Idiopathic Juxtafoveolar Telangiectasis Research Articles

Intravitreal anti-VEGF treatment for subretinal neovascularisation secondary to type 2 idiopathic juxtafoveolar telangiectasia.

To assess the long-term outcome of patients with subretinal neovascular membrane (SRNVM) secondary to type 2 idiopathic juxtafoveolar telangiectasia (IJT) receiving intravitreal anti-VEGF (vascular endothelial growth factor) injections. A total of 14 eyes of 12 patients treated with intravitreal anti-VEGF for SRNVM related to type 2 IJT were retrospectively assessed. Nine men and 3 women with a mean age of 66years (SD 12, range 47-87years) were diagnosed with IJT-related SRNVM. On average, 6.8 injections (SD 5.5, range 3-18) were given per eye. Ten eyes were treated with ranibizumab, 3 eyes with bevacizumab and 1 eye received both substances. The median follow-up after the last injection was 31months (IQR: 18, 48). In 6 eyes, BCVA improved by 1-4 lines (mean Δ +2.0 lines), 1 eye remained stable and 7 eyes showed decline of vision by 1-5 lines (mean Δ -2.1 lines). The baseline central foveal thickness was significantly reduced from a mean of 323 (SD 87) to 266µm (SD 71µm) at the last follow-up visit (p=0.001). SRNVM development is a severe complication of type 2 IJT. Since the establishment of intravitreal anti-VEGF treatment laser coagulation and PDT have lost significance. Intravitreal anti-VEGF therapy seems to be safe and effective for the treatment of IJT-related SRNVM. Frequently multiple intravitreal injections are necessary for stabilisation.

Idiopathic Juxtafoveolar Telangiectasis Type 1A. A Case Report

No Abstract AvailableIdiopathic juxtafoveolar telangiectasisis a descriptive term for various disease entities presenting with incompetence, ectasia, and/or irregular dilations of the capillary network affecting only the juxtafoveolar region of one or both eyes. A 65 years male presented with the chief complaint of painless progressive diminution of vision in his left eye for 6 months' duration. Past ocular, surgical, medical, drug and family history was non contributory. The patient was non hypertensive and non diabetic. His best corrected visual acuity was 6/60 right eye and 6/36 left eye. Right eye, fundus was grossly normal whereas, on the left eye, few microaneurysms and circinate pattern of hard exudates were noticed on the perifoveolar region. Fundal reflex was dull and the foveal depression was absent indicating thickening. Fluorescein angiography showed clusters of telangiectatic vessels around exudates, hyperfluorescent dots of microaneurysms with a circinate pattern of leakage in the late phase. Optical coherence tomography showed macular thickening of 487 micrometer with cystoid changes. Injection Bevacizumab 0.2ml was given after final diagnosis of Left Eye Idiopathic Juxtafoveolar Telangiectasis Type 1A. Three types of idiopathic juxtafoveolar telangiectasis has been defined. Its pathophysiology is also less understood and the treatment modalities are not established yet.This case was an incidental finding, as these patients do not oftenly have profound diminution of vision, unless neovascularization has occurred and the clinical features too are very subtle.DOI: http://dx.doi.org/10.3126/jucms.v2i3.11827 Journal of Universal College of Medical Sciences Vol.2(3) 2014: 38-40

Subretinal neovascular membrane associated with type 2a idiopathic juxtafoveolar telangiectasis in pseudoxanthoma elasticum

The objective was to report a case of pseudoxanthoma elasticum associated with type 2A idiopathic juxtafoveolar telangiectasis (T2AIJT) complicated by bilateral subretinal neovascular membranes (SRNVM). The right eye with juxtafoveal SRNVM and the left eye with subfoveal SRNVM underwent laser photocoagulation and photodynamic therapy respectively. The follow-up duration was 48 months. Best corrected visual acuity was 20/32 in both eyes at baseline, and 20/32 in the right eye and 20/200 in the left eye at the end of the follow-up. Atrophic changes in the retinal pigment epithelium developed around the SRNVM treated with photodynamic therapy (PDT). Pseudoxanthoma elasticum may be associated with T2AIJT complicated by bilateral SRNVM. Further studies are needed to assess both efficacy and appropriate PDT regime in T2AIJT-related SRNVM, especially if associated with other conditions.

Optical Coherence Tomography in Group 2A Idiopathic Juxtafoveolar Telangiectasis

To describe the optical coherence tomographic features in patients with group 2A idiopathic juxtafoveolar telangiectasis. Forty eyes of 20 consecutive patients with idiopathic juxtafoveolar telangiectasis seen between August 2002 and January 2004 were included in the study. All of the patients underwent color fundus photography, fluorescein angiography, and optical coherence tomography. The main outcome measure was optical coherence tomography findings. The most consistent finding between stages 2 through 5 of group 2A idiopathic juxtafoveolar telangiectasis seen in 35 (87.5%) eyes on optical coherence tomography was the presence of hyporeflective intraretinal spaces in the absence of retinal thickening. Other findings included the presence of hyperreflectivity in the middle or inner retinal layers, suggesting retinal pigment epithelium proliferation and migration corresponding to the stellate foci of pigmentation in stage 4 and features of choroidal or subretinal neovascular membrane in stage 5. The optical coherence tomography findings in group 2A idiopathic juxtafoveolar telangiectasis were characteristic and may be helpful in making the diagnosis and defining, as far as possible, the anatomical staging.

