Three cases of persistent hyperplastic primary vitreous (PHPV) showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented. In two cases, there was evidence that the lens had been clinically clear in the presence of a ruptured capsule and posterior cortical granulomatous reaction. The capsular rupture and granulomatous reaction can begin in utero, as shown by the third case, a premature who died at one day of age. It is suggested that a phacoanaphylactoid reaction can contribute to the lens swelling, cataract, and anterior chamber narrowing which often initiate the downhill course in eyes with PHPV. The immunological implications of this reaction are discussed.
- persistent hyperplastic primary vitreous (PHPV)
- trisomy 13
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