Clinical features and treatment of glaucoma secondary to familial exudative vitreoretinopathy_Chinese Journal of Ocular Fundus Diseases

Authors：

Yuan Mingzhen , Li Songfeng , Liu Jinghua , Deng Guangda , Li Liang , Ma Jing ,  Lu Hai

Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing 100730, China;

Corresponding?author：

Lu Hai, Email: trdr_luhai@163.com

Keywords：

Familial exudative vitreoretinopathy; Secondary glaucoma; Clinical features; Treatment methods

DOI：

10.3760/cma.j.cn511434-20220512-00294

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Objective To investigate the etiology, clinical features and treatment of familial exudative vitreoretinopathy (FEVR) secondary glaucoma. Methods A retrospective clinical study. From January 1, 2016 to January 1, 2022, 15 patients (17 eyes) were diagnosed with FEVR secondary glaucoma in Beijing Tongren Hospital, Capital Medical University were included in the study. All patients underwent systematic ophthalmological evaluation. According to the patient's age, visual acuity, intraocular pressure, anterior segment, vitreous body and retina condition, the choice of translimbal lensectomy combined with vitrectomy, goniectomy, cyclophotocoagulation, intravitreal injection of anti-vascular endothelial growth factor (VEGF) treatment were chosen. The follow-up time was 3 to 37 months. The clinical characteristics of the affected eye, and the changes of intraocular pressure, anterior chamber depth and complications after surgery were observed. Results Among the 15 patients, there were 11 males with 13 eyes, and 4 females with 4 eyes. Age was 6.14±7.37 years old. FEVR stages 2B, 3B, 4A, 4B, 5A, and 5B were 1, 1, 5, 6, 3, and 1 eye, respectively. The intraocular pressure of the affected eye was 42.74±9.06 mm Hg (1 mm Hg=0.133 kPa). All eyes had shallow anterior chamber and angle closure, anterior or posterior iris adhesions, lens opacity, retinal detachment, iris neovascularization in 4 eyes, and vitreous hemorrhage in 2 eyes. Sixteen eyes were treated with translimbal lensectomy combined with vitrectomy and goniotomy, of which 8 eyes were treated with anti-VEGF treatment; 1 eye was treated with cyclophotocoagulation combined with anti-VEGF treatment. After operation, the intraocular pressure of 16 eyes returned to normal range, and the depth of anterior chamber of 16 eyes returned to normal, and no obvious complications occurred. Conclusions The main etiology of secondary glaucoma in FEVR is the structural and functional abnormalities of the anterior chamber and angle, which are found in the 2B and above stages of FEVR. The lensectomy and vitrectomy via limbal approach can effectively control the intraocular pressure and restore the anterior chamber, with no serious complications.

Citation： Yuan Mingzhen, Li Songfeng, Liu Jinghua, Deng Guangda, Li Liang, Ma Jing, Lu Hai. Clinical features and treatment of glaucoma secondary to familial exudative vitreoretinopathy. Chinese Journal of Ocular Fundus Diseases, 2023, 39(1): 17-21. doi: 10.3760/cma.j.cn511434-20220512-00294 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.	Tao T, Xu N, Li J, et al. Ocular features and mutation spectrum of patients with familial exudative vitreoretinopathy[J]. Invest Ophthalmol Vis Sci, 2021, 62(15): 4. DOI: 10.1167/iovs.62.15.4.
3.	Tian T, Chen CL, Zhang X, et al. Clinical and genetic features of familial exudative vitreoretinopathy with only-unilateral abnormalities in a Chinese cohort[J]. JAMA Ophthalmol, 2019, 137(9): 1054-1058. DOI: 10.1001/jamaophthalmol.2019.1493.
4.	Selvan H, Swamy DR, Temkar S, et al. Familial exudative vitreoretinopathy and glaucoma: observations, insights, and management strategies[J/OL]. J Glaucoma, 2018, 27(1): e1-6[2018-01-01]. https://pubmed.ncbi.nlm.nih.gov/29088053/. DOI: 10.1097/IJG.0000000000000810.
5.	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.
6.	Ranchod TM, Ho LY, Drenser KA, et al. Clinical presentation of familial exudative vitreoretinopathy[J]. Ophthalmology, 2011, 118(10): 2070-2075. DOI: 10.1016/j.ophtha.2011.06.020.
7.	Tauqeer Z, Yonekawa Y. Familial exudative vitreoretinopathy: pathophysiology, diagnosis, and management[J]. Asia Pac J Ophthalmol (Phila), 2018, 7(3): 176-182. DOI: 10.22608/APO.201855.
8.	Kondo H, Matsushita I, Nagata T, et al. Retinal features of family members with familial exudative vitreoretinopathy caused by mutations in KIF11 gene[J]. Transl Vis Sci Technol, 2021, 10(7): 18. DOI: 10.1167/tvst.10.7.18.
9.	Hubbard GB, Li AL. Analysis of predisposing clinical features for worsening traction after treatment of familial exudative vitreoretinopathy in children[J]. Am J Ophthalmol, 2021, 223: 430-445. DOI: 10.1016/j.ajo.2020.07.013.
10.	Nishin S, Chen CL, Zhang X, et al. Clinical features of congenital retinal folds[J]. Am J Ophthalmol, 2012, 153(1): 81-87. DOI: 10.1016/j.ajo.2011.06.002.
11.	Azuma N, Shiraga F. Guidance of medical care for familial exudative vitreoretinopathyresearch on rare and intractable diseases, health and labour sciences research grants[J]. Nippon Ganka Gakkai Zasshi, 2017, 121(6): 487-497.
12.	Gao F, Wang JJ, Chen JY, et al. Correction to: etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study[J]. Graefe's Arch Clin Exp Ophthalmol, 2021, 259(8): 2389-2390. DOI: 10.1007/s00417-021-05260-7.
13.	Fei P, Yang WJ, Zhang Q, et al. Surgical management of advanced familial exudative vitreoretinopathy with complications[J]. Retina, 2016, 36(8): 1480-1485. DOI: 10.1097/IAE.0000000000000961.
14.	Boldrey EE, Egbert P, Gass JD, et al. The histopathology of familial exudative vitreoretinopathy: a report of two cases[J]. Arch Ophthalmol, 1985, 103(2): 238-241. DOI: 10.1001/archopht.1985.01050020090029.
15.	Alsheikheh A, Lieb W, Grehn F. Criswick-Schepens syndrome-familial exudative vitreoretinopathy. Report of six cases in two consanguineous families[J]. Ophthalmologe, 2004, 101(9): 914-918. DOI: 10.1007/s00347-004-0998-0.
16.	Azuara-Blanco A, Pesin SR, Katz LZ, et al. Familial exudative vitreoretinopathy associated with nonneovascular chronic angle-closure glaucoma[J]. J Glaucoma, 1997, 6(1): 47-49.
17.	Kashani AH, Brown KT, Chang E, et al. Diversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(11): 2220-2227. DOI: 10.1016/j.ophtha.2014.05.029.

