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Enfermedad de Coats

Enfermedad de Coats. Enfermedad de Coats'. Desarrollo de anomalias vasculares retinianas ,vasos telangiectasicos y aneurismas que producen fuga y se asocian con exudacion lipidica. Ocurre de manera unilateral en varones jovenes dentro de la 1era decada. George Coats 1876 - 1915.

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Enfermedad de Coats

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  1. Enfermedad de Coats

  2. Enfermedad de Coats' Desarrollo de anomalias vasculares retinianas ,vasos telangiectasicos y aneurismas que producen fuga y se asocian con exudacion lipidica. Ocurre de manera unilateral en varones jovenes dentro de la 1era decada

  3. George Coats1876 - 1915

  4. Enfermedad de Coats • Anillo de Coats • Sindrome de Coats • Contribuciones a la oftalmologia veterinaria

  5. Historia • 1908 George Coats. Clasificacion incial de 3 estadios • Von Hippel. Angiomatosis retinae • 1912-1915 .Theodore Leber. Aneurismas Miliares Multiples • Reese años 50

  6. Etiologia • Infeccioso. Asociado a Toxoplasmosis • Inflamatorio • Metabolico • Alteraciones en la membrana basal endotelial • Mutaciones CRB1 y NDP

  7. Genetica Describen una mujer con enf. de Coats, que dio a luz a un niño con enfermedad de Norrie, portando ambos una mutación en el gen NDP en el cromosoma Xp11.2. Coats' disease of the retina caused by somatic mutation in the NDP gene: a role for norrin in retinal angiogenesisBlack University Department of Medical Genetics and Regional Genetics Service, St Mary's Hospital, Manchester M13 OJH, UK.

  8. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Giffor MemorialLecture • Primer sintoma o signo fue disminucion AV 68 (34%) • Estrabismo 37 (23%) • leukocoria 31 (20%) • 13 (8%) asintomatico

  9. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture • Telangiectasia en periferia o media periferia 156 (99%) • macular (1%) • temporal 66 (42%) • inferior 41 (26%)

  10. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture • Exudacion 12 horas 86 (55%) • 6 o mas 115 (73%). • DR total 74 (47%) • GNV 12 (8%). • Retinal macrocysts 18 (11%), • retinal neovascularization 4 (3%).

  11. Estadios • Estadio 1 solamente telangiectasia • Estadio 2 telangiectasia y exudados: 2a y 2b • Estadio 3 : DR3a y 3b. • Estadio 4 DR y glaucoma • Estadio 5 Classification and management of Coats disease: the 2000 Proctor Lecture. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J.Oncology Service, Wills Eye Hospital

  12. Am J Ophthalmol. 2001 May;131(5):561-71. LinksClinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture • AF 49 (37%) fuga contraste • fuga mas EM 18 • ECO modo B DR, no LOE

  13. Diagnostico Diferencial Juvenil. Leucocoria y Estrabismo • DR • cataratas congenita • PVPH • FEV • Retinoblatoma

  14. Diagnostico diferencial • Adultos • Eales • R Diabetica • BRVO • Enfermedades colageno • Tumores

  15. tratamientos • Laser • Cryotherapia • Avastin   • Macugen, Lucentis y Retaane • Cirugia de DR

  16. Pauleikoff et al. • 292 ojos • 197 laser, crio, diatermia • Resto amerito cirugia • 52.1% exudados 67.3% vasculares • 33% éxito en pacientes con DR

  17. Ridley et al. • Estabilizacion y mejoria de AV en 21 de 28 Crio, fotocoagulacion Drenaje y vasoablacion, buckle escleral

  18. Siliodor et al. • Infusion intraocular, drenaje y crioterapia DR bulloso • 6 GNV no tratados • 7 cosmeticamente aceptables

  19. Machemer and Williams • Casos seleccionados • VPP, peeling, drenaje interno • Vasoablacion • Gas o silicona

  20. Shields Am J Ophthalmol. 2001 May;131(5):572-83.Classification and management of Coats disease: the 2000 Proctor Lecture. • 124 ojos • 55 meses ( 6 meses a 25 años) • observacion 22 (18%) • cryo 52 (42%) • laser 16 (13%) • cirugia 20 (17%) • Enucleacion 14 (11%). • 76% éxito anatomico • av 20/50 17( 14 %)

  21. Pronostico visual • Baja AV(20/200 o peor) 0% estadio 1 • 53% estadio 2 • 74% estadio 3 • 100% estadio 4 y 5 Shields Am J Ophthalmol. 2001 May;131(5):572-83.Classification and management of Coats disease: the 2000 Proctor Lecture.

  22. Factores de riesgo • AV (20/200 o peor) • postequatorial (P =.01), difusa (P =.01), o superior (P =.04) localizacion de telangiectasias y exudacion, • Falla en la reabsorcion de fluido(P =.02) • macroquistes retinianos(P =.02) Shields Am J Ophthalmol. 2001 May;131(5):572-83.Classification and management of Coats disease: the 2000 Proctor Lecture.

  23. Factores de riesgo • Enucleacion PIO elevada neovascularizacion del iris Shields Am J Ophthalmol. 2001 May;131(5):572-83.Classification and management of Coats disease: the 2000 Proctor Lecture.

  24. Coats bilateral De Blauwe A Bilateral Coats' disease with unusual presentation--a case report. Department of Ophthalmology, University Hospitals Leuven, BelgiumBull Soc Belge Ophtalmol. 2005;(295):35-9.

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