Table 1 Original and revised Ghent nosology
From: Possible extracardiac predictors of aortic dissection in Marfan syndrome
Organ system (involvement) | Major | Minor |
|---|---|---|
Skeletal | The presence of more than 4: | moderate pectus excavatum |
(2 maj OR 1 maj and 2 min) | - pectus carinatum | joint hypermobility |
- pectus excavatum requiring surgical treatment | highly arched palate crowding of teeth | |
- reduced USLS ratio or ASHR greater than 1.05 | Facial abnormalities: | |
- wrist and thumb signs | - dolichocephaly | |
- scoliosis of more than 20° or spondylolisthesis | - malar hypoplasia | |
- reduced extension of the elbows | - enophtalmus | |
- medial displacement of the medial malleolus, pes planus | - retrognathia | |
- protusio acetabuli of any degree | - down-slanting palpebral fissures | |
Ocular | ectopia lentis | abnormally flat cornea |
(2 min) | increased axial length of the eyeball | |
hypoplastic iris, ciliary muscle, decreased miosis | ||
Cardiovascular | ascending aortic dilatation | mitral valve prolapse |
(1 min OR 1 maj) | dissection of the ascending aorta | pulmonary arteria dilatation |
mitral annulus calcification | ||
type B aortic dissection <50 years | ||
Pulmonary (1 min) | spontaneous pneumothorax | |
apical pulmonary blebs | ||
Skin (1 min) | striae atrophicae | |
Dura (1 maj) | Lumbosacral dural ectasia |