Progress in diagnosis and treatment of adult Coats disease_Chinese Journal of Ocular Fundus Diseases

Authors：

He Lu ,  Chen Changzheng

Eye Center, People’s Hospital of Wuhan University, Wuhan 430060, China;

Corresponding author：

Chen Changzheng, Email: whuchenchzh@163.com

Keywords：

Retinal telangiectasis/diagnosis; Retinal telangiectasis/therapy; Review

DOI：

10.3760/cma.j.issn.1005-1015.2018.03.024

Video：

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Adult Coats disease is characterized by abnormal expansion of retinal capillaries, often accompanied by massive lipid exudation and exudative retinal detachment. Unlike Coats disease in young children, adult Coats disease is mostly limited to peripheral retina, with slow progress and better prognosis. Adult Coats disease should be identified with Coats-like diseases such as exudative age-related macular degeneration, diabetic retinopathy, obsolete retinal vein occlusion, idiopathic macular telangiectasia 1, obsolete posterior uveitis, retinal vasculitis, or acute retinal necrosis. Because the pathogenesis of Coats disease is not clear, it lacks specific treatment measures for the cause of disease. The purpose of simple or combined laser photocoagulation, freezing, vitreous intravitreal injection against vascular endothelial growth factor drugs or triamcinolone and surgery is to eliminate abnormal blood vessels and exudation, maintain visual function, which can also improve retinal detachment and prevent neovascular glaucoma and other complications. To explore the similarities and differences of adult Coats disease with Coats disease in young children, to further promote the study of the pathogenesis of adult Coats disease and to provide new targets for its treatment are the direction of future research.

Citation： He Lu, Chen Changzheng. Progress in diagnosis and treatment of adult Coats disease. Chinese Journal of Ocular Fundus Diseases, 2018, 34(3): 299-302. doi: 10.3760/cma.j.issn.1005-1015.2018.03.024 Copy

