Persistent hyperplastic primary vitreous
Leukocoria, right monocular blindness.
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The right eye globe is small in size.
An elongated thick echogenic band is seen in the posterior segment extending from the lens up to the optic nerve head. This echogenic band shows a small sized artery showing continuous biphasic blood flow on doppler, in keeping with persistent hyaloid artery.
Ciliary process is thick and echogenic. Lens is small, echogenic with irregular contour.
Embryologically the primary vitreous forms around 7th week of gestational life and starts regressing at around 20-21 weeks of gestational life and is completely regressed at the time of birth.
Pathologically it is a condition in which the embryonic hyaloid artery fail to regress. Persistence of this leads to fibrosis resulting in cataract, retinal detachment, and ciliary process hypertrophy.