Poland Syndrome
What is Poland syndrome?
Poland syndrome is a rare disorder that is characterized by underdeveloped or entirely missing muscles on one side of the body. Severity and extent vary widely among affected individuals, but typically the chest, hand, shoulder, and arm are impacted.
The incidence of Poland syndrome is estimated to be one of every 20,000 births, although this number may be skewed by the number of people who go undiagnosed. Males are twice as likely to be affected by this condition than females.
What are the symptoms of Poland syndrome?
Age of onset, severity, and extent of symptoms can vary widely among affected individuals. Symptoms include:
- Underdeveloped or entirely missing muscles
- The pectoralis major is most commonly impacted, which may cause the chest to appear concave
- Other muscles that may be affected are in the side, chest wall, and shoulder
- Rib cage abnormalities
- Sparse and/or abnormally placed armpit hair
- Nipple and breast abnormalities
- Brachydactyly
- Syndactyly
- Vestigial fingers
- Shortened bones in the forearm
- Abnormalities of internal organs like the heart, kidneys, and lungs
- This is a very rare and severe symptom
What causes Poland syndrome?
Medical professionals do not know what causes Poland syndrome, but they suspect that it might be a disruption of blood flow during prenatal development.
How is Poland syndrome diagnosed?
Diagnosis typically happens at birth, as doctors will notice the characteristic symptoms. They may use a number of tests to aid in diagnoses, such as a CT scan, X-ray, and clinical evaluation.
What are the treatments for Poland syndrome?
Treatment is symptomatic; a team of specialists should work to address the specific symptoms present in each patient. Plastic surgery, physical therapy, and surgery to correct skeletal abnormalities are often used.
