Long-Term Visual Function and Relative Amblyopia in Posterior Persistent Hyperplastic Primary Vitreous (PHPV)

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Background: Favorable results in the management of PHPV, made possible by improved surgical techniques, are described in the literature. However, the functional results are in general in the low-vision sector (rarely better than 0.025–0.1 maximum). Purpose: To introduce long-term acuity results and estimate the relative amblyopia in early operated PHPV. Material and Methods: Six patients suffering from PHPV of varying degrees (posterior polar cataract, posterior lenticonus, traction and malformation of the optic nerve head and macula region, persistent vitreous vessels) underwent pleoptic treatment after surgery (lentectomy in all, additional stalk-cutting in three cases) at the age of 3 to 7 months. The patients were followed up to age 7 to 12 years. Visual acuity was assessed by Landolt ring single and closely interspaced linear optotypes (C-Test developed by Haase/Hohmann). Results: Compliance with the amblyopia treatment was good in five patients. Visual acuity (VA) improvement in the affected eye could be seen up to early school age (6–8 years). Final VA outcome was 0.1 (minimum) to 0.7 (maximum) with single optotypes. VA for crowded optotypes was maximum 1 octave below single optotype VA. The visual development of the phakic eye was not delayed, i.e. not disturbed by long-term patching. Discussion: Compliance with amblyopia treatment and long-term visual acuity in the cases presented here are even more favorable than described in the literature. As the amount of crowding is small, we conclude that there is little relative (additional) amblyopia and that the functional outcome is limited only by the degree of ocular malformation. Surgical treatment in comparable cases is encouraged.