1.	Morris B, Foot B, Mulvihill A. A population-based study of Coats disease in the United Kingdom Ⅰ: epidemiology and clinical features at diagnosis [J]. Eye (Lond), 2010, 24(12): 1797-1801. DOI: 10.1038/eye.2010.126.
2.	张承芬.外层渗出性视网膜病变[M]//张承芬. 眼底病学. 2版. 北京: 人民卫生出版社, 2010: 307-314.Zhang CF. External exudative retinopathy[M]//Zhang CF. Diseases of ocular fundus. Beijing: People's Medical Publishing House, 2010: 307-314.
3.	Rishi E, Rishi P, Appukuttan B, et al. Coats' disease of adult-onset in 48 eyes [J]. Indian J Ophthalmol, 2016, 64(7): 518-523. DOI: 10.4103/0301-4738.190141.
4.	张承芬, 董方田, 王韧琰, 等. 成年Coats病的临床特征和诊治[J]. 中华眼底病杂志, 2008, 24(4): 279-282.Zhang CF, Dong FT, Wang RY, et al. Clinical characteristics and treatment of Coats' disease in adulthood[J]. Chin J Ocul Fundus Dis, 2008, 24(4): 279-282.
5.	Smithen LM, Brown GC, Brucker AJ, et al. Coats' disease diagnosed in adulthood [J]. Ophthalmology, 2005, 112(6): 1072-1078.
6.	Shields JA, Shields CL, Honavar SG, et al. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture [J]. Am J Ophthalmol, 2001, 131(5): 561-571.
7.	Otani T, Yasuda K, Aizawa N, et al. Over 10 years follow-up of Coats' disease in adulthood [J]. Clin ophthalmol, 2011, 5: 1729-1732. DOI: 10.2147/OPTH.S27938.
8.	Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats disease: the 2000 Proctor Lecture [J]. Am J Ophthalmol, 2001, 131(5): 572-583.
9.	Kumar V, Chandra P, Kumar A. Ultra-wide field imaging in the diagnosis and management of adult-onset Coats' disease [J]. Clin Exp Optom, 2017, 100(1): 79-82. DOI: 10.1111/cxo.12418.
10.	Yonekawa Y, Todorich B, Trese MT. Optical coherence tomography angiography findings in Coats' disease [J]. Ophthalmology, 2016, 123(9): 1964. DOI: 10.1016/j.ophtha.2016.05.004.
11.	曹绪胜, 彭晓燕. Coats病临床诊断中的问题分析[J]. 中华眼底病杂志, 2005, 21(6): 377-380.Cao XS. Analysis of the problems in the clinical diagnosis of Coats disease[J]. Chin J Ocul Fundus Dis, 2005, 21(6): 377-380.
12.	Sigler EJ, Randolph JC, Calzada JI, et al. Current management of Coats disease [J]. Surv Ophthalmol, 2014, 59(1): 30-46. DOI: 10.1016/j.survophthal.2013.03.007.
13.	胡立影, 李志清, 于荣国, 等. 特发性黄斑毛细血管扩张症的研究现状与进展[J]. 中华眼底病杂志, 2016, 32(4): 440-444. DOI: 10.3760/cma.j.issn.1005-1015.2016.04.026.Hu LY, Li ZQ, Yu RG, et al. The status and progress of studies of idiopathic parafoveal telangiectasis[J]. Chin J Ocul Fundus Dis, 2016, 32(4): 440-444. DOI: 10.3760/cma.j.issn.1005-1015.2016.04.026.
14.	Egbert PR, Chan CC, Winter FC. Flat preparations of the retinal vessels in Coats' disease [J]. J Pediatr Ophthalmol, 1976, 13(6): 336-339.
15.	Fernandes BF, Odashiro AN, Maloney S, et al. Clinical-histopathological correlation in a case of Coats' disease [J]. Diagn Pathol, 2006, 1(1): 24. DOI: 10.1186/1746-1596-1-24.
16.	Steidl SM, Hirose T, Sang D, et al. Difficulties in excluding the diagnosis of retinoblastoma in cases of advanced Coats' disease: a clinicopathologic report [J]. Ophthalmologica, 1996, 210(6): 336-340. DOI: 10.1159/000310735.
17.	Kase S, Rao NA, Yoshikawa H, et al. Expression of vascular endothelial growth factor in eyes with Coats' disease [J]. Invest Ophthalmol Vis Sci, 2013, 54(1): 57-62. DOI: 10.1167/iovs.12-10613.
18.	He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats' disease and possible therapeutic role of bevacizumab [J]. Graefe's Arch Clin Exp Ophthalmol, 2010, 248(10): 1519-1521. DOI: 10.1007/s00417-010-1366-1.
19.	Sun Y, Jain A, Moshfeghi DM. Elevated vascular endothelial growth factor levels in Coats disease: rapid response to pegaptanib sodium [J]. Graefe's Arch Clin Exp Ophthalmol, 2007, 245(9): 1387-1388. DOI: 10.1007/s00417-007-0559-8.
20.	Zhao Q, Peng XY, Chen FH, et al. Vascular endothelial growth factor in Coats' disease [J]. Acta Ophthalmol, 2014, 92(3): 225-228. DOI: 10.1111/aos.12158.
21.	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 [J]. Hum Mol Genet, 1999, 8(11): 2031-2035.
22.	Mulvihill A, Morris B. A population-based study of Coats disease in the United Kingdom Ⅱ: investigation, treatment, and outcomes [J]. Eye (Lond), 2010, 24(12): 1802-1807. DOI: 10.1038/eye.2010.127.
23.	Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture [J]. Retina, 2002, 22(1): 80-91.
24.	Zheng XX, Jiang YR. The effect of intravitreal bevacizumab injection as the initial treatment for Coats' disease [J]. Graefe's Arch Clin Exp Ophthalmol, 2014, 252(1): 35-42. DOI: 10.1007/s00417-013-2409-1.
25.	Wang KY, Cheng CK. A combination of intravitreal bevacizumab injection with tunable argon yellow laser photocoagulation as a treatment for adult-onset Coats' disease [J]. J Ocul Pharmacol Ther, 2011, 27(5): 525-530. DOI: 10.1089/jop.2011.0088.
26.	Bohm MR, Uhlig CE. Use of intravitreal triamcinolone and bevacizumab in Coats' disease with central macular edema [J]. Graefe's Arch Clin Exp Ophthalmol, 2011, 249(7): 1099-1101. DOI: 10.1007/s00417-011-1629-5.
27.	Kim J, Park KH, Woo SJ. Combined photodynamic therapy and intravitreal bevacizumab injection for the treatment of adult Coats' disease: a case report [J]. Korean J Ophthalmol, 2010, 24(6): 374-376. DOI: 10.3341/kjo.2010.24.6.374.
28.	Park S, Cho HJ, Lee DW, et al. Intravitreal bevacizumab injections combined with laser photocoagulation for adult-onset Coats' disease [J]. Graefe's Arch Clin Exp Ophthalmol, 2016, 254(8): 1511-1517. DOI: 10.1007/s00417-016-3391-1.
29.	Goel N, Kumar V, Seth A, et al. Role of intravitreal bevacizumab in adult onset Coats' disease [J]. Int Ophthalmol, 2011, 31(3): 183-190. DOI: 10.1007/s10792-011-9436-x.
30.	杨琼, 魏文斌, 刘月明, 等. 单纯玻璃体腔注射雷珠单抗及其联合其他方法治疗3期以上Coats病的疗效观察[J]. 中华眼底病杂志, 2014, 30(5): 462-465. DOI: 10.3760/cma.j.issn.1005-1015.2014.05.008.Yang Q, Wei W, Liu Y, et al. Intravitreal injection of ranibizumab and combined treatment for severe Coats disease[J]. Chin J Ocul Fundus Dis, 2014. 30(5) : 462-465. DOI: 10.3760/cma.j.issn.1005-1015.2014.05.008.
31.	Ramasubramanian A, Shields CL. Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction [J]. Br J Ophthalmol, 2012, 96(3): 356-359. DOI: 10.1136/bjophthalmol-2011-300141.
32.	Jun JH, Kim YC, Kim KS. Resolution of severe macular edema in adult coats' disease with intravitreal triamcinolone and bevacizumab injection [J]. Korean J Ophthalmol, 2008, 22(3): 190-193. DOI: 10.3341/kjo.2008.22.3.190.

