Long-term efficacy and complications of intravitreal anti-vascular endothelial growth factor agents combined with ablative therapies in juvenile Coats disease: a five year follow-up study.

PURPOSE: To evaluate the long-term safety and efficacy of adjuvant intravitreal anti-VEGF therapy in juvenile Coats disease. METHODS: This retrospective, observational study included a total of 62 eyes in 62 pediatric patients with juvenile Coats disease treated with intravitreal anti-VEGF agents followed for a mean of 67.08 months (ranged from 60 to 93 months). All affected eyes were managed initially with one session of ablative treatment plus adjuvant intravitreal anti-VEGF agent (0.5 mg/0.05 ml ranibizumab or conbercept). Ablative treatment was repeated if telangiectatic retinal vessels were not completely regressed or recurred. Anti-VEGF therapy was repeated if subretinal fluid or macular edema still existed. Treatments above were repeated every 2 to 3 months. We reviewed clinical and photographic records of patients including the demographics, clinical characteristics and interventions. RESULTS: At final visit, all 62 affected eyes had partially or completely disease resolution; none progressed to advanced stage namely neovascular glaucoma or phthisis bulbi, respectively. No ocular or systemic side effects related to intravitreal injections were observed during follow-up. In terms of 42 affected eyes that could cooperate with visual examination, best corrected visual acuity improved in 14 (14/42, 33.3%) eyes, stabled in 25 (25/42, 59.5%) eyes, and worsened in 3 (3/42, 7.1%) eyes. In the field of complications, 22 (22/62, 35.5%) eyes developed cataracts; 33 (33/62, 53.2%) eyes developed vitreoretinal fibrosis, of whom 14 (14/33, 42.4%) eyes in the subgroup of stage 3B developed progressive TRD; 40 (40/62, 64.5%) eyes developed subretinal fibrosis. Multivariate regression analysis showed increased clinical stage may be associated with the development of vitreo- and subretinal fibrosis (adjusted odds ratio:16.77,17.59; 95% CI:4.50-62.53, 3.98-77.86, respectively, all P < 0.001). CONCLUSION: Adjuvant intravitreal ranibizumab or conbercept combined with ablative therapies may be a long-term safe and effective treatment for juvenile Coats disease.

Tamoxifen induced maculopathy presenting like macular telangiectasia type 2 in a patient with breast cancer.

INTRODUCTION: Drug-induced crystalline maculopathy has been reported secondary to tamoxifen use for breast cancer treatment. It could be misdiagnosed as macular telangiectasia type 2 (MacTel type 2). CASE REPORT: A 56-year-old woman with a history of diabetes mellitus and breast cancer was referred to our clinic with painless, bilateral, gradual onset of central vision loss for several months. The fundus examination showed the macular pigmentary change in both eyes and a few refractile crystalline deposits in the parafoveal area in the left eye. However, the rest of the retina was normal in both eyes. MANAGEMENT AND OUTCOME: With the diagnosis of tamoxifen-induced maculopathy, the drug was discontinued and supplementary treatment was started. DISCUSSION: In this report, patient medical and drug history was an important and powerful measure. Due to the side effects of long-term use of tamoxifen, we need further studies on the need for retinal screening in these patients.

One-year follow-up of optical coherence tomography angiography microvascular findings: macular telangiectasia type 2 versus tamoxifen retinopathy.

PURPOSE: To compare microstructural and microvascular changes in eyes with macular telangiectasia type 2 (MacTel2) and in those with tamoxifen retinopathy (TR) at baseline and at the 1-year follow-up using optical coherence tomography (OCT) and OCT angiography (OCTA). METHODS: We followed up patients diagnosed with MacTel2 or TR for at least 1 year. We included 17 patients with MacTel2 (31 eyes) and 15 with TR (25 eyes) who discontinued tamoxifen use after a TR diagnosis. We performed OCT and OCTA at baseline and after 1 year. RESULTS: Patients with MacTel2 and TR showed intraretinal cavitation, ellipsoid zone (EZ) loss, and capillary telangiectasia in the superficial and deep plexuses. EZ disruption predominantly affected the temporal region in MacTel2 (32%) and was limited to the foveal center in TR (24%). Vascular density (VD) was significantly reduced within the deep temporal parafovea and superficial fovea in MacTel2 and TR eyes, respectively. After 1 year, the MacTel2 eyes showed enlarged EZ loss, proliferative vascular invasion, and increased VD (p = 0.021) in the temporal deep plexus compared with TR eyes. CONCLUSIONS: After 1-year follow-up, the MacTel2 eyes showed proliferative vascular remodeling, particularly in the temporal parafovea of the deep plexus with EZ loss progression, whereas the TR eyes maintained their baseline capillary rarefaction.

