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People also ask
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What is the Ellsworth classification system?
The Reese-Ellsworth classification system for intraocular tumors is the traditional method for stratifying the intraocular extent of retinoblastoma. The system takes into account the number, size, and location of tumors and the presence or absence of vitreous seeding.
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How rare is retinoblastoma?
Frequency. Retinoblastoma is diagnosed in 250 to 350 children per year in the United States. It accounts for about 4 percent of all cancers in children younger than 15 years.
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What is a PHPV eye condition?
Persistent hyperplastic primary vitreous (PHPV) is a common congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress.
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What is the disease PFV?
Persistent fetal vasculature (PFV), previously known as persistent hyperplastic primary vitreous, is a developmental malformation of the eyes that is caused by a failure of the hyaloid vasculature to regress in utero.
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What is the Reese Ellsworth classification system?
The Reese-Ellsworth staging system divides intraocular retinoblastoma into 5 groups. The higher the group number, from 1 to 5, the lower the chance of controlling the retinoblastoma or of saving the eye or any useful vision.
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What is Group E retinoblastoma?
Intraocular retinoblastomas that are classified as group E have damaged the eye extensively. There is almost no chance of saving the affected eye(s). Large tumor(s) extending toward the front of the eye. Neovascular glaucoma. Glaucoma is damage caused by pressure within the eye.
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What is PFV in medical terms?
Persistent fetal vasculature (PFV) is a human disease in which the fetal vasculature of the eye fails to regress normally. The fetal, or hyaloid, vasculature nourishes the lens and retina during ocular development, subsequently regressing after formation of the retinal vessels.
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What percentage of retinoblastoma patients have calcifications?
Histology reports show that retinoblastomas calcify in ≤95% of cases5; CT has a designated sensitivity in detecting calcifications in retinoblastoma of 81%–96%,6–10 whereas the sensitivity of sonography has been reported to be ≤92.5% of cases.
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What is the difference between PFV and retinoblastoma?
Retinal detachment in PFV is more often tractional and tent shaped whereas retinoblastoma retinal detachments are more likely exudative with subretinal fluid and possible seeds. If present, these features can help differentiate PFV from retinoblastoma.
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What syndrome is PHPV associated with?
Although traditionally a sporadic, unilateral, and isolated finding, PHPV also has been reported in trisomy 13, Norrie disease, Warburg syndrome, incontinentia pigmenti, cerebro-oculo-dysplasia-muscular dystrophy,1 and fetal alcohol syndrome.
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What is the difference between PFV and retinoblastoma?
Retinal detachment in PFV is more often tractional and tent shaped whereas retinoblastoma retinal detachments are more likely exudative with subretinal fluid and possible seeds. If present, these features can help differentiate PFV from retinoblastoma.
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