POLAND SYNDROME

In patients with Poland’s syndrome, some of the most uncomfortable physical alterations are the transversal skin fold in the anterior axillary pillar (caused by the absence or hypoplasia of the pectoral muscles), the infra-clavicular depression, and an anomalous breast contour. The resulting aesthetic derangement is difficult to hide, leads to thoracic asymmetry, and imposes significant psychological trauma and social withdrawal in both men and women.

 

Classic case of complete Poland Syndrome
Classic case of complete Poland Syndrome

 

Historically, in 1841, Alfred Poland presented a 27-year-old patient with unilateral pectoralis major muscle absence and syndactyly on the same side. However, Poland’s syndrome received its name only after Clarkson treated a similar patient in 1962.

Patients with Poland’s syndrome may present with numerous ailments such as absence of the sternal-costal portion of the pectoralis major muscle, upper extremity hypoplasia, brachysyndactyly, and syndactyly. Various other muscles may also be affected: pectoralis minor, latissimus dorsi, serratus anterior, external oblique, and deltoid. Skeletal deformities such as partial agenesis of the ribs, sternum, and spine (sometimes with scoliosis) may occur. Breast hypoplasia or aplasia, nipple abnormalities, skin atrophy, and absence of the sweat glands and surrounding structures are other features.

In Poland’s syndrome, thoracic wall deformities are not as obvious at birth as hand deformities. However, when female patients reach adolescence, the thoracic deformity seems to become more evident as absence or asymmetry of the developing breasts occurs. To minimize this, a tissue expander may be placed in the developing breast to accompany contra-lateral breast growth. Unfortunately, however, surgical treatment of the breast deformities cannot be accomplished before 17-19 years of age, when development of the body is complete.

 

Pré-operatório
Patient with Poland’s Syndrome with silicon prosthesis before the use of Omentum flap.
Pós-operatório com OMENTO
The same patient after the use of Omentum flap over the prosthesis and under the skin. Reconstruction of the right nipple and areola.

Various options have been proposed to reconstruct the breast’s volume, including expanders and implants, transposition of the latissimus dorsi muscle flap (when unaffected by the syndrome), and the rectus abdominis muscle flap when the latissimus dorsi is absent. Although these techniques may achieve excellent results depending on the degree of deformity, satisfaction with aesthetic reconstruction of the anterior axillary pillar and filling of the infra-clavicular depression have been disappointing. Moreover, an additional scar is left in the patient’s donor region of those muscular flaps. Besides a scar, the latissimus dorsi flap also leaves an additional deformity in the dorsal contour due to the absence of muscle filling the posterior axillary pillar.

In an attempt to solve these problems, the laparoscopically harvested omentum flap can be considered an excellent reconstructive option that offers a very interesting aesthetic result in Poland’s syndrome.
The utilization of the omentum flap, in our experience, offers the possibility to treat these deformities with excellent cosmetic results.

 

 

Reconstructive Surgery Center
R. Florêncio Ygartua, 288 / 1004 - Moinhos de Vento
Porto Alegre - RS - Brazil - Phone +55 51 3333.9409