Plentiful melanin pigment containing histiocyte-like cells in Coats disease: Awareness avoids diagnostic pitfall.

Coats disease is an exudative retinal vasculopathy characterised by presence of yellow-golden deposits in the retina and retinal detachment. Subretinal fluid drainage performed as a part of therapeutic management makes the fluid amenable to cytological examination. Infection by Toxoplasma may closely simulate the ocular symptoms seen in Coats disease. Awareness of the cytological findings in Coats disease helps to clinch accurate diagnosis. We herein present a case of Coats disease with many histiocyte-like cells with plentiful intracytoplasmic melanin pigment in cytology smears from subretinal fluid, where cytological diagnosis was challenging and a correct diagnosis was made with the aid of ancillary techniques.

Beyond Performance Metrics: Automatic Deep Learning Retinal OCT Analysis Reproduces Clinical Trial Outcome.

PURPOSE: To validate the efficacy of a fully automatic, deep learning-based segmentation algorithm beyond conventional performance metrics by measuring the primary outcome of a clinical trial for macular telangiectasia type 2 (MacTel2). DESIGN: Evaluation of diagnostic test or technology. PARTICIPANTS: A total of 92 eyes from 62 participants with MacTel2 from a phase 2 clinical trial (NCT01949324) randomized to 1 of 2 treatment groups METHODS: The ellipsoid zone (EZ) defect areas were measured on spectral domain OCT images of each eye at 2 time points (baseline and month 24) by a fully automatic, deep learning-based segmentation algorithm. The change in EZ defect area from baseline to month 24 was calculated and analyzed according to the clinical trial protocol. MAIN OUTCOME MEASURE: Difference in the change in EZ defect area from baseline to month 24 between the 2 treatment groups. RESULTS: The difference in the change in EZ defect area from baseline to month 24 between the 2 treatment groups measured by the fully automatic segmentation algorithm was 0.072±0.035 mm2 (P = 0.021). This was comparable to the outcome of the clinical trial using semiautomatic measurements by expert readers, 0.065±0.033 mm2 (P = 0.025). CONCLUSIONS: The fully automatic segmentation algorithm was as accurate as semiautomatic expert segmentation to assess EZ defect areas and was able to reliably reproduce the statistically significant primary outcome measure of the clinical trial. This approach, to validate the performance of an automatic segmentation algorithm on the primary clinical trial end point, provides a robust gauge of its clinical applicability.

Levels of cytokines in the aqueous humor guided treatment of refractory macular edema in adult-onset coats' disease.

BACKGROUND: Two cases with refractory macular edema secondary to adult-onset Coats' disease underwent unsatisfactory treatment by intravitreal injections of anti-vascular endothelial growth factor (VEGF) drugs and retinal photocoagulation. CASE PRESENTATION: The authors highlight the guiding effect of the measurement of cytokines in the aqueous humor for the treatment of adult-onset Coats' disease with refractory macular edema. In the two cases, typical Coats' disease changes, including telangiectasis, subretinal exudation and macular edema were observed. Initial treatment consisted of intravitreal anti-VEGF drugs and retinal laser photocoagulation; however, the response was poor. Then, the aqueous humor was acquired and the cytokine concentrations were measured (Flow Cytometry Analysis, Beijing Giantmed Medical Diagnostics Lab). When the cytokine levels were tested every time there would be quality control, with a fixed concentration of cytokines samples to detect before the results reported. A low level of VEGF and a high level of inflammatory cytokines were found. Then, treatment was switched to intravitreal injection of dexamethasone implant (Ozurdex®) (Allergan, Inc., Irvine, Calif., USA), which resulted in resolution of the refractory macular edema and improvement of visual acuity in both cases. CONCLUSIONS: For refractory macular edema secondary to adult-onset Coats' disease, measurement of the levels of VEGF and inflammatory cytokines can help clinic doctors precisely investigate the molecular mechanism of macular edema and thereby find a suitable treatment.

