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Deformity of chest muscles and breast
Poland syndrome consists of a deficiency of subcutaneous fat
and muscles on one side of the body. It may include under development
of the arm, hand, and fingers on the same side, and may be associated
with other conditions such as Moebius syndrome or Klippel-Feil syndrome.
The right side of the body is affected twice as often as the left.
It is difficult to determine how many individuals are affected
by Poland syndrome, since mild cases may go unnoticed or be misdiagnosed.
One study estimated that it may occur in 1 in 30,000 individuals.
Symptoms
Poland syndrome has several distinctive symptoms:
- Chest muscle deformities - absence of the pectoralis minor and
the breastbone part of the pectoralis major
- Under development or absence of breast or nipple on the affected
side
- Patchy absence of hair under the arm on the affected side
Additional symptoms on the affected side may include:
* Under developed or missing ribs
* Under developed arm, hand, and fingers
* Abnormally short, webbed fingers
* Small, elevated scapula (shoulder bone), called Sprengel deformity
Diagnosis
In mild cases of Poland syndrome, problems with the chest muscles
or breast may not be identified until an individual is older and
seeks treatment.
If Poland syndrome is identified in an individual, he or she will
be examined for the presence of other syndromes such as Moebius
or Klippel-Feil syndromes.
Treatment
In many cases, reconstruction of the chest wall muscles, breast,
and nipple on the affected side can be accomplished by a plastic
surgeon. The existing chest muscles can be used to rebuild the chest.
If there is not enough chest muscle for rebuilding, muscle can be
taken from other parts of the body. In males, chest reconstruction
can be done as early as 13 years old. In females, surgery is postponed
until breast development is complete. Plastic surgeons may also
be able to improve finger deformities through reconstruction and
separating webbed fingers.
Information for this article was taken from:
- National Organization for Rare Disorders. Poland syndrome.
- Wilhelmi, B. J. (2002). Poland syndrome breast. eMedicine, accessed
at http://www.emedicine.com/plastic/topic132.htm
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