Poland's Anomaly: Natural History and Long-Term-Results of Chest Wall Reconstruction in 33 Patients.

摘要

Poland's anomaly is an uncommon congenital aberration of the chest wall characterized by absence of the pectoralis major muscle and other nearby musculoskeletal components. In this series, a wide spectrum of thoracic deformities was associated with the Poland anomaly, ranging from segmental agenesis of the ribs, sternum, and nearby muscles, to simple aplasia of the pectoralis major muscle. Although little disability was associated with the syndrome, the patients primarily sought operative correction due to the asymmetry and their perception of adverse cosmesis. Over a 10-year period, 53 operations were performed on 27 individuals with the goal of correcting the abnormal contour of the chest wall. The most successful reconstructions involved the use of the latissimus dorsi muscle, which was detached and transferred to the anterior chest wall while preserving the neurovascular pedicle. In women, this was accomplished by insertion of a mammary prothesis. Reconstruction of the so-called 'herniation' of the lung with the rib grafts or alloplastic materials was found to be unnecessary, and the use of custom-made chest wall prosthesis is not recommended, since four of five of these devices had to be removed due to migration, erosion of local tissues, and adverse cosmesis. Keywords: Reprints; Congenital malformity; Therapy. (kt)