Eclipse solaire ou ectopie du cristallin?

Abstract

Ectopia lentis is defined as an acquired or hereditary malposition of the crystalline lens due to a zonular deficiency. It can be caused by a trauma or secondary to an underlying systemic disease, such as Marfan syndrome, Weill-Marchesani syndrome and homocystinuria. Marfan Syndrome (MFS) is a disease of the connective tissue mainly affecting the ocular, cardiovascular and skeletal system. Up to 80% of patients with Marfan syndrome develop ectopia lentis, 50% of which occurs before the age of 5. Lens displacement is usually bilateral and symmetrical, and frequently occurs superotemporally. We here report the case of a 17-year-old girl born to consanguineous parents. She presented with behavioral disorders and school problems reported by her parents. On general examination, she was tall for her age and had long, thin fingers. Clinical examination showed visual acuity 2/10 and good pupillary light reflex; the examination of the anterior segment objectified clear cornea, with an increase in anterior chamber depth and bilateral ectopia lentis in the nasal area. Fundus examination was normal. Etiological research didn’t uncover causal effects. The patient underwent phacophagia with implantation of an iris-fixated implant and skin biopsy aimed to confirm the diagnosis of Marfan syndrome.