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.)
Congenital absence of or defects in structures of the eye; may also be hereditary.
The transparent, semigelatinous substance that fills the cavity behind the CRYSTALLINE LENS of the EYE and in front of the RETINA. It is contained in a thin hyaloid membrane and forms about four fifths of the optic globe.
Procedures performed to remove CAPSULE OPACIFICATION that develops on the POSTERIOR CAPSULE OF THE LENS following removal of a primary CATARACT.
An increase in the number of cells in a tissue or organ without tumor formation. It differs from HYPERTROPHY, which is an increase in bulk without an increase in the number of cells.
A transparent, biconvex structure of the EYE, enclosed in a capsule and situated behind the IRIS and in front of the vitreous humor (VITREOUS BODY). It is slightly overlapped at its margin by the ciliary processes. Adaptation by the CILIARY BODY is crucial for OCULAR ACCOMMODATION.
Partial or complete opacity on or in the lens or capsule of one or both eyes, impairing vision or causing blindness. The many kinds of cataract are classified by their morphology (size, shape, location) or etiology (cause and time of occurrence). (Dorland, 27th ed)

Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous. (1/13)

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A nonautonomous role for retinal frizzled-5 in regulating hyaloid vitreous vasculature development. (2/13)

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Bilateral persistent hyperplastic primary vitreous. (3/13)

A case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 3-month-old male infant, who had bilateral leukokoria, is presented. The child was referred for imaging with a clinical suspicion of retinoblastoma. Gray-scale ultrasound evaluation revealed an echogenic band in the posterior segment of both globes, extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of arterial flow in the band in both globes. Associated echogenic hemorrhage was also seen, which was confirmed by computed tomography. Most cases of PHPV are sporadic and unilateral, and bilateral PHPV is rare. The imaging features in this case suggest the diagnosis of bilateral PHPV and differentiate it from retinoblastoma. This entity, although infrequent, should be considered in the differential diagnosis while evaluating bilateral leukokoria.  (+info)

A developmental defect in astrocytes inhibits programmed regression of the hyaloid vasculature in the mammalian eye. (4/13)

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Infant Aphakia Treatment Study: effects of persistent fetal vasculature on outcome at 1 year of age. (5/13)

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Congenital fibrovascular pupillary membranes: clinical and histopathologic findings. (6/13)

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The deletion of Math5 disrupts retinal blood vessel and glial development in mice. (7/13)

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Deletion of HIF-1alpha partially rescues the abnormal hyaloid vascular system in Cited2 conditional knockout mouse eyes. (8/13)

PURPOSE: Cited2 (CBP/p300-interacting transactivators with glutamic acid (E) and aspartic acid (D)-rich tail 2) is a member of a new family of transcriptional modulators. Cited2 null embryos exhibit hyaloid hypercellularity consisting of aberrant vasculature in the eye. The purpose of the study is to address whether abnormal lenticular development is a primary defect of Cited2 deletion and whether deletion of hypoxia inducible factor (HIF)-1alpha or an HIF-1alpha target gene, vascular endothelial growth factor (VEGF), could rescue abnormal hyaloid vascular system (HVS) in Cited2 deficient adult eyes. METHODS: Le-Cre specific Cited2 knockout (Cited2(CKO)) mice with or without deletion of HIF-1alpha or VEGF were generated by standard Cre-Lox methods. Eyes collected from six-eight weeks old mice were characterized by Real Time PCR and immunohistological staining. RESULTS: Cited2(CKO) mice had smaller lenses, abnormal lens stalk formation, and failed regression of the HVS in the adult eye. The eye phenotype had features similar to persistent hyperplastic primary vitreous (PHPV), a human congenital eye disorder leading to abnormal lenticular development. Deletion of HIF-1alpha or VEGF in Cited2 knockout eyes partially rescued the abnormal HVS but had no effect on the smaller lens and abnormal lens stalk differentiation. Intravitreal injection of Topotecan (TPT), a compound that inhibits HIF-1alpha expression, partially eliminated HVS defects in Cited2(CKO) lenses. CONCLUSIONS: Abnormal HVS is a primary defect in Cited2 knockout mice, resulting in part from dysregulated functions of HIF-1 and VEGF. The Cited2(CKO) mouse line could be used as a novel disease model for PHPV and as an in vivo model for testing potential HIF-1 inhibitors.  (+info)

Persistent Hyperplastic Primary Vitreous (PHPV) is a rare congenital eye condition that occurs during fetal development. It is characterized by the failure of the primary vitreous, a gel-like substance in the eye, to completely regress or disappear. Instead, the primary vitreous persists and undergoes hyperplasia, leading to the formation of abnormal tissue within the eye.

