Marfan syndrome (MFS) is a disorder of connective tissue affecting primarily the musculoskeletal system, the cardiovascular system and the eye.
Patients with Marfan syndrome have tall stature, long arms and legs and characteristic changes in the extremities, particularly long and spider-like fingers, an arm span greater than height. There is poorly developed musculature, meager subcutaneous fat, a laxity of the joints and ligaments, and a tendency to double-jointedness.
Frequently there is either a pigeon breast or funnel-shaped deformity of the chest, kyphosis, dorsal scoliosis, flat feet, and hammer toes. The face may present an elderly appearance and a melancholy expression. Mentality is usually normal. The lens of the eye is dislocated and there is a high frequency of glaucoma or retinal detachment. There is a variety of heart abnormalities and malfunctions, some more severe than others. Rupture of the aorta is the commonest cause of death, and the risk is increased during pregnancy. The mean age of survival is 43 years for men and 46 for women.
Pregnant women with Marfan syndrome (MFS) pose a special challenge. because it's associated with the potential for a catastrophic and even fatal acute aortic dissection and the risk of having a child who will inherit the syndrome.Those with advanced syndrome especially a dilated aorta or an aortic aneuryms are at increased risk of getting sick or dying. The mode of delivery depends on the degree of the dilatation of the aorta, the aortic root. More information: