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Persistent Hyperplastic Primary Vitreous
PHPV
Presentation
- A combination of anterior and posterior presentations is the most commonly seen clinical presentation. Case report.[ncbi.nlm.nih.gov]
Entire Body System
Weakness
- The strengths and weaknesses of each of these diagnostic methods is detailed in regard to patients who present with unilateral leucocoria, microphthalmos, lens opacity, and retinal detachment.[ncbi.nlm.nih.gov]
Asymptomatic
- During follow-up, the patient was asymptomatic except for a single episode of hyphema at about one year after the initial presentation. On repeat MRI, right globe appeared small in size compared to fellow eye.[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]
Eyes
Blurred Vision
- The symptoms are leukocoria, strabismus, nystagmus and blurred vision, blindness.[en.wikipedia.org]
- This leads to blurred vision. PHPV can lead to other eye problems developing The scarred vitreous is often stuck to the back of the lens and areas of the retina.[ssc.education.ed.ac.uk]
Chemosis
- The child had severe lid edema, erythema and tenderness along with chemosis and congestion of conjunctiva. There was no purulent discharge or any evidence of keratitis and corneal ulcer.[ncbi.nlm.nih.gov]
Conjunctival Hyperemia
- (c) Inflammatory membrane in anterior chamber with conjunctival hyperemia after subsidence of cellulitis.[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]
Red Eye
- These include: White pupil (leucocoria) Squint (when both eyes don't appear to point in the same direction) Painful red eye Blurred vision Nystagmus (fast to and fro movements of the eyes) The most common way PHPV presents is when the normally black pupil[ssc.education.ed.ac.uk]
Musculoskeletal
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]
Osteoporosis
- We had the opportunity to investigate the early abnormalities of the eyes in a family with osteoporosis-pseudoglioma syndrome. This syndrome combines severe premature osteoporosis with a bilateral eye disorder, leading to early onset blindness.[ncbi.nlm.nih.gov]
Neurologic
Headache
- Patient also had complaint of headache with signs of meningeal irritation. Ultrasonography of right eye showed disorganized intraocular structure with no definite mass lesion.[ncbi.nlm.nih.gov]
Irritability
- 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]
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]
Spastic Quadriplegia
- quadriplegia, microcephaly and deafness).[ncbi.nlm.nih.gov]
Ataxia
- 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[ncbi.nlm.nih.gov]
Workup
- Workups were done and prolonged partial thromboplastin time with normal platelet count, normal bleeding time, and prothrombin time were detected.[ncbi.nlm.nih.gov]
Treatment
- Surgical treatment in comparable cases is encouraged.[ncbi.nlm.nih.gov]
Prognosis
- 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]
Etiology
- 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
- 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]
Sex distribution
Age distribution
Pathophysiology
- 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]
Prevention
- 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]