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
 Activity intolerance
 Disturbance in self-esteem
 Fear
 Fatigue
 Disturbed body image
 Impaired physical mobility
Treatment
Marfan syndrome has no cure. However,
treatments can help delay or prevent
complications, especially when started early.
Marfan syndrome can affect many parts of your
body, including your heart, bones and joints, eyes,
nervous system, and lungs. The type of treatment
you receive will depend on your signs and
symptoms.
Surgery
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.
Nursing Interventions
1. Observe safety precautions all the time,
keep side rails up all the time.
2. Observe cleanliness and sterility all the
time to avoid possible contamination and
spread of infection.
3. Provide correct information about the
disease process, complications and possible
treatment.
4. Encourage patient to acknowledge and to
express feelings to be relieved from anxiety.
5. Ascertain ability to stand and move about
and degree of assistance necessary or use of
equipment.
6. Discover patient’s inner abilities and help
to improve them to boost up their self-esteem.
7. Provide rest periods, avoid unnecessary
disturbance.
8. Improve other qualities and help the
patient accept their problems and help them
comfort them.

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.