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Subject: What Are the Signs and Symptoms of Marfan Syndrome?
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                  <H1>What Are the Signs and Symptoms of Marfan =
Syndrome?</H1>
                  <P>The signs and symptoms of Marfan syndrome vary from =
one=20
                  person to another, even within the same family. Some =
people=20
                  have mild signs and symptoms, while others may have =
severe=20
                  problems and discomfort. Signs and symptoms occur in =
many=20
                  parts of the body, including:</P>
                  <UL>
                    <LI>The bones and ligaments=20
                    <LI>The heart and blood vessels=20
                    <LI>The eyes=20
                    <LI>The lungs=20
                    <LI>The skin </LI></UL>
                  <H2>Appearance and Body Build</H2>
                  <P>Some of the major signs of Marfan syndrome are the =
common=20
                  physical features seen in people with the condition. =
People=20
                  with Marfan syndrome often have:</P>
                  <UL>
                    <LI>A tall, slender body build. They may be very =
tall or=20
                    taller than other family members who do not have the =

                    condition. However, it should be noted that short, =
heavy=20
                    people also can have Marfan syndrome.=20
                    <LI>Long arms, legs, fingers, and toes. A person=92s =
arm span=20
                    (the distance from the fingertips of one hand to the =

                    fingertips of the other with the arms stretched out =
from the=20
                    sides) may be greater than his or her height.=20
                    <LI>A long and narrow face.=20
                    <LI>A highly arched roof of the mouth with crowded =
teeth.=20
                    <LI>A receding lower jaw, causing an overbite.=20
                    <LI>A protruding or sunken chest.=20
                    <LI>A curved spine.=20
                    <LI>Flat feet that are rotated inward (some people, =
however,=20
                    have exaggerated arches). </LI></UL>
                  <H2>Bones, Cartilage, and Ligaments</H2>
                  <P>The bones of the limbs, hands, and feet often grow =
too long=20
                  in people with Marfan syndrome. This typically leads =
to a=20
                  tall, thin body with disproportionately long arms, =
fingers,=20
                  legs, and toes. People with Marfan syndrome have =
loose,=20
                  relaxed ligaments and are usually loose jointed.</P>
                  <P>Chest abnormalities may occur due to an overgrowth =
of the=20
                  ribs. There are two types of chest abnormalities:</P>
                  <UL>
                    <LI>Pigeon breast, also called pectus carinatum. The =
chest=20
                    protrudes outward like a bird=92s chest. This can =
affect heart=20
                    and lung function.=20
                    <LI>Funnel chest, also called pectus excavatum. The =
chest is=20
                    sunken or indented, reducing the space between the=20
                    breastbone and the backbone. As a result, the heart =
and=20
                    lungs are displaced. Heart and lung function may be=20
                    affected, leading to breathing and endurance =
problems.=20
                  </LI></UL>
                  <P>Curvature of the spine may occur. It usually =
develops=20
                  during childhood, often gets worse during the teenage =
growth=20
                  spurt, and may require surgical treatment. The three =
main=20
                  types of abnormal spine curvature are:</P>
                  <UL>
                    <LI>Scoliosis=97a side-to-side curvature=20
                    <LI>Lordosis=97an inward curvature of the spine in =
the lower=20
                    back, just above the buttocks=20
                    <LI>Kyphosis=97an outward curvature of the spine in =
the upper=20
                    back (hunchback) </LI></UL>
                  <P>Curvature of the spine can cause back pain, posture =

