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Results for “Retinal Telangiectasis”

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At least 19 recordsLinked to original sources

PubMed1987

[Peripheral retinal telangiectasis in retinitis pigmentosa].

The association between retinitis pigmentosa and retinal telangiectasis has been rarely reported. The case of a young woman affected with retinitis pigmentosa and telangiectasis involving bilaterally and symmetrically the inferior retinal periphery is described. Moreover, the patient showed a high degree edema of the retina produced by the permeabilization of the retinal capillaries (especially foveal capillaries) and by the passage of fluid from the choroid through the damaged retinal pigment epithelium. The telangiectasis were successfully treated with cryotherapy. The telangiectasis found in patients with retinitis pigmentosa have to be distinguished from other forms of telangiectasis, as those found in young males or those of the temporal retinal periphery of adults, in which respect they have particular characteristics. The cause of the telangiectasis and of the breakdown of the blood-retinal barrier in retinitis pigmentosa is not know. It is probable that they are produced by a toxic or inflammatory action on the vessels mediated by retinal antigens.

Adult↗

PubMed2001

[Exudative idiopathic juxtafoveolar retinal telangiectasis].

PURPOSE: To study the different clinical presentation of the idiopathic juxtafoveolar retinal telangiectasis and its treatment with argon laser. MATERIAL AND METHOD: We studied retrospectively nine eyes of eight patients, seen from 1990 to 1997, with diagnosis of idiopathic juxtafoveolar retinal telangiectasis. They have been studied with fluorescein angiography and treated with argon laser. RESULT: The most frequent clinical presentation was large areas of retinal telangiectasis affecting the horizontal rafe temporal to the macula. The diagnosis was stabilised as regards clinical manifestation and after ruling out other causes. Six out of eight patient with visual disfunction for macular edema and exudation improved considerably after laser photocoagulation. In 3 patients the vascular abnormalities were progressive after the treatment. CONCLUSIONS: The idiopathic juxtafoveolar retinal telangiectasis with exudation is an uncommon disease, that causes visual disturbance, but may be treated with laser photocoagulation.

Adult↗

PubMed1993

Retinal telangiectasis and proliferation of the retinal vessels in a family.

A 20-year-old woman presented with a marked proliferative retinopathy of the entire central region of the retina in both eyes, with veil-like epiretinal gliovascular membranes and isolated spots of haemorrhaging. The outer region of the retina was unremarkable. Her 49-year-old mother showed perimacular telangiectases in both eyes. The vascular system of both patients were medically sound, and, in particular, the blood pressure, blood sugar and blood picture were normal, with no signs of heart, kidney, lung or liver disease. The first patient had been a heavy smoker for 6 months, and the second did not smoke. The 84-year-old mother of the second patient was blind following bilateral Horton's temporal arteritis.

Adult↗

PubMed1980

Parafoveal retinal telangiectasis. Light and electron microscopy studies.

The clinical, histopathological, and ultrastructural features of a case of parafoveal telangiectasis are reported. No telangiectasis of retinal vessels was found. Instead, there was thickening of the wall of the retinal capillaries and narrowing of the calibre of the lumen. Thickening was due to marked proliferation of basement membrane in a multilayered configuration, Cellular debris from degenerated endothelial cells and pericytes, and multimembranous lamellar lipid material were entrapped between layers of basement membrane. Extensive degeneration of pericytes was observed, and occasional areas showed degenerated endothelial cells. These changes were not restricted to the clinical-affected parafoveal area but were also seen to a lesser degree throughout the retina. The changes are interpreted as being due to primary endothelial cell degeneration and regeneration, with successive waves of basement membrane production and secondary degeneration of pericytes.

Basement Membrane↗

PubMed2001

Idiopathic juxtafoveal retinal telangiectasis: case report and literature review.

BACKGROUND: Idiopathic juxtafoveal retinal telangiectasis (IJRT) is an uncommon cause of vision loss. It is characterized by irregularly dilated capillaries in the macula, often found temporal to the fovea and straddling the horizontal raphe. These telangiectatic vessels appear similar to microaneurysms and can be difficult to detect without fluorescein angiography. Varying degrees of vessel leakage and exudation are also common features. Three distinct types of IJRT have been described on the basis of clinical features. Within the spectrum of disease encompassed by IJRT, it can affect either gender--present unilaterally or bilaterally--and occur at any age. CASE REPORT AND REVIEW: Presented here is a case with type 1A idiopathic juxtafoveal retinal telangiectasis and a review of all types.

