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Pectoralis muscle absence with syndactyly: A unique pattern of one-sidedmalformations characterized by a defect of the chest (pectoralis) muscle onone side of the body and webbing of the fingers (cutaneous syndactyly) ofthe ipsilateral hand (the hand on the same side). Often called Polandsyndrome. It is right-sided three times more often than it is left-sided.It is not common, affecting 1 child in about 20,000. For reasons that areunfathomable, Poland syndrome is 3 times more frequent in boys than girls. The cause of Poland syndrome is uncertain. The disorder is currentlyconsidered a nonspecific developmental field defect occurring at about thesixth week of fetal development. Diminished blood flow through thesubclavian artery that goes to the arm has been blamed, but final proof forthis idea is lacking. In Poland syndrome there is aplasia of the sternalhead of the pectoralis major. In other words, the end of the main chestmuscle that normally attaches to the breastbone is missing. On that side ofthe body, nearby chest muscles (the serratus anterior and latissimus dorsimuscles) may also be absent as may be the armpit (axillary) hair. In girls,the breast on that side is also usually absent. The fingers show webbing andshortening (symbrachydactyly) on the hand on the same side. The child withPoland syndrome usually is entirely normal except for the problems alreadynoted. Upon rare occasions, the Poland syndrome is associated with moresevere finger and arm involvement or vertebral or kidney problems.Intelligence is not impaired by Poland syndrome.
The syndrome occurs sporadically (out of the blue) and is not familial.The risk of recurrence of Poland syndrome in the family is minute, except inthe very small fraction of cases where there is a parent or other relativeknown with Poland syndrome. Reconstructive surgery has in the past been themain recourse. Now bioengineered cartilage may be implanted to help give thechest a more normal look. Other names for Poland syndrome include: Poland sequence, Poland anomaly, and Poland syndactyly.
The syndrome is named forAlfred Poland. Born in 1822 in London, he became demonstrator in anatomy in1839 at Guy's Hospital. There he dissected the body of a deceased convictnamed George Elt whom he reported had Deficiency of the pectoral muscles(Guy's Hosp. Rep. 6:191, 1841). Poland became a celebrated surgeon andophthalmologist but had to retire in 1867 due to a chronic cough. He died in1872 at the age of 51 of consumption of the lungs (pulmonary tuberculosis). Although Poland could never have discovered this syndromewithout George Elt, Mr. Elt has not been credited nor has his name ever beenassociated with the syndrome.