The macular structure and blood flow in familial exudative vitreoretinopathy with inner retinal layer persistence_Chinese Journal of Ocular Fundus Diseases

Authors：

Shao Yirun ^1,2 , Mao Jianbo ³ , Chen Yijing ² , Fang Yuyan ² ,  Shen Lijun ³

1. Hangzhou Traditional Chinese Medicine Hospital, Hangzhou 310007, China;
2. The Affiliated Eye Hospital of Wenzhou Medical University at Hangzhou, Hangzhou 310020, China;
3. Zhejiang Provincial People's Hospital (Affiliated People's Hospital, Hangzhou Medical College) Hangzhou Medical College, Hangzhou 310014, China;

Corresponding?author：

Shen Lijun, Email: slj@mail.eye.ac.cn

Keywords：

Familial exudative vitreoretinopathy; Optical coherence tomography angiography; Regional blood flow

DOI：

10.3760/cma.j.cn511434-20230210-00058

Video：

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Objective To compared the changes of macular microvascular architecture in early stage familial exudative vitreoretinopathy (FEVR) patients with inner retinal layer (IRL) persistence and without IRL persistence. Methods A retrospective clinical study. From 2017 to 2022, 94 patients with stage 1 FEVR with or without IRL residue and 45 age- and sex-matched healthy volunteers with 45 eyes (normal control group) who were confirmed by ophthalmology examination in Hangzhou Hospital of Optometry Affiliated to Wenzhou Medical University and Zhejiang Provincial People's Hospital were included in the study. According to whether there was IRL residue, the patients were divided into IRL group and non-IRL group, with 22 patients (22 eyes) and 72 patients (72 eyes), respectively. Best corrected visual acuity (BCVA) and optical coherence tomography angiography (OCTA) were performed in all eyes. Superficial vessel density (SCP) and deep vessel density (DCP) of whole image, fovea and parafovea, the area and perimeter of fovea avascular area (FAZ), A-circularity index (AI, perimeter/standard circle perimeter with equal area) and vessel density around the 300 μm width of the FAZ (FD), central macular thickness (CMT) on macular 3 mm × 3 mm scan on OCTA were measured. Results SCP and DCP of whole image (F=10.774, 4.583) and parafovea (F=10.433, 3.912), CMT (F=171.940) in IRL group and non-IRL group on macular 3 mm × 3 mm scan on OCTA were significantly lower than that in normal persons (P＜0.05). There were significant differences among three groups of the area of FAZ (F=4.315), AI (F=3.413), FD-300 (F=13.592) (P＜0.05). BCVA were worst in IRL group (P＜0.05). Conclusions Blood flow density decreased in macular area of FEVR patients. CMT is significantly thicker than normal population. The FAZ area of the foveal IRL residual eyes is small and irregular, with worse BCVA and lower macular blood density.

Citation： Shao Yirun, Mao Jianbo, Chen Yijing, Fang Yuyan, Shen Lijun. The macular structure and blood flow in familial exudative vitreoretinopathy with inner retinal layer persistence. Chinese Journal of Ocular Fundus Diseases, 2023, 39(7): 560-564. doi: 10.3760/cma.j.cn511434-20230210-00058 Copy

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4.	Rao P, Lertjirachai I, Yonekawa Y, et alEtiology and clinical characteristics of macular edema in patients with familial exudative vitreoretinopathy[J]. Retina202040713671373. DOI:10.1097/iae.0000000000002623.
5.	Chen C, Liu C, Wang Z, et alOptical coherence tomography angiography in familial exudative vitreoretinopathy: clinical features and phenotype-genotype correlation[J]. Invest Ophthalmol Vis Sci2018591557265734. DOI:10.1167/iovs.18-25377.
6.	Chen X, Viehland C, Carrasco-Zevallos OM, et alMicroscope-integrated optical coherence tomography angiography in the operating room in young children with retinal vascular disease[J]. JAMA Ophthalmol20171355483486. DOI:10.1001/jamaophthalmol.2017.0422.
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17.	Mao J, Chen Y, Fang Y, et alClinical characteristics and mutation spectrum in 33 Chinese families with familial exudative vitreoretinopathy[J]. Ann Med202254132863298. DOI:10.1080/07853890.2022.2146744.
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21.	王文亭, 李姝嬋, 蔣可可, 等家族性滲出性玻璃體視網膜病變患眼黃斑區微血管改變觀察[J]. 中華眼底病雜志20213712932936. DOI: 10.3760/cma.j.cn511434-20210207-00065..Wang WT, Li SC, Jiang KK, et alMacular microvascular findings in familial exudative vitreoretinopathy on optical coherence tomography angiography[J]. Chin J Ocul Fundus Dis2021371293293610.3760/cma.j.cn511434-20210207-00065.

