Bilateral Ectopia Lentis In Marfan Syndrome: A Case Report.
Published 2025 - 43rd Congress of the ESCRS
Reference: PO1025 | Type: Poster | DOI: 10.82333/324c-pf75
Authors: Hiba Alami* 1 , Narjiss Taouri 2 , Nourdine Boutimzine 2 , Abdellah Amazouzi 2 , Lalla Ouafa Cherkaoui 2
1Hôpital des Spécialités ,Rabat,Morocco, 2Hôpital des Spécialités,Rabat,Morocco
Purpose
Ectopia lentis (EL) is a key diagnostic criterion for Marfan syndrome, ranging from a mild, asymptomatic displacement to a significant subluxation.
The purpose of this work is to present the case of a young patient which bilateral subluxation suggested the diagnosis of Marfan Syndrome and who received treatment at our institution.
Setting
We report the case of a 14-year-old patient who was admitted to the ophthalmology emergency department with progressive decline in visual acuity over the past three years without any history of ocular trauma. Ophthalmologic examination revealed visual acuity limited to "finger counting" at 2 meters in both eyes, with anterior and bilateral superolateral dislocation of a clear lens with clear visualization of the zonules.
Methods
The cornea was clear, the anterior chamber deep and the ocular tone measured at 17 mmHg in the right eye and 18 mmHg in the left eye.
Her medical history includes high myopia of – 13.00 D in the right eye and -12.00 D in the left eye and spontaneous fractures of both forearms at the age of 7.
The patient was referred to pediatrics for a general examination, and the pediatrician also suspected Marfan syndrome, requesting a genetic test. The homocysteinemia levels returned normal. A cardiovascular evaluation, including a transthoracic ultrasound, showed no abnormalities.
The patient was then scheduled for lens extraction by phacoemulsification combined with anterior vitrectomy.
Results
Marfan syndrome was first described in 1896. It is a hereditary connective tissue disorder transmitted in an autosomal dominant manner. It primarily affects mesoderm-derived organs, mainly the eye, skeleton, and aorta.
Ocular manifestations occur in 60% to 87% of cases, making ophthalmologic examination essential for the diagnosis and follow-up of this disease.
Anterior lens dislocation is a rare ocular complication, particularly when bilateral. It can occur spontaneously or following even minor trauma.
The major surgical challenge in managing lens dislocation in Marfan syndrome arises from the fragility of the zonular support.
Phacoemulsification combined with anterior vitrectomy is the most commonly used surgical technique.
Conclusions
The ophthalmological management of Marfan patients is challenging and periodical follow-up is needed. Surgical versus conservative management is controversial, each case needs to be evaluated individually to analyze the risks and benefits of the procedures.