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PURPOSE: To report retinal neovascularization associated with retinoblastoma in a 14-month-old infant. DESIGN: Observational case report. METHODS: Review of clinical and pathologic findings. RESULTS: A large frond of retinal neovascularization was present posterior to the lens in the right eye, which also contained a retinoblastoma. CONCLUSIONS: Retinal neovascularization is an unusual association with retinoblastoma.
Retinal vascular tumors can be classified into four distinct clinical entities, which include retinal capillary hemangioma, retinal cavernous hemangioma, retinal arteriovenous communications (Wyburn-Mason syndrome), and retinal vasoproliferative tumor.
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.
PURPOSE: To describe a case of exudative retinal capillary hemangioma treated with photodynamic therapy that resulted in an improvement in clinical appearance and visual acuity. DESIGN: Interventional case report. METHODS: A 55-year-old Asian man presented with a large exudative peripheral retinal capillary hemangioma of the left eye causing serous retinal detachment, cystoid macular edema, and a best-corrected visual acuity of 20/200. The hemangioma was treated with three successive sessions of verteporfin photodynamic therapy (PDT). RESULTS: With 10 months of follow-up after the initial treatment, involution of the hemangioma, reduction of subretinal fluid, and improvement of best-corrected visual acuity to 20/80 was seen. CONCLUSION: Photodynamic therapy may be an effective treatment option in select cases of exudative retinal capillary hemangioma.
PURPOSE: To report a case of peripheral retinal nonperfusion and chronic myeloid leukemia in a 23-year-old woman. DESIGN: Observational case report. METHODS: A complete ophthalmic and systemic evaluation was performed. RESULTS: Ophthalmic examination revealed peripheral retinal nonperfusion with retinal neovascularization in both eyes. Fluorescein angiography of both eyes showed a marked midperipheral and peripheral avascular retina temporally with arteriovenous anastomosis and seafan neovascularizations. Blood work showed no abnormalities, although marked leucocytosis (up to 750 x 10(9)/l) and thrombocytosis (646 x 10(9)/l) were present in 1998 when the patient was diagnosed with leukemia. Following treatment, the patient has been in remission. CONCLUSIONS: Peripheral retinal nonperfusion with retinal neovascularization may occur as a complication of chronic myeloid leukemia. In contrast to other studies describing this association, our patient had a bilateral peripheral retinal nonperfusion with seafan neovascularizations without relapse of the myeloid leukemia and without any of the other retinal signs associated with chronic myeloid leukemia, such as tortuosity of veins, intraretinal or preretinal hemorrhages, and cotton-wool exudates.
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.
PURPOSE: To report atypical clinical features of Bartonella henselae neuroretinitis treated with combination antibiotics. METHOD: Case report. RESULTS: A 20-year-old man with a positive B. henselae titer developed a unilateral neuroretinitis, a large peripapillary angiomatous lesion, branch artery occlusion with ischemic maculopathy, and vision loss that failed to improve with clindamycin. Treatment with doxycycline and rifampin led to rapid clinical improvement. The severe vision loss in this case is atypical. CONCLUSIONS: Ocular findings associated with B. henselae infection may include retinal angiomatous lesion and branch retinal artery occlusion. Doxycycline and rifampin were successful in treating the infection.
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The diagnosis of Rb can usually be made by clinical examination; however, patients should also be studied by CT and MR imaging to help determine the extent of the intraocular tumor, document intralesional calcification, confirm the diagnosis, and delineate orbital or intracranial involvement. These imaging techniques should aid the clinician in differentiating Rb from lesions that simulate Rb.
Coats' disease is an idiopathic disorder in which telangiectatic and aneurysmal retinal vessels leak a lipoproteinaceous exudate, with consequent bullous retinal detachment. It is a diagnostic challenge, and CT and MR imaging provide valuable information to differentiate it from other pathologies, particularly from retinoblastoma. Typical, advanced Coats' disease shows on CT a denser substance posterior to the vitreous, which on MR is hyperintense on all pulse sequences. Contrast administration on both CT and MR might give slight linear enhancement at the boundary between vitreous and exudation. Persistent hyperplastic primary vitreous (PHPV) is a unilateral disorder in a microphthalmic eye, seen in full-term infants. PHPV rarely is bilateral in patients with Norrie's disease, Warburg syndrome, or patients with retinal dysplasia. Persistent fetal vasculature leads to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts. The CT appearance in the disorder is quite variable; however, MR imaging may be superior in demonstrating the enhancing retrolental mass and the elongated ciliary processes.
