Idiopathic Juxtafoveolar Retinal Telangiectasis Research Articles

Role of focal laser ablation in the treatment of type 1 idiopathic juxtafoveolar retinal telangiectasis

Introduction 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 is based mainly on disease classification and includes laser photocoagulation and observation besides other modalities. Aim The aim of the work was to study the role of focal laser treatment for type 1 IJRT. Patients and methods A retrospective noncomparative study was carried out at the Ophthalmology Department, Menoufia University, Egypt, during the period from January 2007 to December 2011, wherein four patients (three male and one female) diagnosed with type 1 (exudative) IJRT on the basis of fluorescein angiography and after ruling out other causes were included in the study. Four eyes with macular edema and exudation were subjected to focal laser treatment (double-frequency YAG) with green laser (wavelength 532 nm). The follow-up period after laser treatment extended from 6 to 24 months. Results Four patients diagnosed with type 1 IJRT were included in the study (three male and one female). Their age ranged from 40 to 42 years, with a mean age of 40.75 years. Focal double-frequency YAG green laser (wavelength 532 nm) treatment resulted in the reduction of both macular edema and exudation with improvement in visual acuity from one to two lines on follow-up. Conclusion Focal laser treatment of type 1 IJRT lesions proved to be effective. The treatment is a safe method for managing leakage and stabilizing vision.

Fundus autofluorescence patterns in type 2A idiopathic juxtafoveolar retinal telangiectasis

To describe fundus autofluorescence (FAF) patterns in patients with type 2A idiopathic juxtafoveolar retinal telangiectasia (IJRT). We reviewed FAF images, color photographs, and fluorescein angiography (FA) images of 30 eyes from 16 patients with type 2A IJRT. Eyes with presence of subretinal neovascularization or any other retinal pathology were excluded. All the imaging modalities were obtained with a Heidelberg Retina Angiograph (HRA) confocal laser scanning system. The mean age of the patients was 50.7 years, 68.5% female. At baseline, the median best-corrected visual acuity was 20/50. Loss of normal foveal hypoautofluorescence was noted in 93.3% of eyes. All the eyes showed hypoautofluorescence corresponding to intraretinal crystals and pigment clumps. Increased FAF around the pigments was noted in 93.3% of eyes. Increased FAF corresponding to the angiographic leakage from telangiectatic and nontelangiectatic areas was noted in 86.6% of eyes and 80% of eyes, respectively. Loss of foveal hypoautofluorescence and increased FAF corresponding to the nontelangiectatic angiographic leakage areas were predominant features. Fundus autofluorescence patterns corresponding to color photography and FA findings may further add to the understanding of morphologic alterations in type 2A nonproliferative IJRT at early stages.

Retinal Pseudocysts in Age-Related Geographic Atrophy

To report the presence of pseudocysts in retinal layers of eyes with geographic atrophy (GA) attributable to age-related macular degeneration (AMD) and to estimate their prevalence. Retrospective study. setting: Clinical practice. patients: Consecutive patients with GA, assessed by spectral-domain optical coherence tomography (OCT). main outcome measures: Assessment of pseudocyst prevalence in retinal layers. Statistical analysis by the chi(2) test, Fisher exact test, Mann-Whitney U test, and Cramer test, performed to explore links between the presence of pseudocysts and demographic features and/or pattern of atrophy, ie, horseshoe, homogeneous area, homogeneous area or patchy atrophy with foveal sparing, and patchy atrophy. Eighty-eight eyes of 68 GA patients aged between 61 and 94 years (mean: 79.8) were examined. In 20 patients, GA was bilateral. Twenty-four eyes (27.2%) exhibited pseudocysts corresponding to small cystoid spaces frequently located in the inner nuclear layer of the retina. There was no macular edema. Fluorescein angiography, performed in 71 eyes (80%), ruled out possible choroidal neovascularization. No correlation was found between 1) patients' age (P = .7) or gender (P > .99) and the presence of pseudocysts or 2) patterns of atrophy (Cramer test: V = 0.183) and the presence of pseudocysts. Pseudocysts seemed to be frequent in atrophic AMD. They might correspond to Müller cell degeneration, as suspected in other degenerative retinal disorders like tamoxifen retinopathy or group 2A idiopathic juxtafoveolar retinal telangiectasis. The present results indicate that pseudocysts are frequently seen on OCT in eyes with atrophic AMD and their presence should not be considered as a manifestation of neovascular AMD that requires prompt treatment.

