Sven Crafoord, MD, PhD (Orebro, Sweden)


To describe the surgery of Posterior PFV which is a rare ocular maldevelopment syndrome seen in children mostly unilateral (90%). Usually the diagnosis is detected among infants with congenital cataract.


Three consecutive children with posterior PFV were included in this interventional study. They all underwent surgery with lensectomy and vitreoretinal surgery.


No eyes were lost. In two eyes amblyopic treatment with contact lenses were initiated. One patient did not get a contact lens due to severe malformation in the posterior segment including retina and macula.


The natural course of posterior PFV is cataract and traction on the ciliary body, retina and the optic disc. This can lead to blindness and loss of the eye. With modern vitreoretinal surgery and removal of the lens and the traction these complications can usually be avoided. The visual function, however, depends on the degree of amblyopia and the persistent pathology of the posterior region.