1 Answers
Poland syndactyly: A unique pattern of one-sided malformations characterizedby a 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-sidedthree 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 nonspecificdevelopmental field defect occurring at about the sixth week of fetaldevelopment. Diminished blood flow through the subclavian artery that goesto the arm has been blamed, but final proof for this idea is lacking. InPoland syndrome there is aplasia of the sternal head of the pectoralismajor. In other words, the end of the main chest muscle that normallyattaches to the breastbone is missing. On that side of the body, nearbychest muscles (the serratus anterior and latissimus dorsi muscles) may alsobe absent as may be the armpit (axillary) hair. In girls, the breast on thatside is also usually absent. The fingers show webbing and shortening(symbrachydactyly) on the hand on the same side. The child with Polandsyndrome usually is entirely normal except for the problems already noted.Upon rare occasions, the Poland syndrome is associated with more severefinger and arm involvement or vertebral or kidney problems. Intelligence isnot impaired by Poland syndrome.
The syndrome occurs sporadically (out ofthe blue) and is not familial. The risk of recurrence of Poland syndromein the family is minute, except in the very small fraction of cases wherethere is a parent or other relative known with Poland syndrome.Reconstructive surgery has in the past been the main recourse. Nowbioengineered cartilage may be implanted to help give the chest a morenormal look. Other names for Poland syndrome include Poland sequence, Polandanomaly, Poland syndactyly, absence of the pectoralis muscle withsyndactyly.
The syndrome is named for Alfred Poland. Born in 1822 in London,he became demonstrator in anatomy in 1839 at Guy's Hospital. There hedissected the body of a deceased convict named George Elt whom he reportedhad Deficiency of the pectoral muscles (Guy's Hosp. Rep. 6:191, 1841).Poland became a celebrated surgeon and ophthalmologist but had to retire in1867 due to a chronic cough.He died in 1872 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.