Persistent fetal vasculature is a rare benign developmental disorder of the globe in which the embryonic vasculature fails to regress normally, resulting in abnormal lenticular development and secondary changes of the retina and globe. This disorder is sporadic and 90% unilateral.

Signs and symptoms include nystagmus, cataracts, retinal detachment, glaucoma or microphthalmia. There are usually no associated systemic findings.

Differential Diagnosis:

• Leukokoria