Preview

Russian Ophthalmological Journal

Advanced search

Coats' disease: current treatment trends. A literature review

https://doi.org/10.21516/2072-0076-2025-18-4-185-189

Abstract

In 1908, G. Coats first described a group of patients with eye damage, manifested by telangiectasia with massive intraand subretinal exudation, leading to local elevation of the retina and/or its complete detachment. Until now, a generally accepted pathogenesis model has not been developed and the causes of Coats disease (CD) have not been identified. It was assumed that the cause of the disease could be an infectious process, an inflammatory reaction, but there is currently no consensus on this issue. A study of the cytokine profile showed an increased concentration of VEGF in the intraocular, subretinal fluid and vitreous humor, an increase in the concentration of IL-6, IL-1b and MCP-1 levels was also noted. Complications of CD include vasoproliferative tumors, neovascular glaucoma, intraretinal and intraocular hemorrhages, vitreous fibrosis, traction retinal detachment. CD treatment includes laser coagulation, сryotherapy, intravitreal administration of glucocorticosteroids and angiogenesis inhibitors. In severe forms and advanced stages of CD, vitreoretinal surgery is resorted to. Enucleation may be necessary is some cases.

About the Authors

N. V. Neroeva
Helmholtz National Medical Research Center of Eye Diseases
Russian Federation

Natalia V. Neroeva — Dr. of Med. Sci., head of the department of pathology of the retina and optic nerve

14/19, Sadovaya-Chernogryazskaya St., Moscow, 105062



V. E. Tankovskiy
Helmholtz National Medical Research Center of Eye Diseases
Russian Federation

Vladimir E. Tankovskiy  — Dr. of Med. Sci., senior researcher of the department of pathology of the retina and optic nerve, associate professor of chair of continuing medical education

14/19, Sadovaya-Chernogryazskaya St., Moscow, 105062



A. I. Ushakov
Helmholtz National Medical Research Center of Eye Diseases
Russian Federation

Alexander I. Ushakov — junior researcher of the department of retina and optic nerve pathology

14/19, Sadovaya-Chernogryazskaya St., Moscow, 105062



References

1. Coats G. Forms of retinal diseases with massive exudation. Roy Lond Ophthalmol Hosp Rep. 1908; 17: 440–525.

2. Katsnel'son L.A., Forofonova T.I., Bunin A.Ya. Vascular eye diseases. Moscow: Meditsina; 1990 (In Russ.).

3. Sun Y, Jain A, Moshfeghi DM. Elevated vascular endothelial growth factor levels in Coats disease: rapid response to pegaptanib sodium. Graefes Arch Clin Exp Ophthalmol. 2007 Sep; 245 (9): 1387–8. doi: 10.1007/s00417-007-0559-8

4. He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats' disease and possible therapeutic role of bevacizumab. Graefes Arch Clin Exp Ophthalmol. 2010 Oct; 248 (10): 1519–21. doi: 10.1007/s00417-010-1366-1

5. Yang Q, Wei W, Shi X, Yang L. Successful use of intravitreal ranibizumab injection and combined treatment in the management of Coats' disease. Acta Ophthalmol. 2016 Jun; 94 (4): 401–6. doi: 10.1111/aos.13067

6. Cakir M, Ceki O, Yilmaz OF. Combined intravitreal bevacizumab and triamcinolone injection in a child with Coats disease. J AAPOS. 2008 Jun; 12 (3): 309–11. doi: 10.1016/j.jaapos.2008.01.009

7. Saatci AO, Doruk HC, Yaman A. Intravitreal dexamethasone implant (ozurdex) in coats' disease. Case Rep Ophthalmol. 2013 Sep 21; 4 (3): 122–8. doi: 10.1159/000355363

8. Smithen LM, Brown GC, Brucker AJ, et al. Coats' disease diagnosed in adulthood. Ophthalmology. 2005 Jun; 112 (6): 1072–8. doi: 10.1016/j.ophtha.2004.12.038

9. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J. Classification and management of Coats disease: the 2000 Proctor Lecture. Am J Ophthalmol. 2001 May; 131 (5): 572–83. doi: 10.1016/s0002-9394(01)00896-0

10. Jones JH, Kroll AJ, Lou PL, Ryan EA. Coats' disease. Int Ophthalmol Clin. 2001 Fall; 41 (4): 189–98. doi: 10.1097/00004397-200110000-00017

11. Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture. Retina. 2002 Feb; 22 (1): 80–91. doi: 10.1097/00006982-200202000-00014

12. 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 May; 131 (5): 561–71. doi: 10.1016/s0002-9394(00)00883-7

13. Imre G. Coats' disease and hyperlipemic retinitis. Am J Ophthalmol. 1967 Oct; 64 (4): 726–33. doi: 10.1016/0002-9394(67)92855-3

14. Tripathi R, Ashton N. Electron microscopical study of Coat's disease. Br J Ophthalmol. 1971 May; 55 (5): 289–301. doi: 10.1136/bjo.55.5.289

