Poland Syndrome

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Hands of a child with Poland syndrome. The right hand (at left) is underdeveloped and significantly smaller. Poland syndrome is a congenital (present at birth) condition where the chest muscles on one side are absent or underdeveloped. There are often also arm and hand abnormalities on the same side of the body, such as underdevelopment, and fusion of the fingers. Poland syndrome is a rare condition that is thought to be caused by disruption to blood flow during embryonic development. It is more than twice as common in males as females.

Poland Syndrome is a rare congenital disorder that typically affects the development of muscles on one side of the body, most commonly the chest and upper limb. The condition is named after Sir Alfred Poland, who first described it in 1841. It is usually present from birth and varies in severity, but often includes underdeveloped or missing muscles, bones, and other structures.

Symptoms of Poland Syndrome

The most common signs and symptoms include:

  • Underdeveloped or missing chest muscles: The pectoralis major muscle (the large muscle in the chest) is often underdeveloped or missing, usually on one side.
  • Asymmetry: The affected side may have less body hair and the nipple or breast may be underdeveloped or absent.
  • Upper limb abnormalities: The hand on the affected side may have shortened or webbed fingers (syndactyly).
  • Underdeveloped or missing ribs: In some cases, ribs on the affected side may also be underdeveloped.

The condition predominantly affects males more than females and typically impacts the right side of the body.

Causes of Poland Syndrome

The exact cause of Poland Syndrome is unknown. However, it is believed to be due to a vascular disruption during embryonic development. Specifically, it is thought that the blood supply to the chest and arm is reduced during the sixth week of fetal development, affecting the growth of tissues in these areas.

Poland Syndrome is not usually inherited, and the majority of cases occur sporadically without a family history.

Treatment for Poland Syndrome

Treatment depends on the severity of the symptoms and the specific areas affected. It typically focuses on improving function and cosmetic appearance.

  1. Reconstructive surgery: This is the most common treatment, especially for those with chest or breast asymmetry. Options include:
    • Chest muscle reconstruction: Using muscle or tissue from other parts of the body to rebuild the chest.
    • Breast reconstruction: For females, breast implants or tissue expanders can be used to correct asymmetry.
    • Hand surgery: In cases with hand abnormalities, corrective surgery can improve function and appearance.
  2. Physical therapy: This may help improve muscle strength and function, especially for people with limited mobility in their arm or hand.
  3. Prosthetics: In cases of significant hand or finger deformities, prosthetics may be used.

Prevention

There is no known way to prevent Poland Syndrome, as the cause is still unclear. Since it is not inherited, genetic counseling is generally not necessary unless there are other related conditions present.

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