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Coats' disease

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111: 72: 186: 374:, this treatment is not recommended as accidental damage to the nerve itself can result in permanent blindness. Although Coats' disease tends to progress to visual loss, it may stop progressing on its own, either temporarily or permanently. Cases have been documented in which the condition even reverses itself. However, once total retinal detachment occurs, sight loss is permanent in most cases. Removal of the eye (enucleation) is an option if pain or further complications arise. 387: 323: 334: 279: 309:
On MRI, the subretinal exudate shows high signal intensity on both T1- and T2-weighted images. The exudate may appear heterogeneous if hemorrhage or fibrosis is present. The subretinal space does not enhance with gadolinium contrast. Mild to moderate linear enhancement may be seen between the exudate
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On CT, the globe appears hyperdense compared to normal vitreous due to the proteinaceous exudate, which may obliterate the vitreous space in advanced disease. The anterior margin of the subretinal exudate enhances with contrast. Since the retina is fixed posteriorly at the optic disc, this
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On funduscopic eye examination, the retinal vessels in early Coats' disease appear tortuous and dilated, mainly confined to the peripheral and temporal portions of retina. In moderate to severe Coats' disease, massive retinal detachment and hemorrhage from the abnormal vessels may be seen.
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is caused by a reflection off blood vessels in the back of a normal eye, an eye affected by Coats' will glow yellow in photographs as light reflects off cholesterol deposits. Children with yellow-eye in photographs are typically advised to immediately seek evaluation from an optometrist or
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Coats' usually affects only one eye (unilateral) and occurs predominantly in young males 1/100,000, with the onset of symptoms generally appearing in the first decade of life. Peak age of onset is between 6–8 years of age, but onset can range from 5 months to 71 years.
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Microscopically, the wall of retinal vessels may be thickened in some cases, while in other cases the wall may be thinned with irregular dilatation of the lumen. The subretinal exudate consists of cholesterol crystals, macrophages laden with cholesterol and pigment,
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Statland JM1, Sacconi S, Farmakidis C, Donlin-Smith CM, Chung M, Tawil R. Coats syndrome in facioscapulohumeral dystrophy type 1: frequency and D4Z4 contraction size. Neurology. 2013 Mar 26;80(13):1247-50. doi: 10.1212/WNL.0b013e3182897116. Epub 2013 Feb
158:. Deterioration of sight may begin in either the central or peripheral vision. Deterioration is likely to begin in the upper part of the vision field as this corresponds with the bottom of the eye where blood usually pools. Flashes of light, known as 634: 619: 205:
Coats' disease results in a gradual loss of vision. Blood leaks from the abnormal vessels into the back of the eye, leaving behind cholesterol deposits and damaging the retina. Coats' disease normally progresses slowly. At advanced stages,
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GlasierCM, Brodsky MC, Leithiser RE Jr, Williamson SL, Seibert JJ. High resolution ultrasound with Doppler: a diagnostic adjunct in orbital and ocular lesions in children. Pediatr Radiol 1992; 22(3): 174–178.
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Chung EM, Specht CS, Schroeder JW. Pediatric Orbit Tumors and Turmorlike Lesions: Neuroepthelial Lesions of the Ocular Globe and Optic Nerve. Radiographics. 2007 Jul-Aug;27(4):1159-86.
302:. On ultrasound, Coats' disease appears as a hyperechoic mass in the posterior vitreous without posterior acoustic shadowing; vitreous and subretinal hemorrhage may often be observed. 473:
EdwardDP, 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(6): 1119–1131.
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KremerI, Nissenkorn I, Ben-Sira I. Cytologic and biochemical examination of the subretinal fluid in diagnosis of Coats' disease. Acta Ophthalmol (Copenh) 1989; 67(3): 342–346.
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Coats' disease itself is painless. Pain may occur if fluid is unable to drain from the eye properly, causing the internal pressure to swell, resulting in painful glaucoma.