15. Zhao Q, Peng XY, Chen FH, et al. Vascular endothelial growth factor in Coats' disease. Acta Ophthalmol. 2014 May; 92 (3): e225-8. doi: 10.1111/aos.12158

16. Feng J, Zheng X, Li B, Jiang Y. Differences in aqueous concentrations of cytokines in paediatric and adult patients with Coats' disease. Acta Ophthalmol. 2017 Sep; 95 (6): 608–612. doi: 10.1111/aos.13151

17. Kase S, Rao NA, Yoshikawa H, et al. Expression of vascular endothelial growth factor in eyes with Coats' disease. Invest Ophthalmol Vis Sci. 2013 Jan 7; 54 (1): 57–62. doi: 10.1167/iovs.12-10613

18. Zhang J, Jiang C, Ruan L, Huang X. Associations of cytokine concentrations in aqueous humour with retinal vascular abnormalities and exudation in Coats' disease. Acta Ophthalmol. 2019 May; 97 (3): 319–24. doi: 10.1111/aos.13971

19. Liang T, Xu Y, Zhu X, et al. Aqueous humour cytokines profiles in eyes with Coats disease and the association with the severity of the disease. BMC Ophthalmol. 2020 May 5; 20 (1): 178. doi: 10.1186/s12886-020-01421-0

20. Sen M, Shields CL, Honavar SG, Shields JA. Coats disease: An overview of classification, management and outcomes. Indian J Ophthalmol. 2019 Jun; 67 (6): 763–71. doi: 10.4103/ijo.IJO_841_19

21. Pesch KJ, Meyer-Schwickerath G. Light coagulation in morbus Coats and Leber’s retinitis. Klin Monbl Augenheilkd. 1967; 151 (6): 846–53. German. PMID: 5592979.

22. Egerer I, Tasman W, Tomer TT. Coats disease. Arch Ophthalmol. 1974 Aug; 92 (2): 109–12. doi: 10.1001/archopht.1974.01010010115006

23. Ridley ME, Shields JA, Brown GC, Tasman W. Coats' disease. Evaluation of management. Ophthalmology. 1982 Dec; 89 (12): 1381–7. doi: 10.1016/s0161-6420(82)34634-5

24. McGrand JC. Photocoagulation in Coats’ disease. Trans Ophthalmol Soc UK. 1970; 90: 47–56. PMID: 5283418.

25. Shchuko A.G., Bukina V.V., Yuryeva T.N., et al. Tactics of managing patients with Coats' disease. Modern technologies in ophthalmology. 2017; 14 (1): 359–62 (In Russ.).

26. Adam RS, Kertes PJ, Lam WC. Observations on the management of Coats' disease: less is more. Br J Ophthalmol. 2007 Mar; 91 (3): 303–6. doi: 10.1136/bjo.2006.103382

27. Glaser BM, Vidaurri-Leal J, Michels RG, Campochiaro PA. Cryotherapy during surgery for giant retinal tears and intravitreal dispersion of viable retinal pigment epithelial cells. Ophthalmology. 1993 Apr; 100 (4): 466–70. doi: 10.1016/s0161-6420(93)31620-9

28. Nagy JA, Benjamin L, Zeng H, Dvorak AM, Dvorak HF. Vascular permeability, vascular hyperpermeability and angiogenesis. Angiogenesis. 2008; 11 (2): 109–19. doi: 10.1007/s10456-008-9099-z

29. Vavvas D, D'Amico DJ. Pegaptanib (Macugen): treating neovascular age-related macular degeneration and current role in clinical practice. Ophthalmol Clin North Am. 2006 Sep; 19 (3): 353–60. doi: 10.1016/j.ohc.2006.05.008

30. Bai J, Song Z, Li G, Dong L, Zhang C. Efficacy and safety of anti-vascular endothelial growth factor drugs for coats' disease treatment: A systematic review. J Ocul Pharmacol Ther. 2023 Sep; 39 (7): 418–29. doi: 10.1089/jop.2023.0028

31. Ramasubramanian A, Shields CL. Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction. Br J Ophthalmol. 2012 Mar; 96 (3): 356–9. doi: 10.1136/bjophthalmol-2011-300141

32. Bhat V, D'Souza P, Shah PK, Narendran V. Risk of tractional retinal detachment following intravitreal Bevacizumab along with subretinal fluid drainage and cryotherapy for stage 3B Coats' Disease. Middle East Afr J Ophthalmol. 2016 Apr-Jun; 23 (2): 208–11. doi: 10.4103/0974-9233.175895

33. Jun JH, Kim YC, Kim KS. Resolution of severe macular edema in adult coats' disease with intravitreal triamcinolone and bevacizumab injection. Korean J Ophthalmol. 2008 Sep; 22 (3): 190–3. doi: 10.3341/kjo.2008.22.3.190

34. Jarin RR, Teoh SC, Lim TH. Resolution of severe macular oedema in adult Coat's syndrome with high-dose intravitreal triamcinolone acetonide. Eye (Lond). 2006 Feb; 20 (2): 163–5. doi: 10.1038/sj.eye.6701828

