Poland syndrome

23.2 Poland syndrome






Introduction


Poland syndrome is a unilateral congenital chest deformity characterized by the absence of the sternal portion of the pectoralis major muscle. The morphological consequence of the missing muscle is that the anterior axillary fold is absent. The incidence of this anomaly ranges from 1 in 10 000 to 1 in 100 000 births. In females the diagnosis is often made only after breast development and patients are often referred to specialists because of breast asymmetry. Ipsilateral breast anomalies range from mild hypoplasia to total agenesia. Breast tissues are often fibrotic since the missing pectoralis major muscle is replaced by fibrotic bands or by a compact fibrous layer. In males, Poland syndrome is often misdiagnosed as a “chest asymmetry” and it is often recognized only after full development. The absence of the sternal component of the pectoralis major and breast anomalies can be associated with one or more of the following malformations:



There is a known male predilection of 2 : 1 and a corresponding right-sided predilection in males of 2–3 : 1. A sidedness predilection does not however exist in females with Poland syndrome.



History


Sir Alfred Poland (1822–1872) was a 19th century British surgeon best known for his description of a congenital deformity described as an underdevelopment or absence of the pectoralis major muscle on one side of the body as well as syndactyly on the ipsilateral hand.


Poland described the condition in 1841, in a paper entitled “Deficiency of the pectoral muscles”.1 This was based on dissections of the body of a deceased convict, Marc DeYoung. These dissections were conducted while Poland was still a medical student. He did not receive the eponym for the condition for more than a century. In 1962, Patrick Wensley Clarkson (1911–1969), a British surgeon operated on a case similar to that of Poland and attributed the description, quite rightly, to Poland.





Treatment/surgical technique



Correction of the thoracic anomaly in males



Reconstruction of the missing muscle


In all groups of patients, the absent sternal head of the pectoralis muscle should be replaced by anterior transposition of the ipsilateral latissimus dorsi muscle. The following minimal incisions are used in these usually young patients:



1. The latissimus muscle flap is harvested through a dorsal incision positioned above the lateral border of the muscle: this 5–8 cm long incision can be either vertically or horizontally oriented. The S-shaped vertical incision proposed in 1985 provides easy visualization of the whole muscle.2,3 However, a straight incision along the brassiere line is easier to hide and is the authors’ current preferred option.4 Through this approach, the entire surface of the anterior fascia of the latissimus dorsi is detached from the subcutaneous layer. The deep surface of the muscle is then freed from underlying structures. Care must be taken at the inferior angle of the scapula in order to avoid dissection under the Teres major muscle. The inferior border of the muscle flap is then severed as distally as possible using bipolar forceps.


2. An incision positioned in an axillary horizontal crease is used to gain access to the posterior axillary fold: the latissimus dorsi muscle is freed until the tendon is reached. The tendon is then transected at its insertion on the humerus. The whole muscle is isolated on the thoracodorsal neurovascular pedicle as in a microsurgical operation. The tendon is then sutured to the anterior bicipital sulcus of the humerus, in the position where the pectoralis major is inserted when anatomy is normal.

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Feb 21, 2016 | Posted by in General Surgery | Comments Off on Poland syndrome

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