more details about this article) |
6/43. Repair of late retinal detachment after successful treatment of retinoblastoma.PURPOSE: To analyze the results of vitreous surgery for late retinal detachment (RD) after successful treatment of retinoblastoma. methods: The records of all patients with retinoblastoma seen at a single ocular oncology service between 1982 and 1998 were reviewed to identify patients treated for late RD. Previous treatments, characteristics of the RD, surgical techniques used, and visual and anatomic results of the surgery were recorded. RESULTS: Of more than 500 charts reviewed, four patients treated for late RD were identified. All four had received previous, whole-eye, external beam radiotherapy and subsequently required cataract surgery. Other previous treatments included radioactive plaque, cryotherapy, xenon photocoagulation, and chemotherapy. At presentation, some patients had shifting subretinal fluid. None had a tear identifiable preoperatively, but two patients had a definite small slit tear at a tumor edge identified at surgery. One patient had a primary scleral buckle that failed. All patients had vitreous surgery with silicone oil. Average postsurgical follow-up was 30 months. Preoperative visual acuity ranged from 20/80 to light perception and improved postoperatively in two patients. The retina remained completely attached in three patients. CONCLUSIONS: Despite shifting subretinal fluid and no identifiable tear, a rhegmatogenous RD should be considered if it occurs late in patients with otherwise stable, treated retinoblastoma. Tumor reactivation must be excluded carefully. Vitreous surgery can be used to repair the RD successfully and improve vision.- - - - - - - - - - ranking = 5keywords = retinal detachment, detachment (Clic here for more details about this article) |
7/43. Acquired tumors arising from congenital hypertrophy of the retinal pigment epithelium.BACKGROUND: Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is widely recognized to be a flat, stationary condition. Although it can show minimal increase in diameter, it has not been known to spawn nodular tumor that is evident ophthalmoscopically. OBJECTIVES: To report 5 cases of CHRPE that gave rise to an elevated lesion and to describe the clinical features of these unusual nodules. methods: Retrospective medical record review. RESULTS: Of 5 patients with a nodular lesion arising from CHRPE, there were 4 women and 1 man, 4 whites and 1 black. Three patients were followed up for typical CHRPE for longer than 10 years before the tumor developed; 2 patients were recognized to have CHRPE and the elevated tumor concurrently. visual acuity was decreased in 3 patients, mainly due to cystoid macular edema. The tumor was located between the equator and ora serrata in all 5 patients. There was no predilection for quadrant of the fundus. The flat part of the lesion was black and had visible lacunae in all 5 patients. The CHRPE ranged in basal diameter from 3 x 3 mm to 13 x 11 mm. The size of the elevated lesion ranged from 2 x 2 x 2 mm to 8 x 8 x 4 mm. The nodular component in all cases was supplied and drained by slightly prominent, nontortuous retinal blood vessels. Yellow retinal exudation occurred adjacent to the nodule in all 5 patients and 1 patient developed a secondary retinal detachment. Two tumors that showed progressive enlargement, increasing exudation, and progressive visual loss were treated with iodine 125-labeled plaque brachytherapy, resulting in deceased tumor size but no improvement in the visual acuity. CONCLUSIONS: Congenital hypertrophy of the retinal pigment epithelium can spawn a nodular growth that slowly enlarges, attains a retinal blood supply, and causes exudative retinopathy and chronic cystoid macular edema. Although no histopathologic evidence is yet available, we believe that the tumor probably represents either an acquired adenoma or a reactive proliferation of the retinal pigment epithelium. The best treatment of these lesions is not yet established.- - - - - - - - - - ranking = 10.213542271606keywords = retinal pigment, retinal detachment, detachment, pigment (Clic here for more details about this article) |
8/43. Normal EOG values in intraretinal metastasis from cutaneous melanoma: a case report.PURPOSE: The electro-oculogram (EOG) is a powerful test to diagnose primary and metastatic choroidal tumors. While in benign tumors light-peak to dark-trough ratio values are in the range of normal subjects, these values appear highly altered in eyes affected by malignant choroidal tumors. Here we report a clinical case of a patient with intraretinal metastasis from cutaneous melanoma; notwithstanding the malignancy of the tumor, the EOG doesn't present alterations. methods AND RESULTS: Standard electro-oculographic recordings were performed before and after local excision of the tumor: recordings from the normal eye were taken as control. The EOG values were always normal in both eyes. Histological sections showed no evident change in the cell population of the retinal pigment epithelium (RPE) and Bruch's membrane. CONCLUSION: Our results suggest that the presence of an intact RPE is a crucial requirement to obtain a normal EOG.- - - - - - - - - - ranking = 1.5252543007232keywords = retinal pigment, pigment (Clic here for more details about this article) |
9/43. Rhegmatogenous retinal detachment in retinoblastoma patients undergoing chemoreduction and cryotherapy.PURPOSE: To report the development of rhegmatogenous retinal detachment, with the retinal break adjacent to a cryotherapy scar, in three patients undergoing systemic chemotherapy for intraocular retinoblastoma. METHOD: Case series. RESULTS: Three patients with intraocular retinoblastoma were referred after poor response to systemic chemotherapy and local cryotherapy; three eyes of these three patients were noted to have rhegmatogenous retinal detachment and active retinoblastoma, with retinal breaks adjacent to cryotherapy scars. CONCLUSION: Rhegmatogenous retinal detachment may result from retinal necrosis associated with cryotherapy in the setting of intraocular retinoblastoma treated with systemic chemotherapy and local cryotherapy.- - - - - - - - - - ranking = 7keywords = retinal detachment, detachment (Clic here for more details about this article) |
10/43. Retinal abnormalities associated with a mutation of the nucleotide 683 in von hippel-lindau disease.BACKGROUND: von hippel-lindau disease (VHL) is a hereditary cancer syndrome in which affected individuals are at risk of developing tumors in multiple organs, including eyes, cerebellum, spinal cord, kidneys, inner ear, adrenal glands and pancreas. methods: We performed a fundus examination and fluorescein and indocyanine green (ICG) angiography in both eyes of a young woman affected by VHL with bilateral pheochromocytoma, retinal angioma, retinal microaneurysms and unusual alterations of the deep retinal layers. A molecular analysis of the VHL gene was carried out. RESULTS: ophthalmoscopy disclosed in her right eye a small retinal hemangioma, some microaneurysms in both eyes at the posterior pole and multiple, small, whitish, dome-shaped lesions scattered in the retinal pigment epithelium (RPE) of the posterior retina. Fluorescein angiograms revealed in the early phase multiple hyperfluorescent spots that showed progressive discoloration in the late phase of angiography. Some of these spots were ophthalmoscopically undetectable. The late phase of ICG angiography showed some small hyperfluorescent points located at the level of the RPE, and some of them corresponded to the hyperfluorescent spots seen on fluorescein angiography. The molecular analysis revealed the presence of a "missense" mutation of the VHL gene at nucleotide 683. CONCLUSIONS: Alterations in the RPE have never been observed in the VHL syndrome. We describe an unusual case of VHL with a capillary hemangioma associated to diffuse alterations with the RPE of the posterior retina. The possibility exists that these lesions form part of the eye modifications in VHL.- - - - - - - - - - ranking = 1.5252543007232keywords = retinal pigment, pigment (Clic here for more details about this article) |
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