Bodily systems

Bodily systems for an individual with Marfan syndrome:

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  • The child may be tall and have long limbs
    • Increased height may lead people to treat older than actual age.
  • The child may be very thin and may lack muscle bulk and strength
  • Joints may be hyper extensible and prone to dislocating. They may have joint contractures.
  • Combination of underdeveloped muscles and joint hypermobility can contribute to poor coordination and delay in acquiring gross and fine more skills
  • Scoliosis may limit that range of motion of back
    • May need brace which limits movement even more
  • Chest wall deformities (pectus) are usually just cosmetic, but occasionally may affect normal function of lungs
    • They may require surgery or a brace
    • This may cause problems in lifting, exercise capacity, and range of motion
  • High narrow and highly arched palate
    • May not be able to use standard mouth guard

  • May require glasses
  • Child may have difficulty with visual perception and with hand eye coordination
  • May have difficulty in following the flight of an object
  • May have difficulty to tolerating bright light

  • Children with Marfan syndrome will have regular echocardiograms to monitor the size of their aorta.
    • Spontaneous aortic rupture (without a trauma) is rare in school aged children
  • Exercise modifications and beta-adrenergic blockers medications are part of management
    • They help by reducing the force with which blood is pumped from the heart thus reducing stress on aorta.
    • Beta blockers may cause-fatigue, feeling sleepy, and reduced ability to concentrate.
    • Repair or replacement of mitral valve may be needed in school age child     
    • If child on blood thinners, they may be prone to spontaneous bleeding and easy bruising.

  • The lungs can have weakened areas of tissue that overinflate and can break.  This can cause the lungs to collapse.
    • Improper breathing techniques can put further stress on lungs and may lead to lung collapse
    • Lungs may be underdeveloped with reduced capacity for gaseous exchange.
    • Children with this lung problem may have reduced exercise tolerance