In PHPV, this persistent tissue can cause various problems, including a small pupil, a cloudy area in the center of the lens (cataract), a white mass behind the lens, and abnormal blood vessels growing from the retina towards the center of the eye. These abnormalities can lead to visual impairment or even blindness, depending on the severity of the condition.

PHPV is typically diagnosed during infancy or early childhood, through a comprehensive eye examination that includes a detailed view of the internal structures of the eye using a specialized lens (slit lamp) and other diagnostic tests. Treatment options may include surgery to remove the abnormal tissue and improve vision, but the success of treatment depends on the extent and location of the PHPV.

Eye abnormalities refer to any structural or functional anomalies that affect the eye or its surrounding tissues. These abnormalities can be present at birth (congenital) or acquired later in life due to various factors such as injury, disease, or aging. Some examples of eye abnormalities include:

1. Strabismus: Also known as crossed eyes, strabismus is a condition where the eyes are misaligned and point in different directions.
2. Nystagmus: This is an involuntary movement of the eyes that can be horizontal, vertical, or rotatory.
3. Cataracts: A cataract is a clouding of the lens inside the eye that can cause vision loss.
4. Glaucoma: This is a group of eye conditions that damage the optic nerve and can lead to vision loss.
5. Retinal disorders: These include conditions such as retinal detachment, macular degeneration, and diabetic retinopathy.
6. Corneal abnormalities: These include conditions such as keratoconus, corneal ulcers, and Fuchs' dystrophy.
7. Orbital abnormalities: These include conditions such as orbital tumors, thyroid eye disease, and Graves' ophthalmopathy.
8. Ptosis: This is a condition where the upper eyelid droops over the eye.
9. Color blindness: A condition where a person has difficulty distinguishing between certain colors.
10. Microphthalmia: A condition where one or both eyes are abnormally small.

These are just a few examples of eye abnormalities, and there are many others that can affect the eye and its functioning. If you suspect that you have an eye abnormality, it is important to consult with an ophthalmologist for proper diagnosis and treatment.

The vitreous body, also known simply as the vitreous, is the clear, gel-like substance that fills the space between the lens and the retina in the eye. It is composed mainly of water, but also contains collagen fibers, hyaluronic acid, and other proteins. The vitreous helps to maintain the shape of the eye and provides a transparent medium for light to pass through to reach the retina. With age, the vitreous can become more liquefied and may eventually separate from the retina, leading to symptoms such as floaters or flashes of light.

A posterior capsulotomy is a surgical procedure that involves making an opening in the back part (posterior) of the lens capsule, which is a thin, clear membrane that holds the lens in place inside the eye. This procedure is typically performed to treat after-cataract, also known as posterior capsular opacification (PCO), which can cause vision loss or disturbance after cataract surgery. During cataract surgery, the cloudy natural lens of the eye is removed and replaced with an artificial intraocular lens (IOL). However, sometimes the back part of the lens capsule may become hazy or opaque over time, leading to visual symptoms similar to those experienced before cataract surgery.

In a posterior capsulotomy, a laser (usually a YAG laser) is used to create an opening in the cloudy posterior capsule, allowing light to pass through and restoring clear vision. The procedure is typically quick, painless, and performed as an outpatient procedure in a doctor's office or clinic. Patients may experience some side effects such as floaters, glare, or flashes of light after the procedure, but these usually resolve within a few days or weeks.

Hyperplasia is a medical term that refers to an abnormal increase in the number of cells in an organ or tissue, leading to an enlargement of the affected area. It's a response to various stimuli such as hormones, chronic irritation, or inflammation. Hyperplasia can be physiological, like the growth of breast tissue during pregnancy, or pathological, like in the case of benign or malignant tumors. The process is generally reversible if the stimulus is removed. It's important to note that hyperplasia itself is not cancerous, but some forms of hyperplasia can increase the risk of developing cancer over time.

The crystalline lens is a biconvex transparent structure in the eye that helps to refract (bend) light rays and focus them onto the retina. It is located behind the iris and pupil and is suspended by small fibers called zonules that connect it to the ciliary body. The lens can change its shape to accommodate and focus on objects at different distances, a process known as accommodation. With age, the lens may become cloudy or opaque, leading to cataracts.

A cataract is a clouding of the natural lens in the eye that affects vision. This clouding can cause vision to become blurry, faded, or dim, making it difficult to see clearly. Cataracts are a common age-related condition, but they can also be caused by injury, disease, or medication use. In most cases, cataracts develop gradually over time and can be treated with surgery to remove the cloudy lens and replace it with an artificial one.

Eye Wiki: Persistent Hyperplastic Primary Vitreous GeneReviews/NIH/NCBI/UW entry on NDP-Related Retinopathies Algernon B. "AB" ... Young J. "Persistent hyperplastic primary vitreous". Archived from the original on 2017-12-12. Retrieved 2009-05-11. Payabvash ... "Persistent hyperplastic primary vitreous - EyeWiki". eyewiki.aao.org. Retrieved 2023-01-03. Apushkin MA, Apushkin MA, Shapiro ... Hunt A, Rowe N, Lam A, Martin F (July 2005). "Outcomes in persistent hyperplastic primary vitreous". The British Journal of ...
... and persistent hyperplastic primary vitreous: role of MR imaging and CT. Radiol Clin North Am 1998; 36(6): 1119- ... vitreous and subretinal hemorrhage may often be observed. On CT, the globe appears hyperdense compared to normal vitreous due ... Persistent color patterns may also be perceived in the affected eye. Initially, these may be mistaken for psychological ... Mild to moderate linear enhancement may be seen between the exudate and the remaining vitreous. The exudate shows a large peak ...
"Case of chromosome 6p25 terminal deletion associated with Axenfeld-Rieger syndrome and persistent hyperplastic primary vitreous ... Kleinmann, R. E., Kazarian, E. L., Raptopoulos, V., & Braverman, L. E. (1981). Primary Empty Sella and Rieger's Anomaly of the ... atrial septal defects or persistent truncus arteriosus. Other alterations described are craniofacial anomalies associated with ...
... for Cataract Secondary To Persistent Hyperplastic Tunica Vasculosa Lentis And Persistent Hyperplastic Primary Vitreous in a ... The main causes of death were similar in both breeds; the primary causes were canine cancer and old age. However, the Pembroke ...
Drexel was born with persistent hyperplastic primary vitreous in her right eye, which required several surgeries and rendered ...
... (PHPV) is also referred to as persistent ... PHPV is a congenital anomaly of the eye that results following failure of embryological primary vitreous and hyaloid ... It is characterized by persistence of various portions of the primary vitreous (embryonic hyaloid vascular system) with ... It has previously been called persistent tunica vasculosa lentis, persistent posterior fetal fibrovascular sheath of the lens, ...
... (PHPV) is also referred to as persistent ... PHPV is a congenital anomaly of the eye that results following failure of embryological primary vitreous and hyaloid ... It is characterized by persistence of various portions of the primary vitreous (embryonic hyaloid vascular system) with ... It has previously been called persistent tunica vasculosa lentis, persistent posterior fetal fibrovascular sheath of the lens, ...
... caused by the failure of regression of the primary vitreous. It is divided into anterior and posterior types and is ... also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, ... Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital developmental ... Persistent hyperplastic primary vitreous: congenital malformation of the eye Barkur S Shastry. Clin Exp Ophthalmol. 2009 Dec. ...
Persistent hyperplastic primary vitreous (PHPV) is a congenital eye disease of children, because its clinical manifestations ... manifestations and pathological characteristics of retrolental membranes secondary to persistent hyperplastic primary vitreous ... Child; Eye abnormalities/etiology; Eye diseases/congenital; Hyperplasia; Vitreous body/abnormalities; Retrolental membranes; ... and the white band through the vitreous in 2 eyes.Pathological examination showed that the retrolental membrane was mainly ...
Eye Wiki: Persistent Hyperplastic Primary Vitreous GeneReviews/NIH/NCBI/UW entry on NDP-Related Retinopathies Algernon B. "AB" ... Young J. "Persistent hyperplastic primary vitreous". Archived from the original on 2017-12-12. Retrieved 2009-05-11. Payabvash ... "Persistent hyperplastic primary vitreous - EyeWiki". eyewiki.aao.org. Retrieved 2023-01-03. Apushkin MA, Apushkin MA, Shapiro ... Hunt A, Rowe N, Lam A, Martin F (July 2005). "Outcomes in persistent hyperplastic primary vitreous". The British Journal of ...
