[2] Defects which arise from this lack of vascular regression are diverse; as a result, the presentation, symptoms, and prognosis of affected patients vary widely, ranging from clinical insignificance to irreversible blindness.
[4] Persistent fetal vasculature heightens the lifelong risk of glaucoma, cataracts, intraocular hemorrhages, and Retinal detachments, accounting for the visual loss of nearly 5% of the blind community in the developed world.
Additionally, the structural anomalies inherent to PFV do not grow with time, which may alter visual or cosmetic prospects in the long term, as the muscles surrounding the eye react and adjust to the impairment.
However, in bilateral cases of PFV with cardiovascular and central nervous system abnormalities, trisomy 13 (Patau syndrome) should be suspected and ruled out.
A differential diagnostic guide to conclusively determine PFV over disorders and anomalies of similar presentations was compiled by Dr. Olga Cerón, et al. & International Ophthalmology Clinics, pp 53–62, Spring 2008:The differential diagnosis of PHPV includes congenital cataract, retinopathy of prematurity, Norrie disease, familial exudative vitreoretinopathy and retinoblastoma.
PHPV can be distinguished from an uncomplicated congenital cataract by the presence of a fibrovascular stalk and other features such as elongated ciliary processes and a shallow anterior chamber.
The primary goal is visual preservation and the protection of healthy eye tissue, with cosmetic concerns as a secondary, and often long term, consideration.
When diagnosed early, surgical intervention is statistically correlated with a higher probability of viable visual preservation in the impacted eye.
Posterior PFV is usually associated with a poor visual outcome regardless of intervention due to retinal and optic nerve abnormalities, though individualized surgical options are noting greater success since the 1990s.
[28] Despite this, surgery is increasingly preferred as a treatment to both presentations, as it mitigates a number of chronic risks, and may help maintain at least subpar visual capacity.
[29] In patients with a unilateral disease, “patching” is commonly prescribed as a post-operative recovery tool to maximize neurological plasticity in the ocular nerve.
[30] Surgical practices since the late 20th century have advanced considerably, increasingly allowing for the preferable preservation of functionally sightless eyes as a bulwark against chronic disease.
[32] While individual outcomes are as diverse as PFV itself, those affected by the disease often maintain outwardly normal lives, and on account of its early presentation, many fully adapt to impairment, having not known any other form of vision.
Damage to depth perception and reaction speed may impair certain capabilities, though in unilateral expressions especially, PFV will not necessarily prevent patients from driving safely, engaging in athletics, and developing advanced fine motor skills.
Bilateral cases experience less adaptable visual outcomes, and additional consultation may be necessary to determine if genetic components of the disease are inheritable.