Symptoma

Persistent Hyperplastic Primary Vitreous

PHPV

A combination of anterior and posterior presentations is the most commonly seen clinical presentation. Case report. [ncbi.nlm.nih.gov]

  • Turkish

    The authors present a Turkish family (two generations, five affected persons) with symptoms of Rieger's eye anomaly as well as Persistent Hyperplastic Primary Vitreous (PHPV). [ncbi.nlm.nih.gov]

  • Band-Shaped Keratopathy

    Diffuse corneal haze and band shaped keratopathy were present and therefore anterior chamber details could not be made out. There was no fundal glow. Extraocular movements were grossly restricted in all quadrants. [ncbi.nlm.nih.gov]

  • Conjunctival Hyperemia

    (c) Inflammatory membrane in anterior chamber with conjunctival hyperemia after subsidence of cellulitis. [ncbi.nlm.nih.gov]

  • Scotoma

    Various ocular side effects have been described in women taking the drug, including decreased vision, mydriasis, flashing lights, central scotoma, photophobia, diplopia, allergic reactions, retinal vasospasms, detachment posterior vitreous, and possibly [ncbi.nlm.nih.gov]

  • Central Scotoma

    Various ocular side effects have been described in women taking the drug, including decreased vision, mydriasis, flashing lights, central scotoma, photophobia, diplopia, allergic reactions, retinal vasospasms, detachment posterior vitreous, and possibly [ncbi.nlm.nih.gov]

  • Short Arm

    Cytogenetic analysis by high resolution GTG banding showed extra chromosomal material on the short arm of one chromosome 1 of the patient, but neither parent. [ncbi.nlm.nih.gov]

  • Suggestibility

    Although there are two previous reports of PHPV in siblings suggesting autosomal recessive inheritance, this is the first report compatible with autosomal dominant inheritance. [ncbi.nlm.nih.gov]

  • Spastic Quadriplegia

    quadriplegia, microcephaly and deafness). [ncbi.nlm.nih.gov]

  • Quadriplegia

    RESULTS: Neurologic abnormalities were identified in three of the children with unilateral PHPV (hemiparesis, impaired coordination and homonymous hemianopia) and seven of the children with bilateral PHPV (ataxia, impaired coordination, hypotonia, spastic quadriplegia [ncbi.nlm.nih.gov]

  • Meningism

    Paediatric neurology opinion was sought for signs of meningeal irritation which was labeled as non-specific and secondary to orbital inflammation. [ncbi.nlm.nih.gov]

Workups were done and prolonged partial thromboplastin time with normal platelet count, normal bleeding time, and prothrombin time were detected. [ncbi.nlm.nih.gov]

  • Central Scotoma

    Various ocular side effects have been described in women taking the drug, including decreased vision, mydriasis, flashing lights, central scotoma, photophobia, diplopia, allergic reactions, retinal vasospasms, detachment posterior vitreous, and possibly [ncbi.nlm.nih.gov]

Surgical treatment in comparable cases is encouraged. [ncbi.nlm.nih.gov]

Glaucoma, retinal or optic nerve pathology, surgery later than three months of age or combinations of these factors were associated with poor prognosis. Isolated microphthalmos was not correlated with poor prognosis. [ncbi.nlm.nih.gov]

Abstract The diagnosis of PHPV is often made difficult by its extremely broad array of clinical manifestations, its etiologic heterogeneity, and frequently opaque ocular media. [ncbi.nlm.nih.gov]

Etiology: Described by Reese in 1949, PHPV is thought to be congenital. As the ectoderm of the lens plate and neuroectoderm of the optic vesicle separate, vasoformative mesenchymal tissue normally grows in to the space. [missionforvisionusa.org]

The exact etiology of PHPV remains unknown, but it characteristically presents as unilateral leukocoria. PHPV is divided into subtypes: anterior, posterior and combined. [healio.com]

Goldberg suggested replacing the term PHPV with the more comprehensive PFV, which emphasizes the etiology of the disorder [4]. [link.springer.com]

Epidemiology, onset & clinical features Epidemiology Though the exact prevalence remains unknown, PHPV is considered to be not a very rare disease. [institut-vision.org]

EPIDEMIOLOGY AND PATHOGENESIS PFV appears sporadically in the vast majority of cases. [medtextfree.wordpress.com]

Pathophysiology The fetal vasculature, both the tunica vasculosa and hyaloid systems, are presumed to nourish the fetus’s developing lens prior to the production of aqueous humor and formation of the anterior chamber. [eyewiki.org]

The fundus was fully visible in 5 cases while in 1 eye remaining and new proliferations prevented visualization of the fundus. Postoperative visual acuity varied from light perception to 0.2 and the intraocular pressure was within normal limits. [ncbi.nlm.nih.gov]

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