Falciform fold of detached dysplastic retina encircles the persistent hyaloid artery that extends from the optic nerve head to the retrolental mass.
Specialty
Ophthalmology
Symptoms
Defects including glaucoma, cataract, falciform retinal folds, funnel- or stalk-shaped retinal detachment, spontaneous fundus hemorrhage, and a congenitally small eye.
Complications
Retinal detachment, blindness, and glaucoma, among other chronic diseases.
Usual onset
Neonatal, most often within 1–2 weeks of birth.
Duration
Permanent.
Types
Unilateral and bilateral presentations of anterior, posterior, and combination types are possible.
Causes
Unknown; genetic factors prevalent in bilateral cases.
Risk factors
Premature birth.
Diagnostic method
Advanced medical imaging required, symptoms of concern include strabismus, nystagmus, and amblyopia.
Prevention
None.
Treatment
Surgical intervention to preserve affected eye(s). Extensive monitoring advised.
Prognosis
Varies; even if treated early, visual outcomes are often poor, though will not generally deteriorate later in life.
Frequency
Unknown.
Persistent fetal vasculature(PFV), also known as persistent fetal vasculature syndrome (PFVS), and until 1997 known primarily as persistent hyperplastic primary vitreous (PHPV),[1] is a rare congenital anomaly which occurs when blood vessels within the developing eye, known as the embryonic hyaloid vasculature network, fail to regress as they normally would in-utero after the eye is fully developed.[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. The underlying structural causes of PFV are considered to be relatively common, and the vast majority of cases do not warrant additional intervention.[3] When symptoms do manifest, however, they are often significant, causing detrimental and irreversible visual impairment.[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.[5] In diagnosed cases of PFV, approximately 90% of patients with a unilateral disease have associated poor vision in the affected eye.[6]
The presentation of persistent fetal vasculature is generally classified into three forms: purely anterior, purely posterior, or a mix of both.[7] Combined expressions of both classifications are by far the most common presentation, accounting for up to 62% of all cases.[8] Purely posterior presentations are often considered to be the most extreme variant and have the least successful surgical and visual outcomes.[9] The majority of PFV cases are unilateral, affecting only one eye, though roughly 2.4% to 11% of cases are bilateral, impacting both eyes.[8] Bilateral cases have generally poor visual outcomes and warrant heightened intervention regardless of presentation. They also may follow an autosomal recessive or autosomal dominant inheritance pattern, while no inheritance patterns have been conclusively identified for unilateral cases.[10]
^"Persistent Fetal Vasculature (PFV)", Encyclopedia of Ophthalmology, Berlin, Heidelberg: Springer Berlin Heidelberg, 2018, p. 1354, doi:10.1007/978-3-540-69000-9_101340, ISBN 978-3-540-68292-9
^Drack A (2005-08-11). "Book review pediatric retina: medical and surgical approaches edited by Mary Elizabeth Hartnett, Michael Trese, Antonio Capone, Jr., Bronya J.B. Keats, and Scott M. Steidl. 559 pp., illustrated. Philadelphia, Lippincott Williams & Wilkins, 2005. $249. 0-7817-4782-1". New England Journal of Medicine. 353 (6): 636–637. doi:10.1056/nejm200508113530623. ISSN 0028-4793.
^Nelson, L. B. (2021). "Color atlas & synopsis of Clinical Ophthalmology. Pediatric Ophthalmology". Детская офтальмология. Цветной атлас и краткое руководство по клинической офтальмологии (in Russian). GEOTAR-Media Publishing Group. pp. 1–304. doi:10.33029/9704-6145-7-oft-2021-1-304. ISBN 978-5-9704-6145-7. S2CID 241019692.
^Hunt A, Rowe N, Lam A, Martin F (July 2005). "Outcomes in persistent hyperplastic primary vitreous". The British Journal of Ophthalmology. 89 (7): 859–863. doi:10.1136/bjo.2004.053595. PMC 1772745. PMID 15965167.
^Taharaguchi S, Yoshida K, Tomioka Y, Yoshino S, Uede T, Ono E (May 2005). "Persistent hyperplastic primary vitreous in transgenic mice expressing IE180 of the pseudorabies virus". Investigative Ophthalmology & Visual Science. 46 (5): 1551–1556. doi:10.1167/iovs.04-0743. PMID 15851549.
^Jain TP (2009). "Bilateral persistent hyperplastic primary vitreous". Indian Journal of Ophthalmology. 57 (1): 53–54. doi:10.4103/0301-4738.44487. PMC 2661510. PMID 19075412.
^Silbert M, Gurwood AS (December 2000). "Persistent hyperplastic primary vitreous". Clinical Eye and Vision Care. 12 (3–4): 131–137. doi:10.1016/s0953-4431(00)00054-0. PMID 11137427.
^ abPollard ZF (1997). "Treatment of persistent hyperplastic primary vitreous". Journal of Pediatric Ophthalmology and Strabismus. 22 (5): 180–183. doi:10.3928/0191-3913-19850901-05. PMID 4045646.
Persistentfetalvasculature (PFV), also known as persistentfetalvasculature syndrome (PFVS), and until 1997 known primarily as persistent hyperplastic...
also disrupts the retinal vascularization in full-term infants. Persistentfetalvasculature that can cause a traction retinal detachment difficult to differentiate...
Chlamydia trachomatis or Moraxella spp. can cause a nonexudative but persistent conjunctivitis without much redness. Bacterial conjunctivitis may cause...
are started. Medical professionals will sometimes lance a particularly persistent or irritating stye with a needle to accelerate its draining. Surgery is...
itching. Histamine also binds to H1 and H2 receptors of the conjunctival vasculature and causes vasodilatation. Mast cell-derived cytokines such as chemokine...
transient or persistent. Inadequate diagnosis and treatment at improper times and tethering or fibrosis of muscles may lead to persistent diplopia. Binocular...
ganglion cells of the optic disc become engorged and bulge anteriorly. Persistent and extensive optic nerve head swelling, or optic disc edema, can lead...
coming from an atherosclerotic carotid artery, any emboli arising from vasculature preceding the retinal artery, ophthalmic artery, or ciliary arteries...
Global Information