Diagnosis, Differential↗

PubMed2001

Classification of the spectrum of Coats' disease as subtypes of idiopathic retinal telangiectasis with exudation.

PURPOSE: An increasing variety of eponymous terms have been used to describe atypical cases of Coats' disease. A group of typical cases of Coats' disease and other cases of differing severity were classified as one of four subtypes of idiopathic retinal telangiectasis with exudation and compared with regard to clinical outcome. METHODS: In a retrospective clinical review patients with typical and atypical Coats' disease were classified as severe, focal, juxtafoveal or associated (with another disease) forms of idiopathic retinal telangiectasis with exudation. RESULTS: 53 eyes in 50 patients were examined of which 62% (n=31) were male. 12 eyes were classified as severe (group 1), 22 focal (group 2), 12 juxtafoveal (group 3) and 7 associated (group 4). The mean age at diagnosis was lowest in group 1 eyes (6.8 years). The best visual acuity at presentation was 6/60 in group 1 whereas high proportions of eyes in the other groups had initial visual acuities of 6/24 or better. In group 1 only one eye was treated, the majority of eyes were blind or had been enucleated whereas 34 (79%) of eyes in the other groups were suitable for treatment and 29 eyes (67%) retained pre-treatment visual acuity or better at last follow-up. CONCLUSIONS: Idiopathic retinal telangiectasis with exudation is a spectrum of disease, which is synonymous with Coats' disease. In this retrospective study eyes with severe idiopathic retinal telangiectasis with exudation corresponding to typical Coats' disease, have poorer vision at presentation, are less suitable for treatment and have worse outcomes than eyes with other subtypes. The spectrum of disease severity seen in idiopathic retinal telangiectasis with exudation may be due to second somatic mutations in genes with an existing germline mutation (the two hit theory) and a mosaic phenotype.

Adolescent↗

PubMed2002

Photodynamic therapy of a subretinal neovascular membrane in type 2A idiopathic juxtafoveolar retinal telangiectasis.

PURPOSE: To report the outcome of a patient with a subretinal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasis treated with photodynamic female therapy. METHODS: Interventional case report. A 43-year-old female with type 2A idiopathic juxtafoveolar retinal telangiectasis presented with a 2-week history of decreased vision and a subretinal neovascular membrane in the left eye. RESULTS: Visual improvement from 20/70-2 to 20/50-2 in the left eye was observed after two photodynamic therapy treatments of a juxtafoveal subretinal neovascular membrane with verteporfin, and was maintained through 7 months of follow-up. Fluorescein angiography showed no leakage specific to the subretinal neovascular membrane, but leakage from the juxtafoveal telangiectasis continued. CONCLUSION: A substantial improvement was seen in this patient after treatment of the juxtafoveal subretinal neovascular membrane by photodynamic therapy. Given the poor prognosis of these lesions, photodynamic therapy may therefore be a useful treatment for patients with subretinal neovascular membranes associated with idiopathic juxtafoveolar retinal telangiectasis.

Adult↗

PubMed2006

Optical coherence tomography in group 2A idiopathic juxtafoveolar retinal telangiectasis.

OBJECTIVE: To describe the changes observed with optical coherence tomography in group 2A idiopathic juxtafoveolar retinal telangiectasis. METHODS: We retrospectively reviewed the medical records of 13 patients (25 eyes). All eyes underwent optical coherence tomography examination consisting of 6 radial scans, fundus color photography, and fluorescein angiography. We calculated retinal foveal and central foveal thicknesses from software mapping results. We compared the optical coherence tomography data with fundus photography and fluorescein angiography findings. RESULTS: Foveal cystoid spaces, very small or more prominent, were present in 20 of 25 eyes. Some degree of disruption of the inner segment/outer segment photoreceptor junction line was observed in 18 eyes as from stage 2 of idiopathic juxtafoveolar retinal telangiectasis, and intraretinal pigmentary proliferation was observed in 9. A foveal detachment without subretinal new vessels was also present in 2 eyes. Despite these abnormalities, central foveal thickness was below or within the range of reference values in all eyes; foveal thickness, in 23 of 25. In the more advanced cases, severe disruption of the inner segment/outer segment photoreceptor junction line and outer retinal atrophy were seen. CONCLUSIONS: Early in the evolution of group 2A idiopathic juxtafoveolar retinal telangiectasis, the optical coherence tomography examination disclosed intraretinal cystoid spaces without foveal thickening and disruption of the inner segment/outer segment photoreceptor junction line. Foveal thinning was present in later stages.