Children with retinoblastoma typically survive their cancer due to advances in early diagnosis and treatment. Despite this success, risk factors persist for metastasis that are thought to be related to patient age, sex, laterality, treatment, genetics, histopathology, and extraocular extension. This review has found that invasion of the uvea, orbit, and optic nerve continue to be the most important predictors of metastatic retinoblastoma. Bilaterality and delays in diagnosis are also important factors. We examine molecular and genetic studies that offer the potential of predicting which tumors are likely to metastasize, which will recur within the eye, and which will undergo senescence. In this review, we describe which clinical evaluations, genetic studies, and histopathologic evaluations of retrieved specimens are currently used widely. This review has been performed to help those caring for patients with retinoblastoma and to aid informed consent.
OBJECTIVE: This study aimed to evaluate the causes of rhegmatogenous retinal detachment in children with retinoblastoma and to assess the surgical approach and results of retinal reattachment surgery. DESIGN: The study design was a retrospective review of all children in whom a rhegmatogenous retinal detachment developed after treatment for retinoblastoma over a 20-year period. PARTICIPANTS: Nine children, 5 to 33 months of age, participated. MAIN OUTCOME MEASURES: Assessment of the treatment methods for retinoblastoma, apparent cause of the rhegmatogenous retinal detachment, interval from the most recent retinoblastoma treatment until repair of the rhegmatogenous retinal detachment, method of surgical repair, and long-term anatomic and visual results were measured. RESULTS: Rhegmatogenous retinal detachment was diagnosed in nine eyes of nine children with treated retinoblastoma. Prior treatment of retinoblastoma included cryotherapy (n = 5), laser photocoagulation (n = 1), external beam radiation therapy (n = 7), and systemic chemotherapy (n = 4). An atrophic retinal hole was present at the most recent site of cryotherapy in three eyes and of laser photocoagulation in one eye. Extreme retinal thinning with a possible retinal break was noted in two eyes at a cryotherapy site. A dialysis was noted in one eye, and a retinal break could not be detected in two eyes. Treatment of retinoblastoma tumors was performed between 1 week and 15 years before retinal reattachment surgery. The retinal detachment was repaired externally with a scleral buckling procedure in seven eyes, with subretinal fluid drainage in five eyes, and without drainage in two eyes. Cytologic evaluation of subretinal fluid was performed in three eyes and showed no tumor cells. The retinal detachment was repaired by pars plana vitrectomy in one eye, and the vitrectomy specimen showed no tumor cells. In one child, the retinal detachment was not repaired. The retina remained attached after surgery in five eyes with follow-up ranging from 5 to 115 months. In three eyes, retinal redetachment associated with recurrent viable intraocular retinoblastoma was noted after surgery, necessitating enucleation. In no case was orbital or metastatic retinoblastoma detected at follow-up. CONCLUSIONS: Rhegmatogenous retinal detachment in children with treated retinoblastoma generally originates from an atrophic retinal hole at the site of prior cryotherapy or laser photocoagulation. If there appears to be complete regression of the intraocular tumor after treatment, surgical repair of the rhegmatogenous retinal detachment may be approached cautiously.
