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: 86 relevant articles (2 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1.	Capone, Antonio: 4 articles (03/2019 - 02/2007)
2.	Trese, Michael T: 3 articles (03/2019 - 06/2003)
3.	Hose, Stacey: 2 articles (01/2020 - 01/2016)
4.	Shang, Peng: 2 articles (01/2020 - 01/2016)
5.	Sinha, Debasish: 2 articles (01/2020 - 01/2016)
6.	Zigler, J Samuel: 2 articles (01/2020 - 01/2016)
7.	Drenser, Kimberly A: 2 articles (03/2019 - 04/2010)
8.	Lu, Lin: 2 articles (01/2019 - 10/2017)
9.	Kohnen, Thomas: 2 articles (01/2016 - 07/2008)
10.	Kuhli-Hattenbach, Claudia: 2 articles (01/2016 - 07/2008)

Drugs and Biologics

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

1.	Retinaldehyde (Retinal)IBA 10/01/2023 - "Morning glory syndrome (MGS) and persistent hyperplastic primary vitreous (PHPV) are congenital abnormity, which may be related to the increased incidence of systemic abnormalities and retinal detachment，diagnosed by ultrasound, identified by CT, MRI, and with the confirmation of fundus examination." 03/01/2020 - "Ocular ultrasound and electroretinogram confirmed the diagnoses of retinal separations and persistent hyperplastic primary vitreous. " 01/01/2020 - "A spatial relationship between persistent hyaloid vasculature and retardation of underlying retinal vascular development was observed in the OIR eyes. " 01/01/2019 - "Myelinated retinal nerve fibers and persistent hyperplastic primary vitreous." 01/01/2016 - "Furthermore, ocular developmental defects of retinal depigmentation, optic disc abnormality, and persistent hyaloid artery were also observed. "
2.	CollagenIBA 01/01/1985 - "Morphological study on the human developing vitreous collagen fibrils and persistent hyperplastic primary vitreous."
3.	pegaptanib (Macugen)FDA 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."
4.	Capsules (Microcapsules)IBA 11/01/1992 - "He had bilateral persistent hyaloid arteries, and in the right eye this vessel penetrated the lens capsule and invaded the lens. " 08/01/1985 - "Three cases of persistent hyperplastic primary vitreous (PHPV) showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented. " 01/01/1984 - "In one, fibrovascular stalk passed forward into the vitreous cavity, while in the second a persistent hyaloid artery extended anteriorly from a tuft of fibrous tissue to the posterior lens capsule. " 01/01/2010 - "A posterior capsulorhexis was performed and the anterior part of the persistent hyaloid artery stalk was resected together with the posterior capsule. " 03/01/2018 - "Intraocular sarcoma associated with lens capsule rupture and persistent hyperplastic primary vitreous in a dog."
5.	Fluorescein (Funduscein)FDA LinkGeneric 01/01/2019 - "To identify imaging characteristics of mouse persistent hyperplastic primary vitreous (PHPV) by Spectralis Optical Coherence Tomography (OCT), as well as to assess and compare the sensitivity and precision of OCT with color photography (CP) and Fundus Fluorescein Angiography (FFA) imaging in detecting mouse PHPV. " 07/01/1978 - "Persistent hyperplastic primary vitreous in an adult: case report with fluorescein angiographic findings." 10/03/2017 - "No persistent hyaloid artery or connection between the cyst and ocular structures was found by fundus fluorescein angiography and B-scan ultrasound. "
6.	SteroidsIBA 01/01/2021 - "Demographic and clinical characteristics were recorded including age at surgery, sex, corneal diameter, CCT pupil diameter, IOP, gonioscopic findings, presence of persistent hyperplastic primary vitreous, surgical approach, primary intraocular lens implantation, and perioperative subconjunctival steroid injection. " 10/01/1999 - "The following glaucoma groups were associated with a poor visual outcome: aniridia, anterior segment developmental anomalies involving the cornea, uveitis with glaucoma other than steroid induced, retinopathy of prematurity, and persistent hyperplastic primary vitreous. "
7.	Crystallins (Crystallin)IBA 01/01/2016 - "In animal models lacking βA3/A1-crystallin, astrocyte numbers are increased and they migrate abnormally from the retina to ensheath the persistent hyaloid artery. " 01/01/2020 - "ACTB: actin, beta; CCND3: cyclin 3; CDK6: cyclin-dependent kinase 6; CHQ: chloroquine; COL4A1: collagen, type IV, alpha 1; CRYBA1: crystallin, beta A1; DAPI: 4'6-diamino-2-phenylindole; EGFR: epidermal growth factor receptor; GAPDH: glyceraldehyde-3-phosphate dehydrogenase; GFAP: glial fibrillary growth factor; KDR: kinase insert domain protein receptor; MAP1LC3/LC3: microtubule-associated protein 1 light chain 3; MKI67: antigen identified by monoclonal antibody Ki 67; MTORC1: mechanistic target of rapamycin kinase complex 1; PARP: poly (ADP-ribose) polymerase family; PCNA: proliferating cell nuclear antigen; PFV: persistent fetal vasculature; PHPV: persistent hyperplastic primary vitreous; RPE: retinal pigmented epithelium; RPS6: ribosomal protein S6; RPS6KB1: ribosomal protein S6 kinase, polypeptide 1; SQSTM1/p62: sequestome 1; TUBB: tubulin, beta; VCL: vinculin; VEGFA: vascular endothelial growth factor A; WT: wild type."
8.	ErbB Receptors (EGF Receptor)IBA 01/01/2020 - "ACTB: actin, beta; CCND3: cyclin 3; CDK6: cyclin-dependent kinase 6; CHQ: chloroquine; COL4A1: collagen, type IV, alpha 1; CRYBA1: crystallin, beta A1; DAPI: 4'6-diamino-2-phenylindole; EGFR: epidermal growth factor receptor; GAPDH: glyceraldehyde-3-phosphate dehydrogenase; GFAP: glial fibrillary growth factor; KDR: kinase insert domain protein receptor; MAP1LC3/LC3: microtubule-associated protein 1 light chain 3; MKI67: antigen identified by monoclonal antibody Ki 67; MTORC1: mechanistic target of rapamycin kinase complex 1; PARP: poly (ADP-ribose) polymerase family; PCNA: proliferating cell nuclear antigen; PFV: persistent fetal vasculature; PHPV: persistent hyperplastic primary vitreous; RPE: retinal pigmented epithelium; RPS6: ribosomal protein S6; RPS6KB1: ribosomal protein S6 kinase, polypeptide 1; SQSTM1/p62: sequestome 1; TUBB: tubulin, beta; VCL: vinculin; VEGFA: vascular endothelial growth factor A; WT: wild type."
9.	Low Density Lipoprotein Receptor-Related Protein-5IBA 02/01/2016 - "Mono- and bi-allelic mutations in the low-density lipoprotein receptor related protein 5 (LRP5) may cause osteopetrosis, autosomal dominant and recessive exudative vitreoretinopathy, juvenile osteoporosis, or persistent hyperplastic primary vitreous (PHPV). "
10.	OpsinsIBA 08/01/1988 - "Immunoreactive opsin and glial fibrillary acidic protein in persistent hyperplastic primary vitreous."

