Fokin Alexander A, Robicsek Francis
The Department of Thoracic and Cardiovascular Surgery, Carolinas Heart Institute, Carolinas Medical Center, Charlotte, North Carolina 28203, USA.
Ann Thorac Surg. 2002 Dec;74(6):2218-25. doi: 10.1016/s0003-4975(02)04161-9.
Poland's syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. Literary data suggest its sporadic nature. The prevailing theory of its cause is hypoplasia of the subclavian artery or its branches, which may lead to a range of developmental changes. The incidence of Poland's syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births. Cases of Poland's syndrome associated with leukemia, carcinoma of the hypoplastic breast, and other conditions, confirm the relationship between developmental defects and tumors, and require oncologic awareness. Various manifestations, age, and gender require different surgical approaches. Our experience, which includes 27 patients (15 male, 12 female), 20 of whom (12 male, 8 female) underwent operation, suggests that the repair should be done in two stages in children and in a single stage in adults. Reconstruction and/or stabilization of the aplastic ribs may be achieved using bone grafts or prosthetic mesh. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.
波兰综合征是一种罕见的先天性异常，其特征为单侧胸壁发育不全和同侧手部畸形。文献资料表明其具有散发性。关于其病因的主流理论是锁骨下动脉或其分支发育不全，这可能导致一系列发育变化。波兰综合征的发病率在不同群体（男性与女性患者、先天性与家族性病例等）之间有所不同，在活产婴儿中的发病率为七千分之一至十万分之一。与白血病、发育不全乳房的癌症及其他病症相关的波兰综合征病例，证实了发育缺陷与肿瘤之间的关系，需要肿瘤学方面的关注。不同的表现、年龄和性别需要不同的手术方法。我们的经验包括27例患者（15例男性，12例女性），其中20例（12例男性，8例女性）接受了手术，这表明儿童应分两期进行修复，而成人则可一期完成。可使用骨移植或人工补片来实现发育不全肋骨的重建和/或稳定。肌瓣和乳房植入物可用于纠正肌肉缺损和乳房发育不全，并有助于实现完全的美容修复。
