Myopia predicts better outcome in persistent hyperplastic primary vitreous

Purpose: Persistent hyperplastic primary vitreous (PHPV) is a congenital disorder that presents with a spectrum of ocular anomalies, including cataracts, microphthalmia, and hyaloid vessel remnants. Severe visual loss due to secondary glaucoma and retinal detachment is common. This report evaluates the visual outcome of a variant of PHPV with myopia not associated with glaucoma. Methods: The records of 23 consecutive patients with the diagnosis of PHPV (all unilateral) from October 1992 to August 1995 were reviewed. All but three patients had a cataract extraction procedure and all underwent amblyopia therapy. Eyes with aphakic myopic refractive error (Rx) or aphakic refractive correction ≤8.5 diopters (D) in the immediate postoperative period were designated as myopic. Results: Six patients were in the myopic group (Group 1) and 17 in were the nonmyopic group (Group 2). The mean age of diagnosis was 21.1 months in Group 1 versus 2.0 months in Group 2, with a comparable follow-up period of 36 months. The mean preoperative Rx of Group 1 was -7.78 D. The mean aphakic Rx of Group 2 was +18.29 D. Average axial length measurement determined by echography was 22.46 mm in Group 1 and 14.03 mm in Group 2. The mean corneal diameter was 11.3 mm in Group 1 vs 8.9 mm in Group 2. In Group 2, seven eyes developed retinal detachment and four developed glaucoma. These complications did not develop in Group 1 during the follow-up period. Overall functional visual aculty was better in Group 1, with a median visual aculty at final follow up of 20/160, as compared with light perception for Group 2. Conclusions: PHPV eyes with myopia were not detected as early as the typical PHPV eyes, primarily because of less media opacification and near-normal corneal diameters. These eyes showed a more favorable visual outcome as they were less likely to develop typical PHPV- related postoperative complications. Myopic PHPV eyes may require a different management approach.