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At least 289 records · Page 16Linked to original sources

Grid laser photocoagulation for macular edema in bilateral juxtafoveal telangiectasis.

OBJECTIVE: The purpose of the study is to report the efficacy of laser photocoagulation treatment for macular edema in bilateral juxtafoveal telangiectasis (BJT). DESIGN: The study design was a retrospective, clinical study. PARTICIPANTS: The authors observed 14 patients with BJT, studying the visual acuities and retinal changes of treated and untreated eyes. MAIN OUTCOME MEASURES: Visual acuity, presence of macular edema, and associated retinal findings were measured. RESULTS: Of 28 eyes, 10 (8 patients) received 15 laser photocoagulation treatments for macular edema. Neither treated nor untreated eyes had visual improvement of two or more lines. After treatment, five eyes (50%) had increased retinal vascular distortion develop, three (30%) had new draining retinal venules, five (50%) had intraretinal fibrovascular tissues, and four (40%) had retinal and preretinal hemorrhages develop. CONCLUSIONS: Grid laser photocoagulation therapy for macular edema in patients with BJT appears to neither improve nor stabilize long-term visual acuity. In addition, treatment is associated with retinal pigment epithelial changes, increased postoperative retinal vascular distortion, postoperative vascularized retinal scars, and postoperative retinal hemorrhages. These changes, however, do not appear to cause a further loss of vision.

Aged↗

Coats' disease.

A 3-year-old boy had leukocoria in his right eye that could have been confused for retinoblastoma because leukocoria is the most common presenting sign of retinoblastoma. Educating parents, nurse practitioners, pediatricians, and fellow health care professionals about such presenting ocular signs will help expedite ophthalmic examinations and accurate diagnosis for these children.

Child, Preschool↗

Resolution of severe macular oedema in adult Coat's syndrome with high-dose intravitreal triamcinolone acetonide.

OBJECTIVE: To report the clinical outcome of a patient who received high-dose intravitreal triamcinolone acetonide as treatment for severe macular oedema secondary to adult Coat's syndrome. METHOD: Case report. RESULTS: A 74-year-old Indian man complaining of chronic gradual blurring of vision in the left eye was found to have adult Coat's syndrome with severe macular oedema. He received 25 mg of intravitreal triamcinolone acetonide following unsuccessful resolution with grid laser. Optical coherence tomography (OCT) demonstrated up to 75% decrease in macular oedema that was evident even after 9 months follow-up. However, there was no significant improvement in visual acuity. CONCLUSION: Intravitreal triamcinolone is a reasonable option in reducing severe macular oedema in cases of adult Coat's syndrome.

Aged↗

Coats-type retinal telangiectasia in case of Kabuki make-up syndrome (Niikawa-Kuroki syndrome).

PURPOSE: To report a case of Kabuki make-up syndrome (KMS) or Niikawa-Kuroki syndrome with Coats-type retinal telangiectasia, which has not been previously reported. METHODS: Observational case report. CONCLUSIONS: There have been only two reports of retinal pigmentation abnormalities and a single case of bilateral macular deposits in KMS, but this is the first report of a Coats type retinal telangiectasia. This case highlights the importance of thorough posterior segment examination in cases of KMS.

Abnormalities, Multiple↗

Cutis marmorata telangiectatica congenita associated with bilateral congenital retinal detachment.

Cutis marmorata telangiectatica congenita is a rare congenital vascular disorder of the skin, characterized by persistent telangiectasia of the cutaneous blood vessels often associated with cutaneous ulcers. The only previously recognized ophthalmic association with this condition has been rare instances of unilateral congenital open angle glaucoma. The authors report their observations in a child in whom this cutaneous disorder was associated with congenital bilateral total retinal detachments and secondary neovascular glaucoma. The retinal detachments produced bilateral leukocoria simulating retinoblastoma. The cutaneous disorder and the ocular findings were confirmed histopathologically.

Corneal Diseases↗

Atypical presentation of Coats disease.

