Juxtapapillary retinal angiomatosis.
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PURPOSE: Retinal ischemia secondary to hypoperfusion of the central retinal artery is recognized as one factor that may contribute to the development of loss of vision in eyes with intraorbital tumors. We study intraorbital tumors which produce motility disturbances and visual problems by color Doppler imaging to evaluate this factor. METHODS: We examined the central retinal artery velocities of 3 patients with disc edema caused by intraconal masses (2 cavernous hemangiomas and 1 presumed optic nerve glioma) via color Doppler imaging. RESULTS: The time-velocity waveform demonstrated abnormally high vascular resistance in the central retinal artery of all affected eyes in the primary position compared with the normal waveform seen in the other eyes. We compared the pulsatility index of eyes with an intraconal mass and contralateral, control eyes using Student's t test for paired samples and significant differences were noted between both groups (p< 0.01). CONCLUSIONS: Intraconal tumors could produce increased pressure in the optic nerve sheath and the optic nerve tissue which could be associated with impaired retinal and optic nerve blood flow and the subsequent amaurosis encountered with intraorbital tumors.
PURPOSE: To assess the prevalence of von Hippel-Lindau (VHL) disease and prognosis of vision in patients with retinal hemangioblastomas (HBs). METHODS: Thirty-six consecutive patients with retinal HBs were treated at Helsinki University Hospital between 1974 and 1998. Detailed neurologic, ophthalmologic, and radiologic examinations; pedigree; mutation analyses; and collection of all relevant clinical, imaging, operative, and autopsy data were performed to identify VHL. RESULTS: The median follow-up time was 10 years. No patient was lost to follow-up. There were three patient groups: 1) 11 patients with clinically definite VHL; 2) 10 patients with clinically suspected VHL with more than one retinal HB (5/10) or visceral cysts (5/10), but with no family history, no detected germ-line mutations, and no VHL-related neoplasms; and 3) 15 patients without VHL with a single retinal HB but no other data suggestive of VHL. In the 11 patients with definite VHL, retinal HBs were detected at a median age of 27 years versus 40 years in the 15 non-VHL patients, and 21 of the 22 eyes were affected. Two VHL patients were totally blind at the end of follow-up compared with one legally blind patient with suspected VHL, but none of the non-VHL patients was blind. The clinical appearance of HBs did not differ among the patient groups. CONCLUSIONS: The prevalence of VHL among patients with retinal HBs was 30% to 58% (11-21 of 36). Visual prognosis was more favorable in non-VHL than VHL patients. All patients with retinal HB should undergo thorough VHL exclusion.
UNLABELLED: Meyer-Schwickerath reported his first results with light coagulation in 1959. This report on the results of treatment in this disease is based on more than 34 years experience at the university eye hospital in Essen. Important prognostic factors are the number of retinal hemangiomas and secondary retinal lesions, such as exudative retinal detachment, lipid deposits, vitreous hemorrhages, preretinal membranes and traction retinal detachment. PATIENTS: Treatment was performed in 156 patients, with 220 involved eyes. The median follow-up was 8.9 years and the average age before treatment, 27.5 years. RESULTS: A positive family history was found in 36.5% and extraocular hemangiomas in 24% of all cases. There were 64 patients with bilateral hemangiomas. In 64.5% of cases the tumor was destroyed without further recurrences. Xenon light coagulation was the only treatment applied in 70%. The other patients received combined procedures with light coagulation, cryocoagulation, Ru 106 plaque and proton beam. Salvage of 90% of all treated eyes was achieved. CONCLUSION: Treatment at an early stage of the disease, before any secondary retinal damage has developed, seems to be crucial for satisfactory results. Light coagulation is extremely effective at this stage. However, the treatment options for advanced cases have also become better recently.
In four of eight cases of reticulum cell sarcoma with ocular involvement diagnosis was made by craniotomy when the signs of a brain tumor developed. A fifth case had an isolated intraocular tumor. Of the other three, two were diagnosed by vitreous aspiration and one by cerebrospinal fluid cytology. Ocular reticulum cell sarcoma frequently accompanies or precedes brain involvement. In this form of the disease the ocular lesion is usually a tumor cell infiltrate of the retina. The associated retinochoroiditis leads to marked vitreous clouding and eventual retinal detachment and glaucoma may occur. On the other hand, when intraocular reticulum cell sarcoma occurs in association with the systemic lymph node visceral form of the disease, choroidal involvement is the rule. Vitreous aspiration may confirm the diagnosis at an early stage when clinical and laboratory evaluation reveal no disease outside the globe. The retinal tumor foci are radiosensitive and local radiation, although not curative, may restore visual acuity. Immunosuppressive therapy given in low doses for anti-inflammatory effect may exacerbate the disease.