INTEGRATED CLINICAL EVALUATION OF TYPE 2A IDIOPATHIC JUXTAFOVEOLAR RETINAL TELANGIECTASIS

To describe spectral high-resolution optical coherence tomography (OCT) findings correlated with fundus autofluorescence (FAF), confocal blue reflectance (CBR), and fluorescein angiography (FA) patterns in patients affected by Type 2A idiopathic juxtafoveolar retinal telangiectasis. We reviewed OCT, FA, CBR, and FAF images of both eyes from 11 patients affected by Type 2A idiopathic juxtafoveolar retinal telangiectasis. One eye had Stage 2 disease, 13 eyes had Stage 3 disease, 5 eyes had Stage 4 disease, and 3 eyes had Stage 5 disease. All the imaging modalities were obtained with a combined instrument for confocal scanning laser ophthalmoscopy and Spectral-domain OCT. Simultaneous acquisition of OCT images with FA, CBR, and FAF images was achieved with perfect point-to-point correspondence. Increased parafoveal CBR was observed in all but 2 eyes that had Stage 5 disease. A correspondence of hyperautofluorescence on FAF and anatomical localization of the intraretinal cyst was observed in the 16 eyes where the cyst was visible by OCT. All eyes showed some alterations of the plexiform layers. A correspondence between increased CBR and late-phase FA hyperfluorescence occurred in the 4 eyes with focal and oval CBR pattern, whereas none of the 10 eyes with ring-pattern lesions had this correspondence. Patterns of increased CBR corresponded to areas of increased FAF in 94% of eligible eyes. The simultaneous acquisition of Spectral-domain OCT and reference images (FA, FAF, and CBR) with point-to-point correlation may further add to the understanding of morphologic alterations in Type 2A idiopathic juxtafoveolar retinal telangiectasis.

Idiopathic juxtafoveolar retinal telangiectasis: A current review

Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

Obliterative idiopathic juxtafoveolar telangiectasis: a 25-year follow-up.

To report findings over 25 years of follow-up on a case of bilateral obliterative idiopathic juxtafoveolar retinal telangiectasis. Case report. A 25-year-old white man with obliterative idiopathic juxtafoveolar retinal telangiectasis presented with a visual acuity of 20/30 in the right eye and 20/40 in the left. Fluorescein angiography revealed bilateral enlarged foveal avascular zones and late perifoveal leakage. Six years after presentation, the foveal avascular zone had enlarged only slightly in both eyes and the leakage had almost entirely subsided. For the next 19 years, the visual acuity and clinical findings have remained unchanged. His most recent visual acuity was 20/25 in the right eye and 20/40 in the left. No treatment was given throughout the entire follow-up period of 25 years. Obliterative idiopathic juxtafoveolar retinal telangiectasis is a rare disorder that may initially progress with subsequent stabilization and good long-term visual outcome.

Early morphological changes and functional abnormalities in group 2A idiopathic juxtafoveolar retinal telangiectasis using spectral domain optical coherence tomography and microperimetry

Aims:To report early morphological changes and functional abnormalities in group 2A idiopathic juxtafoveolar retinal telangiectasis (IJRT) using spectral domain optical coherence tomography (SD-OCT) and microperimetry.Methods:Six eyes (three patients; average age,...

Idiopathic juxtafoveolar retinal telangiectasis in monozygotic twins

Monozygotic twins (aged 63 years old), with group-2A idiopathic juxtafoveolar retinal telangiectasia (JXT), underwent clinical examination, fluorescein angiography (FA) and optical coherence tomography (OCT). Twin 1 : Right fundus showed...

Behandlung idiopathischer juxtafoveolärer retinaler Teleangiektasien mit Bevacizumab (Avastin)

Idiopathic juxtafoveolar retinal telangiectasis presents areas of occult capillary telangiectasis involving the temporal half of the fovea. Its treatment by laser photocoagulation, photodynamic therapy or intravitreal injection of triamcinolone is unsatisfying concerning visual acuity. Recently it has been demonstrated that intravitreal bevacizumab injection could be successful in the treatment of choroidal neovascularisations secondary to ARMD. The principle of this treatment may be successful for the treatment of idiopathic juxtafoveolar retinal telangiectasis as well. We report about 3 cases treated with three monthly intravitreal injections of bevacizumab. Fluorescein angiographies and OCT measurements of retinal thickness were performed. The mean increase of VA was 2 visual steps. In two cases an increase of 5 and 1 visual steps, respectively, could be determined, in the other case no increase was found. The mean retinal thickness decreased from 300 microns to 287 microns. According to our results bevacizumab seems to be less suitable for treatment of idiopathic juxtafoveolar retinal telangiectasis because only a small decrease of retinal thickness can be observed.

