Bilateral Nasal Ectopia Lentis with no Skeletal Abnormality: Is it Marfan Syndrome?

Summary To report three male Marfan patients from one family, with no skeletal anomalies, but with nasally subluxated lenses in two and bilateral total crystalline lens dislocation into the vitreous cavity in one of them. Ophthalmological, cardiological, orthopaedic evaluation. 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 1tricuspid valve insufficiency was present in one patient. Conclusion: Whenever bilateral ectopia lentis is observed, Marfan syndrome should be suspected, and careful systemic evaluation should be carried out in order to avoid overlooking the other systemic life-threatening failures. (TOD Dergisi 2010; 40: 245-7)