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Poland Syndrome



General Considerations

  • Uncommon congenital anomaly characterized by unilateral anomalies of the chest wall and upper extremity
  • Males are more affected than females and the right side is involved more often than left
  • Most likely secondary to a vascular developmental anomaly during the 6th week of gestation

Clinical Findings

  • Functional disability is usually mild
  • All components are rarely present in the same patient, but include
    • Ipsilateral breast and nipple hypoplasia and/or aplasia
      • Involved nipple is smaller and higher than normal
    • Deficiency or absence of subcutaneous fat and axillary hair
    • Absence of the sternal head of the pectoralis major muscle
      • May also include absence of adjacent muscles such as pectoralis minor (common), serratus, latissimus dorsi and external oblique
    • Hypoplasia of the rib cage
      • Usually involves ribs 2 through 5
    • Hypoplasia of the upper extremity

Imaging Findings

  • On chest radiography, it is a cause of a unilateral hyperlucent thorax
  • On mammography, it may manifest as hypoplasia of the breast or of the pectoralis major muscle
  • On CT or MRI, absence of the pectoralis muscles and other anomalies not apparent on chest radiographs may be seen

Associations

  • Well-known association between Poland’s syndrome and Mobius syndrome (bilateral congenital facial nerve palsy with paralysis of the abductors of the eye)
  • May also coexist with Klippel-Feil syndrome
  • Coexist with renal abnormalities like renal agenesis
  • Rare association with leukemia, non-Hodgkin’s lymphoma, cervical cancer and lung cancer

Treatment

  • Cosmetic surgery and reparative may be utilized for chest wall and breast deformities

Poland Syndrome

Poland's Syndrome

Poland's Syndrome. Top: The left hemithorax is more lucent than the right (white arrow). Top and bottom: Axial CT scans through the chest show a normal right pectoralis muscle (blue arrows) and absence of the left pectoralis muscle (yellow arrows).
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Poland Syndrome. eMedicine. BJ Wilhelmi.

Poland’s Syndrome Revisited. AA Fokin and F Robicsek. Annals of Thoracic Surgery. 2002;74:2218