Case report: internal limiting membrane drape sign masking by foveal detachment in macular telangiectasia type 2.

BACKGROUND: Internal limiting membrane (ILM) drape sign is an important OCT characteristic of Macular telangiectasia type 2 (MacTel 2). Described here is a case where masking of the ILM drape sign occurred with bilateral foveal detachments in a patient with MacTel 2. CASE PRESENTATION: A 64-year old female was diagnosed with MacTel 2, four years prior to the current presentation on the basis of an OCT demonstrating bilateral ILM drape sign. Fluorescein angiography showed bilateral dilated, ectatic capillaries and late phase dye leak. At the current presentation there was bilateral gradual visual impairment over two months due to bilateral foveal detachments. Treatment with intravitreal Bevacizumab resulted in unmasking of the pre-existing ILM drape sign at 12 weeks. Visual acuity was reduced to counting fingers in the left eye with the neovascular membrane as a consequence of sub-retinal fibrosis, while the right eye maintained a vision of 6/12. A difference in the stage of the disease at presentation determined the long-term visual outcome after seven years of observation. CONCLUSION: Foveal detachment can influence the OCT detectability of pre-existing foveal cystoid lesions. Visual prognosis at the final follow up was consistent with the interocular disparity of the disease stage at presentation.

ALK1 Loss Results in Vascular Hyperplasia in Mice and Humans Through PI3K Activation.

OBJECTIVE: ALK1 (activin-receptor like kinase 1) is an endothelial cell-restricted receptor with high affinity for BMP (bone morphogenetic protein) 9 TGF-ß (transforming growth factor-ß) family member. Loss-of-function mutations in ALK1 cause a subtype of hereditary hemorrhagic telangiectasia-a rare disease characterized by vasculature malformations. Therapeutic strategies are aimed at reducing potential complications because of vascular malformations, but currently, there is no curative treatment for hereditary hemorrhagic telangiectasia. APPROACH AND RESULTS: In this work, we report that a reduction in ALK1 gene dosage (heterozygous ALK1+/- mice) results in enhanced retinal endothelial cell proliferation and vascular hyperplasia at the sprouting front. We found that BMP9/ALK1 represses VEGF (vascular endothelial growth factor)-mediated PI3K (phosphatidylinositol 3-kinase) by promoting the activity of the PTEN (phosphatase and tensin homolog). Consequently, loss of ALK1 function in endothelial cells results in increased activity of the PI3K pathway. These results were confirmed in cutaneous telangiectasia biopsies of patients with hereditary hemorrhagic telangiectasia 2, in which we also detected an increase in endothelial cell proliferation linked to an increase on the PI3K pathway. In mice, genetic and pharmacological inhibition of PI3K is sufficient to abolish the vascular hyperplasia of ALK1+/- retinas and in turn normalize the vasculature. CONCLUSIONS: Overall, our results indicate that the BMP9/ALK1 hub critically mediates vascular quiescence by limiting PI3K signaling and suggest that PI3K inhibitors could be used as novel therapeutic agents to treat hereditary hemorrhagic telangiectasia.

Successful resolution of coats disease by photodynamic therapy: a case report.

BACKGROUND: Coats disease is a retinal disease characterized by exudative retinal detachment due to abnormal retinal blood vessels. Coats disease is generally treated using laser photocoagulation and cryotherapy to ablate the abnormal retinal blood vessels. However, if abnormal blood vessels are present near the posterior pole of the eye and there is a severe exudative change there, it is difficult to perform these standard treatments. We describe a case of Coats disease with severe exudative retinal change and retinal vascular abnormality near the posterior pole for which we performed photodynamic therapy and successfully suppressed the disease and improved vision. CASE PRESENTATION: A 15-year-old Japanese boy presented to hospital with a chief complaint of decreased vision in his right eye. At the initial examination, corrected visual acuity of the right eye was 20/100. On the right fundus, exudative retinal detachment with subretinal haemorrhage was observed from the upper intermediate periphery to the posterior pole. Abnormal telangiectatic vessels and microaneurysms were found at the nasal peripheral retina. From these findings, we diagnosed the case as Coats disease. We conducted photodynamic therapy for the right eye. At 10 months after treatment, both the subretinal haemorrhage and the exudative retinal detachment had disappeared completely. Further, the retinal structure of the macula had recovered, and right vision had improved to 20/20. CONCLUSION: Photodynamic therapy may be an effective and safe treatment for Coats disease in cases that present with abnormal retinal vessels close to the posterior pole of the eye.

