İskelet anomalisi olmaksızın Bilateral nazal lens ektopisi: Marfan mı?

Objective: To report three male Marfan patients with no skeletal anomalies from the same family; two with nasal subluxated lenses, and one with bilateral total crystalline lens dislocation into the vitreous cavity. Design: Case series. Methods: Ophthalmological, cardiological, orthoapedic evaluation. Results: Although none of the patients had any skeletal abnormalities, all three had ophthalmological involvement. Cardiological examination revealed mitral valve prolapse and aortic root dilatation in all patients. Associated grade 1 tricuspid valve insufficiency was positive in one patient. Conclusion: Whenever bilateral ectopia lentis is observed, Marfan syndrome should be suspected, and careful systemic evaluation should be exercised in order to avoid overlooking the other systemic life-threatening failures.Objective: To report three male Marfan patients with no skeletal anomalies from the same family; two with nasal subluxated lenses, and one with bilateral total crystalline lens dislocation into the vitreous cavity. Design: Case series. Methods: Ophthalmological, cardiological, orthoapedic evaluation. Results: Although none of the patients had any skeletal abnormalities, all three had ophthalmological involvement. Cardiological examination revealed mitral valve prolapse and aortic root dilatation in all patients. Associated grade 1 tricuspid valve insufficiency was positive in one patient. Conclusion: Whenever bilateral ectopia lentis is observed, Marfan syndrome should be suspected, and careful systemic evaluation should be exercised in order to avoid overlooking the other systemic life-threatening failures