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马凡综合征晶状体半脱位手术中 Cionni 改良囊袋张力环的视力结果。

Visual outcomes of lens subluxation surgery with Cionni modified capsular tension rings in Marfan syndrome.

机构信息

Department of Ophthalmology and Vision Science, Eye and ENT Hospital of Fudan University, 83 Fenyang Rd, Shanghai, 200031, China.

Key Laboratory of Myopia of State Health Ministry, and Key Laboratory of Visual Impairment and Restoration of Shanghai, Shanghai, China.

出版信息

Sci Rep. 2021 Feb 4;11(1):2994. doi: 10.1038/s41598-021-82586-6.

Abstract

Marfan syndrome (MFS) is a hereditary disease with an incidence of 0.3% in the general population. Approximately 60% of MFS patients with FBN1 gene mutation will suffer ectopia lentis (EL) from the age of 3. With the development of EL, severe loss of vision will accrue because of lens tilt and glaucoma. Cionni modified capsular tension rings (MCTR) has been applied in the surgery for EL in MFS patients. To evaluate visual acuity and safety of using MCTR during lens subluxation surgery in MFS patients, 66 MFS patients (110 eyes) were included in our study, with the mean duration of follow-up of 4.7 months (SD 1.76 months). The capsular bags were preserved in 101 eyes (91.81%) with MCTR implantation. There was an overall significant improvement in BCVA at 1-month follow-up which was maintained at 3 months. Multivariable linear regression revealed that older age at first visit was associated with greater postoperative BCVA at the 1-month follow-up (P = 0.007). A significant difference was found between different degrees of lens subluxation and the length of surgical time and complications. At follow-up, only two eyes (1.98%) were identified to have developed retinal detachments. In conclusion, better visual outcomes can be achieved when patients received an early operation with MCTR implantation.

摘要

马凡综合征(MFS)是一种遗传性疾病,在普通人群中的发病率为 0.3%。大约 60%的 FBN1 基因突变的 MFS 患者会在 3 岁时出现晶状体异位(EL)。随着 EL 的发展,由于晶状体倾斜和青光眼,严重的视力丧失会随之而来。Cionni 改良的囊袋张力环(MCTR)已应用于 MFS 患者的 EL 手术中。为了评估 MCTR 在 MFS 患者晶状体半脱位手术中应用的视力和安全性,本研究纳入了 66 例 MFS 患者(110 只眼),平均随访时间为 4.7 个月(SD 1.76 个月)。在植入 MCTR 的 101 只眼中保留了囊袋。在 1 个月的随访中,BCVA 总体显著提高,3 个月时保持不变。多变量线性回归显示,首次就诊时年龄越大,术后 1 个月的 BCVA 越高(P=0.007)。不同程度的晶状体半脱位、手术时间和并发症与术后 BCVA 存在显著差异。随访时,仅发现 2 只眼(1.98%)出现视网膜脱离。总之,MCTR 植入术能使患者获得更好的视力预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5406/7862488/a34c1f3cdb52/41598_2021_82586_Fig1_HTML.jpg

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