Table 2 Clinical scoring system for CCA
From: A clinical scoring system for congenital contractural arachnodactyly
Clinical feature | Sensitivity (%) | Specificity (%) | PPV (%) | NPV (%) | Accuracy (%) | Chi2 (p) | Score |
|---|---|---|---|---|---|---|---|
Crumpled ears | 78.0 | 41.8 | 33.3 | 83.6 | 51.7 | 0.024 | 3 |
Arachnodactyly | 97.6 | 17.7 | 30.6 | 95.2 | 39.4 | 0.015 | 3 |
Camptodactyly | 92.3 | 25.7 | 30.0 | 90.6 | 42.8 | 0.021 | 3 |
Contractures of large joints | 88.1 | 25.4 | 30.3 | 85.3 | 42.3 | NS | 3 |
Pectus deformity | 40.5 | 61.5 | 30.0 | 71.8 | 55.5 | NS | 2 |
Dolichostenomelia | 50.0 | 60.0 | 45.5 | 64.3 | 56.0 | NS | 2 |
Kyphoscoliosis | 61.5 | 47.9 | 32.4 | 75.4 | 51.9 | NS | 1 |
Muscle hypoplasia | 54.5 | 27.1 | 34.0 | 46.4 | 38.3 | NS | 1 |
Highly arched palate | 66.7 | 45.8 | 34.8 | 76.0 | 52.1 | NS | 1 |
Micrognathia | 34.3 | 52.2 | 21.8 | 67.1 | 47.2 | NS | 1 |
