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A clinical case of mesenchymal iris dysplasia for the first time in a family

Frank-Kamenetsky syndrome is a rather rare ocular anomaly, which is characterized by hypoplasia of the iris stroma with exposure of its pigment layer and the development of secondary glaucoma, more often in the 2-3rd decade of life. Gonioscopy reveals goniodysgenesis in the form of "dentate" and anterior attachment of the root of the iris of a dirty gray color, as well as the inclusion of mesodermal tissue in the trabecular zone. The severity of the course of glaucoma is determined by the age of onset and is often malignant, gradually leading to blindness. Frank-Kamenetsky syndrome is observed in men and refers to a recessive disease linked to the X chromosome. The article presents a clinical case of mesenchymal iris dysplasia, first identified in a family, similar to the clinical picture of Frank-Kamenetsky syndrome. Previously, retinal pigment degeneration was observed in the male line of the family. Key words: Frank-Kamenetsky syndrome, glaucoma, congenital glaucoma.