Abstract
Purpose: To report a case presented with unilateral acute angle closure glaucoma secondary to ectopia lentis (EL) as first manifestation of Marfan syndrome(MFS) Material and Methods: case report Results: A 37-year-old man with patent laser peripheral iridotomy(LPI) in both eyes presented with repeated episodes of acute angle closure glaucoma in his right eye. Scheimpflug photography (Pentacam, Oculus demonstrated bilateral and superotemporal EL. Pupillary dilated slit lamp examination confirmed EL later. Bilateral EL was also diagnosed in the patient's elder brother Transthoracic echocardiography demonstrated mitral valve prolapse in this patient and aortic root dilatation in the patient's elder brother. Genetic evaluations revealed fibrillin1 (FBN1) gene mutation in this patient, his son and the patient's elder brother. The patient and his elder brother were finally diagnosed as MFS based on Ghent nosology. Conclusion: EL should be considered when repeated episodes of acute angle closure glaucoma occurred in young patients with patent LPI. Scheimpflug photography is valuable in providing the correlation between EL and iris plane with fixed-dilated pupil. Early diagnosis and appropriate surgical management of EL can preserve vision in patients with MFS.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
