Table 2: Revised Ghent Criteria: Systemic features score |
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• Wrist AND thumb sign – 3 (Wrist OR thumb sign – 1) |
• Pectus carinatum deformity – 2 (pectus excavatum or chest asymmetry – 1) |
• Hindfoot deformity – 2 (plain pes planus – 1) |
• Pneumothorax – 2 |
• Dural ectasia – 2 |
• Protrusio acetabuli – 2 |
• Reduced US/LS AND increased arm/height AND no severe scoliosis – 1 |
• Scoliosis or thoracolumbar kyphosis– 1 |
• Reduced elbow extension – 1 |
• Facial features (3/5) – 1 (dolichocephaly, enophtalmos, downslanting palpebral fissures, malar hyoplasia, retrognathia) |
• Skin striae – 1 |
• Mitral valve prolapse (all types) – 1 |
• Myopia >3 diopters – 1 |
Maximum total: 20 points; score ≥7 indicates systemic involvement |