[Table/Fig-1]:
Systemic score (revised Ghent nosology for the Marfan syndrome)
| Systemic Score | |
|---|---|
| Characteristic | Score |
| Wrist and thumb sign | 3 |
| Wrist or thumb sign | 1 |
| Bulging chest | 2 |
| Hollow chest or thoracic asymmetry | 1 |
| Deformity of the rear foot | 2 |
| Flat foot | 1 |
| Spontaneous pneumothorax | 2 |
| Dural ectasia | 2 |
| Protrusion of the acetabulum | 2 |
| Reduction in the ratio between the length of the upper skeletal segment/lower skeletal segment and increased ratio between the length of the upper limb/lower limb and non grave scoliosis. | 1 |
| Scoliosis or thoracolumbar kyphosis | 1 |
| Reduced elbow extension | 1 |
| Cutaneous striae | 1 |
| Myopia > 3 diopters | 1 |
| Prolapse of the mitral valve (of all types) | 1 |
| Cranio-facial aspects (3/5) from: dolicocephaly, malar hypoplasia, retrognathia, enophthalmos and reduction of the palpebral rima | 1 |