Poland Syndrome

Poland syndrome consists of a deficiency of subcutaneous fat and muscles on one side of the body. It may include underdevelopment 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. Poland syndrome does seem to affect males more commonly than females.

Symptoms
Poland syndrome has several distinctive symptoms:

• Chest muscle deformities - absence of the pectoralis minor and the breastbone part of the pectoralis major
• Undedevelopment 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:

• Underdeveloped or missing ribs
• Underdeveloped 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. Computerized tomography (CT) scan may help identify which muscles are involved. In more severe cases, especially those which include hand and arm deformities, Poland syndrome may be identified at birth or shortly after.

If Poland syndrome is identified in an individual, he or she will be exmained 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