Abstract
Purpose: To identify the clinical features of the syndrome Frank-Kamenetsky and determine the criteria of early formation of glaucoma. Materials and Methods: We observed 52 patients. Follow up period was from 5 to 22 years. The first group (juvenile) consisted of males who had the first signs of glaucoma diagnosed before the age of 12 (n = 22). The average age of the group was 10.1 ± 2.4 years. The control group included healthy males (n = 30) in the same age range (average age 7.2 ± 1.6 years). The second group (adults) consisted of patients who had the first signs of glaucoma diagnosed after the age of 18 and elder. The average age of the group was 32.44 ± 6.28 years. The control group had males (n = 30) in the same age range (average age 26.59 ± 4.12 years). The inclusion criterion was: the presence of congenital bilateral mesodermal iris leaf hypoplasia, tra-becular dysgenesis signs, the presence of blood relatives on the maternal line (grandfather, uncle) male with similar changes iridociliary zone and glaucoma. Criteria of glaucoma formation were: increased IOP more than 21 mmHg with accompanying it expansion of the cup/disc ratio, reducing the thickness of the nerve fiber layer (RNFL) according to OCT. Results: It was found that Frank-Kamenetsky Syndrome had an X-linked with sex, recessive inheritance and was characterized by bilateral congenital irisdysgenesis and goniodysgenesis with the accession glaucoma. Predictors of glaucoma formation in early childhood are a combination of: 1) congenital subtotal atrophy of iris mesodermal layer (from 0 to 30 mkm) with signs of progressive dystrophy; 2) nonprogressive congenital megalocornea (cornea diameter 12 - 14 mm); 3) iridotrabecular dysgenesis of II-III degree; 4) hyperopic refraction in axial myopia.
Highlights
This syndrome was first described in Russia in 1925, was characterized by congenital atrophy of the mesodermal layer of the iris and goniodysgenesis and led to the development of glaucoma [1]-[3]
According to the study results, the initial mesodermal layer thickness of the iris at birth is a sign of dysgenesis, which is directly correlated with the degree of goniodysgenesis, abnormalities of the cornea, and the presence of partial or complete posterior embryotoxon
Formation of glaucoma in children is caused by a combination of congenital abnormalities of the cornea, iris, and the iridocorneal angle, as well as the presence of coarse dysgenesis of the anterior segment of the eye
Summary
This syndrome was first described in Russia in 1925, was characterized by congenital atrophy of the mesodermal layer of the iris and goniodysgenesis and led to the development of glaucoma [1]-[3]. It was later named as Frank-Kamenetsky Syndrome in honor of the discoverer. It is suggested that the mechanisms of glaucoma development in different ages can have fundamental differences These differences are dependent on the combination of several characteristics of mesenchymal dysgenesis inherited in this syndrome
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