Poland’s Syndrome

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Poland’s syndrome is a rare nonfamilial disease characterized by ipsilateral ( one sided) absence or underdeveloped chest wall muscles( Pectoralis major, pectoralis minor ), ribs cartilage and breast tissue with weebing of the fingers of the hand on the same side. The name Poland’s syndrome come from the British anatomist Sir Alfred Poland in 1841.

What causes the Poland’s syndrome?

The exact cause of this syndrome is still unknown but believed that it results from vascular development anomaly during sixth week of gestation, with undeveloped  subclavian artery causing musculoskeletal abnormalities .

What are the symptoms and signs of Poland’s syndrome ?

  1. Absence or underdevelopment of chest muscles( Pectoralis major and pectoralis minor)
  2. Hypoplasia of the breast
  3. Hypoplasia of costal cartilage (rib bone)
  4. Rib cage abnormalities
  5. Absence or decreased of subcutaneous fat and axillary hair.
  6. Upper extremity abnormalities, short forearm, arm and fingers (brachysymphalangism)
  7. Gastrointestinal tract abnormalities.
  8. Liver and biliary tract abnormalities
  9. Underdevelopment of kidneys
  10. Small head .

How is Poland’s syndrome diagnosed ?

Diagnosis relies on clinical findings and radiological imaging .

X-ray, CT and MRI is used to determining chest wall and rib cage abnormalities.

How is Poland’s syndrome managed ?

Reconstruction surgery is the only treatment option for Poland syndrome.

In reconstruction surgery , remaining chest wall muscle or muscle from other parts of the body is used to rebuild the chest wall.

References:

  1. Sabiston textbook of surgery 18th edition
  2. The Washington manual of surgery, 5th edition.
  3. emedicine.medscape.com
  4. http://www.medicinenet.com
  5. http://www.diepflap.com
  6. http://en.wikipedia.org

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