Adult↗

PubMedDate not supplied

Cavernous hemangioma of the retina and retinal telangiectasis. Distinct or related vascular malformations?

Cavernous hemangioma of the retina and retinal telangiectasis are two rare developmental vascular lesions of the retina. They are considered to be distinct clinical entities, but they share some common features, so that the differential diagnosis of the two diseases is an important clinical challenge. In the right eye of a young woman, a cavernous hemangioma of the retina close to an area of retinal telangiectasis was found. This association supports the possibility that these developmental anomalies could be strictly related.

Adult↗

PubMed2002

Idiopathic juxtafoveolar retinal telangiectasis: case report and literature review.

BACKGROUND: Idiopathic Juxtafoveolar Retinal Telangiectasis (IJRT) is a retinal vascular anomaly, characterized by irregular dilation and incompetence of retinal vessels. Although the etiology of the condition is unknown, its various presentations have been characterized into three groups. Treatment includes laser photocoagulation and observation and is based on disease classification. CASE REPORT: The case report presents a patient with Group 2A, Stage 3 IJRT. The clinical appearance, differential diagnosis, and management of IJRT and its subgroups are discussed. CONCLUSION: Appropriate classification of IJRT is essential to the successful management of this condition.

Diagnosis, Differential↗

PubMed1999

Coats' disease of the retina (unilateral retinal telangiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal angiogenesis.

Coats' disease is characterized by abnormal retinal vascular development (so-called 'retinal telangiectasis') which results in massive intraretinal and subretinal lipid accumulation (exudative retinal detachment). The classical form of Coats' disease is almost invariably isolated, unilateral and seen in males. A female with a unilateral variant of Coats' disease gave birth to a son affected by Norrie disease. Both carried a missense mutation within the NDP gene on chromosome Xp11.2. Subsequently analysis of the retinas of nine enucleated eyes from males with Coats' disease demonstrated in one a somatic mutation in the NDP gene which was not present within non-retinal tissue. We suggest that Coats' telangiectasis is secondary to somatic mutation in the NDP gene which results in a deficiency of norrin (the protein product of the NDP gene) within the developing retina. This supports recent observations that the protein is critical for normal retinal vasculogenesis.

Amino Acid Substitution↗

PubMed1978

Focal parafoveal retinal telangiectasis.

We describe four patients, two of whom are sisters, with an unusual form of retinal telangiectasis. The vascular abnormality is localized to the temporal parafoveal retina and is virtually identical in appearance in every patient. Other characteristics include: both men and women are involved, both eyes are generally affected, and symptoms develop in middle life. Laser photocoagulation succeeds in improving the visual acuity in the treated eyes. The consistency of the appearance and location, and the familial tendency indicates these cases probably form a subgroup of retinal telangiectasis.

Adult↗

PubMed1997

Surgical removal of subfoveal neovascularization in idiopathic juxtafoveolar retinal telangiectasis.

PURPOSE: To investigate the efficacy of the surgical removal of subfoveal choroidal neovascularization in patients with type 2A idiopathic juxtafoveolar retinal telangiectasis. METHODS: Two patients with bilateral acquired idiopathic juxtafoveolar retinal telangiectasis and a subfoveal choroidal neovascular membrane underwent surgical excision of the membrane using standard subretinal surgical techniques. RESULTS: In both cases, surgical removal of the neovascular membrane was complicated by an intimate adherence of the membrane with the overlying neurosensory retina in an area of retinochoroidal anastomosis. A retinal dehiscence occurred in both instances at the time of surgery. Postoperative visual outcome was poor. CONCLUSION: The retinochoroidal anastomoses often visualized in cases of subretinal neovascularization complicating bilateral acquired IJFRT could indicate that there is a fairly marked adherence of the neovascular membrane to the neurosensory retina. Attempts at surgical removal of subretinal membranes in this disease entity with current techniques may be contraindicated.

