Management and prognosis


Today, the goals in treatment are saving the eye from the complications of untreated PHPV (mainly glaucoma and phthisis bulbi), saving of useful vision, and achieving acceptable cosmetic outcome.

When the cataract is significant, some authors suggest preventive lens extraction in the first few weeks of life. Lensectomy with or without anterior or total vitrectomy, and trabeculectomy are the surgical procedures that are performed depending on the clinical presentation. It should be noted however, that surgery for PHPV may be complicated by retinal detachment. Strict case selection is needed, as preoperative retinal or optic nerve abnormalities are particularly associated with poor outcomes.

Strabismus surgery may also be required though fusional potential is usually poor in these patients.

Visual rehabilitation (aphakic contact lens and amblyopia therapy) is possible with eyes that are fairly normal in structure after the lensectomy and membranectomy. In case of the numerous abnormalities of the posterior segment, visual rehabilitation may not be possible. In patients in whom surgery is not possible, the use of a cosmetic contact lens to give a black pupil is necessary. Enucleation of the blind eye may be indicated in case of intractable glaucoma and phthisis bulbi.


Visual prognosis is usually guarded as the disease is often progressive. Early surgery may result in relatively good visual results in selected patients.

The most important factor to predict the visual outcome in PHPV patients is whether or not the posterior pole is involved.

Treatment of posterior and combined anteroposterior PHPV usually has a less favorable outcome.