Table 1.

Calculation of the Systemic Score

FeatureValueEnter Value if Feature Is Present
Wrist AND thumb sign3
Wrist OR thumb sign1
Pectus carinatum deformity2
Pectus excavatum or chest asymmetry1
Hindfoot deformity2
Plain flat foot (pes planus)1
Pneumothorax2
Dural ectasia2
Protrusio acetabulae2
Reduced upper segment to lower segment AND increased arm span to height ratios1
Scoliosis or thoracolumbar kyphosis1
Reduced elbow extension1
3 of 5 facial features1
Skin striae1
Myopia1
Mitral valve prolapse1
Total

A systemic score calculator and a complete description of each component evaluation can be found at the Marfan Foundation website.

Click here for a printable copy (pdf) of this table.

From: FBN1-Related Marfan Syndrome

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