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." 11/01/2023 - "Lens-sparing vitrectomy is a useful approach to addressing the retrolental stalk in selected cases of persistent fetal vasculature syndrome. " 03/01/2019 - "To describe the microstructural features of the macula and vitreomacular interface in persistent fetal vasculature syndrome (PFVS) with posterior involvement managed with early vitrectomy or with observation, with functional correlation. " 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). "
2.	Intraocular Lens Implantation 01/01/2016 - "Congenital cataract surgery without intraocular lens implantation in persistent fetal vasculature syndrome: Long-term clinical and functional results." 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. " 01/01/2021 - "Demographic and clinical characteristics were recorded including age at surgery, sex, corneal diameter, CCT pupil diameter, IOP, gonioscopic findings, presence of persistent hyperplastic primary vitreous, surgical approach, primary intraocular lens implantation, and perioperative subconjunctival steroid injection. "
3.	Slit Lamp 07/04/2019 - "Slit-lamp examination demonstrated a Mittendorf's dot and fundus examination revealed a persistent hyaloid artery. " 01/01/2020 - "Ophthalmologic examination of male and female rats by using a binocular indirect ophthalmoscope and slit lamp revealed high incidences of corneal opacity (61% and 60%, respectively), lenticular opacity (43% and 47%), persistent hyaloid artery (21% and 17%), and retinal folds (27% and 27%). "
4.	Posterior Capsulotomy 01/01/2020 - "Femtosecond laser-assisted anterior and posterior capsulotomies in children with persistent hyperplastic primary vitreous." 07/01/2013 - "Results of cataract surgery and plasma ablation posterior capsulotomy in anterior persistent hyperplastic primary vitreous." 01/01/2020 - "Anterior and posterior capsulotomies were performed in 12 eyes of 12 patients (age range 3 months to 6 years) with congenital cataracts and primary persistent hyperplastic primary vitreous (PHPV) syndrome using a femtosecond laser. "
5.	Telescopes 01/01/2020 - "A rare case of binocular persistent hyperplastic primary vitreous (PHPV) in a pregnant woman during two consecutive pregnancies." 01/01/2020 - "Ophthalmologic examination of male and female rats by using a binocular indirect ophthalmoscope and slit lamp revealed high incidences of corneal opacity (61% and 60%, respectively), lenticular opacity (43% and 47%), persistent hyaloid artery (21% and 17%), and retinal folds (27% and 27%). "