歯学ジャーナル
オープンアクセス

概要

Fixation of the Capsular Bag-Intraocular Lens Complex as a Surgical Treatment of Lens Subluxation in Marfan Syndrome

Wojciech Mazurek, Barbara Rekas, Karolina Krix-Jachym, Natalia Blagun, Marek Rekas

Purpose: Marfan Syndrome (MFS) is a congenital systemic connective tissue disorder. It is caused by mutations in the fibrillin-1 gene, a structural protein found in many tissues. The defects lead to widespread clinical manifestation, particularly ocular symptoms. The most common ophthalmic manifestation is Ectopia Lentis (EL), which occurs in up to 75% of patients with Marfan Syndrome. This article describes a novel surgical technique used at the Department of Ophthalmology of the Military Institute of Medicine-National Research Institute. The method involves fixation of the capsular bag-intraocular lens complex with iris retractors. This surgical technique applies to treating lens dislocations of various etiologies, including Marfan syndrome.

Case presentation: An 18-year-old female patient with Marfan syndrome was admitted to the clinic due to deterioration of visual acuity. The patient was diagnosed with lens subluxation in both eyes. Preoperative visual acuity was 20/1000 in both eyes. The novel surgical technique was performed. A binocular visual acuity of 20/20 was achieved. The patient has been followed up for about 3 years after the surgery.

Conclusion: Patients with Marfan syndrome are at a much higher risk of lens subluxation than the general population. Therefore, prompt detection and appropriate treatment are essential. In this case, a novel, minimally invasive and effective technique was used to treat lens subluxation. This technique reduces the risk of postoperative decentration of the capsular bag-intraocular lens complex.