Comparison of Visual Outcomes in Coats' Disease: A 20-Year Experience.

PURPOSE: To report differences in visual acuities among patients with Coats' disease who sought treatment at a tertiary care university-based practice. DESIGN: Single-center retrospective cohort study. PARTICIPANTS: Patients with Coats' disease diagnosed clinically, angiographically, or both from 1995 through 2015. METHODS: Patients were divided into 2 groups based on date of presentation: decade 1 (1995-2005) and decade 2 (2006-2015). MAIN OUTCOME MEASURES: Visual acuity (VA). RESULTS: Thirty-nine eyes of 39 patients were included with 19 eyes presenting in decade 1 and 20 eyes presenting in decade 2. Three patients demonstrated bilateral disease, but only the worse eye was included for analysis. Forty-seven percent of eyes in decade 1 demonstrated advanced stages of disease (stage 3B or worse) compared with 20% of eyes in decade 2. There was a trend for the mean initial presenting VA (±standard deviation) for decade 1 eyes to be worse (2.05±1.29 logarithm of the minimum angle of resolution [logMAR]) than for decade 2 eyes (1.45±0.99 logMAR; P = 0.1). From initial to final follow-up visit, mean VA also worsened for decade 1 eyes (P = 0.03), but remained stable for decade 2 eyes (P = 1.0). At the end of follow-up, there was a trend for mean VA for decade 1 eyes (2.28±1.17 logMAR) to be worse than for decade 2 eyes (1.60±1.15 logMAR; P = 0.07). Eight eyes were observed initially in decade 1 compared with 1 eye in decade 2, and only 1 of the observed eyes (in decade 2) developed painful glaucoma requiring enucleation. Decade 2 eyes had a higher average number of procedures per eye (6.5±4.9) compared with decade 1 eyes (1.4±1.7; P < 0.001). CONCLUSIONS: The earlier presentation of disease in decade 2 suggests improvements in disease detection over time. Furthermore, there was a trend for eyes to have better final VA in this decade. This is due to a combination of factors, including earlier presentation of disease, fewer eyes being observed without treatment, and eyes, when treated, receiving a higher number of procedures.

EFFICACY OF INTRAVITREAL AFLIBERCEPT IN MACULAR TELANGIECTASIA TYPE 1 IS LINKED TO THE OCULAR ANGIOGENIC PROFILE.

PURPOSE: To evaluate intravitreal aflibercept in macular telangiectasia Type 1 (MacTel 1) patients and measure their ocular angiogenic profile. METHODS: Eight subjects with MacTel 1 refractory to bevacizumab, ranibizumab, or laser therapy and switched to aflibercept were included. Best-corrected visual acuity, central macular thickness, and cystic areas quantified on optical coherence tomography B-scans were assessed during 12 months. Perifoveal capillary densities were measured on optical coherence tomography angiography. Aqueous humor was sampled from six patients and eight control subjects undergoing cataract extraction. Growth factors were quantified using a multiarray immunoassay. RESULTS: Over 12 months, patients received 6.6 ± 1.4 (range, 5-8) intravitreal aflibercept injections. Twelve months after switching to aflibercept, best-corrected visual acuity increased by ≥5 letters in 5 of 8 patients, compared with preaflibercept levels. Mean best-corrected visual acuity improved from 79.6 (∼20/50) to 88.0 (∼20/35) Early Treatment Diabetic Retinopathy Study letters (P = 0.042), and central macular thickness decreased from 434 ± 98 µm to 293 ± 59 µm (P = 0.014). Compared with control subjects, the profile of angiogenic factors in MacTel 1 eyes revealed no difference in vascular endothelial growth factor-A levels but significantly higher levels of placental growth factor (P = 0.029), soluble vascular endothelial growth factor receptor-1 (sFlt-1; P = 0.013), vascular endothelial growth factor-D (P = 0.050), and Tie-2 (P = 0.019). Placental growth factor levels inversely correlated with both superficial and deep capillary plexus densities on optical coherence tomography angiography (P = 0.03). CONCLUSION: The clinical response to aflibercept coupled to the angiogenic profile of MacTel 1 eyes support the implication of the placental growth factor/Flt-1 pathway in MacTel 1.

