Surgical treatment and current advances of persistent fetal vasculature syndrome_Chinese Journal of Ocular Fundus Diseases

Authors：

Ren Jianing , Peng Jie , Chen Shuangshuang , Zou Yihua ,  Zhao Peiquan

Department of Ophthalmology, Affiliated Xinhua Hospital, Affiliated Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China;

Corresponding author：

Zhao Peiquan, Email: zhaopeiquan@126.com

Keywords：

Persistent hyperplastic primary vitreous; Vitrectomy; Review

DOI：

10.3760/cma.j.cn511434-20210104-00003

Video：

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Persistent fetal vasculature syndrome (PFVS) is a rare congenital vitreous dysplasia, which is classified as anterior, posterior and combined types according to the location of the vascular abnormalities. The clinical manifestations of PFVS are diverse, and early surgical intervention is very important. The main objective of surgical treatment is to remove the anterior and posterior traction between fibrovascular membranes and retina as well as lens, and to reconstruct clear visual axis. Surgical treatments include pupilloplasty, lensectomy with or without intraocular lens implantation and vitrectomy via limbal or scleral approach. For new technologies, the applications of ophthalmic viscosurgical device and femtosecond lasers have desirable results . In addition to focusing on improving the success rate of surgery, it is also necessary to systematically and comprehensively assess the overall preoperative condition and postoperative visual function of the patients. PFVS eyes have limited improvement in postoperative vision, which is related to the extent of lesion involvement and the occurrence of complications. Eyes with macular dysplasia and tractional retinal detachment, as well as elongated ciliary process, have a poor prognosis of vision after surgery. How to improve postoperative vision in the eye affecting the posterior segment of the eye with PFVS from the microscopic anatomical relationship between the fibrous vascular pedicle and the retina is worth further study. On the other hand, reducing surgical trauma and optimizing surgical procedures in order to improve postoperative visual acuity and reduce postoperative complications are also the key research directions of future PFVS treatments.

Citation： Ren Jianing, Peng Jie, Chen Shuangshuang, Zou Yihua, Zhao Peiquan. Surgical treatment and current advances of persistent fetal vasculature syndrome. Chinese Journal of Ocular Fundus Diseases, 2022, 38(2): 163-167. doi: 10.3760/cma.j.cn511434-20210104-00003 Copy

