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Persistent fetal vasculature

Congenital Malformations and Chromosomal Abnormalities

Persistent fetal vasculature, abbreviated PFV and also called persistent fetal vasculature syndrome, is a rare congenital anomaly of the eye. It arises when the hyaloid vasculature, the fetal blood vessels inside the developing eye, fails to regress in the normal way before birth. Until 1997 the condition was known mainly as persistent hyperplastic primary vitreous. The defects that follow are diverse, so presentation, symptoms and outlook vary widely, from clinical insignificance to irreversible blindness. It is generally classified as purely anterior, purely posterior or a mix of both, and the combined form is by far the most common. Most cases affect only one eye, and most do not need further intervention, but when symptoms appear they can be severe. The condition raises the lifelong risk of glaucoma, cataract, bleeding within the eye and retinal detachment.

Facts
Classification
Body System
Eye 1
ICD-10 Chapter
XVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 1
Communicable or Non-communicable
Non-Communicable 1
Sources
1. Persistent fetal vasculature (Wikipedia)
  • Lead, paragraph 1
    is a rare congenital anomaly which occurs when blood vessels called the hyaloid vasculature fail to regress
  • Lead, paragraph 1 [body-system]
    fail to regress as normally in-utero after the eye is fully developed
  • Lead: disease category
    Persistent fetal vasculature (PFV), also known as persistent fetal vasculature syndrome (PFVS), and until 1997 known primarily as persistent hyperplastic primary vitreous (PHPV), is a rare congenital anomaly which occurs when blood vessels called the hyaloid vasculature fail to regress as normally in-utero after the eye is fully developed.
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