OBJECTIVE: This study aimed to evaluate the efficacy and safety of ruthenium-106 brachytherapy of large peripheral retinal capillary hemangiomas. DESIGN: A retrospective case series. PARTICIPANTS: In 25 eyes of 24 patients, peripheral capillary retinal hemangiomas were treated. INTERVENTION: Brachytherapy using 106-ruthenium/106-rhodium plaques was performed. MAIN OUTCOME MEASURES: Eyes were reviewed for hemangioma regression after brachytherapy, occurrence of retinal detachment, requirement of additional vitreoretinal surgery, final visual outcome, and final retinal status. RESULTS: Preoperative mean visual acuity of all eyes treated was 20/60, mean hemangioma diameter was 3.8 mm, corresponding to approximately 2 disc diameters. In 14 eyes, the retina was attached before surgery, 8 eyes showed an exudative detachment, and 3 eyes showed a traction detachment. Fifteen patients had definite von Hippel-Lindau syndrome. Twenty-three of 25 hemangiomas could be destroyed by single brachytherapy. In 16 eyes, a favorable outcome could be achieved. In nine eyes, outcome was unfavorable, characterized by a severe drop in visual acuity, a persisting exudative retinal detachment, or a recurrent traction detachment. In one eye requiring repeated brachytherapy, irradiation retinopathy occurred. Hemangiomas up to a size of approximately 5.0 mm without preoperative exudative detachment could be treated safely by brachytherapy, whereas a larger hemangioma size or a pre-existing exudative retinal detachment predisposed to an unfavorable outcome. CONCLUSION: Solitary peripheral retinal hemangioma can be ablated effectively by ruthenium-106 brachytherapy. A favorable outcome can be expected if the hemangioma diameter is 5.0 mm or smaller and if there is no preoperative exudative retinal detachment.
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.
A case is presented of a patient with bilateral retinoblastoma, treated at infancy with surgery, chemotherapy and radiotherapy, who subsequently developed at least four additional histologically distinct malignancies: a Ewing sarcoma of the left fibula, two extraskeletal osteosarcomas of the left lower extremity, a mucoepidermoid carcinoma of the right parotid gland and a squamous cell carcinoma of the left paranasal cavity. In addition to retinoblastoma, patients with a germline RB-1 mutation are at high risk of second primary malignancies. An additive carcinogenic effect of cytotoxic therapy in these patients has been assumed. Patients with hereditary retinoblastoma should be under life-long follow-up programmes including a regular head and neck examination for detection of new primaries, especially in the radiation field of the presenting retinoblastoma.
There are many lesions that can present as an elevation of the optic disc. Since each of them has different ophthalmic and systemic significance, it is important for the ophthalmologist to recognize them and to plan strategies of management when necessary. Important inflammatory and benign congenital conditions should be differentiated from malignant neoplasms. This review covers the differential diagnosis of lesions that can cause an elevation of the optic disc. It includes selected congenital lesions, inflammatory conditions, non-inflammatory non-neoplastic disorders, benign hamartomas, and primary and secondary malignant neoplasms.
BACKGROUND: Macular holes have been described in association with benign vascular tumours of the retina. However, their successful treatment has not been reported. PATIENTS AND METHODS: Three patients with macular holes associated with retinal vascular tumours had vitrectomy, peeling of the internal limiting membrane, and intraocular gas tamponade. RESULTS: In all three cases, the macular holes were successfully closed, and the visual acuity improved. CONCLUSION: Macular holes associated with benign retinal vascular tumours respond to conventional macular hole surgical techniques.
DEFINITION OF THE PROBLEM: Our goal is to determine the minimum of information necessary for elementary reading, using a retinal implant. This concerns particularly the fragmentation (pixellisation) of the presented image and its position in the visual field. Fragmentation corresponds to the number of electrodes available, the position of the image in the visual field is equivalent to the site of the implant on the retina. MATERIAL AND METHODS: 10 degrees x 10 degrees windows, containing isolated words or letters, were presented to six healthy subjects on a computer screen. A coupling between the computer and an eye tracker stabilizes these images in an area of the visual field. This coupling constantly corrects the position of the image on the screen according to the direction of gaze. RESULTS: 1) A rapid decrease of the performance is observed at a certain threshold of pixellisation, dependent on the eccentricity of presentation of the images. 2) In central vision, about 400 pixels are sufficient to recognize 80% of the four-letters words. At 10 degrees of eccentricity, about 1225 pixels are needed. 3) An acceptable comprehension of a text (identification of four words out of five), is impossible at eccentricities higher than 10 degrees 4) About 50 pixels are sufficient for a satisfactory recognition of isolated letters, independently of their eccentricity. CONCLUSION: These data validate the method of investigation and provide valuable indications regarding minimal visual requirements in prosthetic vision.