Marfan Syndrome Treatment

...less medical jargon in a 'Quick Glance' format!

Marfan Syndrome Treatment:
No treatment exists for Marfan syndrome itself. Instead, therapy often focuses on preventing the various complications of the disease. For that reason, the treatment you receive will depend on the nature and severity of your symptoms.

. . . Cardiovascular problems
Two main approaches exist for treating cardiovascular complications:

  • Medications Doctors often prescribe blood pressure lowering drugs to help prevent the aorta from enlarging and to reduce the risk of dissection, even though your blood pressure may be normal. The most commonly used drugs are beta blockers, which cause your heart to beat more slowly and with less force, and reduce your risk of aortic dissection and rupture.
    If you can't tolerate the side effects of beta blockers — which may include unusual tiredness, diarrhea or nausea — your doctor may prescribe a calcium channel blocker, angiotensin-converting enzyme inhibitor or angiotensin receptor blocker instead. Children often receive the same medications as adults, sometimes beginning as early as 2 years of age.
    Doctors may recommend medications for treatment of dissections involving the descending aorta.
  • Surgery If your aorta enlarges quickly or reaches a certain size, your doctor may recommend an elective operation to replace a portion of your aorta and often the aortic valve. If your aortic valve is functioning well, the surgeon may recommend replacement of the aorta only. The extent of the operation depends on how much of your aorta is involved. Some people with Marfan syndrome may require multiple operations.
    In life-threatening situations such as aortic rupture or dissection involving the ascending aorta — the portion arising first from your heart — surgery is the only treatment option.

  • . . . Skeletal problems
    Many of the skeletal problems that affect people with Marfan syndrome also occur in the general population and are treated the same way. They include:

  • Scoliosis For some children and adolescents, doctors recommend a back brace — a custom-made, plastic garment that extends from the shoulders to the waist. Although it can't permanently straighten the spine, it may help stop spinal curvature from becoming worse while children are growing. For that reason, it's worn nearly continuously until growth is complete.
    If the curve in your child's spine is too great, a brace may not be effective, however. In that case, an operation to straighten the spine may be an option. Doctors typically recommend surgical treatment for children or adolescents whose curves are greater than 40 to 50 degrees, and the operation is generally successful in improving both posture and back function.
    The most common surgical procedure for scoliosis is posterior spinal fusion and instrumentation. In this procedure, your surgeon removes pieces of bone from your child's pelvis and places them between two or more vertebrae. Eventually, the vertebrae and the pieces of bone grow together. Metal rods, hooks, screws and wires are inserted to keep the curve from moving until the fusion becomes solid. Scoliosis surgery is one of the longest and most complicated orthopedic surgical procedures performed on children. After surgery, your child will need to curtail normal activities for several months.
  • Concave chest When a concave chest affects your child's breathing, surgery may be an option. The operation consists of raising the sternum and ribs and holding them in place. This may be accomplished using a metal bar, which is later removed. A convex chest doesn't cause functional problems, but it may be a cosmetic concern and can also be corrected surgically.

  • . . . Eye problems
    Because of the eye problems that can occur with Marfan syndrome, doctors usually recommend an annual eye exam for people with the disease. These problems include:

  • Dislocated lens In younger children, a dislocated lens can be treated effectively with glasses or contact lenses that refract around or through the lens. Adolescents who find glasses cosmetically unacceptable or their visual field too restricted may be candidates for a type of intraocular lens implant. This operation should be performed by an ophthalmologist experienced in treating people with Marfan syndrome.
  • Glaucoma Having Marfan syndrome makes you far more likely to develop glaucoma. Glaucoma can't be cured, and damage to your optic nerve caused by the disease can't be reversed. But the disease can be controlled with early detection and treatment. Eyedrops, oral medications and surgical procedures can prevent or slow further damage. You'll need to continue treatment for the rest of your life, and because the disease can progress or change without you being aware of it, your treatment may change over time.
  • Cataracts This clouding of the normally clear lens of the eye is much more common in people with Marfan syndrome than it is in people who don't have the disorder. If you have cataracts, you may want to consider surgery when vision loss starts to interfere with everyday activities. In general, cataract surgery is very successful at restoring vision. But because people with Marfan syndrome are at risk of surgical complications, it's important to have the surgery performed by a doctor familiar with Marfan syndrome.

  • Prevention

    There's no known way to prevent Marfan syndrome. Even if people with the genetic defect that causes the disease choose not to have children, new mutations can occur. Still, if you have Marfan syndrome and are considering having children, you may find it helpful to talk with a medical geneticist or genetic counselor. These professionals can help you and your partner understand the inheritance pattern of Marfan syndrome and the options that are available to you.

    If you have an infant with signs of Marfan syndrome, have your baby monitored closely on an ongoing basis — even if your doctor isn't able to make a definitive diagnosis. Early detection and treatment of the various aspects of the disorder can help your child live an active, productive life.

    What you can do

    Depending on your risk of heart, eye or skeletal complications, your doctor may recommend that you avoid contact sports, such as football or basketball, and other strenuous activities that strain your muscles, ligaments and aorta, such as weightlifting.

    Still, try to remain as active as possible, engaging in noncompetitive activities approved by your doctor — usually ones that allow you to control when you start and stop. Regular exercise helps to control blood pressure and improve muscle tone, which in turn can help prevent some of the complications of Marfan syndrome.


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    ...less medical jargon in a 'Quick Glance' format!