What is Poland Syndrome?

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, possibly webbed fingers on the same side. The syndrome does not affect intelligence or cognitive functions, only mobility. Constructive surgery can typically correct the abnormalities.

Beyond the absence of a properly-formed pectoral muscle and shorter, webbed fingers, there are many other symptoms of Poland Syndrome. These might include the 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 nipple that is smaller or unformed. 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’s Syndrome, Poland sequence or Poland’s anomaly. There is, however, a theory that it is brought on by interrupted blood flow to a fetus around six weeks of age. The disorder is extremely rare, with only approximately 1 in 30,000 people having reported cases. Poland Syndrome affects twice as many men as women, and deformities on the right side are three times as common as on the left.

The underdeveloped pectoral muscle is the most common indicator of the syndrome, but little more than 13% of people with pectoral aplasia have Poland Syndrome. The same or 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 for the presence of Poland Syndrome.

Treatment of the disorder is mostly done through constructive surgery. Webbed fingers are separated early in childhood, but chest surgery is usually performed sometime in the teen years, when development has reached maturity. Chest muscle tissue can be taken from other parts of the body to construct the missing parts of the pectoral muscles.

Rarely do other problems occur within victims of Poland Syndrome, but when they do, they can be quite serious. Disorders linked to Poland Syndrome include scoliosis, leukemia and lymphoma. Sometimes there can be the underformation of renal tissues which can lead to severe 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 it and studied it. Clarkson then went on to publish a study of three cases, and named the syndrome after Poland.