Table 2 Revised Ghent nosology

3

OR

Wrist AND thumb sign

I

Aortic involvement AND Ectopia lentis

1

Wrist OR thumb sign

II

Aortic involvement AND FBN1 mutation

2

OR

Pectus carinatum

III

Aortic involvement AND Systemic involvement

1

Pectus excavatum OR chest asymmetry

IV

Aortic involvement AND Family history

2

OR

Hindfoot deformity

V

Ectopia lentis AND FBN1 mutation

1

Plain pes planus

VI

Ectopia lentis AND Family history

2

Pneumothorax

VII

Systemic involvement AND Family history

2

Dural ectasia

2

Protusio acetabuli

1

USLS↓ AND ASHR↑ AND no scoliosis

1

Scoliosis OR thoracolumbar kyphosis

1

Reduced elbow extension

1

Facial abnormalitis (see above)

1

Striae atrophicae

1

Myopia > 3 diopters

1

Mitral valve prolapse