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Coats' disease.

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Adult↗

Hemorrhagic retinal macrocysts in advanced Coats disease.

The authors report a case of advanced unilateral Coats disease with associated hemorrhagic retinal macrocysts. Fluorescein angiography showed the macrocysts to be intensely hypofluorescent due to absence of perfused retinal blood vessels in the inner wall and presence of intracavitary blood blocking outer wall and choroidal fluorescence. Around the cystic lesions and in other areas of the peripheral retina, characteristic telangiectatic retinal blood vessels were evident. The mechanism responsible for the development of hemorrhagic retinal macrocysts in Coats disease appears to be coalescence of microcystic spaces in edematous and degenerated chronically detached retina.

Cryosurgery↗

Retinal angiopathy and polypoidal choroidal vasculopathy.

PURPOSE: To describe the clinical and angiographic features of patients with polypoidal choroidal vasculopathy, exudative detachment of the macula, and an associated retinal microangiopathy. METHODS: Case series. RESULTS: Four patients with chronic exudative detachment of the macula with a variable degree of lipid deposition are described. The retina in the detached area, but not beyond, was noted to have a microangiopathy. There was capillary telangiectasia, microaneurysm formation, patchy nonperfusion, and intraretinal leakage. In each patient, there were no other retinal vascular changes in the fundus of either eye. The fluorescein angiogram showed subretinal leakage suspicious for occult choroidal neovascularization. The indocyanine green angiogram showed the presence of underlying polypoidal choroidal neovascularization, accounting for the exudative detachment. After photocoagulation, the retinal angiopathy improved, but not completely. CONCLUSION: Retinal microangiopathy may occur in a chronic macular detachment secondary to polypoidal choroidal neovascularization. The development of these secondary retinal changes is not clearly understood; however, hypoxia from the chronic detachment, a neurotoxic effect from the lipid deposition, or a biochemically induced microvascular abnormality from secretion of vasogenic mediators are possible mechanisms. Indocyanine green angiography is helpful in making a definitive diagnosis. Clinicians should be aware that a retinal microangiopathy may occur in such eyes so that the proper diagnosis can be made and appropriate treatment administered.

Aged↗

Anecortave acetate for the treatment of idiopathic perifoveal telangiectasia: a pilot study.

PURPOSE: To investigate the use of anecortave acetate, a new angiostatic cortisene, for the treatment of the leakage and/or neovascularization associated with idiopathic perifoveal telangiectasia (IPT) in an open label prospective pilot study. METHODS: Seven eyes of six patients were treated with posterior juxtascleral administration of anecortave acetate delivered adjacent to the macula using a specially designed curved cannula. A full clinical examination and fluorescein angiography were performed at baseline and at 3-month intervals. The primary efficacy outcome for this pilot study was the mean change in visual acuity (Early Treatment Diabetic Retinopathy Study) from baseline. RESULTS: The visual acuity remained unchanged in two eyes of two patients with nonproliferative disease after 24 months. The five eyes of four patients presenting with subretinal neovascularization, the proliferative stage of IPT, showed stabilization or improvement of lesion size, resolution of leakage, and stabilization of vision at last follow-up. CONCLUSION: The results of this study suggest that anecortave acetate may inhibit retinal and subretinal permeability as well as neovascular proliferation in patients with IPT. A larger study accordingly should be designed in the future to evaluate the effectiveness and treatment of IPT with this drug.

Adult↗

Subfoveal nodule in Coats' disease.

An atypical presentation of Coats' disease is reported with a prominent subfoveal nodule with peripheral retinal exudates. A 6-year old boy presented with 6/120 vision in the left eye associated with an elevated 1 mm subfoveal, circular lesion with peripheral exudates. The fluorescein angiogram showed peripheral retinal telangiectasias with leakage consistent with Coats' disease. The prominent subfoveal nodule is an uncommon initial presentation of Coats' disease and physicians should be aware of this atypical finding.

Child↗