Abstract
Purpose
To describe the frequency, characteristics, and treatment outcome of persistent fetal vasculature (PFV) in children undergoing surgery for congenital and infantile cataract in the first 2 years of life.
Patients and methods
Observational population-based cohort study with case identification through active surveillance and standardised data collection via a national clinical network, the British Isles Congenital Cataract Interest Group (BCCIG).
Results
The IoLunder2 cohort comprises 246 children undergoing surgery for bilateral and unilateral congenital and infantile cataract in the first 2 years of life. A total of 58/246 (24%) children had PFV (%): overall, 46/95 (46%) with unilateral cataract, and 12/141 (8%) with bilateral disease. Anterior segment vascular remnants were more common in bilateral than unilateral disease (75 vs 11%, P=0.01). At 1 year after surgery, 20% of children with bilateral PFV and 24% with unilateral had achieved normal vision for age within the operated eye. The prevalence of post-operative glaucoma was 9% (of children with bilateral disease) and 4% (unilateral).
Conclusion
PFV is significantly more common than previously reported, and outcomes are comparable to that for congenital and infantile cataract overall.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
References
Goldberg MF . Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture. Am J Ophthalmol 1997; 124 (5): 587.
Lambert SR, Buckley E, Lenhart P, Zhang Q, Grossniklaus H . Congenital fibrovascular pupillary membranes: clinical and histopathologic findings. Ophthalmology 2012; 119 (3): 634–641.
Reese AB . Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955; 40 (3): 317–331.
Rahi JS, Dezateux C . Congenital and infantile cataract in the United Kingdom: underlying or associated factors. British Congenital Cataract Interest Group. Invest Ophthalmol Vis Sci 2000; 41 (8): 2108–2114.
Sisk RA, Berrocal AM, Feuer WJ, Murray TG . Visual and anatomic outcomes with or without surgery in persistent fetal vasculature. Ophthalmology 2010; 117 (11): 2178–2183.
Morrison DG, Wilson ME, Trivedi RH, Lambert SR, Lynn MJ . Infant Aphakia Treatment Study: effects of persistent fetal vasculature on outcome at 1 year of age. J AAPOS 2011; 15 (5): 427–431.
Mullner-Eidenbock A, Amon M, Moser E, Klebermass N . Persistent fetal vasculature and minimal fetal vascular remnants* 1: a frequent cause of unilateral congenital cataracts. Ophthalmology 2004; 111 (5): 906–913.
Solebo AL, Russell-Eggitt I, Cumberland PM, Rahi JS . Risks and outcomes associated with primary intraocular lens implantation in children under 2 years of age: the IoLunder2 cohort study. Br J Ophthalmol 2015; 99 (11): 1471–1476.
Lim Z, Rubab S, Chan YH, Levin AV . Pediatric cataract: the Toronto Experience -Etiology. Am J Ophthalmol 2010; 149 (6): 887–892.
Lambert SR, Lynn MJ, Hartmann EE, DuBois L, Drews-Botsch C, Freedman SF et al. Comparison of contact lens and intraocular lens correction of monocular aphakia during infancy: a Randomized Clinical Trial of HOTV Optotype Acuity at age 4.5 years and clinical findings at age 5 years. JAMA Ophthalmol 2014; 132: 676–682.
Chak M, Rahi JS . Incidence of and factors associated with glaucoma after surgery for congenital cataract: findings from the British Congenital Cataract Study. Ophthalmology 2008; 115 (6): 1013–1018.
Gillespie RL, O'Sullivan J, Ashworth J, Bhaskar S, Williams S, Biswas S et al. Personalized diagnosis and management of congenital cataract by next-generation sequencing. Ophthalmology 2014; 121 (11): 2124–2137.
Lang RA, Bishop JM . Macrophages are required for cell death and tissue remodeling in the developing mouse eye. Cell 1993; 74 (3): 453–462.
Hahn P, Lindsten T, Tolentino M, Thompson CB, Bennett J, Dunaief JL . Persistent fetal ocular vasculature in mice deficient in bax and bak. Arch Ophthalmol 2005; 123 (6): 797.
Lobov IB, Rao S, Carroll TJ, Vallance JE, Ito M, Ondr JK et al. WNT7b mediates macrophage-induced programmed cell death in patterning of the vasculature. Nature 2005; 437 (7057):417–421.
Gogat K, Le Gat L, Van Den Berghe L, Marchant D, Kobetz A, Gadin S et al. VEGF and KDR gene expression during human embryonic and fetal eye development. Invest Ophthalmol Vis Sci 2004; 45 (1): 7.
Acknowledgements
This work was undertaken at UCL Institute of Child Health/Great Ormond Street Hospital for Children, which received a proportion of funding from the Department of Health’s NIHR Biomedical Research Centres funding scheme. Lola Solebo is funded, and Jugnoo Rahi is supported in part by the National Institute for Health Research (NIHR) Biomedical Research Centre based at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology. Lola Solebo was supported by a personal fellowship awarded by the Ulverscroft Vision Research Group. The views expressed are those of the author(s) and not necessarily those of the funders, the NHS, the NIHR, or the Department of Health.
Disclaimer
The funding organisations had no role in the design or conduct of this research.
Author information
Authors and Affiliations
Consortia
Corresponding author
Ethics declarations
Competing interests
The authors declare no conflict of interest.
Additional information
Supplementary Information accompanies this paper on Eye website
Supplementary information
Rights and permissions
About this article
Cite this article
Solebo, A., Russell-Eggitt, I., Cumberland, P. et al. Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2. Eye 30, 1204–1209 (2016). https://doi.org/10.1038/eye.2016.159
Received:
Accepted:
Published:
Issue date:
DOI: https://doi.org/10.1038/eye.2016.159
This article is cited by
-
Surgical outcomes of cataract surgery in anterior and combined persistent fetal vasculature using a novel surgical technique: a single center, prospective study
Graefe's Archive for Clinical and Experimental Ophthalmology (2021)
-
Cataract management in children: a review of the literature and current practice across five large UK centres
Eye (2020)
-
Intraocular lens implantation in combination with lensectomy and vitrectomy for persistent fetal vasculature
Graefe's Archive for Clinical and Experimental Ophthalmology (2020)
