Defining Poland Syndrome
Poland syndrome is described as a malformation involving the under-development or complete absence of the chest muscle on one side of the body. In addition, there is often webbing of the fingers on the same side as the chest wall anomaly. Although Poland’s syndrome can affect either side, it is three times more likely on the right side of the body.
Poland syndrome is listed as a rare disease by the US National Institutes of Health. It is not hereditary, and for reasons not yet understood by medical professionals, it is more frequent in men than women.
What causes Poland Syndrome
The exact cause of Poland syndrome is unknown, although some clinicians speculate that it may involve a lack of blood flow through the arteries at a critical point during fetal growth around the 46th week of life. It is thought that the diminished blood flow may be responsible for the malformation as at that time the fetal fingers and chest muscles would be in development. However, there is not yet any clear proof, or concrete clinical studies proving this suggestion.
Clinical Features of Poland Syndrome
There are many physical features of Poland syndrome. However, the main feature is absent chest muscle on one side of the body. Other chest muscles located in proximity and on the same side may also be missing. In women, the nipple and breast on that side may also be totally absent. For both sexes, the fingers will display webbing of various degrees and will be shortened. There may also be absence of the underarm hair on the affected side.
The severity of the syndrome varies in each person born with it; therefore, additional features of the syndrome will vary. Additional deformities of the hands and arms include abnormal bone of the lower or upper arm, arms that are not symmetrical, and fused or missing fingers. Deformity of the shoulder blade is also often seen. Gastrointestinal and liver problems are also experienced, as well as dextrocardia, in which the heart is instead on the right side of the body.
Plastic and Reconstructive Surgery of Chest
Today, the outcome for those born with this syndrome is excellent. For many, Dr. Agha will reconstruct the breast, nipple, and can utilize the existing muscles to rebuild the chest. If there is not enough muscles present for rebuilding, results can be obtained by taking muscle from other parts of the body. If reconstructive surgery is attempted, it can be performed as early as the age of 13 for boys, but should be postponed until breast development is complete in girls. CLICK HERE to learn more about Pectus Excavatum – Chest Wall Deformity.