Poland Syndrome is a genetic and rare congenital abnormality characterised by unilateral chest wall hypoplasia 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 is either 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 hypoplasia 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.
Reconstruction using autologe tissue.
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.
Specialised shaped implants designed to fill in the chest wall defect in Poland Syndrome are available. Many men with Poland Syndrome seek this option for reconstruction. Pectoral implants are made of silicone gel and are custom-ordered after a complete history, physical examination and detailed measurements are taken by Dr. med. Strouthou in her office. Insertion of a pectoral implant requires creation of a potentially large scar, usually beneath the breast area or in the armpit region. Because subcutaneous fat in the chest region is also missing, a pectoral implant may have a visible edge and look less natural than using the body’s own tissue.
Free Fat Grafting (“Lipofilling”).
Occasionally, Free Fat Grafting (also known as “Lipofilling”) can help to fill in contour defects above the breast in the upper chest region, where the pectoralis major muscle is missing. Fat can be taken (“harvested”) from one area of the body using a Liposuction wand and vacuum and is transferred to fill in an area that is not corrected by traditional reconstructive methods (for instance, above a breast implant below the clavicle, or collarbone). Fat Grafting is only used in special reconstructive situations and is generally not recommended in routine cosmetic procedures.