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Ann Thorac Surg 2002;74:2218-2225
© 2002 The Society of Thoracic Surgeons

Review

Poland’s syndrome revisited

Alexander A. Fokin, MD, PhDa, Francis Robicsek, MD, PhDa*

a The Department of Thoracic and Cardiovascular Surgery, Carolinas Heart Institute, Carolinas Medical Center, Charlotte, North Carolina, USA

* Address reprint requests to Dr Robicsek, Dept. of Thoracic and Cardiovascular Surgery, Carolinas Medical Center, 1001 Blythe Blvd #300, Charlotte, NC 28203, USA
e-mail: tjohn{at}sanger-clinic.com

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.




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D. A. Sonetti, J. Peters, and S. M. Levine
POLAND SYNDROME ASSOCIATED WITH EXTRAGONADAL SEMINOMA
Chest Meeting Abstracts, October 1, 2009; 136(4): 21S - 21S.
[Abstract]

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A. Eroglu, D. Yildiz, and H. Tunc
Dextrocardia is a component of left-sided Poland syndrome
J. Thorac. Cardiovasc. Surg., November 1, 2005; 130(5): 1471 - 1472.
[Full Text] [PDF]


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