Table 1. Prevalence of pathologies of MFS in children and young adults.
Pathology | Prevalence (<18 y) (%) | Prevalence (>18 y) (%) | P (prevalence) |
---|---|---|---|
Dilatation of sinus Valsalva | 73/115 (63.5) | 24/34 (70.6) | ns |
Mitral valve prolapse | 59/115 (51.3) | 19/34 (55.9) | ns |
Ectopia lentis | 21/115 (18.3) | 8/34 (23.5) | ns |
Systemic features | 54/115 (47.0) | 18/34 (52.9) | ns |
Skin striae | 27/115 (23.5) | 19/34 (55.9) | 0.002 |
Dural ectasia | 37/69 (53.6) | 19/31 (61.3) | ns |
Wrist and thumb sign | 52/115 (45.2) | 25/34 (73.5) | 0.016 |
Thumb sign | 34/115 (29.6) | 16/34 (47.1) | ns |
Wrist sign | 32/115 (27.8) | 16/34 (47.1) | ns |
Chest asymmetry | 47/115 (40.9) | 21/34 (61.7) | ns |
Pectus excavatum | 27/115 (32.2) | 8/34 (23.5) | ns |
Pectus carinatum deformity | 25/115 (21.7) | 10/34 (29.4) | ns |
Scoliosis or thoracolumbar kyphosis | 41/115 (35.7) | 19/34 (55.9) | ns |
Reduced elbow extension | 11/115 (9.6) | 10/34 (29.4) | 0.011 |
Protrusio acetabuli | 9/115 (7.8) | 2/34 (5.8) | ns |
Reduced upper segment/lower segment ratio and increased arm/height and no severe scoliosis | 48/115 (41.7) | 21/34 (61.8) | ns |
Plain pes planus | 61/115 (53.0) | 22/34 (64.7) | ns |
Hindfoot deformity | 30/115 (26.1) | 5/34 (14.7) | ns |
Facial features (3/5) (dolichocephaly, enophthalmos, downslanting palpebral fissures, malar hypoplasia, retrognathia) | 31/115 (27.0) | 13/34 (38.2) | ns |
Myopia >3 diopters | 21/115 (18.3) | 3/34 (8.8) | ns |
Pneumothorax | 4/115 (3.5) | 4/34 (11.8) | ns |
MFS, Marfan syndrome; y, years; ns, not significant.