1/8. 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 = 1keywords = vessel (Clic here for more details about this article) |
2/8. Transretinal feeder vessel ligature in von hippel-lindau disease.PURPOSE: To present a new technique called transretinal feeder vessel ligature for the treatment of retinal angiomas. methods: Case report of a patient with peripheral retinal angiomas previously treated unsuccessfully with photocoagulation who responded to this new, alternative surgical treatment. RESULTS: The retinal angiomas decreased in size although two new feeder vessels appeared and the lesions showed a regression pattern after additional laser therapy over the vascular tumors. CONCLUSIONS: A transretinal feeder vessel ligature in association with vitrectomy and photocoagulation may be useful for some advanced or non-responsive cases of retinal angiomas.- - - - - - - - - - ranking = 7keywords = vessel (Clic here for more details about this article) |
3/8. Human T-cell lymphotropic virus type-1 associated t-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis.OBJECTIVE: To report a case of adult T-cell leukemia/lymphoma (ATL) presenting as a bilateral retinal vasculitis and diagnosed by molecular detection of a rearrangement in the T-cell receptor (TCR) and the presence of the human T-cell lymphotropic virus type 1 (HTLV-1) pol gene in the malignant lymphoid cells. DESIGN: Case report. methods: Routine histologic and immunohistochemical analyses were performed on the retinal biopsy specimen before referral to the National eye Institute. Lymphoid cells associated with granulomatous inflammation infiltrating the retina and surrounding retinal blood vessels were microdissected from the paraffin sections of the retinal biopsy specimen. The polymerase chain reaction (PCR) was performed using primers for the TCR gene and HTLV-1 pol and gag genes. RESULTS: Microscopic examination showed a necrotizing granulomatous retinal vasculitis with a predominant T-cell infiltrate detected by immunohistochemistry. Molecular analysis demonstrated a clonal rearrangement of the TCR and the presence of the HTLV-1 pol gene in the microdissected lymphoid cells diagnostic of ATL. CONCLUSIONS: Necrotizing retinitis and retinal vasculitis are rare manifestations of ATL. Human T-cell lymphotropic virus type 1 infection should be considered in the differential diagnosis of patients from endemic areas who have retinal vasculitis at presentation. This case further demonstrates the usefulness of microdissection and PCR for the diagnosis of ocular disease, including HTLV-1 infection.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/8. radiation-induced chorioretinal degeneration: a clinicopathological report of three cases.BACKGROUND: With the shift in radiotherapy toward the posterior segment and with the use of lead screens to shield the anterior segment, posterior segment lesions have become more readily recognized. The purpose of this study is to highlight the effects of ionizing radiation on the choroid and to demonstrate how this can result in visual loss, particularly if the macula is involved. methods: Histopathological study of three enucleated eyes of three patients who had received ionizing radiation: a 27-year-old woman who had received radiation as a child for a hemangioma of the left side of the face, a 16-year-old girl who had received radiation at age 11 years for a malignant mesenchymoma of the right maxilla, and a 4-year-old girl who had received radiation at age 1 year for a retinoblastoma of the right eye. RESULTS: Histopathological examination of the three globes showed extensive chorioretinal degeneration, among other ocular findings. In all cases the fellow eye did not show similar chorioretinal lesions. As all three patients were relatively young, the degree of chorioretinal degeneration was considered to be secondary to radiation treatment. INTERPRETATION: Vascular damage from ionizing radiation is not limited to the retina. It can also affect the choroid in the form of chorioretinal degeneration. Since most of the intraocular circulation arises from the uveal vessels, chorioretinal degenerative lesions may be extensive and may even involve the macula.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/8. Retinal capillary hemangioma treatment by indocyanine green-mediated photothrombosis.PURPOSE: To describe the clinical, angiographic and optical coherence tomography (OCT) findings of a patient with a large retinal capillary hemangioma who was treated by indocyanine green-mediated photothrombosis.Interventional case report. methods: An 18-year-old male patient was treated at a tertiary referral center with a novel laser-dye procedure that uses low-irradiance 810-nm laser and intravenous indocyanine green injection and was prospectively evaluated with fluorescein and indocyanine green angiography, as well as OCT. RESULTS: Vision improved from 20/125 to 20/32 1 at 1-year follow-up. Fundus photography showed marked reduction of a 3 disk diameters retinal capillary hemangioma. Both fluorescein and indocyanine green angiography demonstrated reduced tumor perfusion and narrowing of the lesion feeder and draining vessels as early as 5 days after indocyanine green-mediated photothrombosis; OCT showed immediate decrease in retinal elevation and complete resolution of subretinal fluid within 2 months of treatment. Minimal laser-induced effects were noted in the vicinity of the tumor. CONCLUSIONS: Photothrombosis using low-intensity, 810-nm light to direct laser energy continuously at the tumor after intravenous indocyanine green infusion was effective in restoring macular architecture and improving vision in a patient with a large peripheral retinal capillary hemangioma by means of substantial occlusion of the tumor vasculature and resolution of subretinal fluid.