Atlas of Ophthalmology

Coats' disease (Colour Photography Posterior Pole, Fluorescein Angiography, OCT)

Retina -> Aquired Macular Diseases
Patient: 9 years of age, male, BCVA 1.0 at OD, 0.1 at OS, IOP 15/15 mmHg. Ocular Medical History: loss of vision in OS, no pain. General Medical History: herpes labialis, ASL: 64,2, ACE 20,1 U/l, CMV-IgM: negative, CMV-IgG: negative, EBV-Anti-VCA-IgG: negative, EBV-Anti-VCA-IgM: negative, HSV-IgM: negative, HSV-IgG: positive, Herpes simplex Type 1-Antibody: positive, Herpes simplex Type 2-Antibody: negative, TPHA: negative, Toxocara canis: negative. Purpose: to present unilateral teleangiectasis, exudative subretinal lipid deposits in a young boy. Methods: Colour Photography Posterior Pole, Fluorescein Angiography, OCT. Findings: Colour Photography Posterior Pole: teleangiectasias, preretinal hemorrhages, exudative subretinal lipid deposits. Fluorescein Angiography: peripheral retinal ischaemia OCT: subretinal fluid, retinal exudation. Discussion: Coats' disease (retinal telangiectasis) is a sporadic disorder characterized by a defect in retinal vascular development resulting in vessel leakage, subretinal exudation and retinal detachment. Black GC et al. (1) has indicated probable role of somatic mutation in the NDP gene in the pathogenesis of Coats' disease. Vascular endothelial growth factor (VEGF) has been identified (2) as a key regulator in angiogenesis and vascular permeability. Coats' disease demonstrates vascular abnormalities, associated exudation, and manifests as teleangiectasia and aneurysms. Exudative subretinal lipid deposits can be extensive. Coats' disease is unilateral and affects predominantly healthy young males. Joussen AM (3) suggested that Coats' disease as a progressive disease requires lifelong follow-up and therapy. Treatment consisted of cryotherapy, laser photocoagulation and bevacizumab intravitreal injection. Literature: (1) Black GC, Perveen R, Bonshek R, et al. Coats' disease of the retina (unilateral retinal telangiectasis) caused by somatic mutation in the NDP gene: a role for norrin in retinal angiogenesis. Hum Mol Genet 1999;8:2031–5. (2) Lin KL, Hirose T, Kroll AJ, et al. Prospects for treatment of pediatric vitreoretinal diseases with vascular endothelial growth factor inhibition. Semin Ophthalmol 2009;24:70–6. (3) Joussen AM, Gordes RS, Heußen FA, Müller B. Retinal exudative disease in childhood: Coats' disease and familial exudative vitreoretinopathy (FEVR). Klin Monbl Augenheilkd. 2013 Sep;230(9):902-13.

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