Transpupillary thermotherapy for subfoveal neovascularization secondary to group 2A idiopathic juxtafoveolar telangiectasis

Purpose To evaluate the efficacy and safety of transpupillary thermotherapy (TTT) for subfoveal neovascularization (SRNVM) in patients with group 2A Idiopathic Juxtafoveolar Telangiectasis (IJFT). Design Nonrandomized interventional case series. Methods We performed TTT for subfoveal SRNVM in 14 eyes of 13 patients with group 2A IJFT, who were referred to our tertiary care center. We evaluated visual outcome and SRNVM closure rate in these patients. Results After a mean follow-up period of 8.65 months, 92.3% of treated eyes had stabilization or improvement in visual acuity as well as regression of SRNVM by fluorescein angiography (FA). One SRNVM showed persistent leakage. One patient worsened by more than 2 Snellen lines; one required retreatment. Conclusion Transpupillary thermotherapy may be a safe and useful alternative treatment option for patients with group 2A IJFT with subfoveal SRNVM.

Neovascular membranes associated with idiopathic juxtafoveolar telangiectasis.

To report the visual outcome in patients with a neovascular membrane (NVM) associated with idiopathic juxtafoveolar telangiectasis (IJFT). We performed a retrospective, noncomparative analysis of 26 eyes of 16 patients with an NVM associated with bilateral IJFT (Gass classification group 2A). Eyes were divided into 2 groups: group WO (n = 11) included eyes with IJFT without evidence of an NVM on initial examination; eyes in group W (n = 15) had an NVM at the initial diagnosis of IJFT. In group WO, the initial visual acuity and the time between the initial examination to the diagnosis of an NVM were evaluated. Characteristic fundus findings, including the presence or absence of a chorioretinal anastomosis, intraretinal pigmentary plaques, and crystalline deposits, as well as the final visual acuity were reviewed for both groups. The initial visual acuity for eyes in group WO ranged from 20/20 to 20/70 (median, 20/30); in group W, from 20/20 to 4/200 (median, 20/70). The average time from initial diagnosis of IJFT to the development of an NVM was 73 months (range, 5-142 months). In group WO, chorioretinal anastomosis and concurrent perivascular retinal pigment epithelial hyperplasia were observed before the development of an NVM. The final visual acuity for all eyes ranged from 20/40 to 2/200 (median, 20/200). Eighty-one percent of eyes (21/26) had a final visual acuity of 20/200 or worse. The stable final visual acuity in patients with an NVM associated with IJFT is generally poor, with 80% of eyes in this series having a final visual acuity of l20/200 or worse. In patients with IJFT, the presence of a chorioretinal anastomosis and retinal pigment epithelial hyperplastic plaques always preceded the development of an NVM.

Idiopathic Juxtafoveolar Retinal Telangiectasis: Clinical Pattern in 19 Cases

Background: Idiopathic juxtafoveolar telangiectasis (IJT) is a rare retinal vasculopathy of adulthood that may cause central loss of visual acuity. The purpose of this paper is to evaluate the type and distribution of fundus changes in patients with this disorder. Methods: Medical records of 19 patients with IJT using the Gass & Blodi classification were studied. Eight patients in group 1A (42%) and 11 in group 2A (58%) were analyzed. By projecting color photographs we divided the macula into four quadrants and studied the fundus changes in each one. Results: All patients in group 1A presented unilateral disease with easily seen telangiectasis, lipid deposits and cystic macular edema. The mean age of this group was 55.8 years. Five (63%) patients were male and 3 (37%) were female. All patients in group 2A, 2 males (18%) and 9 females (82%), had bilateral disease evidenced by fluorescein angiography. The mean age of this group was 54.5 years. Fundoscopic findings included: intraretinal pigment plaques, grayish coloring of the macular retina, superficial crystalline deposits, right-angle venules, subretinal and intraretinal neovascularization. Conclusions: All patients in this survey were adults. In group 1A, men were affected 2 times more often than women, and the main affected macular quadrant was the inferior temporal, followed by the inferior nasal. In group 2A, women were affected 4 times more often than men and all eyes had inferior and superior temporal macular quadrant involvement.

Bilateral juxtafoveolar telangiectasis in monozygotic twins

PURPOSE: To report the clinical and angiographic features of two monozygotic twins affected by bilateral group 2 idiopathic juxtafoveolar telangiectasis. METHOD: Case reports. RESULTS: Two 64-year-old women, who were identical twins, were suffering from visual loss. One twin had suffered from visual loss for 1 year and had a visual acuity of 20/25 in both eyes, and the other twin had suffered for 2 years and had a visual acuity of 20/30 in both eyes. Fluorescein angiography disclosed similar fundus features. An analogous area of capillary telangiectasis and leakage was observed in the right macula, where no intraretinal pigment was seen; the left macula showed a similar amount of intraretinal pigment associated with tiny right-angle venules and minimal leakage. CONCLUSION: This observation raises the issue of genetic influences in the pathogenesis of this disease.

Idiopathic Juxtafoveolar Retinal Telangiectasis: Update of Classification and Follow-up Study

Idiopathic juxtafoveolar retinal telangiectasis may cause visual loss. The treatment of this disease is controversial. 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. 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. 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.

Foveal Avascular Zone in Idiopathic Juxtafoveolar Telangiectasia

We measured angiographically the foveal avascular zone (FAZ) in 26 subjects with idiopathic juxtafoveolar telangiectasia (IJFT) and 27 control subjects. Quantitatively, FAZ was significantly smaller in IJFT (median = 0 mm2) than in controls (median = 0.405 mm2). Qualitatively, the telangiectasia in IJFT was temporal (92.5% of eyes) and appeared as microaneurysms interconnected with several capillaries, spider-shaped microaneurysms, or Coats'-like telangiectasia. IJFT is characterized by temporal telangiectasia and small FAZ.