1. Morris B, Foot B, Mulvihill A. A population-based study of Coats disease in the United Kingdom Ⅰ: epidemiology and clinical features at diagnosis [J]. Eye (Lond), 2010, 24(12): 1797-1801. DOI: 10.1038/eye.2010.126.
2. 张承芬.外层渗出性视网膜病变[M]//张承芬. 眼底病学. 2版. 北京: 人民卫生出版社, 2010: 307-314.Zhang CF. External exudative retinopathy[M]//Zhang CF. Diseases of ocular fundus. Beijing: People's Medical Publishing House, 2010: 307-314.
3. Rishi E, Rishi P, Appukuttan B, et al. Coats' disease of adult-onset in 48 eyes [J]. Indian J Ophthalmol, 2016, 64(7): 518-523. DOI: 10.4103/0301-4738.190141.
4. 张承芬, 董方田, 王韧琰, 等. 成年Coats病的临床特征和诊治[J]. 中华眼底病杂志, 2008, 24(4): 279-282.Zhang CF, Dong FT, Wang RY, et al. Clinical characteristics and treatment of Coats' disease in adulthood[J]. Chin J Ocul Fundus Dis, 2008, 24(4): 279-282.
5. Smithen LM, Brown GC, Brucker AJ, et al. Coats' disease diagnosed in adulthood [J]. Ophthalmology, 2005, 112(6): 1072-1078.
6. Shields JA, Shields CL, Honavar SG, et al. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture [J]. Am J Ophthalmol, 2001, 131(5): 561-571.
7. Otani T, Yasuda K, Aizawa N, et al. Over 10 years follow-up of Coats' disease in adulthood [J]. Clin ophthalmol, 2011, 5: 1729-1732. DOI: 10.2147/OPTH.S27938.
8. Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats disease: the 2000 Proctor Lecture [J]. Am J Ophthalmol, 2001, 131(5): 572-583.
9. Kumar V, Chandra P, Kumar A. Ultra-wide field imaging in the diagnosis and management of adult-onset Coats' disease [J]. Clin Exp Optom, 2017, 100(1): 79-82. DOI: 10.1111/cxo.12418.
10. Yonekawa Y, Todorich B, Trese MT. Optical coherence tomography angiography findings in Coats' disease [J]. Ophthalmology, 2016, 123(9): 1964. DOI: 10.1016/j.ophtha.2016.05.004.
11. 曹绪胜, 彭晓燕. Coats病临床诊断中的问题分析[J]. 中华眼底病杂志, 2005, 21(6): 377-380.Cao XS. Analysis of the problems in the clinical diagnosis of Coats disease[J]. Chin J Ocul Fundus Dis, 2005, 21(6): 377-380.
12. Sigler EJ, Randolph JC, Calzada JI, et al. Current management of Coats disease [J]. Surv Ophthalmol, 2014, 59(1): 30-46. DOI: 10.1016/j.survophthal.2013.03.007.
13. 胡立影, 李志清, 于荣国, 等. 特发性黄斑毛细血管扩张症的研究现状与进展[J]. 中华眼底病杂志, 2016, 32(4): 440-444. DOI: 10.3760/cma.j.issn.1005-1015.2016.04.026.Hu LY, Li ZQ, Yu RG, et al. The status and progress of studies of idiopathic parafoveal telangiectasis[J]. Chin J Ocul Fundus Dis, 2016, 32(4): 440-444. DOI: 10.3760/cma.j.issn.1005-1015.2016.04.026.
14. Egbert PR, Chan CC, Winter FC. Flat preparations of the retinal vessels in Coats' disease [J]. J Pediatr Ophthalmol, 1976, 13(6): 336-339.
15. Fernandes BF, Odashiro AN, Maloney S, et al. Clinical-histopathological correlation in a case of Coats' disease [J]. Diagn Pathol, 2006, 1(1): 24. DOI: 10.1186/1746-1596-1-24.
16. Steidl SM, Hirose T, Sang D, et al. Difficulties in excluding the diagnosis of retinoblastoma in cases of advanced Coats' disease: a clinicopathologic report [J]. Ophthalmologica, 1996, 210(6): 336-340. DOI: 10.1159/000310735.
17. Kase S, Rao NA, Yoshikawa H, et al. Expression of vascular endothelial growth factor in eyes with Coats' disease [J]. Invest Ophthalmol Vis Sci, 2013, 54(1): 57-62. DOI: 10.1167/iovs.12-10613.
