Long-Term Visual Function and Relative Amblyopia in Posterior Persistent Hyperplastic Primary Vitreous (PHPV)

Schulz, E.; Griffiths, Birgit

doi:10.1080/09273970600701242

Cited by 19 publications

(7 citation statements)

References 7 publications

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“…Posterior PHPV on the other hand is much less common and occurs in the posterior segment characterized by a white dense opaque membrane or a prominent retinal fold. Macular as well as optic disc abnormalities have been reported 11–14 . Despite its clinical importance, very little is known about the molecular mechanisms responsible for the development of PHPV.…”

Section: Introductionmentioning

confidence: 99%

Persistent hyperplastic primary vitreous: congenital malformation of the eye

Shastry

2009

Clinical Exper Ophthalmology

119

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Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. The condition can be of an isolated type or can occur with other ocular disorders. Most cases of PHPV are sporadic, but it can be inherited as an autosomal dominant or recessive trait. Inherited PHPV also occurs in several breeds of dogs and cats. In a limited number of cases, Norrie disease and FZD4 genes are found to be mutated in unilateral and bilateral PHPV. These genes when mutated also cause Norrie disease pseudoglioma and familial exudative vitreoretinopathy that share some of the clinical features with PHPV. Mice lacking arf and p53 tumour suppressor genes as well as Norrie disease pseudoglioma and LRP5 genes suggest that these genes are needed for hyaloid vascular regression. These experiments also indicate that abnormalities in normal apoptosis and defects in Wnt signalling pathway may be responsible for the pathogenesis of PHPV. Identification of other candidate genes in the future may provide a better understanding of the pathogenesis of the condition that may lead to a better therapeutic approach and better management.

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Section: Introductionmentioning

confidence: 99%

Persistent hyperplastic primary vitreous: congenital malformation of the eye

Shastry

2009

Clinical Exper Ophthalmology

119

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“…22 Amblyopia is a major factor contributing to poor visual outcome. 3,23 In our case, lens subluxation and spontaneous absorption of the lens are presumed to have reduced the impact of form deprivation on visual development somewhat. However, surgery was still indicated in this case to clear the visual axis and correct the refractive errors.…”

Section: Discussionmentioning

confidence: 60%

Bilateral persistent fetal vasculature in an adult: Clinical manifestations and surgical outcomes

Zhao

Ding

2010

Journal of Cataract and Refractive Surgery

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“…Follow-up beyond visual maturation is rare; only one series identifies three children, aged 7–10 years, with a final acuity of 20/40 or better who were managed with lensectomy and vitrectomy, with or without stalk cauterisation 7. The timing of surgical intervention in PHPV is critical to the visual outcome; intervention before 1 year confers a 10-fold increase in the probability of ‘counting fingers’ vision or better 2.…”

Section: Discussionmentioning

confidence: 99%

“…Parental compliance is critical to visual outcome in PHPV7; amblyopia therapy requires their maintained interest and frequent, long-term regular hospital visits are vital. Parental motivation and aggressive amblyopia management was a significant factor in the excellent visual outcome in this child.…”

Section: Discussionmentioning

confidence: 99%

Unilateral persistent hyperplastic primary vitreous: intensive management approach with excellent outcome beyond visual maturation

Yusuf¹,

Patel

Salmon

2015

BMJ Case Reports

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Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract and secondary glaucoma, meticulous monitoring of refraction, visual acuity and intraocular pressure and motivated parents who engaged in the management.

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Long-Term Visual Function and Relative Amblyopia in Posterior Persistent Hyperplastic Primary Vitreous (PHPV)

Cited by 19 publications

References 7 publications

Persistent hyperplastic primary vitreous: congenital malformation of the eye

Persistent hyperplastic primary vitreous: congenital malformation of the eye

Bilateral persistent fetal vasculature in an adult: Clinical manifestations and surgical outcomes

Unilateral persistent hyperplastic primary vitreous: intensive management approach with excellent outcome beyond visual maturation

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