PURPOSE: To highlight the possibility of intraocular calcification in Coats disease and evaluate the ultrasound and computed tomographic findings. METHOD: A 7.5-year-old boy had a 2-week history of unilateral glaucoma and leukocoria with retinal detachment, suggestive of Coats disease. The possibility of retinoblastoma, however, could not be excluded by ultrasound or computed tomographic examination, which revealed a retinal detachment overlying a subretinal mass with calcification. The blind, painful eye was subsequently enucleated. RESULTS: Histopathologically, there were telangiectatic retinal vessels in a fold of the detached retina peripherally and proteinaceous exudate in the subretinal space containing cholesterol clefts and foamy histiocytes, characteristic of Coats disease. There was also a fibro-osseous nodule in the macular area that correlated with intraocular calcification clinically. CONCLUSION: This case provides the first documentation of ancillary corroboration of intraocular bone formation in Coats disease, which, although rare, is an important consideration in the differential diagnosis of retinoblastoma.

Calcinosis↗

Two sisters with telangiectases [corrected], spondyloepiphyseal dysplasia, hypothyroidism, neovascularization and tractional retinal detachments: a new entity?

Two sisters had an unusual constellation of findings, including telangiectases [corrected] involving the face and limbs, unclassifiable spondyloepiphyseal dysplasia, hypothyroidism, and tractional retinal detachment preceded by retinal and iris neovascularization. To our knowledge, this combination of defects has not been previously documented in the literature.

Abnormalities, Multiple↗

Coat's disease: clinical, angiographic, histopathological findings and clinical management.

Twenty-four consecutive patients with Coats's disease are reported. In 9 cases the blind eye was enucleated because of total exudative detachment of the retina or because of untreatable secondary glaucoma. No treatment had been given. Fifteen patients between 22 months and 52 years of age were managed by argon laser or xenon photocoagulation and/or cryocoagulation. The number of treatment sessions varied from 1 to 9 per patient and totalled 49 sessions. Fluorescein angiography was performed in all cases. In most eyes the arterial system seemed to be more damaged than the venous side. Many arterioles ended in macroaneurysm-like dilatations surrounded by avascular areas or complete vascular closure in more advanced cases. In 11 out of 14 eyes the vision improved after treatment or remained unchanged. The follow-up varied from 1 to 8 years. Persistent and aggressive long-term treatment of Coats's disease is recommended because the prognosis without treatment is poor.

Adolescent↗

Cerebroretinal microangiopathy with calcifications and cysts.

BACKGROUND: Extensive cerebral calcifications and leukoencephalopathy have been reported in two rare disorders Coats plus and leukoencephalopathy with calcifications and cysts. In the latter, a progressive formation of parenchymal brain cysts is a special feature, whereas Coats plus is characterized by intrauterine growth retardation, bilateral retinal telangiectasias and exudations (Coats disease), sparse hair, and dysplastic nails without cyst formation. METHODS: We identified 13 patients, including two pairs of siblings, with extensive cerebral calcifications and leukoencephalopathy. We reviewed clinical, ophthalmologic, radiologic and neuropathologic data of seven deceased patients and studied five patients prospectively. RESULTS: Eleven patients were small for gestational age; the other symptoms emerged from infancy to adolescence. All patients had neurologic symptoms including seizures, spasticity, dystonia, ataxia, and cognitive decline. Progressive intracerebral calcifications involved deep gray nuclei, brainstem, cerebral and cerebellar white matter, and dentate nuclei and were accompanied by diffuse white matter signal changes and, in five patients, cerebral cysts. Eleven patients had retinal telangiectasias or angiomas. Additional features were skeletal and hematologic abnormalities, intestinal bleeding, and poor growth. Neuropathologic examination showed extensive calcinosis and abnormal small vessels with thickened, hyalinized wall and reduced lumen. CONCLUSIONS: Our data suggest that Coats plus syndrome and leukoencephalopathy with calcifications and cysts belong to the same spectrum. The primary abnormality seems to be an obliterative cerebral angiopathy involving small vessels, leading to dystrophic calcifications via slow necrosis and finally to formation of cysts and secondary white matter abnormalities.