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An unusual case of choroidal malignant melanoma with heterochromia iridis, massive subretinal hemorrhage and fibrosis, spontaneously resolving retinal detachment, and subretinal seeding is reported. Clinical and pathological findings are presented and discussed. The unusual aspects of the case are further considered by Drs. Andrew Ferry and Mark Tso.
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A 20-year-old man was diagnosed with a left mixed germ cell testicular tumor and clinical staging with computerized tomography suggested left para-aortic subhilar retroperitoneal adenopathy. The patient received 4 cycles of cisplatin, vinblastine and bleomycin chemotherapy but the mass in the left renal hilus area remained unchanged. Subsequent retroperitoneal lymphadenectomy revealed the mass to be a retroaoritc left renal vein type 2. Further confusion occurred during followup in differentiating this anomaly from recurrent neoplasm necessitating evaluation by magnetic resonance imaging. Retroaortic left renal vein represents a potential imaging pitfall in testicular cancer that may facilitate suboptimal staging, treatment and followup.
PURPOSE: To describe further the clinical and immunological features of cutaneous melanoma-associated retinopathy, which is an infrequent form of paraneoplastic syndrome. METHODS: We studied the salient clinical and immunological aspects of a 66-year-old man with metastatic cutaneous melanoma to lymph nodes of unknown primary site who developed melanoma-associated retinopathy. RESULTS: There was gradual loss of vision in the left eye. Colour vision and night vision were not affected. Visual fields showed arcuate defects. A full-field electroretinogram demonstrated attenuation of the b-wave amplitude in the left eye. The a-wave was intact. Indirect immunofluorescence techniques showed that the antibody reactions took place mainly in the outer plexiform layer of the retina. CONCLUSIONS: Bipolar cells seem to be the target in melanoma-associated retinopathy. Contrary to previous reports, night blindness may not be a universal finding.
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PURPOSE: To evaluate the causes of chemoreduction failure in retinoblastoma and to analyze the associated factors for eventual treatment with external beam radiotherapy and enucleation. DESIGN: Prospective noncomparative case series. PARTICIPANTS: Seventy-one patients with 105 eyes with intraocular retinoblastoma that underwent chemoreduction therapy between October 1998 and January 2003. INTERVENTION: A 6-treatment cycle of chemoreduction therapy with vincristine, etoposide, and carboplatin was administered at monthly intervals. Unresponsive disease was defined as persistence of retinal tumors, vitreous seeds, or subretinal seeds after the second treatment cycle, with no appreciable sign of regression. Eyes with unresponsive disease were enucleated after the second treatment. Eyes that responded to chemoreduction therapy received focal treatment, including indirect laser photocoagulation, transpupillary thermotherapy, cryotherapy, and ruthenium 106 episcleral plaque radiotherapy after the second chemoreduction treatment, if necessary, to achieve complete tumor regression. Recurrence was defined as the regrowth of retinal tumors, vitreous or subretinal seeds after an initial favorable response, and regression. Recurrent retinal tumor, vitreous seeds, or subretinal seeds were treated with focal treatments and 2 to 3 additional chemoreduction treatments. When these methods failed or were not applicable, external beam radiotherapy and/or enucleation was administered. MAIN OUTCOME MEASURES: The use of external beam radiotherapy and enucleation for chemoreduction failure, which was defined as unresponsive or recurrent disease. RESULTS: The mean follow-up was 25.7 months (range: 6-49). Ten of 105 eyes (9.5%) with unresponsive disease were enucleated after the second treatment. Of the remaining 95 eyes, 42 (44.2%) developed recurrence after chemoreduction. Recurrent disease failing to be treated successfully by other methods was treated with external beam radiotherapy in 26 of 95 eyes (27.4%) and enucleation in 22 of 95 eyes (23.2%). External beam radiotherapy was successful in preventing enucleation in 20 of 26 eyes (76.9%). Overall, the globe salvage rate was 69.5%, ranging from 36.1% for Reese-Ellsworth group V disease to 87.0% for groups I to IV disease. Histopathologically, 29 of 31 enucleated eyes (93.5%) had poorly differentiated or moderately differentiated retinoblastoma. Using multivariate logistic regression analysis, factors predictive of eventual treatment with external beam radiotherapy were female gender (P = 0.010), presence of subretinal seeds (P = 0.023), and a greater number of chemoreduction treatments (P = 0.027). By multivariate analysis, the factors associated with the need for eventual treatment with enucleation were recurrence of retinal tumors (P = 0.004), presence of vitreous seeds (P = 0.008), greater tumor thickness (P = 0.015), presence of subretinal fluid (P = 0.040), and older patient age (P = 0.042). CONCLUSIONS: Chemoreduction failure in this article was defined as unresponsive or, more commonly, recurrent retinoblastoma. Older patient age, greater tumor thickness, presence of vitreous seeds and subretinal fluid at baseline, and retinal tumor recurrence after chemoreduction were factors associated with the need for enucleation.
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