Surgery for Marfan syndrome

Marfan syndrome

Marfan syndrome is a disorder which causes damage to connective tissues. Marfan syndrome can affect the heart, blood vessels, skeleton and eyes. It is an inherited syndrome which can be extremely severe or mild. The symptoms of Marfan syndrome may be very tall and slim appearance, unusually long arms and legs (in some cases even fingers and toes), shortsightedness, hums on the heart, curvature of the spine, flat feet, curved palate and problems with eyes. Since there is no cure for Marfan syndrome itself, the treatment is determined according to the particular condition of each patient. Even if the patient does not have any health problems or the symptoms of Marfan syndrome are mild, it is the important for the patient to have regular visits to the doctor. Health problems caused by Marfan syndrome usually progress with age.

Treatment

The aim of the treatment for Marfan syndrome is to prevent worsening of the heart problems which can be life-threatening, and also to reduce the complications with the eyes and bones. Problems with the heart can be managed with medications and aorta surgery. Your doctor will prescribe medications which will reduce the blood pressure to reduce the risk of further complications with the heart. The treatment for the bone problems caused by Marfan syndrome includes special braces or surgery for curved spine. Mild eye problems can be managed with contact lenses or glasses. More serious eye disorders include medications and surgery.

Surgery

Aorta surgery is performed if the diameter of the aorta is much wider than normal. The surgical procedure includes a removal of the wider part of the aorta and replacement with an implant. In traditional method, the mechanic implant is connected to the openings of the aorta. In valve sparing method, the dilated part of the aorta can be replaced with the tube or repaired with the native valve. After the surgery, the patient needs to be hospitalized for about a week. Further home treatment requires about seven weeks. Spine surgery is performed if the curvature of the spine is so severe that it causes further problems. Since eye problems may cause glaucoma, detachment of the retina and cataract, an eye surgery which involves a replacement of the damaged lenses with the artificial ones or repairing the damaged part of the eye can reduce the risk of vision loss.

Complications

The complications of Marfan syndrome include heart failure, vision loss and various problems with the lungs. In addition, Marfan syndrome may cause problems with the pregnancy especially if the woman has heart problems.