Quantitative analysis of ocular fundus vascular manifestations in familial exudative vitreoretinopathy based on ultra-widefield fundus fluorescein angiogarphy_Chinese Journal of Ocular Fundus Diseases

Authors：

Sun Yongcheng , Ge Xing ,  Liu Haiyang

Department of Ophthalmology, Xuzhou Municipal Hospital Affiliated to Xuzhou Medical University, Xuzhou First People's Hospital, Xuzhou Institute of Eye Disease Prevention and Control, Xuzhou 221002, China;

Corresponding?author：

Liu Haiyang, Email: liuhaiyang86@126.com

Keywords：

Familial exudative vitreoretinopathy; Ultra-widefield fundus fluorescein angiogarphy; Quantitative analysis; Retinal vessel; Vessel count; Intervascular distance

DOI：

10.3760/cma.j.cn511434-20251015-00449

Video：

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Objective To observe the number and distribution characteristics of the peripheral retinal vessels in the eyes of patients with familial exudative vitreoretinopathy (FEVR). Methods A retrospective clinical study. From October 2020 to April 2025, 33 patients (33 eyes) diagnosed with FEVR (FEVR group) and 27 healthy volunteers (27 eyes) during the same period (normal control group) at the Department of Ophthalmology of Xuzhou First People's Hospital were enrolled in the study. All subjects underwent ultra-wide-angle fluorescein angiography, and clear venous-phase images were selected. Centered on the fovea centralis, the retina was divided into two concentric circular zones, sequentially defining the posterior pole, mid-periphery, and far-periphery regions. The measurement areas were manually outlined using ImageJ software: the nasal far-peripheral region was defined as the area extending 1 400 pixels nasally and 2 000 pixels vertically from the fovea; the temporal mid-peripheral region was defined as the area extending 1 000 pixels temporally and 1 500 pixels vertically from the fovea. The nasal and temporal vertical ranges were subdivided into the entire range, and the upper, middle, and lower subgroups. The total number of vessels and inter-vessel distances in the four regions (nasal and temporal) of both groups were compared and observed. Comparisons between the two groups were performed using independent samples t test. Results Compared with the normal control group, the total number of retinal vessels in the entire range and different directions of both the nasal far-peripheral region (t=?3.88, ?2.34, ?2.23, ?3.80) and the temporal mid-peripheral region (t=?5.50; Z=?2.78; t=?3.53, ?5.00) increased in the FEVR group, and the differences were statistically significant (P＜0.05). There were statistically significant differences between the two groups in the inter-vessel distances of the entire range in the nasal far-peripheral region and the middle subgroup in the temporal mid-peripheral region (t=?2.71, ?2.24; P＜0.05). Conclusion Compared with the normal control group, the number of vessels increases and the inter-vessel distances decreases in both the nasal far-peripheral region and the temporal mid-peripheral region of FEVR eyes.

Citation： Sun Yongcheng, Ge Xing, Liu Haiyang. Quantitative analysis of ocular fundus vascular manifestations in familial exudative vitreoretinopathy based on ultra-widefield fundus fluorescein angiogarphy. Chinese Journal of Ocular Fundus Diseases, 2026, 42(7): 578-582. doi: 10.3760/cma.j.cn511434-20251015-00449 Copy

