Marfan Syndrome Visual difficulties
Flat feet
(Arachnodactyly) Learning disability
Thin, narrow face
Marfan syndrome is a heritable condition that
Hypotonia
affects the connective tissue. The primary
purpose of connective tissue is to hold the Joint laxity
body together and provide a framework for Highly arached palate
growth and development. Crowded teeth
Dislocation of lens
Relatively rare disorder characterized by Malformed cornea
elongation of bones associated abnormalities Retinal detachment
of eyes and cardiovascular system.
Complications
Marfan syndrome affects men, women, and
Dilated aortic root
children, and has been found among people
Dissecting aortic aneurysm
of all races and ethnic backgrounds. It is
estimated that at least 1 in 5,000 people. Aortic rupture
Bacterial endocarditis
Heart failure
Marfan syndrome is named after Antoine
Mitral valve prolapsed
Marfan, the French doctor who first described
Frequent palpations
the disorder in 1896.
High blood
Pneumothorax
Etiology Scoliosis
Marfan syndrome is a genetic disorder. A Diagnostic tests
mutation, or change, in the gene that controls
Physical examination
how the body makes fibrillin causes Marfan
Echocardiogram
syndrome. Fibrillin is a protein that plays a
Eye examination
major role in your body's connective tissue. X-ray
Doppler echo test
Signs and Symptoms MRI
CT scan
Marfan syndrome often affects the long bones of Biopsy
the body. This can lead to signs, or traits, such as: CBC
Blood chemistry
Long, lanky frame Urine analysis
Long thin ribs
Arm span significantly greater than body Nursing Diagnoses
height
Long, spidery fingers (arachnodactyly) Risk for injury
Funnel chest Risk for infection
Scoliosis Knowledge deficit
Anxiety Nursing Interventions
Activity intolerance
Disturbance in self-esteem 1. Observe safety precautions all the time,
Fear keep side rails up all the time.
Fatigue 2. Observe cleanliness and sterility all the
Disturbed body image time to avoid possible contamination and
Impaired physical mobility spread of infection.
3. Provide correct information about the
Treatment disease process, complications and possible
treatment.
4. Encourage patient to acknowledge and to
Marfan syndrome has no cure. However, express feelings to be relieved from anxiety.
treatments can help delay or prevent 5. Ascertain ability to stand and move about
complications, especially when started early.
and degree of assistance necessary or use of
equipment.
Marfan syndrome can affect many parts of your
body, including your heart, bones and joints, eyes, 6. Discover patient’s inner abilities and help
nervous system, and lungs. The type of treatment to improve them to boost up their self-esteem.
you receive will depend on your signs and 7. Provide rest periods, avoid unnecessary
symptoms. disturbance.
8. Improve other qualities and help the
Surgery patient accept their problems and help them
comfort them.
If your aorta stretches, it's more likely to tear (a
condition called aortic dissection). To prevent this,
your doctor may recommend surgery to repair or
replace part of your aorta.
Surgery may involve:
A composite valve graft. For this surgery,
part of the aorta and the aortic valve are
removed. The aorta is replaced with a
man-made tube called a graft. A man-
made valve replaces the original valve.
Aortic valve-sparing surgery. If your aortic
valve is working well, your doctor may
recommend valve-sparing surgery. For
this surgery, your doctor replaces the
enlarged part of your aorta with a graft.
Your aortic valve is left in place.
