Macular vessel defects for Coats' disease associated with subfoveal fibrotic nodule_Chinese Journal of Ocular Fundus Diseases

Authors：

Zhang Juan , Ruan Lu , Jiang Chen , Yang Qian , Ju Yuqiao , Chang Qing ,  Huang Xin

Eye and ENT Hospital, Shanghai Medical College, Fudan University, Shanghai 200030, China;

Corresponding?author：

Huang Xin, Email: fd2017huangxin@163.com

Keywords：

Retinal telangiectasis; Subfoveal fibrotic nodule; Macular vessel defects; Tomography, optical coherence; Regional blood flow

DOI：

10.3760/cma.j.cn511434-20220520-00311

Video：

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Abstract Full text Figures/Tables Video References Cited by

Objective To investigate macular microvascular abnormalities in eyes with subfoveal fibrotic nodules secondary to Coats' disease. Methods A cross-sectional study. From January 1, 2018 to July 30, 2021, 45 eyes of 45 patients diagnosed with Coats' disease with or without subfoveal fibrotic nodules in Eye and ENT Hospital, Shanghai Medical College of Fudan University were included in this study. There were 40 eyes in 40 males and 5 eyes in 5 females. All were under 21 years old. According to the presence or absence of subfoveal fiber nodules, the patients were divided into fibrotic group (26 cases, 26 eyes) and non-fibrotic group (19 cases, 19 eyes). Optical coherence tomography angiography was used to scan 3 mm×3 mm or 6 mm×6 mm macular area of both eyes. The software of the device automatically processed the images. The presence of FAZ edge anastomotic vascular arch ring breakage and abnormal microvascular branch (AMB) in the foveal avascular zone (FAZ) were observed. Results In 26 eyes of fibrosis group, AMB originating from the parafoveal retinal capillary network was observed, which grew into and destroyed the integrity of the vascular arch ring at the edge of FAZ. AMB was crisscrossing and winding, and its curvature expands. B-scan images showed the blood flow signal in the subfoveal fiber nodule, and the blood flow signal traversed between the inner retina and the fiber nodule in 23 eyes (88.46%, 23/26). In the non-fibrosis group, all the vascular abnormalities were characterized by capillary dilation and defect, and no breakage of FAZ anastomotic vascular arch ring or AMB was observed. Conclusions In Coats' disease with subfoveal fiber nodules, staggered and dilated AMBs emerge from the parafoveal vascular network, grow into and destroy the integrity of the vascular arch ring at the edge of FAZ, and grow down longitudinally into the fiber nodules.

Citation： Zhang Juan, Ruan Lu, Jiang Chen, Yang Qian, Ju Yuqiao, Chang Qing, Huang Xin. Macular vessel defects for Coats' disease associated with subfoveal fibrotic nodule. Chinese Journal of Ocular Fundus Diseases, 2022, 38(11): 880-884. doi: 10.3760/cma.j.cn511434-20220520-00311 Copy

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10.	Rabiolo A, Marchese A, Sacconi R, et al. Refining Coats' disease by ultra-widefield imaging and optical coherence tomography angiography[J]. Graefe's Arch Clin Exp Ophthalmol, 2017, 255(10): 1881-1890. DOI: 10.1007/s00417-017-3794-7.
11.	Smithen LM, Brown GC, Brucker AJ, et al. Coats' disease diagnosed in adulthood[J]. Ophthalmology, 2005, 112(6): 1072-1078. DOI: 10.1016/j.ophtha.2004.12.038.
12.	Zhang J, Ruan L, Jiang C, et al. Updating understanding of macular microvascular abnormalities and their correlations with the characteristics and progression of macular edema or exudation in Coats' disease[J/OL]. Front Med (Lausanne), 2022, 9: 788001[2022-04-13]. https://pubmed.ncbi.nlm.nih.gov/35492340/. DOI: 10.3389/fmed.2022.788001.
13.	Vezzola D, Mapelli C, Canton V, et al. Macular fibrosis in Coats disease[J]. Retina, 2011, 31(10): 2136-2137. DOI: 10.1097/IAE.0B013E318236E861.
14.	Sigler EJ, Calzada JI. Retinal angiomatous proliferation with chorioretinal anastomosis in childhood Coats disease: a reappraisal of macular fibrosis using multimodal imaging[J]. Retina., 2015, 35(3): 537-546. DOI: 10.1097/IAE.0000000000000341.
15.	Ong SS, Cummings TJ, Vajzovic L, et al. Comparison of optical coherence tomography with fundus photographs, fluorescein angiography, and histopathologic analysis in assessing Coats disease[J]. JAMA Ophthalmol, 2019, 137(2): 176-183. DOI: 10.1001/jamaophthalmol.2018.5654.
16.	Tan AC, Dansingani KK, Yannuzzi LA, et al. Type 3 neovascularization imaged with cross-sectional and en face optical coherence tomography angiography[J]. Retina, 2017, 37(2): 234-246. DOI: 10.1097/IAE.0000000000001343.

