Poland syndrome: evaluation and treatment of the chest wall in 63 patients.

BACKGROUND

Poland syndrome is a sporadic, congenital unilateral absence of the sternocostal head of the pectoralis major muscle that can occur with other ipsilateral chest wall and limb derangements. The chest wall deficiency is primarily cosmetic, its incidence is unknown, male patients may be affected more than female patients, the right side is affected more than the left, and associated comorbidities may exist. Chest wall repair depends on anatomical type and gender.

METHODS

Sixty-three patients with Poland syndrome were divided into two treatment groups by chest wall anatomy and gender. Surgical repair was based on this division. Seventy-six operations were performed by the senior author (A.E.S.) during a 30-year period, and long-term outcomes are presented. Corrective methods included use of custom-made chest wall prostheses, mammary prostheses, latissimus dorsi muscle transfers, transverse rectus abdominis musculocutaneous flaps, sternal/rib reconstruction, or a combination of methods. Follow-up ranged from 1 to 21 years.

RESULTS

Two anatomical forms of the disorder are described, each with unique surgical requirements. The simple deformity was effectively repaired with a latissimus dorsi muscle transfer plus, in female patients, a sublatissimus mammary prosthesis. Repair of the complex deformity, in addition to the latissimus transfer, selectively included musculoskeletal chest wall realignment. Custom-made chest wall prostheses carried a higher risk of complications.

CONCLUSIONS

Poland syndrome of the chest wall exists in two forms: the more common simple variety and a complex form (as originally described by Poland). Repair of the chest wall can be effectively tailored to these anatomical types, gender, and patient preference.