Scoring of systemic features11
| ▸ Wrist AND thumb sign | 3 |
| ▸ Wrist OR thumb sign | 1 |
| ▸ Hind foot deformity | 2 |
| ▸ Pes planus | 1 |
| ▸ Pneumothorax | 2 |
| ▸ Pectus carinatum deformity | 2 |
| ▸ Pectus excavatum or chest deformity | 1 |
| ▸ Scoliosis or thoracolumbar kyphosis | 1 |
| ▸ Dural ectasia | 2 |
| ▸ Protrusio acetbuli | 2 |
| ▸ Reduced US/LS AND increased arm/height AND no severe scoliosis | 1 |
| ▸ Reduced elbow extension | 1 |
| ▸ Facial features – Dolicephaly – Enophalmos – Downslanting palpebral fissures – Malar hypoplasia – Retrognathia | Max 3 out 5 |
| ▸ Skin striae | 1 |
| ▸ Myopia, >3 dioptres | 1 |
| ▸ Mitral valve prolapse | 1 |
| Maximum total 20 points | |
| Score ≥7 indicates systemic involvement | |
US/LS, upper segment to lower segment ratio.