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David J. Jacobs, Brett P. Bielory, Audina M. Berrocal; Peripheral Retinal Avascularity in Persistent Fetal Vasculature. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4854.
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Persistent Fetal Vasculature (PFV) or Persistent Hyperplastic Primary Vitreous is a rare congenital malformation of the eye due to the persistence of variable components of the primary vitreous. Characteristic findings include lens opacification, retrolenticular membrane, centrally dragged ciliary processes, fibrovascular stalk, intraocular hemorrhage, microphthalmia, and glaucoma. The vast majority of cases are unilateral and sporadic. No causative gene has yet been demonstrated. PFV occurs in fully developed infants and is not associated with retinopathy of prematurity or familial exudative vitreoretinopathy. Peripheral retinal avascularity is not a defining characteristic of PFV. We report 3 cases of unilateral PFV with bilateral peripheral retinal avascularity demonstrated by fluorescein angiography.
Examination under anesthesia with fundus photography and fluorescein angiography were performed on 3 patients with PFV. The peripheral retinas were evaluated for avascularity.
2 patients were male and 1 was female. Ages ranged from 4 months to 5 years old. None of the patients had a history of prematurity. Each of the 3 patients initially presented with characteristic findings of unilateral PFV including lens opacification, retrolenticular membrane, centrally dragged ciliary processes, and fibrovascular stalk. The right eye was the affected eye in each case. Fundus examination and fluorescein angiography demonstrated peripheral retinal avascularity bilaterally in each case.
Unilateral PFV can present with peripheral retinal avascularity of both the affected and unaffected eyes.
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