In the mid 19th century, a British surgeon dissected a deceased inmate named George Elt who displayed peculiar symptoms, namely a set of malformations localized to one side of his body. The surgeon was Sir Alfred Poland, and Poland Syndrome, or as it is also known, Poland anomaly, continues to be present in patients today.
Features
Poland Syndrome manifests itself as an absence or underdevelopment of chest muscles on one side the body, with webbed fingers occurring in the same side. Four essential features are necessary for a patient to be diagnosed with Poland Syndrome: the shortening of index, ring and long fingers on one side of the body, syndactyly (webbing) on the fingers of one side, hypoplasia of the hand, and the lack of sternocostal portions of the pectoral muscles on one side.
Causes
There is no confirmed or fully understood cause for Poland Syndrome even after over 150 years. However, physicians speculate that the syndrome develops during the sixth week of fetal development. During this stage the fingers and pectoral muscles are in development, so Poland Syndrome may develop from a disruption of blood flow to that area during this crucial time. Physicians are sure that the condition is not genetic and inheritable.
Symptoms
In addition to the missing pectoral muscles and shortened or webbed fingers, other symptoms can occur with Poland Syndrome. These include an abnormal digestive system, missing fingers or arm bones, and the malformation of several bones in the arm on only one side of the body. In addition, some with Poland Syndrome are born with their heart on the right side of their body instead of the left.
Frequency
The frequency of Poland Syndrome is particularly hard to track because the condition is not often reported or diagnosed. Thus the National Genome Research Institute can only specify that the condition occur between 1 in 10,000 to 100,000 births. This means a child afflicted with Poland Syndrome is born every 1 to 10 days in the United States. The syndrome itself affects boys three times as much as girls, and occurs twice as often on the right side of the body.
Treatment
Luckily, Poland Syndrome can be treated through different types of reconstructive surgery. Webbed or fused fingers can be separated, and breast or chest implants can be used to substitute for missing pectoral muscles after full physical development has been reached. In addition, cartilage can be bioengineered and used in the chest.
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