                  problems, and deformity. Scoliosis can sometimes =
reduce lung=20
                  function.</P>
                  <P>Another problem that can occur is dural ectasia=20
                  (ek-TA-ze-a), which is the stretching of the membrane=20
                  surrounding the brain and spinal cord. Dural ectasia =
can wear=20
                  down the bones of the spine over time. Symptoms may=20
                  include:</P>
                  <UL>
                    <LI>Pain in the lowest part of the back=20
                    <LI>Pain in the abdomen=20
                    <LI>Pain, numbness, or weakness of the legs =
</LI></UL>
                  <P>Dural ectasia is a hallmark of Marfan syndrome that =
is very=20
                  rare in the general population.</P>
                  <H2>Heart and Blood Vessels</H2>
                  <P>People who have Marfan syndrome often have problems =
with=20
                  the heart and blood vessels. The valves of the heart =
may not=20
                  work properly and may permit some blood flow to be =
reversed,=20
                  causing the heart to do extra work. The aorta=97the =
large artery=20
                  that carries blood away from the heart to the =
body=97may stretch=20
                  and enlarge. These problems can cause signs and =
symptoms, such=20
                  as:</P>
                  <UL>
                    <LI>A <A=20
                    =
href=3D"http://www.nhlbi.nih.gov/health/dci/Diseases/heartmurmur/hmurmur_=
what.html">heart=20
                    murmur</A>. This can be the result of valves not =
functioning=20
                    properly or other heart problems.=20
                    <LI>Sudden chest pain. This can be a symptom of =
aortic=20
                    dissection, a life-threatening complication that can =
occur=20
                    due to weakened aorta walls. The pain may occur in =
the front=20
                    or in the back, usually in the midline of the body. =
The=20
                    location of the pain may change as the tear in the =
aorta=20
                    grows. In some cases, a dissection does not cause =
dramatic=20
                    pain.=20
                    <LI>Abnormal heart rhythms called <A=20
                    onclick=3D'alert("This link takes you away from the =
DCI Web Site and will open in a new window. To return to the DCI Web =
Site, simply close the window.");'=20
                    =
href=3D"http://www.nlm.nih.gov/medlineplus/ency/article/001101.htm"=20
                    target=3Dnew>arrhythmias</A> (a-RITH-me-as). A =
person with=20
                    Marfan syndrome may develop an arrhythmia as a =
result of=20
                    problems with the heart valves or due to <A=20
                    =
href=3D"http://www.nhlbi.nih.gov/health/dci/Diseases/cm/cm_what.html">car=
diomyopathy</A>=20
                    or <A=20
                    =
href=3D"http://www.nhlbi.nih.gov/health/dci/Diseases/Hf/HF_WhatIs.html">c=
ongestive=20
                    heart failure</A>.=20
                    <LI>Shortness of breath and fatigue (tiredness). =
These can=20
                    be symptoms of heart failure due to an overworked =
heart.=20
                    </LI></UL>
                  <P>Two of the heart and blood vessel complications =
that can=20
                  occur in people with Marfan syndrome are <A=20
                  onclick=3D'alert("This link takes you away from the =
DCI Web Site and will open in a new window. To return to the DCI Web =
Site, simply close the window.");'=20
                  =
href=3D"http://www.nlm.nih.gov/medlineplus/ency/article/000180.htm"=20
                  target=3Dnew>mitral valve prolapse</A> (MVP) and =
enlargement of=20
                  the aorta.</P>
                  <H3>Mitral Valve Prolapse </H3>
                  <P>MVP is a problem with the heart=92s mitral valve. =
The mitral=20
                  valve controls the flow of blood through two of the =
chambers=20
                  in the heart, the left atrium and the left ventricle. =
The=20
                  flaps of the valve are designed to allow blood to flow =
in one=20
                  direction=97from the left atrium into the left =
ventricle=97and to=20
                  prevent blood from flowing the other way.</P>
                  <P>In MVP, the mitral valve does not work correctly. =
The valve=20
                  flaps are large and floppy. They may overlap or not =
close=20
                  completely. One or both flaps may flutter or swing =
back into=20
                  the atrium.</P>
                  <P>The abnormal mitral valve can allow blood to flow =
backward=20
                  through the valve in the wrong direction. This is =
called=20
                  mitral regurgitation. When this happens, the heart has =
to work=20
                  harder to make up for the backward flow of blood. Over =
time,=20
                  the heart can become overworked, leading to heart =
failure.</P>
                  <P align=3Dcenter><IMG height=3D497=20
                  alt=3D"Figure A shows the normal mitral valve =
separating the left atrium from the left ventricle. Figure B shows the =
heart with mitral valve prolapse. Figure C shows the detail of mitral =
valve prolapse. Figure D shows a mitral valve that allows blood to flow =
backward into the left atrium."=20
                  =
src=3D"http://www.nhlbi.nih.gov/health/dci/images/mitralvalve_prolapse.gi=
f"=20
                  width=3D320></P>
                  <P class=3Dcaption>Figure A shows the normal mitral =
valve=20
                  separating the left atrium from the left ventricle. =
Figure B=20
                  shows the heart with mitral valve prolapse. Figure C =
shows the=20
                  detail of mitral valve prolapse. Figure D shows a =
mitral valve=20
                  that allows blood to flow backward into the left =
atrium.</P>
                  <P>MVP occurs in about 3 out of every 4 people with =
Marfan=20
                  syndrome. It also occurs in people who do not have =
Marfan=20
                  syndrome. In many people with MVP, little or no blood =
leaks=20
                  back through the valve, there are no symptoms, and no=20
                  treatment is needed. In some people, blood does leak =
back=20
                  through the valve, and these people may have symptoms =
and=20
                  require treatment.</P>
                  <H3>Enlargement of the Aorta (Aortic Dilation)</H3>
                  <P>The aorta is the large artery that carries =
oxygen-rich=20
                  blood away from your heart out to the rest of the =
body. When=20
                  weak connective tissue causes the walls of the aorta =
to=20
                  weaken, the aorta widens and stretches. Most often, =
the=20
                  enlargement begins where the aorta connects with the =
left=20
                  ventricle of the heart, just above the aortic valve. =
This part=20
                  of the aorta is called the aortic root.</P>
                  <P>Enlargement of the aorta can lead to several =
serious=20
                  complications:</P>
                  <UL>
                    <LI>Aortic root dilation. The aorta can widen and =
bulge=20
                    where it attaches to the heart. This happens due to=20
                    continued weakening of the wall of the aorta. Aortic =
root=20
                    dilation may not cause any symptoms until it becomes =
quite=20
                    large. If symptoms occur, they may include pain in =
the=20
                    chest, upper back, neck, and jaw; coughing and =
hoarseness;=20
                    and trouble swallowing. As part of this dilation, =
one=20
                    section may enlarge and bulge out. This is called an =
aortic=20
                    aneurysm (AN-u-rism). The aneurysm can rupture, =
causing=20
                    massive internal bleeding, which is a =
life-threatening=20
                    medical emergency.=20
                    <LI>Aortic regurgitation. The aortic valve sits =
between the=20
                    left ventricle and the aorta and allows blood to =
flow one=20
                    way, from the ventricle to the aorta. In Marfan =
syndrome, as=20
                    the aorta widens, the aortic valve also is =
stretched, and=20
                    the valve flaps cannot close properly. This allows =
blood to=20
                    leak the wrong way, from the aorta back into the =
left=20
                    ventricle (regurgitation). As a result, the heart =
has to=20
                    work harder to pump blood out to the body. The heart =
can=20
                    become overworked, leading to heart failure. People =
with=20
                    aortic regurgitation may have shortness of breath =
and=20
                    exercise intolerance.=20
                    <LI>Aortic dissection. This is a tear that occurs in =
the=20
                    weakened layers of the inner wall of the aorta. The =
tear=20
                    allows blood to get under the inner layer of the =
wall. As a=20
                    result, the aorta tears further. An aortic =
dissection is a=20
                    life-threatening emergency. Signs and symptoms of an =
aortic=20
                    dissection include abrupt onset of chest pain, which =
may=20
                    range from mild to severe. There may be pain in the =
back or=20
                    the abdomen, or both. The person may sweat, vomit, =
faint,=20
                    and have a weak pulse. </LI></UL>
                  <H2>Eyes and Vision</H2>
                  <P>Nearsightedness (myopia) is the most common eye =
problem in=20
                  people with Marfan syndrome. It can range from mild to =
severe.=20
                  People with Marfan syndrome often have astigmatism=20
                  (a-STIG-ma-tizm), which causes visual distortion and =
blurred=20
                  vision.</P>
                  <P>Dislocation of the lens of the eye (ectopia lentis) =
is=20
                  another common complication of Marfan syndrome. It is=20
                  considered a hallmark of Marfan syndrome because it =
occurs in=20
                  very few other conditions. It may affect one or both =
eyes, and=20
                  the lens may be higher or lower than normal or shifted =
off to=20
                  the side. Vision in the affected eye(s) may be =
severely=20
                  affected.</P>
                  <P>Other, less common eye problems in Marfan syndrome =
include=20
                  detached retina, cataracts, and glaucoma. A detached =
retina=20
                  occurs when there are holes or tears in the inner =
lining of=20
                  the eye. A cataract is a clouding of the lens. =
Glaucoma occurs=20
                  as a result of high pressure in the eye. People with =
Marfan=20
                  syndrome tend to get cataracts and glaucoma at a =
younger age=20
                  than people who do not have Marfan syndrome.</P>
                  <H2>Lungs</H2>
                  <P>In Marfan syndrome, the air sacs in the lungs may =
be larger=20
                  than normal. This can cause breathing problems. People =
with=20
                  Marfan syndrome are at an increased risk of developing =