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FernandesBF, Odashiro AN, Maloney S, Zajdenweber ME, Lopes AG, Burnier MN Jr. Clinical-histopathological correlation in a case of Coats' disease. Diagn Pathol 2006; 1: 24.
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into the retina and subretinal space. Over time, the accumulation of this proteinaceous exudate thickens the retina, leading to massive, exudative retinal detachment.
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WoodsAC, Duke JR. Coats's disease. I. Review of the literature, diagnostic criteria, clinical findings, and plasma lipid studies. Br J Ophthalmol 1963; 47: 385–412.
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BerrocalT, de Orbe A, Prieto C, et al. US and color Doppler imaging of ocular and orbital disease in the pediatric age group. RadioGraphics 1996; 16(2): 251–272.
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A case of Coats' disease, showing total retinal detachment with subretinal exudate containing cholesterol crystals and a fibrous nodule in the posterior pole
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ChangMM, McLean IW, Merritt JC. Coats' disease: a study of 62 histologically confirmed cases. J Pediatr Ophthalmol Strabismus 1984; 21(5): 163–168.
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EisenbergL, Castillo M, Kwock L, Mukherji SK, Wallace DK. Proton MR spectroscopy in Coats disease. AJNR Am J Neuroradiol 1997; 18(4): 727–729.
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are common symptoms. Persistent color patterns may also be perceived in the affected eye. Initially, these may be mistaken for psychological
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A case of Coats' disease, showing total exudative retinal detachment, and subretinal exudate containing cholesterol crystals (H&E)
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Shields CL, Uysal Y, Benevides R, Eagle RC, Malloy B, Shields JA (2006). "Retinoblastoma in an eye with features of Coats' disease".
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G. Coats. Forms of retinal disease with massive exudation. Royal London Ophthalmic Hospital Reports, 1908, 17, 3: 440-525.
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Computed Tomography image of a patient with Coats' disease, showing total exudative retinal detachment in the right eye
233:(FSHD). A single study reported it in 1 percent of FSHD patients, most often those with FSHD type 1 (FSHD1) with large 823: 781: 746: 350:. A granulomatous reaction, induced by the exudate, may be seen with the retina. Portions of the retina may develop 1751: 1547: 1521: 1479: 1474: 1339: 1212: 1200: 1156: 311: 295: 1818: 1217: 1930: 1724: 1571: 1416: 1163: 330:
Grossly, retinal detachment and yellowish subretinal exudate containing cholesterol crystals are commonly seen.
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A young child with the yellow eye of Coats' disease - still in an early stage. Only visible with a flash camera.
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ShieldsJA, Shields CL. Review: Coats disease—the 2001 LuEsther T. Mertz lecture. Retina 2002; 22(1): 80–91.
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ophthalmologist, who will assess and diagnose the condition and refer to a vitreo-retinal specialist.
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can also develop secondary to Coats' disease. In some cases, removal of the eye may be necessary (
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and foreign fluids mechanically interacting with the photoreceptors located on the retina.
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One early warning sign of Coats' disease is yellow-eye in flash photography. Just as the
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and the remaining vitreous. The exudate shows a large peak at 1–1.6 ppm on
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is a rare congenital, nonhereditary eye disorder, causing full or partial
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Clinical photography of patient with Coats' disease, showing
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GeneReviews/NIH/NCBI/UW entry on NDP-Related Retinopathies
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Coats' disease is thought to result from breakdown of the
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In the early stages, there are a few treatment options.
