Poland’s Syndrome

Abstract

Poland’s syndrome is a sporadic, congenital thoracic deformity, mostly unilateral, with a wide spectrum of presentation. The thoracic malformations are distinguished on the basis of the anatomical site in which an embryological development alteration has occurred after the fourth week of gestation (Table 3.1) [1]. Poland’s syndrome is a rare congenital and complex anomaly of the development of thoracic muscles, characterized by hypoplasia of the breast and nipple, scarcity of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, lack of the pectoralis minor muscle, aplasia or deformity of the costal cartilages or ribs II to IV or III to V, alopecia of the axillary and mammary region, anhidrosis, and unilateral brachysyndactyly. The severity of Poland syndrome differs from patient to patient and is often not diagnosed or reported [2]. In 1841 a London medical student, Alfred Poland, reported some unusual anatomic findings, after cadaver dissection, in the chest of a 27 years old man. He noted that the left side of thorax and the left upper extremity of the cadaver were underdeveloped. He wrote “the sternal costal portions of pectoralis major muscle were deficient, the pectoralis minor and serratus magnus muscles were almost absent … and the left hand was shorter than the right” [3]. Lallemand in 1826 and Froriep in 1839 wrote about patients with a similar deformity, but the description by Poland was the most precise and comprehensive. Thomson in 1895 described the full anatomical spectrum of the disease. Clarkson in 1962 coined the name Poland’s syndactyly or “pectoral-aplasia-dysdactylia syndrome.” After the work of Baudinne (1967) the name of this disorder was Poland’s syndrome [4].