Sobolev N P, Teplovodskaya V V, Sudakova E P
S.N. Fedorov National Medical Research Center «MNTK «Eye Microsurgery», Moscow, Russia.
Vestn Oftalmol. 2022;138(1):58-63. doi: 10.17116/oftalma202213801158.
Oculocutaneous albinism is a group of autosomal recessive disorders that directly affect the hypopigmentation of the skin, hair, and eyes. According to the World Health Organization, albinism occurs in 1 out of 20 000 people in Europe and North America. Congenital changes in the organ of vision in albinism lead to a significant decrease in the visual functions of patients, worsening their quality of life.
To study the results of surgical treatment with implantation of an artificial iris as a diaphragm element in patients with albinism.
This article was based on the examination and treatment of 2 patients suffering from oculocutaneous albinism, which is characterized by hypopigmentation of the iris and retinal pigment epithelium of the retina, foveolar hypoplasia, low visual acuity, nystagmus, strabismus and excruciating photophobia. The patients were implanted an artificial iris in both eyes with fixation in the capsular bag in the first patient, and in the ciliary sulcus in the second.
In both patients, complaints of photophobia and optical phenomena have ceased. Visual acuity increased to 0.2 and remained without regression throughout the entire period of observation. Intraocular pressure and hydrodynamic parameters also remained stable throughout the observation period. The artificial irises were observed by ultrabiomicroscopy to remain in a stable position both when it was implanted in the capsule bag, and when it was seamlessly fixed in the ciliary sulcus.
The surgical interventions relieved patients from glare, at the same time improving visual acuity without correction, as well as achieving a pronounced cosmetic effect and visual rehabilitation. Artificial irises have proven to be a safe and effective way to treat patients with oculocutaneous albinism.
眼皮肤白化病是一组常染色体隐性疾病,直接影响皮肤、头发和眼睛的色素减退。据世界卫生组织称,欧洲和北美的白化病发病率为两万分之一。白化病患者眼部的先天性变化导致其视觉功能显著下降,生活质量恶化。
研究在白化病患者中植入人工虹膜作为隔膜元件的手术治疗效果。
本文基于对2例眼皮肤白化病患者的检查和治疗,其特征为虹膜和视网膜色素上皮色素减退、黄斑发育不全、视力低下、眼球震颤、斜视和极度畏光。第一例患者双眼植入人工虹膜并固定于囊袋内,第二例固定于睫状沟。
两名患者的畏光和光学现象主诉均已消失。视力提高到0.2,在整个观察期内保持无下降。眼压和流体动力学参数在整个观察期内也保持稳定。通过超生物显微镜观察,人工虹膜无论是植入囊袋还是无缝固定于睫状沟时均保持稳定位置。
手术干预使患者免受眩光困扰,同时提高了未矫正视力,还实现了显著的美容效果和视觉康复。人工虹膜已被证明是治疗眼皮肤白化病患者的一种安全有效的方法。