Diagnostics and Prediction of Glaucoma in Patients with Familial Congenital Iris HypoplasiaReport as inadecuate




Diagnostics and Prediction of Glaucoma in Patients with Familial Congenital Iris Hypoplasia - Download this document for free, or read online. Document in PDF available to download.

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.

KEYWORDS

Glaucoma, Megalocornea, Congenital Mesodermal Iris Atrophy, Goniodysgenesis

Cite this paper

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.





Author: Tatiana Iureva1,2, Andrey Shchuko1, Yulia Pyatova1

Source: http://www.scirp.org/



DOWNLOAD PDF




Related documents