Skip to main content

Table 1 Systemic score of Marfan syndrom

From: Marfan syndrome and cardiovascular complications: results of a family investigation

Systemic involvement Points
Wrist and thumb sign 3
Wrist or thumb sign 1
Pectus carinatum deformity 2
Pectus excavatum or chest asymmetry 1
Hindfoot deformity 2
Plain flat foot 1
Spontaneous pneumothorax 2
Dural ectasia 2
Protusio acetabuli 2
Scoliosis or thoracolumbar kyphosis 1
Reduced elbow extension 1
3 of 5 facial features 1
Skin striae 1
Severe myopia (> 3 diopters) 1
Mitral valve prolapse 1
Reduced upper segment / lower segment (US/LS) and increased arm span / height 1
  1. (Facial features: dolicocephaly, malar hypoplasia, enophthalmos, retrognathia, and down-slanting palpebral fissures)
  2. Maximum total: 20 points; a score ≥ 7 is considered a positive systemic score