Purpose: To identify the clinical features of the syndrome Frank-Kamenetsky and determine the\ncriteria of early formation of glaucoma. Materials and Methods: We observed 52 patients. Follow\nup period was from 5 to 22 years. The first group (juvenile) consisted of males who had the first\nsigns of glaucoma diagnosed before the age of 12 (n = 22). The average age of the group was 10.1 �±\n2.4 years. The control group included healthy males (n = 30) in the same age range (average age\n7.2 �± 1.6 years). The second group (adults) consisted of patients who had the first signs of glaucoma\ndiagnosed after the age of 18 and elder. The average age of the group was 32.44 �± 6.28 years.\nThe control group had males (n = 30) in the same age range (average age 26.59 �± 4.12 years). The\ninclusion criterion was: the presence of congenital bilateral mesodermal iris leaf hypoplasia, trabecular\ndysgenesis signs, the presence of blood relatives on the maternal line (grandfather, uncle)\nmale with similar changes iridociliary zone and glaucoma. Criteria of glaucoma formation were:\nincreased IOP more than 21 mmHg with accompanying it expansion of the cup/disc ratio, reducing\nthe thickness of the nerve fiber layer (RNFL) according to OCT. Results: It was found that Frank-\nKamenetsky Syndrome had an X-linked with sex, recessive inheritance and was characterized by\nbilateral congenital irisdysgenesis and goniodysgenesis with the accession glaucoma. Predictors\nof glaucoma formation in early childhood are a combination of: 1) congenital subtotal atrophy of\niris mesodermal layer (from 0 to 30 mkm) with signs of progressive dystrophy; 2) nonprogressive\ncongenital megalocornea (cornea diameter 12 - 14 mm); 3) iridotrabecular dysgenesis of II-III degree;\n4) hyperopic refraction in axial myopia.
Loading....