The effect of intravitreal bevacizumab injection as the initial treatment for Coats' disease.

BACKGROUND: In Coats' disease, the most recent development in the treatment has been the intravitreal injection of anti-VEGF agents. The purpose of this article was to evaluate the effect of intravitreal bevacizumab as the initial treatment for Coats' disease in children and adults. METHODS: The study included 14 pediatric patients and five adult patients with Coats' disease. They were treated with intravitreal bevacizumab (1.25 mg/0.05 ml) as the initial treatment, combined with or without other treatments. The analyses included the evaluation of basic clinical conditions. RESULTS: In the pediatric group, after a mean of 9.1 months of follow-up, the differences in visual acuity were significant for the comparisons between the baseline examination and the follow-up examinations carried out at weeks 6, 12, and 24 after the baseline (P = 0.006, P = 0.004, P = 0.005 respectively). Vitreoretinal fibrosis was observed in three patients (n = 3, 21.4 %), among whom two showed fibrosis before treatment. All of the pediatric patients showed a resolution of fluid and exudation, and regression of the telangiectasia. In the adult group, after a mean of 10.6 months of follow-up, the differences in visual acuity were not statistically significant (P > 0.05) between the baseline and follow-up examinations. Vitreoretinal fibrosis (n = 2, 40 %) was observed in two patients who both showed fibrosis before treatment. All of the adult patients showed a resolution of fluid and exudation, and regression of the telangiectasia. The differences in the change of BCVA between children and adults were not significant (P > 0.05) during the follow-up examinations. CONCLUSION: The intravitreal injection of bevacizumab as the initial treatment is associated with a measurable gain in visual acuity in patients with Coats' disease. Resolution of the subretinal fluid and exudation, and regression of the telangiectasia were observed in both pediatric and adult patients. Vitreoretinal fibrosis may be one of the natural courses of Coats' disease, and it remains uncertain whether bevacizumab accelerates the fibrosis phenomenon.

ranibizumab in the management of advanced Coats disease Stages 3B and 4: long-term outcomes.

BACKGROUND: Laser photocoagulation and cryotherapy to completely destroy telangiectatic vessels and ischemic retina in Coats disease is barely applicable in advanced cases with total retinal detachment, and globe survival is notoriously poor in Stages 3B and 4. Anti-vascular endothelial growth factor intravitreal injections may offer new prospects for these patients. METHODS: This study is a retrospective review of all consecutive patients with Coats disease treated with neoadjuvant or adjuvant intravitreal ranibizumab plus conventional and amblyopia treatment as appropriate. RESULTS: Nine patients (median age, 13 months) presenting Coats Stages 3B and 4 (5 and 4 eyes, respectively) were included. Iris neovascularization resolved within 2 weeks and retinal reapplication within 4 months in all patients. At last follow-up, globe survival was 100% with anatomical success in 8 of the 9 eyes. With a median follow-up of 50 months, fibrotic vitreoretinopathy was developed in 5 of the 9 cases, one leading to tractional retinal detachment and ultimately phthisis bulbi. The remaining 4 of the 9 eyes achieved some vision (range, 0.02-0.063). CONCLUSION: To the best of the authors' knowledge, this is the largest reported series of late-stage Coats undergoing anti-vascular endothelial growth factor therapy, a homogenous cohort of patients treated with a single agent and with the longest follow-up. This study supports the role of ranibizumab in advanced disease by transient restoration of the hemato-retinal barrier and suppression of neovascularization to facilitate classic treatment. At the last follow-up, the authors report unprecedented anatomical success and functional outcome.

Decreased macular thickness in nonproliferative macular telangiectasia type 2 with oral carbonic anhydrase inhibitors.