Persistent hyperplastic primary vitreous. Post-scleral bucking surgery. Anterior lens subluxation (ectopia lentis) ... Trabeculectomy for acute primary angle closure. Ophthalmology. 2000 Jul. 107(7):1298-302. [QxMD MEDLINE Link]. [Full Text]. ... Liang YB, Wang NL, Rong SS, Thomas R. Initial Treatment for Primary Angle-Closure Glaucoma in China. J Glaucoma. 2015 Aug. 24 ( ... Peripheral anterior synechia reduce extent of angle widening after laser peripheral iridotomy in eyes with primary angle ...
Persistent primary hyperplastic vitreous. *Retinoblastoma. *Toxocara canis (infection caused by a parasite) ...
This entity does not include other childhood glaucomas outlined below in the listing of other primary developmental and ... Persistent hyperplastic primary vitreous. * Congenital pupillary-iris-lens membrane. * Topiramate therapy. * Ciliary body cysts ... Differential diagnoses of the primary developmental glaucomas. Primary congenital glaucoma. * Newborn primary congenital ... Genetics of primary glaucoma. Curr Opin Ophthalmol. 2011 Sep. 22(5):347-55. [QxMD MEDLINE Link]. ...
Persistent hyperplastic primary vitreous ( PHPV). *Developmental disorder in which there is failure of regression of primary ... Injection into the vitreous of melphalan (with or without topotecan) is useful in cases of vitreous seeding following treatment ... Useful in selected cases, and in vitreous seeds (free floating groups of tumor cells in the vitreous). ... Retinoblastoma is the most common primary cancer of the eye occurring in childhood, with a frequency of 1:14,000 to 1:20,000 ...
PHPV) Persistent hyperplastic primary vitreous. *(PLA) Pectinate ligament abnormality. Inherited conditions that develop later ... There are two types of inherited glaucoma, Primary Closed Angle Glaucoma (PCAG/PACG) and Primary Open Angle Glaucoma (POAG). ...
Persistent primary hyperplastic vitreous. *Retinoblastoma. *Toxocara canis (infection caused by a parasite) ... The fluids in the eye are divided by the lens into the vitreous humor (behind the lens) and the aqueous humor (in front of the ...
Coats disease and persistent hyperplastic primary vitreous. Role of MR imaging and CT.. Edward DP; Mafee MF; Garcia-Valenzuela ...
... persistent tunica vasculosa lentis and persistent hyperplastic primary vitreous (2 dogs; 4 eyes), and trauma-induced cataract ( ... bulging vitreous humor (1), lens subluxation (1), and extrusion of vitreous humor into the anterior chamber (1). ...
Persistent hyperplastic primary vitreous - The martini glass sign. Bezuidenhout, A Fourie.. · texto em Inglês · Inglês ( pdf ) ...
Persistent pupillary membranes Persistent hyperplastic primary vitreous / persistent hyperplastic tunica vasculosa lentis (PHPV ... Less commonly it moves posteriorly into the vitreous were it causes less problems. Primary lens luxation is a bilateral ... Management of primary lens luxation Surgical removal of anteriorly luxated lenses (intracapsular extraction) is indicated. The ... This may be a primary or secondary condition. Secondary lens subluxation is commonly associated with glaucoma (due to ...
Persistent Pupillary Membrane and Secondary Angle Closure Glaucoma: A Case Report ... Read about Persistent Pupillary Membrane and Secondary Angle Closure Glaucoma. ... An integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous. Am J Ophthalmol. ... 2005). Hyperplastic Persistent Pupillary Membrane Associated With Subacute Angle-Closure Glaucoma. Annals of Ophthalmology. 37 ...
The indications included congenital rubella cataract with microphthalmos, traumatic cataract, persistent anterior and posterior ... hyperplastic primary vitreous, postcataract pupillary membranes, and postcataract vitreous prolapse with … ... persistent anterior and posterior hyperplastic primary vitreous, postcataract pupillary membranes, and postcataract vitreous ...