                  emphysema or <A=20
                  =
href=3D"http://www.nhlbi.nih.gov/health/dci/Diseases/Copd/Copd_WhatIs.htm=
l">chronic=20
                  obstructive pulmonary disease</A> (COPD), a serious =
lung=20
                  disease.</P>
                  <P>Sudden collapse of the lung, called spontaneous=20
                  pneumothorax (noo-mo-THOR-aks) can occur in people =
with Marfan=20
                  syndrome. About 1 in every 20 people with Marfan =
syndrome=20
                  develops this problem. Collapsed lung can happen after =
only a=20
                  slight blow to the chest, or even while at rest =
without a=20
                  known cause. Collapsed lung usually causes sudden =
shortness of=20
                  breath and requires immediate medical attention.</P>
                  <P><A=20
                  =
href=3D"http://www.nhlbi.nih.gov/health/dci/Diseases/SleepApnea/SleepApne=
a_WhatIs.html">Sleep=20
                  apnea</A> is a problem that is often associated in the =
general=20
                  population with being overweight or obese. But thin =
people=20
                  with Marfan syndrome can also suffer from it. This may =
be due=20
                  to looseness of the connective tissue in the =
airways.</P>
                  <H2>Skin</H2>
                  <P>Stretch marks on the skin occur in about 2 out of =
every 3=20
                  people with Marfan syndrome. The stretch marks are =
usually on=20
                  the lower back, buttocks, shoulders, breasts, thighs, =
and=20
                  abdomen. They differ from stretch marks in the general =

                  population because, in people with Marfan syndrome, =
they occur=20
                  without excessive weight gain or loss. The marks =
usually=20
                  appear around the time of puberty, but may occur in =
childhood.=20
                  They do not require=20
        =
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