Persistent Hyperplastic Primary Vitreous

A developmental ocular anomaly in which the primary VITREOUS BODY and its surrounding hyaloid vasculature failed to regress. It is usually unilateral and characterized by CATARACT; MICROPHTHALMOS (small eyeballs), and retrolenticular fibrovascular tissue. (from Yanoff: Ophthalmology, 2nd ed.)

Also Known As:

Networked: 75 relevant articles (2 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Retinoblastoma (Glioblastoma, Retinal)
2.	Hamartoma
3.	Cataract (Cataracts)
4.	Retinopathy of Prematurity (Retrolental Fibroplasia)
5.	Retinal Detachment (Retinal Detachments)

Experts

1.	Capone, Antonio: 3 articles (04/2010 - 02/2007)
2.	Trese, Michael T: 2 articles (04/2010 - 06/2003)
3.	Shang, Peng: 1 article (01/2016)
4.	Sinha, Debasish: 1 article (01/2016)
5.	Goldberg, Morton F: 1 article (01/2016)
6.	Valapala, Mallika: 1 article (01/2016)
7.	Zigler, J Samuel: 1 article (01/2016)
8.	Hose, Stacey: 1 article (01/2016)
9.	Kirchhof, Bernd: 1 article (07/2015)
10.	Ikeda, Tsunehiko: 1 article (05/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Persistent Hyperplastic Primary Vitreous:

1.	Retinaldehyde (Retinal)IBA 01/01/2013 - "Persistent hyaloid artery with an aberrant peripheral retinal attachment: A unique presentation." 08/15/2012 - "Our results strongly suggest that autosomal recessive persistent hyperplastic primary vitreous is caused by N46H and is etiologically related to nonsyndromic congenital retinal nonattachment. " 05/01/2010 - "Interestingly, mutations at the same residue but resulting in a different missense change were previously described in subjects with XL-FEVR (p.Arg41Lys) or persistent fetal vasculature syndrome (p.Arg41Ser), indicating that the novel p.Arg41Thr change causes a more severe retinal phenotype. " 01/01/2004 - "The paper presents the case of a 18 years old male suffering from persistent hyperplastic primary vitreous (PHPV) associated with congenital retinal folds. " 01/01/2004 - "[Persistent hyperplastic primary vitreous associated with retinal folds]."
2.	Norrie diseaseIBA 03/01/1998 - "Study of the Norrie disease gene in 2 patients with bilateral persistent hyperplastic primary vitreous." 09/01/1983 - "The three most common causes of pseudoglioma are persistent hyperplastic primary vitreous, Coats's disease and sclerosing endophthalmitis. " 01/01/1992 - "We believe that the retinal and other manifestations of Norrie disease are the result of a primary abnormality of vascular proliferation, probably in relation to persistent hyperplastic primary vitreous after approximately 14 weeks' gestation. "
3.	SodiumIBA 04/01/2010 - "The purpose of this study was to report the successful management of a case of choroidal neovascular membrane secondary to persistent fetal vasculature syndrome in an infant using intravitreal pegaptanib sodium (Macugen). " 04/01/2010 - "Choroidal neovascular membrane in persistent fetal vasculature syndrome managed with intravitreal pegaptanib sodium in an infant."
4.	CollagenIBA 01/01/1985 - "Morphological study on the human developing vitreous collagen fibrils and persistent hyperplastic primary vitreous."
5.	pegaptanibFDA Link 04/01/2010 - "The purpose of this study was to report the successful management of a case of choroidal neovascular membrane secondary to persistent fetal vasculature syndrome in an infant using intravitreal pegaptanib sodium (Macugen). " 04/01/2010 - "Choroidal neovascular membrane in persistent fetal vasculature syndrome managed with intravitreal pegaptanib sodium in an infant."
6.	Peters anomalyIBA 02/01/2010 - "Case of novel PITX2 gene mutation associated with Peters' anomaly and persistent hyperplastic primary vitreous." 12/01/2001 - "To clarify the developmental mechanism and critical period for the uncommon complex of Peters' anomaly and persistent hyperplastic primary vitreous (PHPV). " 12/01/2001 - "Histopathological examination of two cases of anterior staphyloma associated with Peters' anomaly and persistent hyperplastic primary vitreous." 08/01/2009 - "Some Pax6(Leca4)(/+) ocular abnormalities were similar to those present in human Peters' anomaly and persistent hyperplastic primary vitreous (PHPV) so Pax6(Leca4)(/+) mice provide a useful model for some inherited eye diseases."
7.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 05/01/2015 - "A fundus examination showed a persistent hyaloid artery in the vitreous cavity, a widespread loss of fundus pigmentation, transparent choroidal vessels (some choroidal vessel sections were visible), and the absence of a distinct macular reflex. " 11/01/1992 - "He had bilateral persistent hyaloid arteries, and in the right eye this vessel penetrated the lens capsule and invaded the lens. " 05/01/1997 - "Persistent hyperplastic primary vitreous (PHPV) is a congenital disorder that presents with a spectrum of ocular anomalies, including cataracts, microphthalmia, and hyaloid vessel remnants. "
8.	Coloboma of optic nerveIBA 05/15/1987 - "On histologic examination the left eye showed an iris coloboma, ciliary epithelial differentiation to the retina, undifferentiated neuroepithelium beneath the equatorial retina, persistent hyaloid artery, and optic nerve coloboma and pit. " 12/15/1992 - "Spontaneous retinal reattachment in a patient with persistent hyperplastic primary vitreous and an optic nerve coloboma."
9.	tebufenozide (Mimic)IBA 04/01/1999 - "With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. " 01/01/2013 - "Bilateral persistent hyperplastic primary vitreous: a close mimic of retinoblastoma."
10.	ROPIBA 04/01/1988 - "Association of a persistent hyaloid artery and ROP." 09/01/1994 - "When leukokoria is associated with microphthalmia, persistent hyperplastic primary vitreous (PHPV) (28% of cases) or retinopathy of prematurity (ROP) (5% of cases) should be considered. "

