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PURPOSE: To demonstrate that developmental glaucoma (instead of the term goniodysgenetic 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 autosomal 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 had late-onset goniodysgenetic 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 iridocorneal angle tissues from the patients showed thick juxtacanalicular connective tissue with accumulations of a basement membrane-like extracellular matrix. CONCLUSION: Congenital microcoria are considered to be frequently associated with the incidence of late-onset goniodysgenetic glaucoma.
Dr. A. Tawara, Department of Ophthalmology, School of Medicine, University of Occupational and Environmental Health, Japan. Tawara-a@med.uoeh-u.ac.jp
9.1.2 Juvenile glaucoma (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)