Choroid↗

PubMed1993

Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study.

BACKGROUND: Idiopathic juxtafoveolar retinal telangiectasis may cause visual loss. The treatment of this disease is controversial. METHODS: The authors reviewed the records of 140 patients with idiopathic juxtafoveolar telangiectasis. A classification scheme based on biomicroscopic and fluorescein angiographic findings is presented. In addition, the effect of photocoagulation on the natural history of the disorder is evaluated. FINDINGS: Patients are categorized into three groups. Group 1 comprises 39 male patients with nonfamilial, easily visible telangiectasis and intraretinal exudation. The telangiectasis is unilateral in 94% of patients. The telangiectasia in this group is probably of developmental origin (Coats syndrome). Group 2 comprises 94 patients with occult juxtafoveolar telangiectasis, minimal exudation, superficial retinal crystalline deposits, and right-angle venules. Late in the course of the disease, foveolar atrophy, intraretinal pigment plaques, and subretinal neovascularization develop. The telangiectasis is acquired during middle age and is bilateral in 98% of patients. Group 3 comprises seven patients with bilateral easily visible telangiectasis, minimal exudation, and capillary occlusion. All of these patients had systemic disease, which was probably related to their eye disease. CONCLUSION: Slow visual loss beginning in adulthood characterizes most of these patients. The telangiectasis appears to be caused primarily by retinal capillary leakage in group 1, capillary diffusion abnormalities in group 2, and capillary occlusion in group 3. Photocoagulation is probably beneficial for patients in group 1 and not for patients in group 2, at least before their development of subretinal neovascularization.

Adolescent↗

PubMed2006

Iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis.

We describe a case of iris microhaemangiomas (iris vascular tufts) causing spontaneous hyphaema and where examination showed that the patient also had idiopathic juxtafoveolar retinal telangiectasis. A systematic search through PubMed, EMBASE and reference lists revealed a total of 90 reported cases of iris microhaemangiomas. To our knowledge, no previous reports have described concurrent findings of iris microhaemangiomas and idiopathic juxtafoveolar retinal telangiectasis in a patient. Both conditions are acquired vascular diseases, but their pathogenesis and aetiologies are unknown. Iris microhaemangiomas are important clinically because they are occasionally a cause of spontaneous hyphaema and may induce transient elevation of intraocular pressure.

Aged↗

PubMed1992

Severe visual loss associated with retinal telangiectasis and facioscapulohumeral muscular dystrophy.

Facioscapulohumeral (FSH) muscular dystrophy is known to be associated with retinal telangiectasis. However, there are only few reports of severe visual loss due to exudative complications, so the risk to vision has not been established. Because of the possible therapeutic implications, we have described two cases of young girls who developed FSH muscular dystrophy and exudative retinal detachment due to telangiectasis. In the first patient, the severity of the disease precluded visual recovery despite extensive photo- and cryotherapy. In the other, visual acuity in both affected eyes was retained after treatment. Fundus examinations in young children at risk of having the gene for FSH muscular dystrophy may be justified so that retinal vascular disease can be detected before it becomes untreatable.

Blindness↗

PubMedDate not supplied

Atypical retinitis proliferans, retinal telangiectasis, and vitreous hemorrhage in a patient with tuberous sclerosis.

This report describes an unusual case of recurrent vitreous hemorrhage and atypical retinal neovascularization in a patient with tuberous sclerosis. During three years of observation, the patient also developed retinal telangiectasis with macular edema and lipid exudation. Although the patient did not have an obvious astrocytic hamartoma, a diffuse, flat retinal hamartoma within the nerve fiber layer was suspected.

Adult↗

PubMed1993

Subretinal hemorrhage after grid laser photocoagulation for idiopathic juxtafoveolar retinal telangiectasis.

A 56-year-old man had decreased vision secondary to bilateral idiopathic juxtafoveolar retinal telangiectasis. One month after grid laser photocoagulation for macular edema in the right eye, a subretinal hemorrhage developed. Over the next 2 months, the hemorrhage spontaneously resorbed and vision improved to 20/50. At 2-year follow up, without further treatment, vision was still 20/50.

Fluorescein Angiography↗