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J. Ulrich, S. Moyer, M. E. Hartnett; Characteristics and Treatment Outcomes of Pediatric Retinal Vascular Malformations. Invest. Ophthalmol. Vis. Sci. 2010;51(13):3542.
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To describe the characteristics and treatment outcomes of pediatric retinal conditions associated with abnormal retinal angiogenesis.
Medical records from 2005 and 2009 were queried for the diagnoses of retinal telangiectasia or vitreoretinopathy, and those associated with abnormal angiogenesis in patients younger that age 21 years were reviewed for the findings of telangiectasia, intravitreous neovascularization or peripheral avascular retina. Included patients underwent a clinical examination or examination under anesthesia with diagnostic testing (Fluorescence Angiogram [FA] and fundus photography or Retcam [Clarity Medical Systems, Pleasanton, CA] wide angle imaging). Treatment (laser photocoagulation, cryotherapy, intravitreal bevacizumab, pars plana vitrectomy [PPV]) was performed as indicated for the condition. Patients were analyzed based on clinical findings, visual acuity (VA) and outcomes following interventions.
20 patients from 3 months to 20 years of age (mean 7.9 years) were identified. 13 patients had Coats’ disease and were significantly older than 5 patients with familial exudative vitreoretinopathy (FEVR; mean 10.2 yrs, 3.6 yrs, p=0.047). One patient was diagnosed with incontinentia pigmenti, one patient with exudative retinopathy of unknown etiology. Bilateral disease was present in only 2 Coats’ patients (16.7%) but was present in all FEVR patients (p<0.001). Avascular retina was identified with or without FA in 54% of the Coats’ patients (7/13) and 100% of the patients with FEVR (p=0.08).All but one patient underwent treatment (laser: n=15, cryotherapy: n=5, PPV: n=5, bevacizumab: n=1) with regression of exudates or neovascularization. 2 Coats’ patients required PPV (16.7%) compared to 2 FEVR patients (40%, p=0.42). The visual acuity in Coats’ patients stabilized after treatment (mean 20/81 from 20/83 prior to treatment). Due to the young age of FEVR patients, only one VA measurement was possible in this group (20/40).
FEVR is often associated with peripheral avascular retina and growth of blood vessels into the vitreous. Our findings suggest that Coats’ disease, defined by retinal telangiectasia, also has an association with peripheral avascular retina.
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