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Poland syndrome is a rare birth defect characterized by a deformed chest muscle and shorter, possibly webbed fingers on the same side. It does not affect cognitive function and can be corrected through surgery. The cause is unknown, but it may be related to disrupted blood flow to the fetus. Treatment is mainly through constructive surgery. Other problems related to the syndrome include scoliosis, leukemia, and lymphoma. The syndrome is named after Sir Alfred Poland who first noticed the deformity in 1841.
Poland syndrome is a birth defect characterized by a deformity usually on the right side of the body. It involves a malformed chest muscle and shorter fingers, possibly webbed on the same side. The syndrome does not affect intelligence or cognitive function, only mobility. Constructive surgery can usually correct the abnormalities.
In addition to the absence of a properly formed pectoral muscle and shorter, webbed fingers, there are several other symptoms of Poland syndrome. These could include lack of hair under the arm, missing or underdeveloped ribs, or a deformed shoulder bone. Women who have the disorder might also have a missing breast or a smaller or unformed nipple. Obvious cases are detected at birth, but some cases are never reported or treated. Very mild cases might even go completely unnoticed.
There is no known cure or specific cause for Poland syndrome, also called Poland syndrome, Poland sequence, or Poland anomaly. There is, however, a theory that it is caused by disruption of blood flow to a fetus around six weeks of age. The disorder is extremely rare, with only about 1 in 30,000 people reporting cases. Poland syndrome affects twice as many men as women, and right-sided deformities are three times more common than left-sided.
Underdeveloped pectoral muscle is the most common indicator of the syndrome, but just over 13% of people with pectoral aplasia have Poland syndrome. The same percentage or a lower percentage applies to people who have syndactylism, which is the technical term for webbed fingers. The combination of these factors, however, is a strong indicator of the presence of Poland syndrome.
Treatment of the ailment is mainly carried out through constructive surgery. Webbed toes are separated in early infancy, but thoracic surgery is usually done in the teen years when development has reached maturity. Chest muscle tissue can be taken from other parts of the body to build up the missing parts of the pectoral muscles.
Other problems rarely occur in victims of Poland syndrome, but when they do occur they can be quite serious. Disorders related to Poland syndrome include scoliosis, leukemia, and lymphoma. Sometimes there can be an underformation of the kidney tissues which can lead to serious kidney problems.
In 1841, Sir Alfred Poland noticed the deformity of the pectoral muscles and hand in a patient he was dissecting. He saved the hand specimen and in 1962 Patrick Clarkson found and studied it. Clarkson then published a study of three cases and named the syndrome after Poland.
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