Other Vitreoretinal Pathologies in Infants

Abstract

Pediatric retinal diseases can be acquired, such as retinopathy of prematurity, or in addition can certainly be congenital. The hallmark disease is familial exudative vitreoretinopathy which can present in full-term infants with an avascular periphery initially, dragged disc, exudative and tractional retinal detachment, and can lead to blindness due to its potentially progressive course lifelong. In addition to this, diseases such as persistent fetal vasculature syndrome can also be devastating in terms of retinal vascular and neural tissue development, both of which can lead to a poorly seeing or blind eye. Several other diseases which are not discussed in this chapter such as congenital X-linked retinoschisis and Norrie’s disease as well as Coats’ disease can all lead to severe retinal detachment and blindness. These can in part be distinguished by their physical characteristics, but may have some biochemical features in common, which are important relative to retinal vascular and neural cell development. This chapter will concentrate on familial exudative vitreoretinopathy and persistent fetal vasculature syndrome outlining their diagnosis and current and future management.