Author(s)

Tatiana Iureva^1,2, Andrey Shchuko¹, Yulia Pyatova¹

¹Irkutsk Branch, S. Fyodorov Eye Microsurgery Federal State Institution, Irkutsk, Russia.
²Irkutsk State Medical Academy for Postgraduate Education, Irkutsk, Russia.

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.

Glaucoma, Megalocornea, Congenital Mesodermal Iris Atrophy, Goniodysgenesis

Iureva, T. , Shchuko, A. and Pyatova, Y. (2015) Diagnostics and Prediction of Glaucoma in Patients with Familial Congenital Iris Hypoplasia. Open Journal of Ophthalmology, 5, 115-123. doi: 10.4236/ojoph.2015.53018.