Хирургическое лечение врожденной глаукомы при синдроме Франк-Каменецкого (клинический случай)

[摘要] Purpose . To submit clinical features and the effectiveness of surgical treatment of patients with glaucoma associated with the Frank-Kamenetsky’s syndrome. Material and method.We examined two patients with the Frank-Kamenetsky’s syndrome. Microinvasive non-penetrating deep sclerectomy was performed in both eyes of these patients. Results.The examination of patients detected the following features: x-linked recessive type of inheritance, decreased keratometry parameters with an axial length increase of the eye, specific changes in the anterior segment of the eye: posterior embryotoxon, bluish sclera, unusual iris structure, specific goniodisgenesis and glaucoma starting in adolescence. The combination of these features allowed us to diagnose the Frank-Kamenetsky’s syndrome. The operation and postoperative period were uncompli cated. The intraocular pressure was compensated during the entire follow-up periods. Conclusion.The described clinical cases demonstrate the clinical features of the Frank-Kamenetsky’s syndrome with a specific genetic link. A tolerance to the medical treatment with a fast progression is typical for this type of glaucoma. Surgical treatment allows to compensate the stable hydrodynamic indices of intraocular pressure, to preserve and improve visual function in these patients.

[学科分类] 眼科学

[关键词] mesenchymal;dystrophy;syndrome;Frank-Kamenetsky;glaucoma;surgery;non-penetrating deep sclerectomy;мезенхимальная;дистрофия;синдром;Франк-Каменецкий;глаукома;непроникающая глубокая склерэктомия.