Summary: The primary pathological process in FEVR is believed to be a premature arrest of retinal angiogenesis/vasculogenesis or retinal vascular differentiation, leading to incomplete vascularization of the peripheral retina.
Incomplete normal retinal angiogenesis is the hallmark of a group of retinal vascular diseases including familial exudative vitreoretinopathy (FEVR).
The primary pathological process in FEVR is believed to be a premature arrest of retinal angiogenesis/vasculogenesis or retinal vascular differentiation, leading to incomplete vascularization of the peripheral retina (van Nouhuys 1991). This failure to vascularize the peripheral retina is the unifying feature seen in all affected individuals, but by itself usually causes no clinical symptoms.
The visual problems in FEVR result from secondary complications due to the development of hyperpermeable blood vessels, neovascularization and vitreo-retinal traction. These features cause a reduction in visual acuity and in 20% of cases can lead to vitreous hemorrhage and partial or total retinal detachment.
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