Poland syndrome
Overview
Poland syndrome (also Poland's syndrome, Poland's syndactyly, Poland sequence, and Poland's anomaly) is a rare birth defect characterized by underdevelopment or absence of the chest muscle (pectoralis) on one side of the body and (but not always) webbing of the fingers (cutaneous syndactyly) of the hand on the same side (ipsilateral hand) mostly common on the right side of body and found more in males than females. It is usually considered a unilateral condition. Some have claimed that the term can be applied in bilateral presentation, but others recommend using alternate terminology in those cases
Symptoms
The cause of Poland syndrome is not known. The disorder is currently considered "a nonspecific developmental field defect" occurring at about the sixth week of fetal development. Diminished blood flow through the subclavian artery that supplies blood to the arm has been blamed, but final proof for this idea is lacking. Speculation has involved two hypotheses. One proposes that the underlying ribs on the affected side grow too quickly in a forward growth plane and thus reduce the flow of blood in the arteries leading to the overlying pectoralis muscle and arm on the affected side. Another proposal is that a malformation of the embryonic blood vessel serving the pectoralis muscle and arm/hand on that side of the body limits blood flow to these structures. To date no animal research has provided conclusive support for these proposals.
Causes
The cause of Poland syndrome is unknown. However, an interruption of the embryonic blood supply to the arteries that lie under the collarbone (subclavian arteries) at about the 46th day of embryonic development is the prevailing theory.
Treatment
Reconstructive surgery is the main treatment for those with Poland syndrome. Either existing chest muscle or transplanting muscle from another body area may be used to develop symmetry between the affected and unaffected side. If chest-wall ribs are underdeveloped or missing, bioengineered cartilage can be implanted to help give the chest a more normal appearance. Reconstructive surgery may be considered in males as young as 13 years of age. In females, in order to ensure breast similarity in size and character, reconstructive surgery is often postponed until breast development on the uninvolved side has been completed. Therapeutic tattooing can be uses to create the appearance of an areola and nipple.