NDP-related persistent fetal vasculature, NDP-related familial exudative vitreoretinopathy, NDP-related advanced retinopathy of ... NDP-Related Persistent Fetal Vasculature (PFV). Formerly referred to as persistent hyperplastic primary vitreous (PHPV), PFV is ... NDP-related persistent fetal vasculature (PFV). The PFV (formerly referred to as persistent hyperplastic primary vitreous [PHPV ... NDP-related persistent fetal vasculature (PFV; formerly referred to as persistent hyperplastic primary vitreous [PHPV]). ...
Bilateral persistent hyperplastic primary vitreous: an Egyptian family supporting a rare autosomal dominant inheritance. Galal ... Locus for autosomal recessive nonsyndromic persistent hyperplastic primary vitreous. Khaliq S, Hameed A, Ismail M, Anwar K, ... Bilateral persistent hyperplastic primary vitreous: an Egyptian family supporting a rare autosomal dominant inheritance. Genet ... The disorder known as persistent hyperplastic primary vitreous is generally not considered hereditary since it usually occurs ...
Persistent Fetal Vascular Syndrome is also known as Persistent Hyperplastic Primary Vitreous that can cause a traction retinal ... Persistent fetal vasculature / Persistent hyperplastic primary vitreous. *Persistent tunica vasculosa lentis. *Familial ...
PHPV - Persistent hyperplastic primary vitreous Current Synonym true false 458602016 Persistent hyperplastic primary vitreous ... Persistent hyperplastic primary vitreous (disorder). Code System Preferred Concept Name. Persistent hyperplastic primary ... Persistent hyperplastic primary vitreous (disorder) {314270008 , SNOMED-CT } Parent/Child (Relationship Type) ...
Persistent Hyperplastic Primary Vitreous Preferred Term Term UI T689801. Date01/24/2007. LexicalTag NON. ThesaurusID NLM (2008) ... Persistent Hyperplastic Primary Vitreous Preferred Concept UI. M0505780. Scope Note. A developmental ocular anomaly in which ... Persistent Hyperplastic Primary Vitreous. Tree Number(s). C11.250.616. C16.131.384.725. Unique ID. D054514. RDF Unique ... Persistent Hyaloid Artery Persistent Hyaloid Vasculature Previous Indexing. Infant, Newborn, Diseases (1966-2007). Vitreous ...
Persistent Hyperplastic Primary Vitreous Preferred Term Term UI T689801. Date01/24/2007. LexicalTag NON. ThesaurusID NLM (2008) ... Persistent Hyperplastic Primary Vitreous Preferred Concept UI. M0505780. Scope Note. A developmental ocular anomaly in which ... Persistent Hyperplastic Primary Vitreous. Tree Number(s). C11.250.616. C16.131.384.725. Unique ID. D054514. RDF Unique ... Persistent Hyaloid Artery Persistent Hyaloid Vasculature Previous Indexing. Infant, Newborn, Diseases (1966-2007). Vitreous ...
This entity does not include other childhood glaucomas outlined below in the listing of other primary developmental and ... Persistent hyperplastic primary vitreous. * Congenital pupillary-iris-lens membrane. * Topiramate therapy. * Ciliary body cysts ... Differential diagnoses of the primary developmental glaucomas. Primary congenital glaucoma. * Newborn primary congenital ... Genetics of primary glaucoma. Curr Opin Ophthalmol. 2011 Sep. 22(5):347-55. [QxMD MEDLINE Link]. ...
Persistent Hyperplastic Primary Vitreous Entry term(s). Persistent Fetal Vasculature Syndrome Persistent Hyaloid Arteries ... Persistent Hyperplastic Primary Vitreous - Preferred Concept UI. M0505780. Scope note. A developmental ocular anomaly in which ... Persistent Hyaloid Arteries. Persistent Hyaloid Artery. Persistent Hyaloid Vasculature. Persistent Hyaloid Vasculatures. ... A developmental ocular anomaly in which the primary VITREOUS BODY and its surrounding hyaloid vasculature failed to regress. It ...
Persistent Hyperplastic Primary Vitreous: This eye condition occurs prior to birth and is concurrent with Retinal Dysplasia ...
persistent hyperplastic primary vitreous DOID:0060282 * chromosome 2q32-q33 deletion syndrome DOID:0060428 ...
Persistent Hyperplastic Primary Vitreous. Glial remnants anterior to the optic disc, Hyaloid vascular remnant and retrolental ... Cataract, Macular scar, Vitreous haze, Epiretinal membrane, Vitreous floaters, Band keratopathy, .... ORPHA:279914. ... abnormal vitreous body morphology Tmem38bem1(IMPC)Mbp HET Early adult 6.50×10-05 ... Retinal detachment, Vitreous floaters, Optic atrophy, Macular edema, Retinal exudate, Tractional .... ORPHA:209943. ...

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