Prominent radial iris blood vessels created small but definite pupillary notches in five children with persistent hyperplastic primary vitreous and persistence of the tunica vasculosa lentis. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Surgical and amblyopia therapy, therefore, may not provide useful vision, and should be undertaken with caution.
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