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Table 6 The Kids-Short Marfan score (adapted from Mueller GC et al. [39])

From: When flexibility is not necessarily a virtue: a review of hypermobility syndromes and chronic or recurrent musculoskeletal pain in children

Required manifestations Risk category for likelihood of MFS
Dilatation of aortic root + Ectopia lentis Very high risk
• dilatation of aortic root High risk (Complete examination of all symptoms of the revised Ghent Criteria is strictly recommended as soon as possible. Patient should see Marfan Syndrome specialists)
+ mitral valve prolapse + tricuspid valve prolapse or
+ dilatation of pulmonary artery or
+ at least 3 skeletal features of the systemic score of the revised Ghent criteria
• Ectopia lentis
+ mitral valve prolapse + tricuspid valve prolapse or
+ dilatation of pulmonary artery
Family history dilatation of aortic root Moderate risk (Patient needs to be verified or excluded with further diagnostic procedures other than or echocardiography and clinical examination)