Incontinentia pigmenti is a dominant genetic disease linked to chromosome X, lethal for boys. Retinal ischemia develops during the first year of life, and usually affects only one eye due to lionization of chromosome X (Fig. 12.2). Other specific features of this disease are the dermatological lesions that consist in pigmented skin lesions on the legs that are visible during the first months of life [8].

We have described ischemic lesion in shaken baby syndrome that could be due to the shearing effect of the trauma on retinal vessels (Fig. 12.3) [9].

Subretinal exudation is less commonly seen in ROP, but can be observed in advanced stages of stage 4 or 5 disease with very active plus disease.

In FEVR, this feature can be predominant due to the presence of large vessel peripheral anastomosis.

Coats disease is characterized by vascular dilation of capillaries, rarefaction of capillary meshwork and retinal exudation leading to total retinal detachment (Figs. 12.4 and 12.5) [10]. Boys are specifically concerned in 85% of the cases. No vitreous involvement is observed initially in Coat’s disease; neovascularization can be observed after induced retinal ischemia by extensive laser photocoagulation of vascular aneurysms [11].

Retinal detachment is observed in stage 4 and 5 ROP. In late phases of these detachments, the fibrous tissue can be the predominant feature of these tractional detachments, with a very important regression of the vascular component in most cases.

Advanced stages of FEVR are characterized by total retinal detachment (Fig. 12.6). Although babies can be affected, the mean age of retinal detachment in this disease is young infancy or adolescence. Subretinal exudation is often present and asymmetrical disease is a typical presentation in FEVR.