Persistent hyperplastic primary vitreous in male twins

Persistent hyperplastic primary vitreous (PHPV) occurs sporadically and few familial occurrences have been reported. The authors report PHPV unassociated with other congenital anomalies in male twins. One underwent a lensectomy for the management of angle-closure glaucoma and the other was treated for amblyopia. These cases provide further evidence to suggest autosomal recessive inheritance, though the possibility of developmental error cannot be excluded.