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Poland syndrome

What is Poland syndrome?

Poland syndrome, also called Poland syndactyly, Poland sequence, or Poland abnormality, is a rare birth defect characterized by the absence (hypoplasia) or underdevelopment (agenesis) of the pectoralis major muscle on one side of the body. .

Sometimes accompanied by malformations of the hand.  “Webbing” (fusion) of the fingers (cutaneous syndactyly) of the hand on the same side. It is more common on the right side of the body. In 70%, is more frequent in men than in women in a proportion of 75% -25%.

In 1841, Alfred Poland, a 19-year-old medical student at Guy’s Hospital (London) published the classic description of the syndrome that bears his name. It affects one in 20,000 / 32,000 live births.

  • Hypoplasia (absence) of subcutaneous cell tissue.
  • Hypoplasia of the mammary gland.
  • Hypoplasia of the areola and nipple.
  • Absence or decrease of axillary hair of the torso.
  • Hypoplasia of other muscles of the chest.
  • Hypoplasia or abnormalities (dysplasia) in the anterior segment of the ribs.

In 15% of cases Poland syndrome coexists with Moebius syndrome, which is characterized by facial paralysis, inability to smile or frown, or move the eyes from side to side.

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What causes Poland syndrome?

The cause of Poland syndrome is unknown. However, a disruption of embryonic blood from the arteries below the clavicle (subclavian arteries) around the sixth week of management (46th day of embryonic development) is the prevailing theory. This theory is reinforced by presenting patients, in some cases, vascular alterations and Klippel Trenaunay syndrome.

These deficiencies are largely aesthetic, with the most common (simple) form presenting as unilateral absence of the sternocostal head of the pectoralis major muscle. The deformity can also be complex, with ipsilateral absence of ribs, axillary fold, and distortions of the hemitorax. The complex may also incorporate a variety of ipsilateral trunk defects and upper limb defects, including agenesis of the anterior portions of ribs two to five, deformity of the sternum, and absence of latissimus dorsi, serratus, external and internal abdominal obliques. The breast tissues may be small or absent, and the areola and nipple complex may be small, slightly pigmented, and displaced towards the armpit.

How can Poland syndrome be treated?

Poland syndrome in women

In the case of women, the simplest cases can be successfully treated with a breast prosthesis. However, this option can accentuate subclavicular hollowness and, due to the natural narrowness of the parasternal tissues, the implant can migrate to this space. That causes a high malposition. Adjuvant breast implant treatments may include a silicone implant that repairs the chest contour and customized with CT, a TRAM or DIEP flap, and / or a latissimus (latissimus dorsi) muscle transfer, all of which can provide shape and volume to the upper poor wall.

Fat grafting is also a good option to hide the contour of the implant and achieve better symmetry. Another option is the transfer of the latissimus dorsi muscle, a technique that is used when we need to disguise the prosthesis or the expander. Its main advantage gives an adequate shape to the axillary area. The downside is that sacrificed muscle can atrophy in the long run, losing volume.

Poland syndrome in men

In men, the goal is to replace the missing pectoral muscle, and the best technique is to transfer the latissimus muscle to the chest. Although this is considered to be the best option, most patients do not accept using a healthy muscle for this, so in these cases we use a pectoral implant. We currently use custom implants.

We are the only Spanish reference center in Anatomik Modeling. The Anatomik Modeling reference centers have received theoretical and practical training on the computer construction of 3D implants and the different intervention techniques (pectus excavatum, Poland syndrome or other lack of muscles). These centers already carry out different types of interventions.

Fat grafting can be used to disguise the inevitable implant edges that can be seen subcutaneously and to fill in the axilla, which is not corrected by the implant. .

In patients with excess fat in the abdomen and in the healthy chest, autologous fat is obtained from these areas, so its appearance is improved by eliminating excess tissue, and  using this autologous fat for further contouring

In complex cases where there are missing ribs, the defect is treated with a synthetic mesh and the implants are not used because there is no firm bottom. In these cases, autologous tissues are the option of choice for reconstruction.

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