Sporadic.

Unilateral association from:

-        congenital absence of the pectoralis major (or at least its sterno-costal portion), often accompanied by hypoplasia of the  pectoralis minor

-        hypoplasia or aplasia of the mammary gland (in > 30% of the female subjects)

-        hypoplasia or aplasia of the ribs II-IV or III-V

-        ipsilateral upper limb anomaly: from hypoplasia of some phalanxes to syndactyly (mittens hand) or even the absence of hand (ectrodactyly).

Predominantly male (2 to 3:1) and on the right side of the body (60 %) in sporadic cases; no predominance of sex in familial cases (rare). Probable etiology: hypoplasia of the subclavian artery or some of its branches (which explains the variability in clinical expression).

Occasionally associated: renal aplasia, dextrocardia, scoliosis.

Moebius syndrome is associated with Poland syndrome in 15% of cases.

Significant risk of cancer: leukemia, non-Hodgkin's lymphoma, cancer of the neck, breast (on the side of the anomaly) or lung.

Anesthetic implications:

in case of agenesis of part of the rib cage, paradoxical breathing worsens under general anesthesia and requires assisted or controlled ventilation.

References : 

• Sethuraman R, Kannan S, Bala I, Sharma RK. 
  Anaesthesia in Poland syndrome. 
  Can J Anesth 1998; 45: 277-9.
• Kupper HJ. 
  Anesthesia in Poland’s syndrome. 
  Can J Anaesth 1999; 46: 513-4.
• Fokin AA, Robicsek F. 
  Poland’s syndrome revisited. 
  Ann Thor Surg 2002; 74:2218-25.

Updated: October 2018