Poland’s syndrome is a developmental abnormality of the upper limb, shoulder, chest and breast area. The exact cause is not known but relates to an abnormality in the development of the shoulder region at an early foetal stage, possibly from a problem with blood supply to that area.
It is sporadic, and not genetic, so children do not inherit the problem. It presents with a wide range of problems although the most classic is an absence of the pectoralis muscles and underdevelopment or absent breast growth. It is equal between males and females.
Other associated problems are chest wall shape abnormalities, shoulder, elbow and hand problems. There are few functional issues when isolated to the chest wall and breast. The common reasons for presentation are contour abnormalities of the chest in males and the same but also lack breast development in females.
Treatment strategies range from purpose built implants to replace the chest wall and muscle absence, fat transfer and movement of muscles from back to replicate the missing pectoralis muscles (done less often). The role of fat transfer and muscle procedures will be discussed if appropriate.
For the treatment of the absent breast, silicone breast implants are used. These can be used in isolation or after a chest wall implant. This will depend on the deformity and patient requirements.
Why 3D custom implants and how does it work?
Stephen McCulley has been treating this condition since 2002 and custom built implants remain a good option. Previously these implants were made with the traditional methods of creating chest wall casts, wax models and then a silicone implant. This has been replaced by use of more modern technology using 3D CT scans, computer modelling of the required changes, 3D printing an implant and then using this as a template to create a bespoke sterile silicone implant for surgical insertion. The scans are done locally and sent to a company in France (www.anatomikmedelling.com) where the 3D print model is created and then sent to Sebbin (www.sebbin.com) in Paris who are a long established implant company making high quality breast implants.
The silicone implant used for Polands Syndrome is made of a solid but malleable silicone and therefore cannot leak. It is designed to sit on the rib cage replacing the contour deficit of the bones and muscles and designed to be permanent. It takes 10 weeks to produce an implant.
This type of implant creates very good changes but is limited by feeling firmer than the muscle it is intended to at least in part replace. It also is not able to completely fill the sulcus or gap at the front of the shoulder often seen in Poland’s patients.
How is the operation performed?
An incision is made on the lateral chest wall just outside of the planned implant position. The exact implant location will be marked and planned using the actual 3D model created in France.
The skin is elevated off the rib cage to create a pocket for the permanent implant so that it is an exact fit. The implant is then inserted and the wounds closed. Drains are sometimes used and a pressure garment will always be applied.
Recovery from custom 3D implants
You will be kept for one or occasionally two nights in hospital. You will wake up with a compression garment over your chest and may have a drain.
Time off work – You would require two weeks from a sit down job up to 6 weeks for a manual job.
Driving – You need to be relatively pain free and in control of your car and should avoid driving for 1-4 weeks depending upon size of surgery.
Sport and exercise – You will be walking well after the first day. Gentle exercise is allowable after first 10 days. High impact sport should be avoided for 8 weeks.
Risks of custom 3D implant
Bleeding – Although much less invasive than other options this still does involve lifting the skin off the rib cage giving a risk of bleeding in first 24 hours. Although uncommon this would require a return to theatre.
Infection – Very uncommon but could result in the loss of the implant or a washout procedure.
Scarring – The scar heals very well in most patients. However scars can become pink and thickened in some and there is a small risk of long-term hypertrophic or keloid scarring.
Altered sensation to skin. Usually short term
Seroma -This is a fluid collection around the implant in the first few weeks after surgery. It is very common even if a drain is used hence many surgeons do not use drains. The fluid is removed with a needle and may need repeating a few times to achieve the final outcome. It is then very rare for it to recur.
Rotation or movement implant – Very uncommon as implant becomes encased in scar tissue and feels firm once in place. However is a small risk.
Replacement of implant – The procedure is designed to be permanent there is some risk the implant may need replacing if there is evidence of it breaking or weakening over the years.
Costs for custom 3D implant including CT scan, Implant planning and production, Operation and all follow-up requirements; £12,100