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2. Chen C, Xiao H, Ding X. Persistent fetal vasculature[J]. Asia Pac J Ophthalmol (Phila), 2019, 8(1): 86-95. DOI: 10.22608/APO.201854.
3. Khaliq S, Hameed A, Ismail M, et al. Locus for autosomal recessive nonsyndromic persistent hyperplastic primary vitreous[J]. Invest Ophthalmol Vis Sci, 2001, 42(10): 2225-2228.
4. Fei P, Liu Z, He L, et al. Early detection of ocular abnormalities in a Chinese multicentre neonatal eye screening programme-1-year result[J/OL]. Acta Ophthalmol, 2021, 99(3): e415-e422[2020-09-15]. https://pubmed.ncbi.nlm.nih.gov/32930484/. DOI: 10.1111/aos.14586.
5. 聂文英, 吴汉荣, 戚以胜, 等. 新生儿眼病筛查的初步研究[J]. 中华眼科杂志, 2008, 44(6): 497-502. DOI: 10.3321/j.issn:0412-4081.2008.06.005.Nie WY, Wu HR, Qi YS, et al. A pilot study of ocular diseases screening for neonates in China[J]. Chin J Ophthalmol, 2008, 44(6): 497-502. DOI: 10.3321/j.issn:0412-4081.2008.06.005.
6. Anteby I, Cohen E, Karshai I, et al. Unilateral persistent hyperplastic primary vitreous: course and outcome[J]. J AAPOS, 2002, 6(2): 92-99. DOI: 10.1067/mpa.2002.121324.
7. Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment and results[J]. Trans Am Ophthalmol Soc, 1997, 95: 487-549.
8. Jiang H, Liang Y, Long K, et al. Postoperative follow-up of a case of atypical morning glory syndrome associated with persistent fetal vasculature[J]. BMC Ophthalmol, 2019, 19(1): 150. DOI: 10.1186/s12886-019-1154-6.
9. Hu A, Yuan M, Liu F, et al. Ultrasonographic feature of persistent hyperplastic primary vitreous[J]. Eye Sci, 2014, 29: 100-103.
10. Hu A, Pei X, Ding X, et al. Combined persistent fetal vasculature: a classification based on high-resolution B-mode ultrasound and color doppler imaging[J]. Ophthalmology, 2016, 123(1): 19-25. DOI: 10.1016/j.ophtha.2015.09.001.
11. 梁天蔚, 张诚玥, 白大勇, 等. 先天性纤维血管瞳孔膜的临床特点及治疗分析[J]. 中华眼科杂志, 2018, 54(11): 849-854. DOI: 10.3760/cma.j.issn.0412-4081.2018.11.010.Liang TW, Zhang CY, Bai DY, et al. Clinical characteristics and treatment of congenital fibrovascular pupillary membranes[J]. Chin J Ophthalmol, 2018, 54(11): 849-854. DOI: 10.3760/cma.j.issn.0412-4081.2018.11.010.
12. Maheshwari D, Rao S, Pawar N, et al. An infectious cause of congenital pupillary-iris-lens membrane with secondary angle closure glaucoma in an infant[J/OL]. J Glaucoma, 2021, 30(5): e271-e273[2021-05-01]. https://pubmed.ncbi.nlm.nih.gov/33137022/. DOI: 10.1097/IJG.0000000000001726.
13. Yu T, Yin ZQ, Yang H. Surgical management of congenital pupillary-iris-lens membrane[J]. Eur J Ophthalmol, 2011, 21(5): 604-607. DOI: 10.5301/EJO.2011.7734.
14. Kandori M, Saishin Y, Kusaka S, et al. Pupilloplasty for congenital pupillary-iris-lens membrane with 25-gauge vitreous cutter[J/OL]. Acta Ophthalmol, 2010, 88(7): e289-290[2009-10-23]. https://pubmed.ncbi.nlm.nih.gov/19860772/. DOI: 10.1111/j.1755-3768.2009.01698.x.
15. Lambert SR, Buckley EG, Lenhart PD, et al. Congenital fibrovascular pupillary membranes: clinical and histopathologic findings[J]. Ophthalmology, 2012, 119(3): 634-641. DOI: 10.1016/j.ophtha.2011.08.043.
16. Demidenko A, Jakobiec FA, Hanna E, et al. Congenital pupillary-iris-lens membrane with goniodysgenesis: histopathologic findings in an enucleated eye[J]. J Pediatr Ophthalmol Strabismus, 2010, 47(3): 178-182. DOI: 10.3928/01913913-20100505-09.
17. Demidenko A, Jakobiec FA, Hanna E, et al. Congenital pupillary-iris-lens membrane with goniodysgenesis: a rare cause of glaucoma and vision loss[J]. Int Ophthalmol Clin, 2009, 49(1): 83-88. DOI: 10.1097/IIO.0b013e318192442b.
18. Bosjolie A, Ferrone P. Visual outcome in early vitrectomy for posterior persistent fetal vasculature associated with traction retinal detachment[J]. Retina, 2015, 35(3): 570-576. DOI: 10.1097/IAE.0000000000000353.
19. Hunt A, Rowe N, Lam A, et al. Outcomes in persistent hyperplastic primary vitreous[J]. Br J Ophthalmol, 2005, 89(7): 859-863. DOI: 10.1136/bjo.2004.053595.
20. I Gawdat G, M Youssef M, M Bahgat N, et al. Incidence and risk factors of early-onset glaucoma following pediatric cataract surgery in egyptian children: one-year study[J]. J Curr Glaucoma Pract, 2017, 11(3): 80-85. DOI: 10.5005/jp-journals-10028-1229.
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