35. Othman IS, Moussa M, Bouhaimed M. Management of lipid exudates in Coats disease by adjuvant intravitreal triamcinolone: effects and complications. Br J Ophthalmol. 2010 May; 94 (5): 606–10. doi: 10.1136/bjo.2009.168013

36. Jonas JB. Intravitreal triamcinolone acetonide as treatment for extensive exudative retinal detachment. Br J Ophthalmol. 2004 Apr; 88 (4): 587–8. doi: 10.1136/bjo.2003.028688

37. Bhm MR, Uhlig CE. Use of intravitreal triamcinolone and bevacizumab in Coats' disease with central macular edema. Graefes Arch Clin Exp Ophthalmol. 2011 Jul; 249 (7): 1099–101. doi: 10.1007/s00417-011-1629-5

38. Mart nez-Castillo S, Gallego-Pinazo R, Dolz-Marco R, Mar n-Lamb es C, D az-Llopis M. Adult coats' disease successfully managed with the dexamethasone intravitreal implant (ozurdex®) combined with retinal photocoagulation. Case Rep Ophthalmol. 2012 Jan; 3 (1): 123–7. doi: 10.1159/000337481

39. Kumar K, Raj P, Chandnani N, Agarwal A. Intravitreal dexamethasone implant with retinal photocoagulation for adult-onset Coats' disease. Int Ophthalmol. 2019 Feb; 39 (2): 465–70. doi: 10.1007/s10792-018-0827-0

40. Chen Q, Liang S, Wang X, et al. Efficacy and safety outcomes of intravitreal dexamethasone implant therapy for the treatment of adult Coats' disease. J Ophthalmol. 2020 Oct 1; 2020: 9131908. doi: 10.1155/2020/9131908

41. Suesskind D, Altpeter E, Schrader M, Bartz-Schmidt KU, Aisenbrey S. Pars plana vitrectomy for treatment of advanced Coats' disease — presentation of a modified surgical technique and long-term follow-up. Graefes Arch Clin Exp Ophthalmol. 2014 Jun; 252 (6): 873–9. doi: 10.1007/s00417-013-2512-3

42. Mrejen S, Metge F, Denion E, et al. Management of retinal detachment in Coats disease. Study of 15 cases. Retina. 2008 Mar; 28 (3 Suppl): S26-32. doi: 10.1097/IAE.0b013e31816b3158. Erratum in: Retina. 2009 Jan; 29 (1): 127. PMID: 18317340.

43. Li AS, Capone A Jr, Trese MT, et al. Long-term outcomes of total exudative retinal detachments in stage 3B Coats disease. Ophthalmology. 2018 Jun; 125 (6): 887–93. doi: 10.1016/j.ophtha.2017.12.010. Epub 2018 Feb 1. PMID: 29361355.

44. Stanga PE, Jaberansari H, Bindra MS, Gil-Martinez M, Biswas S. Transcleral drainage of subretinal fluid, anti-vascular endothelial growth factor, and wide-field imaging-guided laser in Coats exudative retinal detachment. Retina. 2016 Jan; 36 (1): 156–62. doi: 10.1097/IAE.0000000000000669

45. Cai X, Zhao P, Zhang Q, Jin H. Treatment of stage 3 Coats' disease by endolaser photocoagulation via a two-port pars plana nonvitrectomy approach. Graefes Arch Clin Exp Ophthalmol. 2015 Jul; 253 (7): 999–1004. doi: 10.1007/s00417-015-2984-4

46. Yoshizumi MO, Kreiger AE, Lewis H, Foxman B, Hakakha BA. Vitrectomy techniques in late-stage Coats'-like exudative retinal detachment. Doc Ophthalmol. 1995; 90 (4): 387–94. doi: 10.1007/BF01268124

47. Kusaka S. Surgical management of Coats disease. Asia Pac J Ophthalmol (Phila). 2018 May-Jun; 7 (3): 156–9. doi: 10.22608/APO.201867

48. Jumper JM, Pomerleau D, McDonald HR, et al. Macular fibrosis in Coats disease. Retina. 2010 Apr; 30 (4 Suppl): S9–14. doi: 10.1097/iae.0b013e3181cfd3e7

49. Shukla D, Chakraborty S, Behera UC, Kim R. Vitrectomy for epimacular membrane secondary to adult-onset Coats' disease. Ophthalmic Surg Lasers Imaging. 2008 May-Jun; 39 (3): 239–41. doi: 10.3928/15428877-20080501-15

50. Sigler EJ, Randolph JC, Calzada JI, Wilson MW, Haik BG. Current management of Coats disease. Surv Ophthalmol. 2014 Jan-Feb; 59 (1): 30–46. doi: 10.1016/j.survophthal.2013.03.007


Review

For citations:


Neroeva N.V., Tankovskiy V.E., Ushakov A.I. Coats' disease: current treatment trends. A literature review. Russian Ophthalmological Journal. 2025;18(4):185-189. (In Russ.) https://doi.org/10.21516/2072-0076-2025-18-4-185-189

Views: 22


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2072-0076 (Print)
ISSN 2587-5760 (Online)