Features
Persistent fetal vasculature (PFV) occurs when the fetal vasculature of the eye fails to undergo the normal involution process. The cause of this incomplete regression of the fetal vasculature remains unknown. Formerly referred to as persistent hyperplastic primary vitreous (PHPV), PFV has become the preferred nomenclature as it includes all components of the fetal intraocular vasculature. During fetal development, the hyaloid artery and other blood vessels proliferate from the posterior to the anterior pole of the eye. They form a network of vessels that extend to cover the anterior and posterior surface of the lens (i.e., anterior and posterior tunica vasculosa lentis) and the iris (i.e., pupillary membranes), providing nutrition to the intraocular structures in the setting of an immature anterior chamber and absent aqueous humor. Persistence of these structures ex utero may result in several ocular sequelae, including microphthalmos, cataract, secondary angle-closure glaucoma, tractional retinal detachment, and vitreous hemorrhage. PFV is mostly idiopathic, unilateral, and have no other systemic associations; when bilateral, however, it is often associated with systemic syndromes such as Trisomy 13, Walker-Warburg syndrome, anencephaly, Norrie disease, oculo-dento osseous disease, and oculopalatal cerebral dwarfism.
102.1.1 Common Symptoms
Decreased vision may be present.
102.1.2 Exam Findings
Ocular findings associated with PFV include morning glory anomaly, Peter anomaly, macular hypoplasia, microcornea, and microphthalmos. On presentation, the patient may have a predominant anterior or posterior involvement. The majority, however, have a combination of both anterior and posterior features. While there is likely some overlap in terminology between anterior and posterior PFV, the features of each are listed below.
Anterior PFV
Persistent pupillary membranes: Filamentary remnants of the anterior tunica vasculosa lentis; may rarely cause complications including spontaneous hyphema or corneal clouding when attached to the endothelium.
Retrolental opacification: Due to persistent posterior tunica vasculosa lentis; may be centrally located and small or may extend to the ciliary processes.
Cataract: A common feature associated with retrolental opacification.
Elongated ciliary processes: Can occasionally be seen behind the lens and extending into the retrolental opacity.
Presence of iridohyaloid vessels: Radially oriented blood vessels in the iris stroma act as a connection between the posterior and anterior tunica vasculosa lentis; when persistent can lead to notching in the pupillary sphincter or frank entropion or ectropion uveae.
Shallow anterior chamber angle: May be secondary to an intumescent, cataractous lens or rotation of the ciliary body and lens.
Posterior PFV
Persistent hyaloid artery: The most common remnant of the hyaloid artery is observed as an indistinct vessel in Cloquet’s canal that may extend from the optic nerve to the posterior lens surface (▶ Fig. 102.1, ▶ Fig. 102.2); commonly referred to as the “stalk” in PFV that is visualized on ultrasound. The hyaloid artery may have persistent perfusion, which can be visualized on fluorescein angiography.
Congenital retina nonattachment: Strong adherence of the fetal vasculature to the retinal may induce a tractional retinal detachment; there may also be elevated vitreous membranes emanating from the optic nerve; retina may be drawn up into the stalk in more severe cases.
Retinal folds: May be due to persistent traction from the stalk.
Retinal dysplasia: May manifest as disruption of the retinal laminations or “microscopic” dysplasia with grossly normal macular anatomy; both may limit vision despite adequate surgical and amblyopia therapy.
Macular abnormalities: Typically results from traction from the vascular stalk and may include macular schisis, cystoid spaces, and an absent foveal pit. In severe cases, there may be a macular fold.
Optic nerve head hypoplasia or dysplasia: Both will inevitably limit long-term visual prognosis.
Fig. 102.1 Fundus photograph of a persistent hyaloid artery or “stalk” extending from the optic nerve to the posterior surface of the lens.
(Image courtesy of Maxwell Stem and Michael T. Trese.)
Fig. 102.2 Anterior segment intraoperative image demonstrating a persistent hyaloid artery attaching slightly nasally on the posterior surface of the lens.
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