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波兰综合征

Poland syndrome.

作者信息

Urschel Harold C

机构信息

Department of Cardiovascular & Thoracic Surgery, Baylor University Medical Center and University of Texas Southwestern Medical School, Dallas, Texas 75246, USA.

出版信息

Semin Thorac Cardiovasc Surg. 2009 Spring;21(1):89-94. doi: 10.1053/j.semtcvs.2009.03.004.

Abstract

Poland syndrome is characterized by hypoplasia or absence of the breast or nipple, hypoplasia of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, and absence of costal cartilages or ribs 2, 3, and 4 or 3, 4, and 5. The chest wall defect is often associated with a lung hernia. Clinical manifestations are extremely variable and rarely are all the features recognized in 1 individual. Fortunately it is invariably unilateral, allowing for an easier reconstruction. Single-stage reconstruction of the chest wall combined with simultaneous augmentation mammoplasty and transfer of an island pedicle myocutaneous flap of latissimus dorsi muscle are major improvements over previous multiple-stage procedures that provide less satisfactory cosmetic results in management of patients with Poland syndrome.

摘要

波兰综合征的特征为乳房或乳头发育不全或缺失、皮下组织发育不全、胸大肌肋胸骨部分缺失、胸小肌缺失以及第2、3、4或3、4、5肋软骨或肋骨缺失。胸壁缺损常伴有肺疝。临床表现变化极大,很少在1例患者中发现所有特征。幸运的是,该病总是单侧发病,便于进行更容易的重建。与以往的多阶段手术相比,胸壁一期重建联合同期隆乳术以及背阔肌岛状蒂肌皮瓣转移是重大改进,以往的多阶段手术在波兰综合征患者的治疗中美容效果欠佳。

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