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Coats' disease is a rare extramuscular manifestation of
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Human eye disease causing full or partial blindness
1740:arteritic anterior (AAION or arteritic AION) 720:http://www.orpha.net/data/patho/GB/uk-Coats.pdf 131:. Coats' disease can also fall under glaucoma. 134:It can have a similar presentation to that of 747: 8: 1814:Chronic progressive external ophthalmoplegia 417:Coats' disease is named after George Coats. 1142:Thygeson's superficial punctate keratopathy 2248: 2012: 1801: 1792: 1670: 1657: 1644: 1251: 1069: 1060: 1047: 798: 789: 776: 754: 740: 732: 602: 469: 467: 465: 463: 461: 459: 306:enhancement has a V-shaped configuration. 109: 70: 43: 146:The most common sign at presentation is 426: 231:facioscapulohumeral muscular dystrophy 170:, but are actually the result of both 7: 1622:Familial exudative vitreoretinopathy 1745:non-arteritic anterior (NAION) 1582:Leber's hereditary optic neuropathy 1617:Persistent tunica vasculosa lentis 25: 1345:Polypoidal choroidal vasculopathy 1450:Central retinal artery occlusion 385: 2300:Disorders of choroid and retina 1455:Branch retinal artery occlusion 1184:Terrien's marginal degeneration 437:J Pediatr Ophthalmol Strabismus 1485:Bietti's crystalline dystrophy 1445:Central retinal vein occlusion 1174:Pellucid marginal degeneration 1: 1538:Vitelliform macular dystrophy 1429:Posterior vitreous detachment 1295:Persistent pupillary membrane 2025:Leber's congenital amaurosis 1936:Internuclear ophthalmoplegia 1627:Vogt-Koyanagi-Harada disease 1612:Persistent fetal vasculature 1543:Leber's congenital amaurosis 824:Meibomian gland dysfunction 2316: 1548:Birdshot chorioretinopathy 1522:Central serous retinopathy 1340:Focal choroidal excavation 1213:Corneal neovascularization 1030:Subconjunctival hemorrhage 296:Magnetic Resonance Imaging 29: 2260: 2247: 2015: 1931:Convergence insufficiency 1804: 1673: 1656: 1643: 1572:Primary juvenile glaucoma 1417:Cytomegalovirus retinitis 1059: 1046: 832: 788: 775: 766:Diseases of the human eye 354:as a response to injury. 78: 69: 1441:Ocular ischemic syndrome 286:Imaging studies such as 1941:One and a half syndrome 1695:Foster Kennedy syndrome 292:Computerized Tomography 2180:Argyll Robertson pupil 980:Periorbital cellulitis 338: 327: 312:proton MR spectroscopy 283: 190: 81:conjunctival hyperemia 60:Retinal telangiectasis 2153:Scintillating scotoma 1819:Kearns–Sayre syndrome 1762:Toxic and nutritional 1735:posterior (PION) 1218:Kayser–Fleischer ring 336: 325: 281: 247:blood-retinal barrier 188: 34:. For the plant, see 1926:Conjugate gaze palsy 1797:Paralytic strabismus 1730:anterior (AION) 1512:Retinitis pigmentosa 1507:Macular degeneration 1280:Intermediate uveitis 1196:Keratoconjunctivitis 255:cholesterol crystals 210:is likely to occur. 