Coats Disease

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Coats Disease

  1. 1. Doheny Eye Institute Grand Rounds Presenter Irina Bykhovskaya, MD Discussant Dr. SriniVas Sadda 3/9/2007
  2. 2. Patient Presentation <ul><li>HPI: 16 yo HM from juvenile hall c/o “black cloud” OS x 8 months </li></ul><ul><li>POHx: h/o “fluid in the eye” and laser tx OS at age 8 yo </li></ul><ul><li>PMH / FHx – unremarkable </li></ul><ul><li>SLE: LLL/S/C/K/AC/I/L – wnl OU </li></ul>
  3. 3. Fundus Photos lipid-rich, yellow subretinal exudate in the posterior pole OS
  4. 4. Fundus Photos OS
  5. 5. Red-Free Fundus Photos
  6. 6. Fluorescein Angiogram
  7. 7. Fluorescein Angiogram
  8. 8. Coats’ Disease <ul><li>Idiopathic condition </li></ul><ul><li>Telangiectatic and aneurismal vessels with intraretinal and subretinal exudates </li></ul><ul><li>1 st described by Coats in 1908 </li></ul>
  9. 9. Etiology <ul><li>Vascular: </li></ul><ul><li>Thickening of endothelial basement membrane of the characteristic telangiectatic vessels due to deposition of acid-Schiff (PAS)-positive material </li></ul>
  10. 10. Clinical Presentation <ul><li>Painless </li></ul><ul><li>Males : Females = 3:1 </li></ul><ul><li>80% unilateral </li></ul><ul><li>2/3 cases present before age 10 </li></ul><ul><li>Diagnosed at a median age of 5 years </li></ul><ul><li>No predilection for race or laterality </li></ul><ul><li>Poor vision, strabismus, leukocoria </li></ul><ul><li>Visual acuity at presentation 20/200 to no light perception </li></ul><ul><li>No genetic transmission </li></ul><ul><li>No systemic associations </li></ul>
  11. 11. Clinical Picture <ul><li>Localized, lipid-rich, yellow subretinal exudate </li></ul><ul><li>Associated vascular anomalies: sheathing, telangiectasia, tortuosity, aneurismal dilatations, zones of capillary drop out, and occasionally neovascularization </li></ul>
  12. 12. Clinical Course <ul><li>Variable but generally progressive with acute exacerbations and quiescent stages </li></ul><ul><li>Complications: </li></ul><ul><li>subretinal CMV </li></ul><ul><li>serous RD </li></ul><ul><li>hemorrhagic macrocysts </li></ul><ul><li>secondary iridocyclitis </li></ul><ul><li>Cataract </li></ul><ul><li>neovascular glaucoma </li></ul>
  13. 13. FA <ul><li>Reveals localized anomalies in retinal vasculature </li></ul><ul><li>Telangiectasia, aneurisms, beading of vessel walls, and vascular communication channels </li></ul><ul><li>Breakdown of blood-retinal barrier  Early and persistent leakage </li></ul><ul><li>Microvascular anomalies: Diffuse loss of capillary bed or areas of complete capillary nonperfusion surrounded by areas of arteriolar and venular anomalies </li></ul>
  14. 14. Histopathology Loss of vascular endothelium and pericytes All levels of the retinal vascular circuit are involved Proteinaceous subretinal exudates with prominent cholesterol clefts and prominent histiocytes Total exudative retinal detachment
  15. 15. Histopathology cont’d Subretinal fluid with cholesterol clefts and lipid-laden macrophages Telangiectasic retinal vessels Intraretinal cholesterol deposition triggering a giant cell reaction foreign-body type
  16. 16. Differential Diagnosis <ul><li>Retinoblastoma </li></ul><ul><li>Retinal detachment </li></ul><ul><li>Persistentt hyperplastic primary vitreous </li></ul><ul><li>Congenital cataract </li></ul><ul><li>Norrie’s disease </li></ul><ul><li>Familial exudative vitreoretinopathy </li></ul><ul><li>Eales’ disease </li></ul><ul><li>Vasculitis </li></ul><ul><li>Tumor accompanied by exudation </li></ul><ul><li>Diabetic vasculopathies </li></ul><ul><li>BRVO </li></ul><ul><li>Idiopathic juxtafoveal telangiectasis </li></ul>
  17. 17. Diagnosis and Ancillary Testing <ul><li>Echography </li></ul><ul><li>- Ultrasonography typically shows a retinal detachment but no solid mass </li></ul><ul><li>CT or MRI </li></ul><ul><li>High-resolution Doppler ultrasound </li></ul><ul><li>- to differentiate from retinablastoma </li></ul>Computed Tomography. Total exsudative retinal detachment in the right eye.
  18. 18. Treatment <ul><li>Focal laser photocoagulation </li></ul><ul><li>to close areas of vascular leakage to allow resorption of exudate </li></ul><ul><li>Near yellow wavelength </li></ul><ul><li>100 to 500 microns </li></ul><ul><li>Cryotherapy if retina is exudatively detached </li></ul><ul><li>Subretinal fluid drainage and vasoablation +/- scleral buckle </li></ul>
  19. 19. References <ul><li>Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol. 2001;131:561–571. </li></ul><ul><li>Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina. 2002;22:80–91. </li></ul><ul><li>Shields JA, Parsons HM, Shields CL, Shah P. Lesions simulating retinoblastoma. J Pediatr Ophthalmol Strabismus. 1991;28:338–340. </li></ul><ul><li>Bruno F Fernandes, Alexandre N Odashiro, Shawn Maloney, Moyses E Zajdenweber, Andressa G Lopes, and Miguel N Burnier, Jr. Clinical-histopathological correlation in a case of Coats' disease. Diagn Pathol. 2006; 1: 24. </li></ul><ul><li>L. Smithen, G. Brown, A. Brucker, L. Yannuzzi, C. Klais, R. Spaide. Coats’ Disease Diagnosed in Adulthood. Ophthalmology, Volume 112, Issue 6, Pages 1072-1078 </li></ul><ul><li>Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary vitreous: role of MR imaging and CT. Radiol Clin North Am 1998;36:1119-1131 </li></ul>

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