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. Tao T, Xu N, Li J, et al. Ocular features and mutation spectrum of patients with familial exudative vitreoretinopathy[J]. Invest Ophthalmol Vis Sci, 2021, 62(15): 4. DOI: 10.1167/iovs.62.15.4.
3. Tian T, Chen CL, Zhang X, et al. Clinical and genetic features of familial exudative vitreoretinopathy with only-unilateral abnormalities in a Chinese cohort[J]. JAMA Ophthalmol, 2019, 137(9): 1054-1058. DOI: 10.1001/jamaophthalmol.2019.1493.
4. Selvan H, Swamy DR, Temkar S, et al. Familial exudative vitreoretinopathy and glaucoma: observations, insights, and management strategies[J/OL]. J Glaucoma, 2018, 27(1): e1-6[2018-01-01]. https://pubmed.ncbi.nlm.nih.gov/29088053/. DOI: 10.1097/IJG.0000000000000810.
5. 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.
6. Ranchod TM, Ho LY, Drenser KA, et al. Clinical presentation of familial exudative vitreoretinopathy[J]. Ophthalmology, 2011, 118(10): 2070-2075. DOI: 10.1016/j.ophtha.2011.06.020.
7. Tauqeer Z, Yonekawa Y. Familial exudative vitreoretinopathy: pathophysiology, diagnosis, and management[J]. Asia Pac J Ophthalmol (Phila), 2018, 7(3): 176-182. DOI: 10.22608/APO.201855.
8. Kondo H, Matsushita I, Nagata T, et al. Retinal features of family members with familial exudative vitreoretinopathy caused by mutations in KIF11 gene[J]. Transl Vis Sci Technol, 2021, 10(7): 18. DOI: 10.1167/tvst.10.7.18.
9. Hubbard GB, Li AL. Analysis of predisposing clinical features for worsening traction after treatment of familial exudative vitreoretinopathy in children[J]. Am J Ophthalmol, 2021, 223: 430-445. DOI: 10.1016/j.ajo.2020.07.013.
10. Nishin S, Chen CL, Zhang X, et al. Clinical features of congenital retinal folds[J]. Am J Ophthalmol, 2012, 153(1): 81-87. DOI: 10.1016/j.ajo.2011.06.002.
11. Azuma N, Shiraga F. Guidance of medical care for familial exudative vitreoretinopathyresearch on rare and intractable diseases, health and labour sciences research grants[J]. Nippon Ganka Gakkai Zasshi, 2017, 121(6): 487-497.
12. Gao F, Wang JJ, Chen JY, et al. Correction to: etiologies and clinical characteristics of young patients with angle-closure glaucoma: a 15-year single-center retrospective study[J]. Graefe's Arch Clin Exp Ophthalmol, 2021, 259(8): 2389-2390. DOI: 10.1007/s00417-021-05260-7.
13. Fei P, Yang WJ, Zhang Q, et al. Surgical management of advanced familial exudative vitreoretinopathy with complications[J]. Retina, 2016, 36(8): 1480-1485. DOI: 10.1097/IAE.0000000000000961.
14. Boldrey EE, Egbert P, Gass JD, et al. The histopathology of familial exudative vitreoretinopathy: a report of two cases[J]. Arch Ophthalmol, 1985, 103(2): 238-241. DOI: 10.1001/archopht.1985.01050020090029.
15. Alsheikheh A, Lieb W, Grehn F. Criswick-Schepens syndrome-familial exudative vitreoretinopathy. Report of six cases in two consanguineous families[J]. Ophthalmologe, 2004, 101(9): 914-918. DOI: 10.1007/s00347-004-0998-0.
16. Azuara-Blanco A, Pesin SR, Katz LZ, et al. Familial exudative vitreoretinopathy associated with nonneovascular chronic angle-closure glaucoma[J]. J Glaucoma, 1997, 6(1): 47-49.
17. Kashani AH, Brown KT, Chang E, et al. Diversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy[J]. Ophthalmology, 2014, 121(11): 2220-2227. DOI: 10.1016/j.ophtha.2014.05.029.

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