1.	Criswick VG, Schepens CL. Familial exudative vitreoretinopathy[J]. Am J Ophthalmol, 1969, 68(4): 578-594. DOI: 10.1016/0002-9394(69)91237-9.
2.	Canny CL, Oliver GL. Fluorescein angiographic findings in familial exudative vitreoretinopathy[J]. Arch Ophthalmol, 1976, 94(7): 1114-1120. DOI: 10.1001/archopht.1976.03910040034006.
3.	Shukla SY, Kaliki S, Shields CL. Asymmetry of familial exudative vitreoretinopathy[J/OL]. J Pediatr Ophthalmol Strabismus, 2012, 49[2012-02-28]. https://pubmed.ncbi.nlm.nih.gov/22372757/. DOI: 10.3928/01913913-20120221-01.
4.	Friberg TR, Forrester JV. Ultra-wide angle (200°+) fluorescein angiography using a modified Optos Panoramic200TM imaging system[J]. Invest Ophthalmol Vis Sci, 2004, 45(13): 3001.
5.	杜葵芳, 魏文斌. 超廣角熒光血管造影在視網膜疾病中的應用研究現狀[J]. 眼科新進展, 2021, 41(6): 593-598. DOI: 10.13389/j.cnki.rao.2021.0124.Du KF, Wei WB. Research status of the application of Ultra-wide-field fluorescein angiography in retinal diseases[J]. Rec Adv Ophthalmol, 2021, 41(6): 593-598. DOI: 10.13389/j.cnki.rao.2021.0124.
6.	Pendergast SD, Trese MT. Familial exudative vitreoretinopathy. Results of surgical management[J]. Ophthalmology, 1998, 105(6): 1015-1023. DOI: 10.1016/S0161-6420(98)96002-X.
7.	Kashani AH, Brown KT, Chang E, et al. Diversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(11): 2220-2207. DOI: 10.1016/j.ophtha. 2014.05.029. DOI: 10.1016/j.ophtha.2014.05.029.
8.	Poulter JA, Ali M, Gilmour DF, et al. Mutations in TSPAN12 cause autosomal-dominant familial exudative vitreoretinopathy[J/OL]. Am J Hum Genet, 2016, 98(3): 592[2016-03-03]. https://pubmed.ncbi.nlm.nih.gov/28863275/. DOI: 10.1016/j.ajhg.2016.02.017.
9.	Jiao X, Ventruto V, Trese MT, et al. Autosomal recessive familial exudative vitreoretinopathy is associated with mutations in LRP5[J]. Am J Hum Genet, 2004, 75(5): 878-884. DOI: 10.1086/425080.
10.	Chen ZY, Battinelli EM, Fielder A, et al. A mutation in the Norrie disease gene (NDP) associated with X-linked familial exudative vitreoretinopathy[J]. Nat Genet, 1993, 5(2): 180-183. DOI: 10.1038/ng1093-180.
11.	Robitaille J, MacDonald ML, Kaykas A, et al. Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy[J]. Nat Genet, 2002, 32(2): 326-330. DOI: 10.1038/ng957.
12.	Spitznas M, Bornfeld N. The architecture of the most peripheral retinal vessels[J]. Albrecht Von Graefes Arch Klin Exp Ophthalmol, 1977, 203(3-4): 217-229. DOI: 10.1007/BF00409828.
13.	Kashani AH, Learned D, Nudleman E, et al. High prevalence of peripheral retinal vascular anomalies in family members of patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(1): 262-268. DOI: 10.1016/j.ophtha.2013.08.010.
14.	Simons M. Angiogenesis: where do we stand now?[J]. Circulation, 2005, 111(12): 1556-1566. DOI: 10.1161/01.CIR.0000159345.00591.8F.
15.	Shastry BS, Hejtmancik JF, Plager DA, et al. Linkage and candidate gene analysis of X-linked familial exudative vitreoretinopathy[J]. Genomics, 1995, 27(2): 341-344. DOI: 10.1006/geno.1995.1052.
16.	Shastry BS, Trese MT. Familial exudative vitreoretinopathy: further evidence for genetic heterogeneity[J]. Am J Med Genet, 1997, 69(2): 217-218. DOI: 10.1002/(sici)1096-8628(19970317)69:2<217::aid-ajmg19>3.0.co;2-o.
17.	Shastry BS, Hejtmancik JF, Trese MT. Identification of novel missense mutations in the Norrie disease gene associated with one X-linked and four sporadic cases of familial exudative vitreoretinopathy[J]. Hum Mutat, 1997, 9(5): 396-401. DOI: 10.1002/(SICI)1098-1004(1997)9:5<396::AID-HUMU3>3.0.CO;2-2.
18.	Dickinson JL, Sale MM, Passmore A, et al. Mutations in the NDP gene: contribution to Norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity[J]. Clin Exp Ophthalmol, 2006, 34(7): 682-688. DOI: 10.1111/j.1442-9071.2006.01314.x.
19.	Xu Q, Wang Y, Dabdoub A, et al. Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pair[J]. Cell, 2004, 116(6): 883-895. DOI: 10.1016/s0092-8674(04)00216-8.
20.	Singer M, Sagong M, van Hemert J, et al. Ultra-widelield imaging of the peripheral retinal vascaluture in normal subjeets[J]. Ophthalmology, 2016, 123(5): 1053-1059. DOI: 10.1016/j.ophtha.2016.01.022.
21.	Jiang JW, Yi ZH, Wang XL, et al. Quantitative analysis of retinal vasculature in normal eyes using ultra-widefield fluorescein angiography[J]. Int J Ophthalmol, 2021, 14(12): 1915-1920. DOI: 10.18240/ijo.2021.12.16.

