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