Poland syndrome is a genetic and rare congenital abnormality characterised by unilateral chest wall hyperplasia and ipsilateral hand abnormalities. A cluster of symptoms are usually present, all to varying degrees.
Most commonly, patients notice that they do not have the sternal or the lower part of the pectoralis muscle; this is the largest muscle on the front part of the chest wall; but also may be missing the latissimus dorsi which is the large muscle at the back and may also notice that the breast tissue on the affected side either is smaller than the opposite or fails to develop at all. Not infrequently, the nipple and areolar is significantly smaller and is placed much higher on the affected side than the opposite side.
The cluster of symptoms associated with Poland syndrome may extend into the arm where it is not uncommon to have either general hypoplasia or decrease in size of the hand or the arm or a variety of different syndactyly or symbrachydactyly in which the fingers are fused together. Other less frequent effects of Poland syndrome are hyperplasia or decreased size of the upper ribs, herniation of a lung, a skin web across the anterior axilla and a scapula which is sitting higher than the opposite side.
Surgery is usually conducted in adolescence or in early adulthood and may involve a variety of different muscle transfers to reconstruct the lower part of the pectoralis major muscle. The most common muscle used is the latissimus dorsi which can be transferred either endoscopically or through an open approach. More recently considerable success has been achieved with the sequential augmentation of the soft tissue using fat transfers.
In our experience, custom-made silicone prostheses to reconstruct the pectoralis major muscle in males has not met with the success we had hoped, and not infrequently we find that the prostheses become infected or become painful and more often than not, end up being eventually removed.
Somewhat counterintuitively, management of women with Poland syndrome is significantly easier than with men as we have a large variety of well-made, anatomically shaped breast prostheses which can be inserted underneath the soft tissue and can be used to camouflage or mask the underlying pectoralis major deficiency and abnormality and can be used to reconstruct the breast. By careful placement of a tissue expander and progressive expansion of the upper pole of the breast, it is possible to both enlarge the areolar and move it downwards so it matches the opposite unaffected side. If the areolar remains smaller than the opposite side, its size can be further increased through tattooing of the areolar complex.
If you or one of your family has Poland syndrome and are interested in surgical correction, please do not hesitate to give our rooms a call.