Table 2

Scoring of Systemic Features in the Marfan Syndrome

FeaturePoints
Wrist and thumb sign3
Wrist or thumb sign1
Pectus carinatum deformity2
Pectus excavatum or chest asymmetry1
Hindfoot deformity2
Plain pes planus1
Pneumothorax2
Lumbosacral dural ectasia2
Protrusio acetabuli2
Reduced upper-segment to lower-segment ratio (<0.85 in white adults; <0.78 in black adults) and increased arm span–to-height ratio (>1.05) and no severe scoliosis1
Scoliosis or thoracolumbar kyphosis1
Reduced elbow extension1
Facial features (3 of 5): dolichocephaly, enophthalmos, down-slanting palpebral fissures, malar hypoplasia, retrognathia1
Skin striae1
Myopia (>3 diopters)1
Mitral valve prolapse1

Modified with permission from Loeys et al. (2) Copyright © 2010, British Medical Journal Publishing Group.

Maximum total, 20 points; score >7 indicates systemic involvement.