1. Lyu J, Zhang Q, Wang S, et alUltra-wide-field scanning laser ophthalmoscopy assists in the clinical detection and evaluation of asymptomatic early-stage familial exudative vitreoretinopathy[J]. Graefe's Arch Clin Exp Ophthalmol201725513947. DOI:10.1007/s00417-016-3415-x.
2. Yuan M, Yang Y, Yu S, et alPosterior pole retinal abnormalities in mild asymptomatic FEVR[J]. Invest Ophthalmol Vis Sci2014561458463. DOI:10.1167/iovs.14-15821.
3. Yonekawa Y, Thomas BJ, Drenser KA, et alFamilial exudative vitreoretinopathy: spectral-domain optical coherence tomography of the vitreoretinal interface, retina, and choroid[J]. Ophthalmology20151221122702277. DOI:10.1016/j.ophtha.2015.07.024.
4. Rao P, Lertjirachai I, Yonekawa Y, et alEtiology and clinical characteristics of macular edema in patients with familial exudative vitreoretinopathy[J]. Retina202040713671373. DOI:10.1097/iae.0000000000002623.
5. Chen C, Liu C, Wang Z, et alOptical coherence tomography angiography in familial exudative vitreoretinopathy: clinical features and phenotype-genotype correlation[J]. Invest Ophthalmol Vis Sci2018591557265734. DOI:10.1167/iovs.18-25377.
6. Chen X, Viehland C, Carrasco-Zevallos OM, et alMicroscope-integrated optical coherence tomography angiography in the operating room in young children with retinal vascular disease[J]. JAMA Ophthalmol20171355483486. DOI:10.1001/jamaophthalmol.2017.0422.
7. Koulisis N, Moysidis S, Yonekawa Y, et alCorrelating changes in the macular microvasculature and capillary network to peripheral vascular pathologic features in familial exudative vitreoretinopathy[J]. Ophthalmol Retina201937597606. DOI:10.1016/j.oret.2019.02.013.
8. Hsu ST, Finn AP, Chen X, et alMacular microvascular findings in familial exudative vitreoretinopathy on optical coherence tomography angiography[J]. Ophthalmic Surg Lasers Imaging Retina2019505322329. DOI:10.3928/23258160-20190503-11.
9. Kashani A, Brown K, Chang E, et alDiversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy[J]. Ophthalmology20141211122202227. DOI:10.1016/j.ophtha.2014.05.029.
10. Hasegawa T, Hirato M, Kobashi C, et alEvaluation of the foveal avascular zone in familial exudative vitreoretinopathy using optical coherence tomography angiography[J]. Clin Ophthalmol20211519131920. DOI:10.2147/opth.S305520.
11. Bringmann A, Syrbe S, G?rner K, et alThe primate fovea: structure, function and development[J]. Prog Retin Eye Res2018664984. DOI:10.1016/j.preteyeres.2018.03.006.
12. Wang Z, Liu C, Huang S, et alWnt signaling in vascular eye diseases[J]. Prog Retin Eye Res201970110133. DOI:10.1016/j.preteyeres.2018.11.008.
13. Zhao R, Dai E, Wang S, et alA comprehensive functional analysis on the pathogenesis of novel TSPAN12 and NDP variants in familial exudative vitreoretinopathy[J]. Clin Genet20231033320329. DOI:10.1111/cge.14273.
14. Tao T, Meng X, Xu N, et alOcular phenotype and genetical analysis in patients with retinopathy of prematurity[J]. BMC Ophthalmols202222122. DOI:10.1186/s12886-022-02252-x.
15. Dailey W, Gryc W, Garg P, et alFrizzled-4 variations associated with retinopathy and intrauterine growth retardation: a potential marker for prematurity and retinopathy[J]. Ophthalmology2015122919171923. DOI:10.1016/j.ophtha.2015.05.036.
16. Zhang T, Sun X, Han J, et alGenetic variants of TSPAN12 gene in patients with retinopathy of prematurity[J]. J Cell Biochem201912091454414551. DOI:10.1002/jcb.28715.
17. Mao J, Chen Y, Fang Y, et alClinical characteristics and mutation spectrum in 33 Chinese families with familial exudative vitreoretinopathy[J]. Ann Med202254132863298. DOI:10.1080/07853890.2022.2146744.
18. Wu W, Lin R, Shih C, et alVisual acuity, optical components, and macular abnormalities in patients with a history of retinopathy of prematurity[J]. Ophthalmology2012119919071916. DOI:10.1016/j.ophtha.2012.02.040.
19. Chen P, Kang E, Chen K, et alFoveal hypoplasia and characteristics of optical components in patients with familial exudative vitreoretinopathy and retinopathy of prematurity[J]. Sci Rep20221217694. DOI:10.1038/s41598-022-11455-7.
20. Zhang J, Jiang C, Ruan L, et alMacular capillary dropout in familial exudative vitreoretinopathy and its relationship with visual acuity and disease progression[J]. Retina202040611401147. DOI:10.1097/iae.0000000000002490.
21. 王文亭, 李姝嬋, 蔣可可, 等家族性滲出性玻璃體視網膜病變患眼黃斑區微血管改變觀察[J]. 中華眼底病雜志20213712932936. DOI: 10.3760/cma.j.cn511434-20210207-00065..Wang WT, Li SC, Jiang KK, et alMacular microvascular findings in familial exudative vitreoretinopathy on optical coherence tomography angiography[J]. Chin J Ocul Fundus Dis2021371293293610.3760/cma.j.cn511434-20210207-00065.

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