36:Lupinus flavoculatus 2231:Childhood blindness 2210:Parinaud's syndrome 1780:Extraocular muscles 1568:Ocular hypertension 1532:Epiretinal membrane 1517:Retinal haemorrhage 1372:Congenital cataract 318:Pathologic findings 56:Exudative retinitis 1757:Leber's hereditary 1424:Retinal detachment 1377:Childhood cataract 1189:Post-LASIK ectasia 970:Orbital cellulitis 919:Lacrimal apparatus 725:2021-12-30 at the 687:External resources 397:. You can help by 339: 328: 284: 208:retinal detachment 191: 172:retinal detachment 142:Signs and symptoms 85:mild corneal edema 2282: 2281: 2278: 2277: 2243: 2242: 2239: 2238: 2185:Marcus Gunn pupil 2161: 2160: 2082:Visual impairment 1949: 1948: 1855: 1854: 1842:Fourth-nerve (IV) 1770: 1769: 1707:Optic disc drusen 1639: 1638: 1635: 1634: 1353: 1352: 1241: 1240: 1147:Corneal dystrophy 1042: 1041: 1038: 1037: 913: 912: 710: 709: 415: 414: 251:endothelial cells 118: 117: 41:Medical condition 16:(Redirected from 2307: 2249: 2013: 2004:Vision disorders 1963:Refractive error 1905:Brown's syndrome 1861:Other strabismus 1847:Sixth-nerve (VI) 1837:Oculomotor (III) 1809:Ophthalmoparesis 1802: 1793: 1784:Binocular vision 1717:Optic neuropathy 1683:optic papillitis 1671: 1658: 1645: 1534:(Macular pucker) 1252: 1233:Band keratopathy 1070: 1061: 1048: 975:Orbital lymphoma 862:Blepharophimosis 852:Blepharochalasis 799: 790: 777: 756: 749: 742: 733: 603: 591: 588: 582: 571: 565: 562: 556: 553: 547: 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1965: 1964: 1961: 1960: 1958: 1956: 1952: 1942: 1939: 1937: 1934: 1932: 1929: 1927: 1924: 1923: 1921: 1917: 1911: 1908: 1906: 1903: 1901: 1898: 1894: 1891: 1889: 1886: 1885: 1884: 1881: 1879: 1876: 1874: 1870: 1867: 1866: 1864: 1862: 1858: 1848: 1845: 1843: 1840: 1838: 1835: 1834: 1832: 1830: 1826: 1820: 1817: 1815: 1812: 1810: 1807: 1806: 1803: 1800: 1798: 1794: 1791: 1789: 1788:Accommodation 1785: 1781: 1777: 1773: 1763: 1760: 1758: 1755: 1753: 1750: 1746: 1743: 1741: 1738: 1736: 1733: 1731: 1728: 1727: 1726: 1723: 1722: 1720: 1718: 1714: 1708: 1705: 1703: 1702:Optic atrophy 1700: 1696: 1693: 1692: 1691: 1688: 1684: 1681: 1680: 1679: 1676: 1675: 1672: 1669: 1667: 1663: 1659: 1655: 1651: 1646: 1642: 1628: 1625: 1623: 1620: 1618: 1615: 1613: 1610: 1608: 1605: 1603: 1602:Keratomycosis 1600: 1598: 1597:Globe rupture 1595: 1593: 1590: 1588: 1585: 1583: 1580: 1578: 1575: 1573: 1569: 1565: 1562: 1561: 1559: 1555: 1549: 1546: 1544: 1541: 1539: 1536: 1533: 1530: 1528: 1527:Macular edema 1525: 1523: 1520: 1518: 1515: 1513: 1510: 1508: 1505: 1501: 1498: 1496: 1493: 1491: 1488: 1486: 1483: 1481: 1478: 1476: 1473: 1471: 1468: 1466: 1463: 1462: 1461: 1458: 1456: 1453: 1451: 1448: 1446: 1442: 1439: 1437: 1436:Retinoschisis 1434: 1430: 1427: 1426: 1425: 1422: 1418: 1415: 1413: 1410: 1409: 1408: 1405: 1404: 1402: 1400: 1396: 1390: 1387: 1385: 