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Poland sequence: A unique pattern of one-sided malformations characterizedbya defect of the chest (pectoralis) muscle on one side of the body andwebbing of the fingers (cutaneous syndactyly) of the ipsilateral hand (thehand on the same side). Often called Poland syndrome.
It is right-sided three times more often than it is left-sided. It is not common, affecting 1child in about 20,000. For reasons that are unfathomable, Poland syndrome is3 times more frequent in boys than girls. The cause of Poland syndrome isuncertain. The disorder is currently considered a nonspecific developmentalfield defect occurring at about the sixth week of fetal development.Diminished blood flow through the subclavian artery that goes to the arm hasbeen blamed, but final proof for this idea is lacking. In Poland syndromethere is aplasia of the sternal head of the pectoralis major. In otherwords, the end of the main chest muscle that normally attaches to thebreastbone is missing. On that side of the body, nearby chest muscles (theserratus anterior and latissimus dorsi muscles) may also be absent as may bethe armpit (axillary) hair. In girls, the breast on that side is alsousually absent. The fingers show webbing and shortening (symbrachydactyly)on the hand on the same side. The child with Poland syndrome usually isentirely normal except for the problems already noted. Upon rare occasions,the Poland syndrome is associated with more severe finger and arminvolvement or vertebral or kidney problems. Intelligence is not impaired byPoland syndrome.
The syndrome occurs sporadically (out of the blue) and isnot familial. The risk of recurrence of Poland syndrome in the family isminute, except in the very small fraction of cases where there is a parentor other relative known with Poland syndrome. Reconstructive surgery has inthe past been the main recourse. Now bioengineered cartilage may beimplanted to help give the chest a more normal look. Other names for Polandsyndrome include Poland anomaly, Poland syndactyly, absence of thepectoralis muscle with syndactyly.
The syndrome is named for Alfred Poland.Born in 1822 in London, he became demonstrator in anatomy in 1839 at Guy'sHospital. There he dissected the body of a deceased convict named GeorgeElt whom he reported had Deficiency of the pectoral muscles (Guy's Hosp.Rep. 6:191, 1841). Poland became a celebrated surgeon and ophthalmologistbut had to retire in 1867 due to a chronic cough. He died in 1872 at the ageof 51 of consumption of the lungs (pulmonary tuberculosis). AlthoughPoland could never have discovered this syndrome without George Elt, Mr. Elthas not been credited nor has his name ever been associated with thesyndrome.