Persistent Hyperplastic Primary Vitreous*

Reese, Algernon B.

doi:10.1016/0002-9394(55)91866-3

Cited by 209 publications

(102 citation statements)

References 19 publications

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“…The early outcomes from IoLunder2 should be broadly predictive of later outcomes, 10 although more cases of post-operative glaucoma are likely to emerge. 11 Stretched ciliary processes were once considered pathognomic for PFV, 3 but are now known to be present in other vitreoretinopathies such as retinopathy of prematurity or vitreoretinal dysplasia. 1 Our findings suggest that children with bilateral PFV are less likely to have ciliary traction, but also more likely to have anterior segment manifestations, and more likely to have a nonophthalmic disorder, but the significance of this is unclear.…”

Section: Discussionmentioning

confidence: 99%

“…2 Cataract is a frequent secondary event in PFV due to invasion of the vascular remnants, lenticular swelling, intralenticular haemorrhage, or calcification. 1,3 PFV features have been found in a fifth of cases of unilateral congenital or infantile cataract, but are much less commonly noted in bilateral cataract. 4 Cataract surgery in children with PFV is associated with a higher incidence of adverse events and worse outcomes.…”

Section: Introductionmentioning

confidence: 99%

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Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

Solebo

Russell‐Eggitt

Cumberland

et al. 2016

Eye

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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 postoperative 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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

Solebo

Russell‐Eggitt

Cumberland

et al. 2016

Eye

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“…Eyes from patients with PHPV have the following pathologic characteristics: (i) the presence of retrolental tissue, which can contain melanocytes; (ii) the attachment of the retrolental tissue to the inner neuroretina; (iii) retrolental tissue-induced traction on the neuroretina that causes neuroretina detachment from the retina pigment epithelium; (iv) cellular disorganization and other dysplastic changes in the neuroretina; (v) posterior lens capsule destruction by the retrolental mass; and (vi) cataractous degeneration of the lens (24,25,36,37). The observation that the eye pathology in Arf Ϫ͞Ϫ mice is similar to that in PHPV patients raises the question of whether ARF abnormalities may contribute to this disease.…”

Section: Discussionmentioning

confidence: 99%

The Arf tumor suppressor gene promotes hyaloid vascular regression during mouse eye development

Mckeller

Fowler

Cunningham

et al. 2002

Proc. Natl. Acad. Sci. U.S.A.

117

140

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A key tumor suppressor mechanism that is disrupted frequently in human cancer involves the ARF and p53 genes. In mouse fibroblasts, the Arf gene product responds to abnormal mitogenic signals to activate p53 and trigger either cell cycle arrest or apoptosis. Recent evidence indicates that Arf also has p53-independent functions that may contribute to its tumor suppressor activity. Using Arf Ϫ͞Ϫ and p53 Ϫ͞Ϫ mice, we have discovered a p53-independent requirement for Arf in the developmental regression of the hyaloid vascular system (HVS) in the mouse eye. Arf is expressed in the vitreous of the eye and is induced before HVS regression in the first postnatal week. In the absence of Arf, failed HVS regression causes a pathological process that resembles persistent hyperplastic primary vitreous, a developmental human eye disease thought to have a genetic basis. These findings demonstrate an essential and unexpected role for Arf during mouse eye development, provide insights into the potential genetic basis for persistent hyperplastic primary vitreous, and indicate that Arf regulates vascular regression in a p53-independent manner. The latter finding raises the possibility that Arf may function as a tumor suppressor at least in part by regulating tumor angiogenesis.

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“…Occasionally in humans, instead of regressing between the third and the ninth month of gestation, the structures of the primary vitreous persist to give a disorder known as persistent hyperplastic primary vitreous (PHPV). This congenital ocular disorder has been thoroughly reviewed by Reese in his 1955 Jackson memorial lecture (Reese, 1955). It usually occurs unilaterally with no apparent hereditary influence.…”

Section: Introductionmentioning

confidence: 99%

High frequency of persistent hyperplastic primary vitreous and cataracts in p53-deficient mice

et al. 1998

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In order to investigate whether the p53 gene product plays a role in normal eye development, age matched p53-deficient mice and wild-type controls were sacrificed from day 2 to day 21 after birth. Eyes were paraffin-embedded and sectioned. Serial sections were taken at the level of the tunica vasculosa lentis and the hyaloid artery. The terminal dUTP nick-end labelling technique (TUNEL) was used to detect the number of cells displaying DNA fragmentation within these structures. Eyes were also prepared for scanning electron microscopy and resin embedded for semi-thin sections. Adult wild-type mice and p53-deficient mice were examined ophthalmoscopically in vivo. Ophthalmoscopical examination of mice completely deficient in p53 revealed them to be normal except for the persistence of the hyaloid vasculature, a structure that normally regresses during eye development. In adult animals there was also a high frequency of cataracts. Using morphological assessment and TUNEL we could show that in normal mice, regression of the primary vitreous, which includes the hyaloid artery, the vasa hyaloidea propria as well as the tunica vasculosa lentis, occurs via apoptotic cell death within 5 ± 6 weeks after birth. The number of TUNEL-positive cells within these structures was significantly reduced in the p53-deficient mice in which parts of the hyaloid vasculature persisted and developed into a fibro-vascular retrolental plaque analogous to persistent hyperplastic primary vitreous (PHPV) described in humans. As in humans, PHPV in mice resulted in the development of cataracts. We have identified a role for p53-dependent apoptosis in the regression of the hyaloid vasculature and tunica vasculosa lentis. Our results provide further evidence for the importance of p53 in normal development and provide the first detailed evidence of its role in postnatal development in remodelling the developing eye.

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Persistent Hyperplastic Primary Vitreous*

Cited by 209 publications

References 19 publications

Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

Congenital cataract associated with persistent fetal vasculature: findings from IoLunder2

The Arf tumor suppressor gene promotes hyaloid vascular regression during mouse eye development

High frequency of persistent hyperplastic primary vitreous and cataracts in p53-deficient mice

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