1382: 1378: 1375: 1373: 1370: 1369: 1368: 1365: 1364: 1362: 1360: 1356: 1346: 1343: 1341: 1338: 1334: 1331: 1330: 1329: 1326: 1324: 1323:Choroideremia 1321: 1320: 1318: 1316: 1312: 1306: 1303: 1301: 1300:Iridodialysis 1298: 1296: 1293: 1291: 1288: 1286: 1283: 1281: 1278: 1276: 1273: 1272: 1270: 1265: 1262: 1260: 1257: 1256: 1253: 1250: 1248: 1244: 1234: 1231: 1229: 1228:Arcus senilis 1226: 1224: 1223:Haab's striae 1221: 1219: 1216: 1214: 1211: 1209: 1206: 1202: 1199: 1198: 1197: 1194: 1190: 1187: 1185: 1182: 1180: 1177: 1175: 1172: 1170: 1167: 1166: 1165: 1162: 1158: 1155: 1153: 1150: 1149: 1148: 1145: 1143: 1140: 1138: 1137:Corneal ulcer 1135: 1131: 1128: 1126: 1123: 1121: 1118: 1116: 1115:acanthamoebic 1113: 1111: 1108: 1107: 1106: 1103: 1102: 1100: 1098: 1094: 1088: 1085: 1083: 1080: 1079: 1077: 1075: 1071: 1068: 1066: 1065:Fibrous tunic 1062: 1058: 1054: 1049: 1045: 1031: 1028: 1026: 1023: 1021: 1018: 1016: 1013: 1009: 1006: 1005: 1004: 1001: 999: 996: 995: 993: 991: 987: 981: 978: 976: 973: 971: 968: 966: 963: 961: 958: 957: 955: 953: 949: 943: 942:Xerophthalmia 940: 938: 935: 933: 930: 928: 925: 924: 922: 920: 916: 906: 903: 901: 898: 896: 893: 891: 888: 887: 885: 883: 879: 873: 870: 868: 865: 863: 860: 858: 855: 853: 850: 848: 847:Lagophthalmos 845: 843: 840: 838: 835: 834: 831: 825: 822: 820: 817: 815: 812: 810: 807: 806: 804: 800: 797: 795: 791: 787: 783: 778: 774: 767: 764: 763: 757: 752: 750: 745: 743: 738: 737: 734: 728: 724: 721: 718: 716: 713: 712: 702: 698: 697: 693: 692: 689: 685: 678: 674: 673: 669: 667: 663: 662: 658: 656: 652: 651: 647: 645: 641: 640: 636: 632: 630: 626: 625: 621: 617: 616: 613: 608: 604: 597: 587: 584: 581: 580:Who Named It? 577: 576: 570: 567: 561: 558: 552: 549: 543: 540: 534: 531: 525: 522: 516: 513: 507: 504: 498: 495: 488: 485: 479: 476: 470: 468: 466: 464: 462: 460: 456: 451: 447: 443: 439: 438: 430: 427: 420: 418: 409: 400: 396: 393:This section 391: 388: 384: 383: 377: 375: 373: 369: 365: 364:Laser surgery 357: 355: 353: 349: 345: 335: 331: 324: 317: 315: 313: 307: 303: 301: 297: 293: 289: 280: 273: 271: 264: 262: 260: 256: 252: 248: 240: 238: 236: 232: 227: 225: 221: 217: 213: 209: 203: 196: 194: 187: 183: 180: 175: 173: 169: 165: 161: 157: 153: 149: 141: 139: 137: 132: 130: 126: 122: 112: 106: 105:Ophthalmology 103: 101: 97: 94: 90: 86: 82: 77: 73: 68: 65: 61: 57: 54: 50: 45: 37: 33: 19: 2080: / 2076: / 2055:Monochromacy 1987: / 1883:Heterophoria 1871: / 1570: / 1566: / 1489: 1470:hypertensive 1443: / 1264:Ciliary body 1179:Keratoglobus 1087:Episcleritis 965:Enophthalmos 960:Exophthalmos 905:Trichomegaly 900:Distichiasis 802:Inflammation 694: 670: 659: 648: 633: 618: 586: 573: 569: 560: 551: 542: 533: 524: 515: 506: 497: 487: 478: 444:(5): 313–5. 