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Abstract #59062 Published in IGR 16-3
Progressive supra-aortic stenosis in a young adult with the findings of Singleton Merten Syndrome
Ozyuksel A; Ersoy C; Canturk E; Akcevin ABritish Medical Journal (Clinical Research Edition) Case Reports 2014; 2014:
Singleton Merten Syndrome is an autosomal dominant disorder of unknown origin. Patients often present with muscular weakness, failure to thrive, abnormal dentition, glaucoma, psoriatic skin lesions, aortic calcification and musculoskeletal abnormalities. In this case, we present a young girl with a history of aortic root replacement, who had an unusual progressive supra-aortic stenosis managed with urgent surgery during the course of the syndrome. Cardiovascular involvement needs special attention, since it is the major cause of mortality along with rhythm disturbances in the course of Singleton Merten Syndrome.
Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
Full articleClassification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
