Retinal vascular changes of incontinentia pigmenti

Of 19 patients with incontinentia pigmenti, seven had a bizarre retinal anomaly that consisted of a zone of abnormal arteriovenous connections and preretinal fibrotic tissue at the temporal equator, with no perfusion peripheral to it. In one patient, the vascular changes progressed and required photocoagulation. This retinal lesion may represent an early stage of the pseudoglioma that so commonly is reported with this skin disease. Incontinentia pigmenti should be considered in the differential diagnosis of retinal vascular disease in girls and women of any age.