Therapies and Procedures

1.	Vitrectomy 03/01/1979 - "Pars plana vitrectomy requiring a two-instrument technique successfully cleared the pupillary axis of dense persistent hyperplastic primary vitreous in a 29-year-old patient. " 04/01/2010 - "Early vitrectomy effective for bilateral combined anterior and posterior persistent fetal vasculature syndrome." 07/01/2013 - "To report the feasibility and outcome of lens aspiration, and Fugo blade-assisted capsulotomy and anterior vitrectomy in eyes with anterior persistent hyperplastic primary vitreous (PHPV). " 04/01/2010 - "A 14-month-old male infant developed a peripapillary choroidal neovascular membrane 10 months after lens-sparing vitrectomy that he underwent for persistent fetal vasculature syndrome and confirmed on angiography and optical coherence tomography. " 06/01/2003 - "This series shows that, in selected cases of persistent fetal vasculature syndrome that are predominantly posterior in nature with nonaxial lens opacification, lens-sparing vitrectomy can be of benefit."
2.	Intraocular Lens Implantation 03/01/2001 - "Intraocular lens implantation in a child with monocular cataract and anterior persistent hyperplastic primary vitreous." 12/01/2007 - "The following risk factors were evaluated: age at cataract surgery, presence of systemic anomalies, microcornea, persistent hyperplastic primary vitreous (PHPV), primary capsulotomy/anterior vitrectomy, primary intraocular lens implantation, secondary membrane surgery and duration of postoperative observation. "
3.	Phacoemulsification 03/01/2001 - "A 3-year-old girl had phacoemulsification during which the presence of anterior persistent hyperplastic primary vitreous (PHPV) was discovered. " 01/01/2010 - "A 15-year-old boy underwent phacoemulsification for a membranous cataract during which the presence of anterior persistent hyaloid artery and elongated ciliary processes were discovered. "
4.	Traction 03/01/2005 - "To demonstrate the ultrastructure of vitreomacular traction associated with persistent hyaloid artery. " 03/01/2005 - "Ultrastructure of vitreomacular traction syndrome associated with persistent hyaloid artery." 03/01/2005 - "Pars plana vitrectomy was performed in a 66-year-old man with progressive vitreomacular traction associated with a persistent hyaloid artery. " 07/01/2015 - "Remnants of a persistent hyaloid artery can occasionally cause cataracts and traction on the retina at the posterior pole of the eye.It"
5.	Vitreoretinal Surgery 11/01/2011 - "As new technics in vitreoretinal surgery develop performance of miniinvasive procedures has become possible in children with ocular diseases associated with cataract (persistent hyperplastic primary vitreous, retinal detachment, vitreous synechia), retinopathy of prematurity, retinoblastoma, retinal detachment of different ethiology as well as for prevention of proliferative syndrome. "