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[马凡氏综合征患者的眼部表现及手术结果]

[Ocular manifestations and surgical results in patients with Marfan syndrome].

作者信息

Ladewig M S, Robinson P N, Neumann L M, Holz F G, Foerster M H

机构信息

Augenklinik, Rheinische Friedrich-Wilhelms-Universität, Ernst-Abbe-Strasse 2, 53127 Bonn.

出版信息

Ophthalmologe. 2006 Sep;103(9):777-82. doi: 10.1007/s00347-006-1386-8.

Abstract

AIM

This retrospective study should examine and judge the surgical indications and the therapeutic possibilities as well as their complications in patients with ocular manifestations of Marfan syndrome (MFS) diagnosed according to the criteria of the Ghent nosology.

PATIENTS AND METHODS

The study included 17 patients. Operative indications were increasing subluxation of the lens, retinal detachments and secondary glaucoma. The operative procedure depended on patient age and findings. Eleven MFS patients were operated in both eyes and six MFS patients in one eye.

RESULTS

Stabilization or functional improvement of visual acuity could be achieved in all patients in whom no disorders limiting visual acuity or amblyopia were present preoperatively. In six eyes of five patients, lens insertion was accomplished via a pars plana approach. Lens removal without implantation of an intraocular lens was performed in 16 eyes of 10 patients. Pars plana vitrectomy was accomplished in 12 eyes. Complications were well controlled by pars plana vitrectomy.

CONCLUSIONS

Difficult preoperative situations and postoperative complications are not rare in MFS patients. However, they can be controlled well by means of modern vitreous surgery.

摘要

目的

本回顾性研究旨在根据根特分类法标准,对诊断为马凡综合征(MFS)且有眼部表现的患者的手术指征、治疗可能性及其并发症进行检查和判断。

患者与方法

该研究纳入了17例患者。手术指征为晶状体半脱位加重、视网膜脱离和继发性青光眼。手术方式取决于患者年龄和检查结果。11例MFS患者双眼接受手术,6例MFS患者单眼接受手术。

结果

术前不存在限制视力或弱视的疾病的所有患者,视力均实现了稳定或功能改善。5例患者的6只眼中,通过平坦部入路植入晶状体。10例患者的16只眼中,进行了晶状体摘除且未植入人工晶状体。12只眼进行了平坦部玻璃体切除术。通过平坦部玻璃体切除术,并发症得到了良好控制。

结论

MFS患者术前情况复杂且术后并发症并不罕见。然而,借助现代玻璃体手术,这些情况能够得到很好的控制。

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