Ectopia lentis in Marfan syndrome

Abstract

We report the case of a 6-year-old-child, with history of mitral valve disease, he was diagnosed suffering from Marfan syndrome. He was brought to ophtalmological consultation for decreased visual acuity. The clinical examination found a visual acuity reduced to counting fingers at 1 meter in the right eye and 1/10 in the left eye. The examination after pupillary dilation revealed bilateral lens dislocation in the superonasal direction. While posterior segment examination was normal in both eyes. Considering the visual impact which cannot be improved by an optical correction, we decide to manage it surgically, which consisted in phacophagia for both eyes with interval of 2 months. For the aphakia, the patient benefited from an optical correction by glasses and kept under observation to treat amblyopia. Ectopia lentis is an evolving disease, characterized by a crystalline lens displacement in the frontal plane, due to zonular abnormalities by constitutional anomaly. It is usually bilateral, and symmetric. It may vary from partial to a complete displacement. In the case of our patient the ectopia lentis was a part of systemic diseases which is Marfan syndrome.

Keywords

• marfan syndrome
• ectopic lentis
• zonular abnormalities