441: 435: 429: 416: 403: 399:adding to it 394: 361: 344:erythrocytes 340: 329: 308: 304: 285: 268: 244: 241:Pathogenesis 228: 204: 200: 197:Presentation 192: 176: 145: 133: 120: 119: 87:, posterior 63: 59: 55: 2205:Cycloplegia 2148:Photophobia 2143:Hemeralopia 2100:Hemianopsia 2078:Vision loss 1989:Aniseikonia 1980:Astigmatism 1900:Cyclotropia 1878:Hypertropia 1690:Papilledema 1662:Optic nerve 1495:Sickle cell 1475:Purtscher's 1460:Retinopathy 1328:Choroiditis 1169:Keratoconus 990:Conjunctiva 867:Xanthelasma 819:Blepharitis 372:optic nerve 368:cryotherapy 348:hemosiderin 259:macrophages 224:enucleation 52:Other names 32:Yellow Eyes 2289:Categories 2254:Infections 2175:Anisocoria 2138:Asthenopia 2131:subjective 2115:homonymous 2110:bitemporal 2062:Nyctalopia 2050:Dichromacy 1994:Presbyopia 1955:Refraction 1776:Strabismus 1666:Optic disc 1025:Pinguecula 890:Trichiasis 672:DiseasesDB 421:References 148:leukocoria 18:Yellow eye 2295:Blindness 2226:Nystagmus 2200:Mydriasis 2074:Blindness 2020:Amblyopia 2008:Blindness 1968:Hyperopia 1893:Exophoria 1888:Esophoria 1873:Exotropia 1869:Esotropia 1407:Retinitis 1105:Keratitis 1082:Scleritis 1015:Pterygium 895:Madarosis 842:Ectropion 837:Entropion 814:Chalazion 575:synd/2146 358:Treatment 300:diagnosis 294:(CT) and 265:Diagnosis 220:cataracts 160:photopsia 125:blindness 100:Specialty 89:synechiae 2265:Trachoma 2030:Diplopia 1725:Ischemic 1650:Pathways 1564:Glaucoma 1465:diabetic 1367:Cataract 1305:Synechia 1157:Meesmann 1125:Exposure 1110:herpetic 1008:allergic 998:Chemosis 932:Epiphora 723:Archived 696:Orphanet 450:17022167 212:Glaucoma 164:floaters 156:parallax 93:cataract 2105:binasal 2092:Anopsia 2035:Scotoma 1829:palsies 1592:Red eye 1577:Floater 1384:Aphakia 1315:Choroid 1285:Hyphema 1275:Uveitis 882:Eyelash 666:D058456 378:History 352:gliosis 249:in the 216:atrophy 2195:Miosis 1973:Myopia 1752:Kjer's 1500:photic 1399:Retina 1152:Fuchs' 1120:fungal 1097:Cornea 1074:Sclera 857:Ptosis 794:Eyelid 782:Adnexa 655:300216 644:362.12 448:  346:, and 290:(US), 218:, and 162:, and 129:retina 107:  2219:Other 2167:Pupil 1557:Other 1201:sicca 1053:Globe 952:Orbit 677:31162 629:H35.0 1359:Lens 1259:Iris 809:Stye 661:MeSH 650:OMIM 639:9-CM 446:PMID 154:and 91:and 701:190 635:ICD 620:ICD 578:at 492:27. 401:. 366:or 226:). 2291:: 699:: 675:: 664:: 653:: 642:: 627:: 624:10 458:^ 442:43 440:. 314:. 237:. 214:, 138:. 83:, 62:, 58:, 755:e 748:t 741:v 637:- 622:- 612:D 452:. 408:) 404:( 38:. 20:)

Index

Yellow eye
Yellow Eyes
Lupinus flavoculatus

conjunctival hyperemia
mild corneal edema
synechiae
cataract
Specialty
Ophthalmology
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blindness
retina
retinoblastoma
leukocoria
depth perception
parallax
photopsia
floaters
hallucinations
retinal detachment
red-eye effect

retinal detachment
Glaucoma
atrophy
cataracts
enucleation
facioscapulohumeral muscular dystrophy
D4Z4 deletions

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