Optical Coherence Tomography in Group 2A Idiopathic Juxtafoveolar Retinal Telangiectasis

To describe the changes observed with optical coherence tomography in group 2A idiopathic juxtafoveolar retinal telangiectasis. 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. 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. 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.

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.

Morphologic Features of Group 2A Idiopathic Juxtafoveolar Retinal Telangiectasis in Three-Dimensional Optical Coherence Tomography

To investigate morphologic features of group 2A idiopathic juxtafoveolar retinal telangiectasis (IJRT) with three-dimensional optical coherence tomography (OCT). Observational case series. We performed OCT ophthalmoscopy on six eyes of three consecutive patients with group 2A IJRT. Thin cystic low-intensity space within inner and outer retinal layers was detected with the longitudinal B-scan through the fovea in five eyes. Partial loss of highly reflective line considered as the boundary between photoreceptor inner segments and outer segments was also detected. The transverse C-scan provided en-face images of the low-intensity space shown with the B-scan, if not entirely. These morphologic alterations appeared not to correspond with the staining observed in the late phases of fluorescein angiography. The OCT ophthalmoscope could visualize morphologic alterations indicating degeneration or atrophy of neurosensory retina, including photoreceptor layers. These alterations may play a role in the pathogenesis of group 2A IJRT.

Intravitreal bevacizumab (Avastin) associated with the regression of subretinal neovascularization in idiopathic juxtafoveolar retinal telangiectasis

To describe the clinical, angiographic, and optical coherence tomography findings of a patient with subretinal neovascularization (SRN) in idiopathic juxtafoveolar retinal telangiectasis (IJRT) treated with intravitreal bevacizumab injection. In the setting of a tertiary referral center, a patient with bilateral acquired IJRT, complicated with juxtafoveal SRN in one eye, was treated with a single intravitreal injection of 1.5 mg of bevacizumab and then prospectively followed for 24 weeks. Vision improved from 20/40(-1) to 20/20(-2) by 24 weeks of follow-up. Fluorescein angiography demonstrated the absence of leakage from a previously active SRN. Optical coherence tomography revealed gradual neovascular lesion contraction and the resolution of subretinal fluid over the 24-week follow-up period. No obvious adverse events were noted. In the short-term, intravitreal bevacizumab treatment resulted in partial restoration of the macular architecture and vision improvement by means of neovascular lesion regression and the resolution of associated serohemorrhagic complications.

Photodynamic therapy for subretinal neovascularization in type 2A idiopathic juxtafoveolar telangiectasis

Background: Photodynamic therapy (PDT) with verteporfin is now the standard of care worldwide for the treatment of choroidal neovascularization, but has been used only rarely in those with subretinal neovascular membranes (SRNVM) due to type 2A idiopathic juxtafoveolar retinal telangiectasis (IJT). We performed a retrospective study to examine the outcome of patients treated with PDT for SRNVM secondary to IJT. Methods: Retrospective interventional case series of 7 eyes of 6 IJT patients with SRNVMs treated with PDT. Ophthalmic examination and fluorescein angiography were performed before treatment, with retreatment every 3 months as needed. The main outcome was the proportion of patients avoiding vision loss (change of ± 4 letters, or better). Results: Baseline Snellen acuity ranged from 20/40 to 20/400 (median 20/80). Mean follow-up was 21 months. Patients received 2.4 treatments on average. Five of 7 patients avoided vision loss; acuity improved in 3 eyes (≥ 1 line improvement), stayed the same in 2 eyes (± 4 letters) and decreased in 2 eyes (≥ 1 line decrease) over time. Median final acuity was 20/80. Five of 7 eyes had final acuities of ≥ 20/200. No leakage was observed in any eyes following cessation of treatment. Interpretation: Previously reported outcomes for SRNVM in type 2A IJT, in both natural history and after laser or surgical treatment, have been uniformly poor. PDT may be considered for these patients due to its excellent safety profile and clinically favorable outcomes in this study.

