Myopic subvariant of 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 are common. This report evaluates the visual outcome of a subvariant 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 2 patients had a cataract extraction procedure and all underwent amblyopia therapy. Eyes with a phakic myopic refractive error (Rx) or aphakic refractive correction ≤ 13.5 D in the immediate postoperative period were examined. Results. There were 6 patients in the myopic group (Gp I) and 17 in the non-myopic group (Gp II). The mean age of diagnosis was 21.1 mos in Gp I versus 2.5 mos in Gp II with a comparable follow-up period of 36 mos. The mean pre-op Rx of Gp I was -7.78D and the mean aphakic Rx of Gp II was +17.44D with average A-scan readings of 22.46mm in Gp I and 14.03mm in Gp II. The mean corneal diameter was 11.3mm in Gp I versus 8.9mm in GP II. In Gp II, 5 eyes developed retinal detachment and 3 developed glaucoma. These complications did not develop in Gp I during the follow-up period. Functional visual acuity was overall better in Gp 1 with a median visual acuity at final follow-up of 20/160, as compared to light perception for Gp II. Conclusion. Eyes with the myopic subvariant of PHPV were not detected as early as the typical PHPV, 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. The myopic PHPV subvariant may require a different management approach.

Duke Scholars

Author Terri Lois Young Ophthalmology