Outcomes of surgical (pars plicata and limbal lensectomy, vitrectomy) and non-surgical management of persistent fetal vasculature (PFV): an analysis of 54 eyes.

Purpose: To identify clinical characteristics associated with outcomes of treatment for persistent fetal vasculature (PFV based on a modified classification.

Methods: In this noncomparative case series, 54 eyes of 47 consecutive patients with PFV managed from 1981 until 1998 at a tertiary eye hospital were reviewed. Pars plicata lensectomy and vitrectomy was performed in 34 eyes and translimbal lensectomy and vitrectomy in five. Fifteen eyes were managed non-surgically. The following outcomes were measured: 1) "Visual improvement", defined as at least one Zipf's category improvement of best corrected final vision as compared with initial visual acuity, provided that final vision of CSUM (central, steady, unmaintained) equal to 20/100-20/30 was obtained. 2) "Cosmetic acceptability", defined as the absence of the following: small orbit, externally small appearing eye with microcornea and hypotony, eye deviation more than 35 prism diopters without strabismus surgery and corneal opacity.

Results: Six eyes were inoperable. In 10 out of 33 operated eyes (30.3%) improvement of vision was obtained including four eyes with an anterior form and six eyes with a combined form of PFV. In univariate analysis, factors such as anterior form of PFV surgery in the combined form and having a normal retina, had a weak association with a higher chance of visual improvement. In multivariate regression analysis the likelihood of unacceptable cosmetic appearance was higher in females than males (OR = 10.5), and in bilateral cases (OR = 7.9). Offspring of consanguineous marriages had more severe forms of PFV.

Conclusions: Although visual potential is limited in PFV some eyes with an anterior or combined form and normal retina achieved improvement of visual acuity after surgery. However, statistically no single factor except sex and bilateral PFV (for cosmetic unacceptability) reliably predicted the results of treatment. Therapy should be individualized based on clinical findings. Genetic studies in offspring of consanguineous marriages with PFV may prove informative.