products in the vascular endothelial Wnt signaling pathways demonstrates that multiple genetic hits upon a system of cellular metabolism or structures can lead to diseases that share a common phenotypic spectrum. The knowledge of the Wnt signal pathway and its strong capacity to modulate normal angiogenesis suggests that targets in this pathway might indeed be upregulated in pathological angiogenesis that might be reasonable foci for therapeutic intervention.

13.4.7  Incidence

FEVR is a rare ocular condition.

a

c

e

Fig. 13.9  Subtle macular dragging is seen in this asymptomatic patient with FEVR in the right (a) and left (b) eyes. A more pronounced case is seen in a second patient (c and d). A color montage demonstrates the peripheral changes in the second patient (e)

13.4.8  Natural History and Prognosis

(Signs, Symptoms, Timing, etc.)

Patients with FEVR may present in the first few years of life with signs of decreased vision such as strabismus or leukocoria. Other patients with FEVR are completely asymptomatic with areas of avascular temporal peripheral retina evident only on careful clinical exam. Symptomatic patients can present with retinal exudates, retinal neovascularization, or a peripheral fibrovascular mass [56]. Retinal, macular (Fig. 13.9), and optic nerve dragging are not uncommon. Retinal detachment and vitreous hemorrhage may lead to surgical intervention.

b

d