PURPOSE: To evaluate whether carbonic anhydrase inhibitors reduce the macular thickness and/or cystic spaces in patients with macular telangiectasia (MacTel) Type 2. METHODS: Retrospective review of patients with nonproliferative cystoid changes associated with MacTel seen at the University of Iowa between 2009 and 2012. Carbonic anhydrase inhibitors were used in 8 patients with MacTel Type 2. Five patients with MacTel Type 2 were observed during this period. Initial and final visual acuities were documented. The presence of cystic spaces and the retinal thickness were measured with spectral-domain optical coherence tomography. RESULTS: Patients treated with oral carbonic anhydrase inhibitors showed significant reduction in both the cystoid cavities and central macular thickness when compared with the patients who were observed (-12.2 µm; P = 0.020). The reduction in retinal thickness was more pronounced in patients receiving acetazolamide (-20.13 µm; P = 0.007) compared with methazolamide (-6.25 µm; P = 0.177). There was no significant change in visual acuity in patients receiving carbonic anhydrase inhibitors. Five patients with MacTel Type 2 did not receive treatment and demonstrated no change in visual acuity, cystoid cavities, or central macular thickness. CONCLUSION: Oral carbonic anhydrase inhibitors, particularly acetazolamide, may decrease macular cystic cavities and reduce central macular thickness but does not appear to improve visual acuity. These findings have yet to be confirmed with a prospective treatment trial.

Ranibizumab for macular telangiectasia type 2 in the absence of subretinal neovascularization.

PURPOSE: To evaluate the effects of 0.3 mg or 0.5 mg of ranibizumab in eyes with macular telangiectasia type 2 without subretinal neovascularization. METHODS: Ten eyes were randomized to either 0.3 mg or 0.5 mg ranibizumab group in 1 eye only. Study eye received ranibizumab at baseline and at Months 1 and 2. Injections at Months 3, 4, and 5 were at investigator's discretion. Participants were followed monthly through 6 months with best-corrected visual acuity, fluorescein angiography, and optical coherence tomography. RESULTS: For study eyes at baseline, median best-corrected visual acuity letter score was 60 (20/64 Snellen equivalent) and central subfield retinal thickness was 181.5 µm. Median number of injections was six. Median change in best-corrected visual acuity at Month 3 was 4 letters (range: -5 to 9 letters) at both doses in the study eye and 3 letters (range: -10 to 5 letters) in the untreated fellow eye. At Month 3, retinal leakage decreased 0.87 disk area and 0.76 disk area for 0.3 mg and 0.5 mg ranibizumab, respectively. Median change in central subfield retinal thickness was 1 µm and -11 µm for 0.3 mg and 0.5 mg ranibizumab, respectively. CONCLUSION: Ranibizumab (0.3 mg or 0.5 mg) decreases leakage secondary to macular telangiectasia type 2, but accompanying improvements in best-corrected visual acuity appear similar to improvements in the untreated fellow eye where retinal thickness is relatively unchanged.

Comparison of observation, intravitreal bevacizumab, or pars plana vitrectomy for non-proliferative type 2 idiopathic macular telangiectasia.

PURPOSE: To compare visual and anatomic outcomes in eyes with type 2 idiopathic macular telangiectasia (Mactel) treated with either intravitreal bevacizumab (IVB), observation, or pars plana vitrectomy (PPV) with internal limiting membrane removal. METHODS: Retrospective, consecutive, interventional case series of phakic patients with Mactel. Best-corrected Snellen visual acuity (BCVA) and complete ophthalmic exam was obtained prior to treatment and at subsequent 3-month intervals for a minimum of 6 months. Fluorescein angiographic and spectral-domain optical coherence tomography features were examined, and compared to BCVA at treatment initiation and follow-up. RESULTS: Fifty-six eyes of 28 patients were evaluated. Mean age was 65 ± 12 years, and mean follow-up was 24 ± 13 months. Patients were treated with either observation (n = 33), IVB (n = 15), or PPV (n = 8). Mean number of treatments for the IVB group was 2.5 ± 3.5 intravitreal injections. No significant differences in BCVA change were observed between treatment groups via one-way ANOVA (p = 0.49). Presence of inner retinal cysts was not correlated to BCVA (p > 0.05). Discontinuous outer nuclear layer was significantly related to worse initial and final vision, but not to BCVA change. CONCLUSION: IVB and PPV with ILM removal appear ineffective in improving visual outcome in eyes with non-proliferative Mactel. SD-OCT evidence of disrupted foveal outer nuclear layer is related to decreased BCVA, but not related to BCVA change following treatment.

Long-term course in type 2 idiopathic macular telangiectasia.

INTRODUCTION: To study the long-term course in patients with idiopathic macular telangiectasia and report the effect of anti VEGF and laser treatment. METHODS: A retrospective case series of 19 patients/38 eyes with symptomatic type 2 idiopathic macular telangiectasia was performed. Six eyes received intravitreal injections of bevacizumab (1-3 injections), four eyes received focal laser treatment. Follow up examinations comprised visual acuity, biomicroscopy, fluorescein angiography and assessment of macular morphology and thickness by time and spectral-domain optical coherence tomography (OCT). RESULTS: Mean follow-up time was 81 months (range 15-188 months) - the median added up to 80 months. Visual outcome at final visit varied substantially (20/200-20/20). On average visual acuity decreased 1,2 lines (range -0,5 to 6) by 3 years, 2 lines (range -0,5 to 7) by 5 years and 4,1 lines (range 0 to 12) by 10 years. Development of choroidal neovascularisation was observed in only one eye. There was no significant difference in visual acuity between eyes receiving no treatment, intravitreal bevacizumab or laser treatment after 3 and 5 years. Morphological studies by OCT revealed typical changes with retinal atrophy and intraretinal cysts. Visual acuity correlated with the eccentricity of the main manifestation-visual preservation was associated with mainly extrafoveal disease manifestation. DISCUSSION: Type 2 idiopathic macular telangiectasia is a chronic, often slowly progressing macular disease leading to retinal atrophy and visual impairment over decades. Thorough knowledge about the long term course of this disease is necessary to evaluate possible therapeutic options in the long run.

Reduced-fluence photodynamic therapy combined with ranibizumab for nonproliferative macular telangiectasia type 2.

PURPOSE: To report the efficacy of reduced-fluence photodynamic therapy (PDT) combined with intravitreal ranibizumab for the treatment of nonproliferative macular telangiectasia (MacTel) type 2. METHODS: Noncomparative, interventional, retrospective case series; 5 eyes of 4 patients were studied. Patients were treated with reduced-fluence PDT and intravitreal ranibizumab within 24 h. After initial treatment, follow-up was at least 12 months in all patients. RESULTS: At baseline median logMAR (logarithm of the minimal angle of resolution) best-corrected visual acuity (BCVA) was 1.0 (range, 1.0-0.3). At 3 months of follow-up vision increased in 3 out of 5 eyes and median BCVA was 0.4 (range, 1.0-0.2). The gain of BCVA ranged from 6 lines to 1 line. Visual acuity remained stable in the other 2 study eyes. No eyes lost vision at 3 months of follow-up. At 12 months of follow-up median logMAR BCVA was 0.7 (range, 1.3-0.3). Two eyes had maintained their gain in BCVA compared to baseline. Two eyes lost vision compared to baseline and 1 eye showed unchanged visual acuity at 12 months of follow-up. CONCLUSION: A combination therapy with reduced-fluence PDT and intravitreal ranibizumab might be a valuable treatment option for eyes with progressive vision loss due to nonproliferative MacTel type 2.

Intravitreal bevacizumab for type 2 idiopathic macular telangiectasia.

BACKGROUND/AIMS: The aim of this paper is to report the treatment of type 2 nonproliferative idiopathic macular telangiectasia (IMT) with intravitreal bevacizumab (IVB). METHODS: Retrospective case series of 10 eyes of 5 patients with type 2 IMT. All patients received 3 monthly IVB injections. Visual acuity (VA), fluorescein angiography (FA) and optical coherence tomography (OCT) were performed at baseline and 4 weeks after each injection. RESULTS: Four weeks after the third IVB injection, VA remained stable for all patients. All eyes showed some decrease in fluorescein leakage, and there was a mild decrease in central macular thickness. One year later, VA, OCT and FA findings returned to the baseline levels. CONCLUSION: IVB did not improve VA in cases of type 2 IMT.

Adult-onset Coats disease.

Coats disease is an idiopathic retinal vasculopathy characterized by telangiectasia and aneurysm of retinal vessels along with intra and subretinal exudation and fluid. While Coats disease is classically described in young male population, there is an adult variant of Coats disease presenting in adulthood. Adult onset Coats disease have a similar presentation but a slower progression, localised lipid deposition, both peripheral and juxta-macular involvement. In this review article, we have attempted to describe in detail the characteristic clinical features, pathogenesis, investigation modalities and treatment in adult-onset Coats disease.