Table 2.
Prevalence of the individual features in 17 patients with Marfan syndrome diagnosed in Olmsted County from 1976 to 2005
| Study parameter | Fulfilling Ghent (N = 17) |
|---|---|
| Skeletal system involved | 10 |
| Reduced upper to lower segment ratio (<0.85) or arm span to height ratio >1.05 | 8 |
| Wrist and thumb signs | 3 |
| Scoliosis of >20° or spondylolisthesis | 2 |
| Medial displacement of the medial malleolus causing pes planus | 2 |
| Marfanoid habitus, not otherwise specified | 1 |
| Ocular system involved | 11 |
| Ectopia lentis | 4 |
| Othera | 11 |
| Cardiovascular system involved | 15 |
| Dilatation of ascending aorta | 12 |
| Dissection of ascending aorta | 3 |
| Mitral valve prolapse with or without mitral valve regurgitation | 9 |
| Dilatation of descending thoracic or abdominal aorta at <50 years | 3 |
| Dissection of descending thoracic or abdominal aorta at <50 years | 1 |
| Lungs involved | 1 |
| Spontaneous pneumothorax | 1 |
| Skin and integument involved | 3 |
| Recurrent or incisional hernia | 1 |
| Striae atrophica from puberty | 3 |
| Genetics | 11 |
| Having parent, child, or sibling who meets diagnostic criteria independently | 11 |
| Presence of a mutation in FBN1 known to cause Marfan syndrome | 2 |
Non-criteria-fulfilling ocular involvement: myopia, hyperopia, astigmatism, strabismus, retinal detachment, amblyopia, and blurred vision.