Congenital microcoria associated with late-onset developmental glaucoma.

Purpose: To demonstrate that developmental glaucoma (instead of the term goniodysgenctic glaucoma is used in this paper), defined as glaucoma with goniodysgenesis resulting from a fetal maldevelopment of the iridocorneal angle, develops in association with congenital microcoria. Methods: Three subjects descended from a family with amosoinal dominant congenital microcoria and goniodysgenesis were followed up for more than 25 years. Results: The extended family consisted of 3 generations including 8 males and 10 females. In the second generation, 2 of 7 subjects who presented with a history of congenital microcoria bad late-onset goniodysgenefic glaucoma. In the third generation, all 3 descendants of the second generation subjects with congenital microcoria had congenital microcoria with goniodysgenesis. Two of these subjects developed late-onset goniodysgenetic glaucoma in both eyes during the 25-years follow-up period. They were both treated with a trabeculectomy in both eyes to control the glaucoma. Histologically, the iridocomeal angle tissues from the patients showed thick juxtacanalicular connective tissue with accumulations of a basement membranelike extracellular matrix. Conclusion: Congenital microcoria are considered to be frequently associated with the incidence of late-onset goniodysgenctic glaucoma.