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The vasa hyaloidea propria, a component of the fetal hyaloidal vasculature, is characterized by multiple persistent fetal vasculatures branching into the vitreous. We present a 4-month-old girl with stage 4 familial exudative vitreoretinopathy, with multiple ectopic retinal vessels extending into the vitreous, confirmed with fluorescein angiography, which was consistent with persistent vasa hyaloidea propia/retinae making contact with the retina. The patient underwent vitreoretinal surgery to address the retinal detachment, during which the patent stalks of the persistent vasa hyaloidea propia/retinae were transected.

An 8-week-old boy referred for an abnormal pupil was found to have an atypical presentation of persistent fetal vasculature (PFV) with multiple vascular stalks. Examination under anesthesia with fluorescein angiography (FA) revealed 2 perfused persistent hyaloid vessels, one extending from the optic disc and another from the inferonasal retina. These vessels meet anteriorly to form a vascular network at the lens, which is the remnant of the tunica vasculosa lentis. Although the posterior portion of PFV typically presents as a single stalk attached at the optic disc, this case features an atypical presentation of 2 distinct vascular stalks, which may expand our understanding of ocular development and pathogenesis of PFV. We hypothesize that the aberrant additional stalk may represent failure of the vasa hyaloidea propria (tributaries of the hyaloid artery) to regress.

The hyaloid vasculature is a complex of transient intraocular vessels consisting of the main hyaloid artery, vasa hyaloidea propria , tunica vasculosa lentis , and the pupillary membrane . Its proper development and regression are instrumental in the healthy maturation of the eye. Failure of the hyaloid vasculature to develop and regress in a normal manner is associated with various presentations of persistent fetal vasculature (PFV). The abnormalities seen in PFV can range from causing no obvious physiological irregularities to markedly limiting the visual function of the eye. Recent advancements in surgical techniques have led to advancements in visual potential; however, some cases are still considered poor surgical candidates for visual development. Much has been learned recently regarding the complex biochemical interactions involved in physiological development and regression of the hyaloid vascular system; however, our understanding of all of these interactions remains incomplete.

We describe a simple proteomic approach to investigate the differential protein expression patterns and identify the physiologically relevant angiogenic and anti-angiogenic factors involved in the hyaloid vascular system regression. Differentially-expressed proteins were identified using two-dimensional gel electrophoresis followed by nano-flow chromatography coupled to tandem mass spectrometry. These proteins are expected to provide insight as to their function in the early maintenance and eventual regression of the hyaloid vascular system.

REPORT OF CASE A case in which cystlike remains of the vasa hyaloidea propria were observed is reported as follows: R. L., a boy 13 years of age, was referred for examination because of convergent concomitant squint of the left eye of 20 degrees. Vision of the right eye was 20/20, and that of the left eye was 20/100 and was not improved. The pupils, the tension and the fundi were normal. With direct illumination, there was seen to be present on the posterior pole of the left lens a white opacity resembling a posterior polar cataract. With the slit lamp the detailed examination of this opacity revealed: In the retrolental space and attached by a strand of pigment slightly nasal to the posterior pole of the left lens was a globular, semitransparent cyst, about 1 by 0.5 mm. in diameter.

The part played by undue persistence of portions of the fetal vascular system of the eye has long been recognized in considering the factors involved in the production of congenital anomalies. Indeed, one of the most striking phenomena in the whole developmental history of the mammalian eye is the elaborate formation of a complicated transient vascular system which disappears in adult life. These vessels are peculiar to mammals, only rudiments of them occurring in animals lower in the scale. They reach a very high stage of development in man, and their occasional persistence or abnormality is to be expected and is well known. The transient vascular system referred to comprises the hyaloid artery and its branches (the outer and the inner set of the vasa hyaloidea propria and the terminal branches of the hyaloid on the back of the lens), the posterior vascular capsule of the lens, the lateral or