Marfan syndrome is a genetic disorder (autosomal dominant) characterized by connective tissue damage. This syndrome includes multiple malformations of organs (bones, eyes, skin, intestines, heart and lungs).
Marfan syndrome symptoms
Most of the visible signs or symptoms occur in the skeletal system. The patients suffering from Marfan syndrome are taller than patients without the disease and arachnodactyly ( very thin and long fingers). Another anomaly in the skeletal system is pectus excavatus (deformity of the anterior wall of the chest which penetrates inside sternum) and scoliosis (abnormality of the spine that consists of spine deformity in the form of the letter S). Other signs include abnormal joint flexibility and sometimes pain in joint.The most important signs and symptoms associated with Marfan syndrome are the cardiovascular symptoms. This are mitral or aortic valve prolapse (the most common heart valve abnormality) and aneurysm of the aorta. Many patients with Marfan syndrom have some type of vision problems of which the most important are myopia (a visual anomaly in which distant objects appear blurred), glaucoma and cataracts. This can severely influence Marfan syndrome life expectancy.
Marfan syndrome life expectancy
Marfan syndrome life expectancy may be different from patient to patient. There is no treatment for the Marfan syndrome, but life expectancy may be influenced by monitoring and treatment of complications that can occur. Marfan syndrome life expectancy increased lately because of the advancement in medical techniques and appearance of new treatments.Best way to expend Marfan syndrome life expectancy is tracking the aneurysm of the aorta and mitral valve prolapse. This is done by a cardiologist annual control for monitoring the health of the heart valves and the aorta. This is done by performing an ECG or another radiological method for the visualization of the anomaly (such as CT or MRI image). The goal of treatment in Marfan syndrome is to slow the progression of the aortic dilatation and to prevent the dissection of the aorta. Another goal of the treatment is to eliminate arrythmias and diminuate the heart rate.
The skeletal manifestations of this syndrome are also important for Marfan syndrome life expectancy. They can be serious and can affect patients life. To solve these problems are used different kinds of pain medication or muscle relaxants.
Because some of the Marfan symptoms of the spine may be asymptomatic, but serious if are not treated, they can be solved by surgery and thus increase the Marfan syndrome life expectancy.
Lung damage involves spontaneous pneumothorax and Marfan syndrome life expectancy depends on the volume of air in the pleural space. It may resolve by itself or can be made by chest drainage.
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