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      Intravitreal anti-vascular endothelial growth factor agents as an adjunct in the management of coats' disease in children

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          Abstract

          We describe the role of intravitreal anti-vascular endothelial growth factor (VEGF) agents in Coats' disease in children. In a prospective, interventional, non-randomized case series, three patients (three eyes) aged 16, seven and two years were diagnosed to have Coats' disease. In Case 1 (16 yr/ male) with macular edema, previous laser photocoagulation being unsuccessful, intravitreal pegaptanib sodium (Macugen™) was tried. Case 2 (seven yr/ male) and Case 3 (two yr/ female) were diagnosed to have Stage 4 Coats' and underwent external needle drainage, laser photocoagulation, SF6 gas injection and intravitreal injection of bevacizumab (Avastin™). Reduction of exudation and attached posterior pole (Cases 2 and 3) was seen at a follow-up of six months and two months respectively. Intravitreal anti-VEGF agents may be successfully used as adjunct treatment in select cases of Coats' disease in childhood.

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          Intravitreal bevacizumab (Avastin) therapy for persistent diffuse diabetic macular edema.

          To evaluate the efficacy of bevacizumab (Avastin; Genentech, Inc., South San Francisco, CA) for the treatment of diabetic macular edema. This prospective, consecutive, noncomparative case series included 51 consecutive patients (26 females and 25 males; mean age, 64 years) with diffuse diabetic macular edema. Inclusion criteria were determined independently of the size of edema, retinal thickness, visual acuity, age, metabolic control, type of diabetes, or previous treatments beyond a 6-month period. At each visit, patients underwent complete eye examination, including determination of best-corrected visual acuity, slit-lamp examination, intraocular pressure measurement, stereoscopic biomicroscopy of the macula, retinal thickness measurement by optical coherence tomography, fluorescein angiography, and fundus photography. After written informed consent was obtained, all patients were treated with a 0.05-mL injection containing 1.25 mg of bevacizumab. All patients completed 6 weeks of follow-up; 23 (45%) completed 12 weeks of follow-up. Sixteen patients (70%) had received at least two intravitreal injections. All patients had undergone previous treatments, such as focal laser therapy (35%), full-scatter panretinal laser therapy (37%), vitrectomy (12%), and intravitreal injection of triamcinolone (33%). The mean diameter of the foveal avascular zone was 503 micro m, with 49% with values of >500 micro m. At baseline, mean visual acuity +/- SD was 25.88 +/- 14.43 ETDRS letters (0.86 +/- 0.38 logMAR of Snellen letters). Mean central retinal thickness by optical coherence tomography +/- SD was 501 +/- 163 micro m (range, 252-1,031 micro m). Mean visual acuity +/- SD increased to 0.75 +/- 0.37 logMAR of Snellen letters at 6 weeks after injection (P = 0.001), with some regression to 0.84 +/- 0.41 logMAR of Snellen letters after 12 weeks. Changes in ETDRS letters were not significant throughout follow-up. Mean retinal thickness +/- SD decreased to 425 +/- 180 micro m at 2 weeks (P = 0.002), 416 +/- 180 micro m at 6 weeks (P = 0.001), and 377 +/- 117 micro m at 12 weeks (P = 0.001). Changes of retinal thickness and visual acuity correlated weakly (r = -0.480 and P = 0.03 at 6 weeks; r = -0.462 and P = 0.07 at 12 weeks). The increase of visual acuity after 6 weeks as measured by ETDRS charts could be predicted best by baseline visual acuity. No other factors investigated, such as age, thickness by optical coherence tomography, or previous treatments, were predictive for the increase in visual acuity. Even in cases of diffuse diabetic macular edema not responding to previous treatments such as photocoagulation, intravitreal injection of triamcinolone, or vitrectomy, improvement of visual acuity and decrease of retinal thickness could be observed after intravitreal injection of bevacizumab. Although our follow-up period was too short to provide specific treatment recommendations, the short-term results encourage further prospective studies with different treatment groups and longer follow-up.
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            Elevated vascular endothelial growth factor levels in Coats disease: rapid response to pegaptanib sodium.

            The purpose was to report a case of elevated intraocular vascular endothelial growth factor (VEGF) levels in a 2-year-old boy with stage 4 Coats disease. Interventional case report. A 2-year-old boy with severe Coats disease and complex retinal detachment refractory to surgical intervention underwent two injections of intravitreal pegaptanib sodium in his right eye 8 weeks apart. After intravitreal injection there was a marked reduction in VEGF (908 to 167 pg/ml) levels and improvement of exudation, hemorrhage, and nearly complete reattachment of his retina within 3 weeks of treatment. The patient remains stable 6 months post-treatment. Coats disease may have a component of dysregulation of VEGF-mediated angiogenesis as evidenced by elevated levels in this patient.
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              Classification of the spectrum of Coats' disease as subtypes of idiopathic retinal telangiectasis with exudation.

              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. 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. 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. 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.
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                Author and article information

                Journal
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Medknow Publications (India )
                0301-4738
                1998-3689
                Jan-Feb 2010
                : 58
                : 1
                : 76-78
                Affiliations
                Department of Pediatric Ophthalmology and Strabismology, Aditya Jyot Eye Hospital Pvt Ltd, Mumbai, India
                Author notes
                Correspondence to: Dr. Shalini Kaul, Department of Pediatric Ophthalmology and Strabismology, Aditya Jyot Eye Hospital Pvt. Ltd, Plot no 153, Road no 9. Major Parmeswaran Road, Wadala, Mumbai – 31, India. E-mail: drshalinikaul@ 123456gmail.com
                Article
                IJO-58-76
                10.4103/0301-4738.58480
                2841382
                20029154
                d5eec83a-5af1-4a9b-901e-18045e343093
                © Indian Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 18 July 2008
                : 07 January 2009
                Categories
                Brief Communications

                Ophthalmology & Optometry
                vascular endothelial growth factor,coats' disease
                Ophthalmology & Optometry
                vascular endothelial growth factor, coats' disease

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