1. Criswick VG, Schepens CL. Familial exudative vitreoretinopathy[J]. Am J Ophthalmol, 1969, 68(4): 578-594. DOI: 10.1016/0002-9394(69)91237-9.
2. Canny CL, Oliver GL. Fluorescein angiographic findings in familial exudative vitreoretinopathy[J]. Arch Ophthalmol, 1976, 94(7): 1114-1120. DOI: 10.1001/archopht.1976.03910040034006.
3. Shukla SY, Kaliki S, Shields CL. Asymmetry of familial exudative vitreoretinopathy[J/OL]. J Pediatr Ophthalmol Strabismus, 2012, 49[2012-02-28]. https://pubmed.ncbi.nlm.nih.gov/22372757/. DOI: 10.3928/01913913-20120221-01.
4. Friberg TR, Forrester JV. Ultra-wide angle (200°+) fluorescein angiography using a modified Optos Panoramic200TM imaging system[J]. Invest Ophthalmol Vis Sci, 2004, 45(13): 3001.
5. 杜葵芳, 魏文斌. 超廣角熒光血管造影在視網膜疾病中的應用研究現狀[J]. 眼科新進展, 2021, 41(6): 593-598. DOI: 10.13389/j.cnki.rao.2021.0124.Du KF, Wei WB. Research status of the application of Ultra-wide-field fluorescein angiography in retinal diseases[J]. Rec Adv Ophthalmol, 2021, 41(6): 593-598. DOI: 10.13389/j.cnki.rao.2021.0124.
6. Pendergast SD, Trese MT. Familial exudative vitreoretinopathy. Results of surgical management[J]. Ophthalmology, 1998, 105(6): 1015-1023. DOI: 10.1016/S0161-6420(98)96002-X.
7. Kashani AH, Brown KT, Chang E, et al. Diversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(11): 2220-2207. DOI: 10.1016/j.ophtha. 2014.05.029. DOI: 10.1016/j.ophtha.2014.05.029.
8. Poulter JA, Ali M, Gilmour DF, et al. Mutations in TSPAN12 cause autosomal-dominant familial exudative vitreoretinopathy[J/OL]. Am J Hum Genet, 2016, 98(3): 592[2016-03-03]. https://pubmed.ncbi.nlm.nih.gov/28863275/. DOI: 10.1016/j.ajhg.2016.02.017.
9. Jiao X, Ventruto V, Trese MT, et al. Autosomal recessive familial exudative vitreoretinopathy is associated with mutations in LRP5[J]. Am J Hum Genet, 2004, 75(5): 878-884. DOI: 10.1086/425080.
10. Chen ZY, Battinelli EM, Fielder A, et al. A mutation in the Norrie disease gene (NDP) associated with X-linked familial exudative vitreoretinopathy[J]. Nat Genet, 1993, 5(2): 180-183. DOI: 10.1038/ng1093-180.
11. Robitaille J, MacDonald ML, Kaykas A, et al. Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy[J]. Nat Genet, 2002, 32(2): 326-330. DOI: 10.1038/ng957.
12. Spitznas M, Bornfeld N. The architecture of the most peripheral retinal vessels[J]. Albrecht Von Graefes Arch Klin Exp Ophthalmol, 1977, 203(3-4): 217-229. DOI: 10.1007/BF00409828.
13. Kashani AH, Learned D, Nudleman E, et al. High prevalence of peripheral retinal vascular anomalies in family members of patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(1): 262-268. DOI: 10.1016/j.ophtha.2013.08.010.
14. Simons M. Angiogenesis: where do we stand now?[J]. Circulation, 2005, 111(12): 1556-1566. DOI: 10.1161/01.CIR.0000159345.00591.8F.
15. Shastry BS, Hejtmancik JF, Plager DA, et al. Linkage and candidate gene analysis of X-linked familial exudative vitreoretinopathy[J]. Genomics, 1995, 27(2): 341-344. DOI: 10.1006/geno.1995.1052.
16. Shastry BS, Trese MT. Familial exudative vitreoretinopathy: further evidence for genetic heterogeneity[J]. Am J Med Genet, 1997, 69(2): 217-218. DOI: 10.1002/(sici)1096-8628(19970317)69:2<217::aid-ajmg19>3.0.co;2-o.
17. Shastry BS, Hejtmancik JF, Trese MT. Identification of novel missense mutations in the Norrie disease gene associated with one X-linked and four sporadic cases of familial exudative vitreoretinopathy[J]. Hum Mutat, 1997, 9(5): 396-401. DOI: 10.1002/(SICI)1098-1004(1997)9:5<396::AID-HUMU3>3.0.CO;2-2.
18. Dickinson JL, Sale MM, Passmore A, et al. Mutations in the NDP gene: contribution to Norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity[J]. Clin Exp Ophthalmol, 2006, 34(7): 682-688. DOI: 10.1111/j.1442-9071.2006.01314.x.
19. Xu Q, Wang Y, Dabdoub A, et al. Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pair[J]. Cell, 2004, 116(6): 883-895. DOI: 10.1016/s0092-8674(04)00216-8.
20. Singer M, Sagong M, van Hemert J, et al. Ultra-widelield imaging of the peripheral retinal vascaluture in normal subjeets[J]. Ophthalmology, 2016, 123(5): 1053-1059. DOI: 10.1016/j.ophtha.2016.01.022.
21. Jiang JW, Yi ZH, Wang XL, et al. Quantitative analysis of retinal vasculature in normal eyes using ultra-widefield fluorescein angiography[J]. Int J Ophthalmol, 2021, 14(12): 1915-1920. DOI: 10.18240/ijo.2021.12.16.

Chinese Journal of Ocular Fundus Diseases

Quantitative analysis of ocular fundus vascular manifestations in familial exudative vitreoretinopathy based on ultra-widefield fundus fluorescein angiogarphy

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