1. Shields JA, Shields CL, Honavar SG, et al. Clinical variations and complications of Coats disease in 150 cases: the 2000 Stanford Gifford Memorial Lecture[J]. Am J Ophthalmol, 2001, 131(5): 561-571. DOI: 10.1016/s0002-9394(00)00883-7.
2. Bonnet M. Coats' syndrome[J]. J Fr Ophtalmol, 1980, 3(1): 57-66.
3. 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.
4. Jonas JB, Holbach LM. Clinical-pathologic correlation in Coats' disease[J]. Graefe's Arch Clin Exp Ophthalmol, 2001, 239(7): 544-545. DOI: 10.1007/s004170100299.
5. Spitznas M, Joussen F, Wessing A, et al. Coat's disease. An epidemiologic and fluorescein angiographic study[J]. Albrecht Von Graefe's Arch Klin Exp Ophthalmol, 1975, 195(4): 241-250. DOI: 10.1007/BF00414937.
6. 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.
7. 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. DOI: 10.1016/s0002-9394(01)00896-0.
8. Daruich AL, Moulin AP, Tran HV, et al. Subfoveal nodule in Coat' disease: toward an updated classification predicting visual prognosis[J]. Retina, 2017, 37(8): 1591-1598. DOI: 10.1097/IAE.0000000000001399.
9. Ong SS, Mruthyunjaya P, Stinnett S, et al. Macular features on spectral-domain optical coherence tomography imaging associated with visual acuity in Coats' disease[J]. Invest Ophthalmol Vis Sci, 2018, 59(7): 3161-3174. DOI: 10.1167/iovs.18-24109.
10. Rabiolo A, Marchese A, Sacconi R, et al. Refining Coats' disease by ultra-widefield imaging and optical coherence tomography angiography[J]. Graefe's Arch Clin Exp Ophthalmol, 2017, 255(10): 1881-1890. DOI: 10.1007/s00417-017-3794-7.
11. Smithen LM, Brown GC, Brucker AJ, et al. Coats' disease diagnosed in adulthood[J]. Ophthalmology, 2005, 112(6): 1072-1078. DOI: 10.1016/j.ophtha.2004.12.038.
12. Zhang J, Ruan L, Jiang C, et al. Updating understanding of macular microvascular abnormalities and their correlations with the characteristics and progression of macular edema or exudation in Coats' disease[J/OL]. Front Med (Lausanne), 2022, 9: 788001[2022-04-13]. https://pubmed.ncbi.nlm.nih.gov/35492340/. DOI: 10.3389/fmed.2022.788001.
13. Vezzola D, Mapelli C, Canton V, et al. Macular fibrosis in Coats disease[J]. Retina, 2011, 31(10): 2136-2137. DOI: 10.1097/IAE.0B013E318236E861.
14. Sigler EJ, Calzada JI. Retinal angiomatous proliferation with chorioretinal anastomosis in childhood Coats disease: a reappraisal of macular fibrosis using multimodal imaging[J]. Retina., 2015, 35(3): 537-546. DOI: 10.1097/IAE.0000000000000341.
15. Ong SS, Cummings TJ, Vajzovic L, et al. Comparison of optical coherence tomography with fundus photographs, fluorescein angiography, and histopathologic analysis in assessing Coats disease[J]. JAMA Ophthalmol, 2019, 137(2): 176-183. DOI: 10.1001/jamaophthalmol.2018.5654.
16. Tan AC, Dansingani KK, Yannuzzi LA, et al. Type 3 neovascularization imaged with cross-sectional and en face optical coherence tomography angiography[J]. Retina, 2017, 37(2): 234-246. DOI: 10.1097/IAE.0000000000001343.

Chinese Journal of Ocular Fundus Diseases

Macular vessel defects for Coats' disease associated with subfoveal fibrotic nodule

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