Poland Syndrome

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Classification according to ICD-10
Q79.8 Other congenital malformations of the musculoskeletal system
ICD-10 online (WHO version 2019)
Missing left pectoralis muscle
Malformation of the right breast

The Poland syndrome (named in 1841 after the English surgeon Alfred Poland has described the 1841 syndrome) is a complex malformation with absence of a chest muscle and malformation of the ipsilateral breast.

Epidemiology

Poland syndrome has an incidence between 1: 10,000 and 1: 100,000, is more common in men than in women, and more common on the right side of the body than on the left. Most cases occur sporadically, but familial clusters have also been reported. The etiology is not fully understood.

causes

It is an inhibitory malformation that has its origin in the embryonic period. When the upper ray sprouts, there is an inhibition of varying degrees for unknown reasons, which determines the later clinical picture. Occasionally there are also malformations of internal organs or of the lower beam, but always on the same half of the body.

Symptoms

The malformation of the mammary gland and large pectoral muscle (M. pectoralis major) is obligatory. The breast on the affected side is always smaller than that of the opposite side. It can also be completely absent, but then at least one nipple is created. The chest is higher, the areola is smaller and darker. Structurally, the breast is rich in connective tissue and poor in adipose tissue, which gives it a firm, girlish appearance. The opposite side is richer in fat and therefore hangs more and more. This is important for the success of the reconstruction. The two lower parts of the large pectoral muscle, which can be divided into three parts, are almost always missing, the upper part is also rarely missing, and the small pectoral muscle (M. pectoralis minor) may also be missing.
The other symptoms vary in frequency:

  • Finger malformations such as syndactyly of fingers, absence of fingers 4 and 5
  • Shorter, weaker arm
  • Rib malformations, the whole side of the chest (thorax) can be smaller.
  • If the left side is affected, the heart's movement may be impeded in very rare cases.

treatment

Design of a 3D custom-made implant for Poland Syndrome

The health of those affected is usually little impaired. The missing parts of the chest muscle are well compensated for by other muscles. The cosmetic impairment caused by the uneven breasts usually leads to treatment. Depending on the extent, an internal examination should also be carried out.

The breast correction includes:

Adjusting the breast size by enlarging the affected side, depending on the situation also tightening or reducing the size of the opposite side with a reduction of the areola. The breast is enlarged with a silicone prosthesis; custom-made inlays can also be used to balance the breast muscle. The implant is designed using CAD (Computer Aided Design). A 3D reconstruction of the patient's chest is created by a medical scanner to design a virtual implant that is perfectly adapted to each patient's anatomy. The implant is made of unbreakable, medical grade silicone.

Poland Syndrome, treated with a 3D custom made implant

The operation is performed under general anesthesia and takes less than an hour. The surgeon prepares the site for the size of the implant after making an 8 cm axillary incision and inserts the implant under the skin. The closure takes place in two levels. The implant replaces the pectoralis major muscle so that the thorax is symmetrical and, in women, the breast as well.

The balance with the body's own skin-fat cloths (e.g. DIEP cloths) is possible. However, since these are young patients, it should be considered whether the first operation should not be carried out with a silicone implant and whether the switch to the body's own tissue should only take place in older patients.

The structural difference between the two breasts is retained, which means that, in the long term, the same volume but no shape can be achieved. Some doctors therefore recommend subcutaneously removing the breast on the opposite side and also replacing it with a prosthesis.

The probability of a Poland syndrome is between 1: 20,000 and 1: 100,000, depending on the severity of its severity.

In contrast to Poland's syndrome, Amazon's syndrome refers to the sole absence of the breast anlage ( mammary gland ), although the pectoralis muscle is present. The name goes back to the Greek mythology, according to which the women of the Amazons should have cut off a breast for better handling of bows and arrows.

Poland syndrome occasionally occurs together with Möbius syndrome .

Individual evidence

  1. ^ A. Poland: Deficiency of the pectoral muscles , in: Guy's Hospital Reports VI: 191-193
  2. ↑ A. Fokin, F. Robicsek: Poland's syndrome revisited. 2002 in: Annals of Thoracic Surgery 74 (6): 2218-25. doi : 10.1016 / S0003-4975 (02) 04161-9 . PMID 12643435
  3. ↑ R. Slezak and M. Sasiadek (2000): "Poland's syndrome", Pol Merkur Lekarski 9 (50), 568-71 (article in Polish).
  4. ↑ J.-P. Chavoin; A. André; E. Bozonnet; A. Teisseyre; J. Arrue; B. Moreno; D. Glangloff; JL. Grolleau; I. Garrido (2010). "Mammary implant selection or chest implants fabrication with computer help". Annales de chirurgie plastique esthétique. 55: 471-480.
  5. ↑ A. Chichery; F. Jalbert; L. Foucras; J.-L. Grolleau; J.-P. Chavoin (2006). "Syndrome de Poland". EMC - Techniques chirurgicales - Chirurgie plastique reconstructrice et esthétique. 1 (3): 1.

literature