Group 2A idiopathic juxtafoveolar retinal telangiectasia in monozygotic twins

To report the clinical and fluorescein angiographic characteristics of monozygotic twins affected by group 2A idiopathic juxtafoveolar retinal telangiectasia. Observational case report. Both eyes of identical twins were examined in a clinical practice setting. Two 68-year-old Caucasian, monozygotic, female twins were examined. In one twin, visual acuity was 20/50 in the right eye (OD) and 20/40 in the left (OS). Ophthalmoscopy demonstrated right-angle venules without edema in both eyes (OU). Fluorescein angiography demonstrated areas of leakage temporal to the fovea OU with some nasal leakage OS. In her identical twin sister, with type II diabetes, visual acuity was 20/60 OD and 20/25 OS. Right-angle venules OU, refractile deposits OS, and no macular edema OU were present. Fluorescein angiography demonstrated areas of parafoveal leakage temporal and nasal OD and temporal OS. This is the second set of female monozygotic twins with group 2A idiopathic juxtafoveolar retinal telangiectasia reported in the literature. This finding suggests a genetic component in the pathogenesis of this retinal vascular disease.

Photodynamic therapy of subfoveal neovascular membrane in type 2A idiopathic juxtafoveolar retinal telangiectasis

Purpose To evaluate the effect of photodynamic therapy on subfoveal neovascular membrane related to type 2A idiopathic juxtafoveolar retinal telangiectasia. Design Interventional case series. Methods Retrospective review of four eyes of four patients who underwent photodynamic therapy for subfoveal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasia. Ocular photodynamic therapy with verteporfin was performed in all cases using standard protocols. Results are given in terms of final visual acuity and neovascular membrane activity based on clinical examination, fluorescein and indocyanin green angiography, and, in two cases, optical coherence tomography. Results Baseline visual acuity of 20/30 and 20/40 (×2) was maintained in three patients after one, two, and three sessions of photodynamic therapy respectively, and a follow-up of 23, 21, and 9 months. Leakage specific to the subfoveal neovascular membrane ceased on the fluorescein angiography. In the other patient, the final vision decreased from 20/50 to 20/200 after four sessions of photodynamic therapy and a follow-up of 14 months. Although there was still mild persistent leakage on the fluorescein angiography, neovascular membrane size was unchanged, and no subretinal fluid was demonstrated on optical coherence tomography. Conclusions Data from this case series suggest that photodynamic therapy may be effective in managing subfoveal neovascular membrane associated with idiopathic juxtafoveolar retinal telangiectasia, which usually carries a poor visual prognosis. Prospective study is required to confirm the beneficial effect of this treatment.

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.

Idiopathic juxtafoveolar retinal telangiectasia: a review and case report

Idiopathic juxtafoveolar retinal telangiectasis is a group of retinal vascular anomalies characterized by retinal vessel dilation and tortuosity, multiple aneurysm formations, varying degrees of vascular leakage and lipid exudate deposition. Idiopathic juxtafoveolar retinal telangiectasis may occur as a primary disorder (either congenital or acquired), or may be caused or accompanied by other systemic or ocular diseases. The visual prognosis and effectiveness of therapy is dependent upon the etiology of the retinal telangiectasis. Included in this review is a case report, as well as the classification system used to identify idiopathic juxtafoveolar retinal telangiectasia.

Laser Photocoagulation and Glucose Metabolism in Idiopathic Juxtafoveolar Retinal Telangiectasis

Laser Photocoagulation and Glucose Metabolism in Idiopathic Juxtafoveolar Retinal Telangiectasis

Bilateral idiopathic juxtafoveolar retinal telangiectasis

Bilateral idiopathic juxtafoveolar retinal telangiectasis (JRT) is a condition of unknown etiology characterized by telangiectatic retinal vessels located temporal to the fovea. Most patients with bilateral JRT have associated telangiectasis, dilated capillaries, minimal exudation, retinal crystals, right angle venules, and retinal pigment hyperplasia. An unusual case of bilateral retinal telangiectasis with associated asymmetric cystoid macular edema in a middle-aged female is presented. This case is unusual due to the presence of cystoid macular edema. Classification, differential diagnosis and management of idiopathic juxtafoveolar retinal telangiectasis are reviewed.