Adolescent↗

Coats' disease: A study of cholesterol transport in the eye.

An attempt to establish an animal model (rabbit) to study deposition and transport of cholesterol in the eye has been described. Interesting findings include the deposition of cholesterol in the choroid, sclera, and anterior cornea of rabbits fed on a high cholesterol diet. Injected intravitreal cholesterol is seen to leave through the retina, optic nerve head, and the trabecular meshwork. This process appears to be very slow. During the 4 months of observation the yellow preretinal deposits decreased little in size. Only 1 rabbit showed a picture typical of Coats' disease with serous detachment of the retina, subretinal lipoidal deposits and preretinal neovascularization. Although these results are preliminary, they suggest that fatty materials may pass through the retina into the subretinal space from the vitreous and might explain the pathogenesis of Coats' disease in the human.

Animals↗

Coats' syndrome as a cause of secondary open-angle glaucoma.

A case of Coats' syndrome presenting with acute secondary open-angle glaucoma is described. A 41-year-old woman presented with eye pain and an intraocular pressure of 50 mm Hg in the right eye. Numerous hyperrefringent yellow-white crystals were seen in the anterior chamber and in a recessed anterior chamber angle. Fundus examination revealed an inferior yellow exudative retinal detachment with retinal vascular abnormalities resembling Coats' disease. Following lensectomy, vitrectomy, retinal reattachment, and endolaser photocoagulation, the intraocular pressure stabilized within normal limits. Coats' syndrome may cause increased intraocular pressure by a secondary open-angle mechanism, even in phakic eyes. Removal of the lipid crystals and treatment of the anomalous vessels may be sufficient to control the intraocular pressure.

Adult↗

Pseudoretinoblastoma in enucleated eyes of Asian patients.

INTRODUCTION: Retinoblastomas of the eye are a cause of childhood blindness and have a high rate of mortality, as well as a hereditary mode of transmission. Other conditions that mimic retinoblastomas are known as pseudoretinoblastomas, and are managed differently. Although pseudoretinoblastoma and the accuracy of retinoblastoma diagnosis have been reviewed in Caucasian patients, published studies in Asian patients are lacking. The purpose of this article is to report our experience with pseudoretinoblastomas in two major ophthalmological centres in Asia. METHODS: A case series of 28 enucleations carried out for suspected retinoblastoma at the Singapore National Eye Centre and KK Women's and Children's Hospital, Singapore, between January 1991 and December 2002, is reported. All cases were subjected to a detailed history from parents, followed by external ocular examination, slit-lamp biomicroscopy and binocular indirect ophthalmoscopy. Ancillary studies, such as B-scan ultrasonography and computed tomography, were employed as necessary to confirm the diagnosis. Histology was obtained on all cases. RESULTS: Of the 28 cases, 25 (89 percent) were found on histological analysis to be retinoblastomas. Three (11 percent) were pseudoretinoblastomas. There were two cases of Coat's disease and a case of presumed ocular toxocariasis. These three cases were described in detail. CONCLUSION: Although our sample size is small, the percentage of confirmed retinoblastomas was found to be only slightly higher than that found in western countries. Our findings are consistent with their findings that Coat's disease and presumed ocular toxocariasis are the more common causes of pseudoretinoblastoma.

Asian People↗

[Pigmentary retinopathy and Coats' vasculopathy].

The paper reports on a clinical case of association between pigmentary retinopathy and Coat's retinal vasculopathy. The study of the clinical syndrome of the case presented and of those described in the literature, shows the possibility that vascular telangiecstases might be the consequence of a hereditary tendency towards a form of latent vasculopathy, that develops towards a known clinical form. The disturbances of a generalized retinal vascular permeability, described for the first time in this context seems to be dependent on the genetic type of pigmentary retinopathy and independent of the peripheral telangiectatic modifications. The association of the two affections is, by the serious complications they generate, a factor of aggravating the visual prognosis.

Female↗