Poland Syndrome

Poland syndrome is a rare disorder that is thought to be caused by kinking of the of one of the main arteries to the chest and arm (subclavian artery) during the 6th week of pregnancy leading to five main problems, which can be more or less severe. It was first described by Alfred Poland in 1841.

What is Poland’s Syndrome?

Poland’s Syndrome is thought to occur in every 1:10,000 to 100,000 births but this is not clear as some people are affected only mildly and may never seek medical attention. At the other end of the spectrum Poland’s Syndrome can result in severe chest wall, arm and hand deformities requiring significant reconstructive surgery. On this website we will focus on the breast and chest wall problems but at the St Andrew’s Centre we also can provide a service for those with associated hand issues.

Common features include:

Underdevelopment of part the chest wall muscle (Pectoralis major) and ribs

Underdevelopment of the breast in females

Underdevelopment of hand and forearm

Shortening of the fingers and thumb, mainly index, long and ring fingers

Fusion of the shortened fingers (Syndactyly)

Other less common features include:

Contraction of anterior armpit fold

Absence of other muscles around the chest wall and shoulder

Rib deformities and spinal curvature to one side

What happens when I come to the St Andrew’s Centre

If you think you have Poland’s Syndrome then please visit your GP who will examine you and then refer onto us if appropriate. Here you will be seen by one of our medical team who will take a family and personal history, detail your problems and then examine you. After this you may be asked to have a follow up visit with our specialist breast reconstruction nurses to discuss things further and see some pictures.

Deformities can be considered as mild moderate or severe and we have various options for each. Though males are affected more than females, more women seek attention due to the problems with breast development. Once we have defined the problem and options for treatment then you will be able to choose the right choice for you.

Mild Deformities

If the breast is very underdeveloped (hypoplastic) then this is not an option and the breast volume needs to be increased. Typically, a tissue expander is placed under the breast and the volume increased over some weeks. Expanders are silicone breast implants that have a central chamber which can be filled with saline (salty water) to increase their size. A tube comes out the back of the implant and connects to a 2-3cm rubber, self-sealing button, that is placed under the skin in the armpit area. It is possible then to inject this port in clinic with 50 – 100 mls of saline to achieve breast expansion. (This procedure is usually no more uncomfortable than having a blood test or vaccination). Often a number of repeat expansions are required over a period of weeks to attain the desired volume. This expander can be left in place or converted to a definitive silicone breast implant at a later date when skeletal growth as ceased around the age of 18.

If the anterior armpit (axillary) skin fold is absent due to lack of the underlying pec major muscle then this can be reconstructed using the latissimus dorsi muscle from the back, if it is normal. This is mobilised and swung under the armpit and reattached to the upper arm bone (humerus) to mimic the natural fold, however this will leave a large donor site scar on the back.

Typically, Poland’s Syndrome patients are very slim and present to us in their teenage years. Later when they have stopped growing and perhaps have had children the there are other options that become available. The breast can be reconstructed using the patient’s own tissue if they have enough abdominal excess, uncommon in teenagers but very common after pregnancy. In this case, abdominal fat and skin can be transplanted and buried in the chest to provide more breast volume. These procedures (DIEP/MS-TRAM flaps) are commonly employed at St Andrew’s to reconstruct the breast after breast cancer surgery and are described elsewhere in the website. These techniques take 4-6 hours and are appropriate in a small number of cases.

Sometimes the problem includes defects of the ribs and sternum. Custom made firm silicone implants can be used to camouflage the defect and work well. Disadvantages are that the implants do not move as well as the underlying rib cage, edges can be felt and they can displace.

How long will the treatment take?

As Poland’s Syndrome is rare and quite variable the treatment required will be tailored to your individual needs therefore it is best to discuss the surgical course and options in the outpatient setting on a one to one basis.