18. He YG, Wang H, Zhao B, et al. Elevated vascular endothelial growth factor level in Coats' disease and possible therapeutic role of bevacizumab [J]. Graefe's Arch Clin Exp Ophthalmol, 2010, 248(10): 1519-1521. DOI: 10.1007/s00417-010-1366-1.
19. Sun Y, Jain A, Moshfeghi DM. Elevated vascular endothelial growth factor levels in Coats disease: rapid response to pegaptanib sodium [J]. Graefe's Arch Clin Exp Ophthalmol, 2007, 245(9): 1387-1388. DOI: 10.1007/s00417-007-0559-8.
20. Zhao Q, Peng XY, Chen FH, et al. Vascular endothelial growth factor in Coats' disease [J]. Acta Ophthalmol, 2014, 92(3): 225-228. DOI: 10.1111/aos.12158.
21. 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 [J]. Hum Mol Genet, 1999, 8(11): 2031-2035.
22. Mulvihill A, Morris B. A population-based study of Coats disease in the United Kingdom Ⅱ: investigation, treatment, and outcomes [J]. Eye (Lond), 2010, 24(12): 1802-1807. DOI: 10.1038/eye.2010.127.
23. Shields JA, Shields CL. Review: coats disease: the 2001 LuEsther T. Mertz lecture [J]. Retina, 2002, 22(1): 80-91.
24. Zheng XX, Jiang YR. The effect of intravitreal bevacizumab injection as the initial treatment for Coats' disease [J]. Graefe's Arch Clin Exp Ophthalmol, 2014, 252(1): 35-42. DOI: 10.1007/s00417-013-2409-1.
25. Wang KY, Cheng CK. A combination of intravitreal bevacizumab injection with tunable argon yellow laser photocoagulation as a treatment for adult-onset Coats' disease [J]. J Ocul Pharmacol Ther, 2011, 27(5): 525-530. DOI: 10.1089/jop.2011.0088.
26. Bohm MR, Uhlig CE. Use of intravitreal triamcinolone and bevacizumab in Coats' disease with central macular edema [J]. Graefe's Arch Clin Exp Ophthalmol, 2011, 249(7): 1099-1101. DOI: 10.1007/s00417-011-1629-5.
27. Kim J, Park KH, Woo SJ. Combined photodynamic therapy and intravitreal bevacizumab injection for the treatment of adult Coats' disease: a case report [J]. Korean J Ophthalmol, 2010, 24(6): 374-376. DOI: 10.3341/kjo.2010.24.6.374.
28. Park S, Cho HJ, Lee DW, et al. Intravitreal bevacizumab injections combined with laser photocoagulation for adult-onset Coats' disease [J]. Graefe's Arch Clin Exp Ophthalmol, 2016, 254(8): 1511-1517. DOI: 10.1007/s00417-016-3391-1.
29. Goel N, Kumar V, Seth A, et al. Role of intravitreal bevacizumab in adult onset Coats' disease [J]. Int Ophthalmol, 2011, 31(3): 183-190. DOI: 10.1007/s10792-011-9436-x.
30. 杨琼, 魏文斌, 刘月明, 等. 单纯玻璃体腔注射雷珠单抗及其联合其他方法治疗3期以上Coats病的疗效观察[J]. 中华眼底病杂志, 2014, 30(5): 462-465. DOI: 10.3760/cma.j.issn.1005-1015.2014.05.008.Yang Q, Wei W, Liu Y, et al. Intravitreal injection of ranibizumab and combined treatment for severe Coats disease[J]. Chin J Ocul Fundus Dis, 2014. 30(5) : 462-465. DOI: 10.3760/cma.j.issn.1005-1015.2014.05.008.
31. Ramasubramanian A, Shields CL. Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction [J]. Br J Ophthalmol, 2012, 96(3): 356-359. DOI: 10.1136/bjophthalmol-2011-300141.
32. Jun JH, Kim YC, Kim KS. Resolution of severe macular edema in adult coats' disease with intravitreal triamcinolone and bevacizumab injection [J]. Korean J Ophthalmol, 2008, 22(3): 190-193. DOI: 10.3341/kjo.2008.22.3.190.

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