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/8. Spontaneous regression of retinal angiomatous lesions in v. Hippel-Lindau disease (VHL).BACKGROUND: Very little documentation of spontaneous regression of an angiomatous retinal lesion in v. Hippel-Lindau disease (VHL) exists. It is commonly believed that a spontaneous change of hemangiomas into fibrotic lesions occurred. patients/methods: Follow-up examinations of four patients with VHL in the Freiburg VHL study were carried out. RESULTS: A 16-year-old girl revealed a vascular lesion at the border of the optic disc. Control examination nine years later revealed complete spontaneous regression of the retinal vascular changes. A slight retinal vascular change at the superior border in her right eye was found in a 36-year-old woman. A control examination 20 years later revealed regression of the lesion. A 41-year-old woman showed in the retinal periphery a small fibrotic white hemangioma with a pigmented feeder vessel as sign of spontaneous tumor regression. A 12-year-old boy had a retinal microaneurysm inferior to the optic disc that disappeared several years later. CONCLUSION: Documentations of spontaneous regression of minor angiomatous retinal lesions in VHL exist. Such vascular changes are rare. Every retinal lesion should be controlled by follow-up examination and documentation. In case of retinal lesion growth, treatment is necessary.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/8. Uncommon presentation of pediatric ruptured intracranial aneurysm after radiotherapy for retinoblastoma. Case report.OBJECTIVE: radiation-induced intracranial aneurysms are a rare entity with high mortality. Their pathogenesis is still in debate. Their unique anatomy and behavior should be considered when deciding the proper management. A background of radiation, uncommon anatomic aspects, age of presentation, and location guide us to suspect a radiation-induced etiology. We report the case of a pediatric patient with a ruptured intracranial aneurysm, who previously received radiation therapy to the orbits. We aim to contribute to the literature of this uncommon condition and stress the importance of its prompt diagnosis and treatment. CASE DESCRIPTION: A 12-year-old boy, who received radiation therapy for recurrent bilateral retinoblastomas at age 4 months, suddenly developed severe headache associated with nausea and vomiting. A computed tomography scan revealed subarachnoid hemorrhage. A 4-vessel cerebral angiogram revealed a 2-mm aneurysm in the right A1 segment. The aneurysm was clipped successfully with excellent outcome. After 3 years of follow-up, the patient remains neurologically intact and asymptomatic. A new computed tomography angiogram revealed no new aneurysms. CONCLUSION: Vascular abnormalities develop after radiation injury to the brain. Aneurysm formation after radiation therapy has been previously reported, probably secondary to endothelial injury. In this case, early presentation, unusual anatomy, location, and small size at rupture, in contrast with saccular aneurysms, suggest a radiation-induced etiology.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/8. Late-onset retinoblastoma in a well-functioning fellow eye.PURPOSE: To describe the documented growth, clinical course, and histopathology of retinoblastomas in an untreated and otherwise normal right eye of a 27-year-old white male with a g.153211T>A (p.Tyr606X) mutation in the retinoblastoma 1 gene, whose left eye was enucleated at age 2 years for 2 retinoblastomas. DESIGN AND PARTICIPANTS: Retrospective interventional case report. INTERVENTIONS: Over the years, the right eye was irradiated twice and underwent trans-pars plana vitrectomy, transscleral cryocoagulation, argon laser photocoagulation of tumors and their feeder vessels, extracapsular cataract extraction with posterior chamber lens implantation, and neodymium:yttrium-aluminum-garnet laser treatment of after-cataract in the form of Elschnig's pearls. Finally, the patient received combination chemotherapy with etoposide, methotrexate, actinomycin D, cisplatin, and vincristine. RESULTS: The eye finally had to be removed 12 years later due to tumor recurrences and seeding, pseudohypopyon, and elevated intraocular pressure. Histopathology showed microcellular retinoblastoma cells in the anterior chamber angle and trabecular meshwork without subconjunctival extension and in the nasal ciliary body, pars plana, internal limiting membrane, and optic nerve head anterior to the cribriform plate. The patient is without local or systemic recurrences at age 50, 11 years after the last eye was enucleated. CONCLUSIONS: This report shows that retinoblastoma patients may have tumor growth in their fellow eye 25 years after the first eye and also that Elschnig's after-cataract pearls still can arise after irradiation of a lens with 45 Gy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |