Paediatric Retinal Vascular Diseases
Introduction This chapter will discuss pediatric retinal vascular diseases like Persistent Fetal Vasculature, Familial Exudative Vitreo Retinopathy and Incontinentia Pigmenti Persistent Fetal Vasculature (Persistent Hyperplastic Primary Vitreous) Definition Persistent hyperplastic primary vitreous (PHPV) is a congenital ocular disorder in which fetal vasculature does not regress after birth. It is unilateral approximately 90% of the time. No single gene has been identified. It can vary from very subtle with no visual disturbance to as severe as phthisis bulbi or secondary glaucoma. The prognosis can be good if early intervention is planned. Due to its wide spectrum of presentation, it has now been renamed as persistent fetal vasculature (PFV).1 Pathogenesis The fetal vasculature is composed of two parts: Tunica vasculosa lentis. It is situated anteriorly encircling the lens. It has anterior and posterior divisions. Anterior division has additional attachments to the pupillary frill of

