Ectopia lentis superior, bilateral and symmetrical as a hereditary alteration of Marfán syndrome

Abstract

Introduction: The term ectopia lentis is used to refer the displacement of the lens out of its site. It can occur in some relatively common metabolic, systemic, hereditary disorders such as Marfan syndrome or homocystinuria, as well as in other rarer ones such as Weill-Marchesani syndrome or ectopia lentis et pupillae syndrome.

Objective: To present a case of bilateral ectopia lentis, which shows the management of a patient with subluxated lenses not related to ocular trauma.

Case presentation: The case is presented of a 47-year-old female patient with decreased vision in the left eye. The patient had an individual history of high myopia since childhood and cataract surgery of the right eye ten years ago. She presented superior subluxation of the intraocular lens in the right eye, as well as superior subluxation of mature cataract in the left eye without ocular trauma. Diagnosis: Marfan syndrome. In follow-up consultation, before deciding surgical management, cataract dislocation towards vitreous cavity was observed, a reason why she was referred to vitreoretinal consultation.

Conclusions: A patient with bilateral ectopia lentis should have a general physical examination in addition to the ophthalmologic examination; also, a referral to specific specialties should be made in order to rule out genetic entities.