A very unusual case of poland syndrome with amastia-athelia and dextrocardia
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Poland syndrome is a rare congenital anomaly characterized by unilateral aplasia of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, deformity or aplasia of the costal cartilages or ribs II to IV or III to V, hypoplasia or aplasia of the breast and nipple, athelia, scarcity of subcutaneous tissue, alopecia of the axillary and mammary region and unilateral syndactyly or brachysyndactyly. The prevailing theory regarding its cause is that the hypoplasia of the subclavian artery or its branches may lead to a range of developmental changes.
The congenital unilateral absence of the breast (amastia) and the nipple (athelia) is a very rare deformity and more often encountered in women. We present one very unusual case of Poland syndrome in a woman referred to the Hospital for the treatment of uterine fibroma with amastia-athelia and dextrocardia without complete mirror-image transposition of the adbominal and thoracic viscera and we also review the literature.
Key WordsPoland Syndrome Amastia Athelia Dextrocardia Brachydactyly Syndactyly
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- 3.Ravitch MM: Poland_s syndrome. In: Ravitch MM (ed). Congenital deformities of the chest wall and their operative correction. WB Saunders: Philadelphia, London, Toronto, 1977:pp 233–271.Google Scholar
- 5.Ravitch MM:Poland’s syndrome — a study of an eponym. Plast Reconstr Surg. 1977; 59:508–512.Google Scholar
- 12.Torre M, Baban A, Buluggiu A, Costanzo S, Bricco L, Lerone M, Bianca S, Gatti GL, Sénès FM, Valle M, Calevo MG: Dextrocardia in patients with Poland syndrome: Phenotypic characterization provides insight into the pathogenesis. J Thorac Cardiovasc Surg. 2009 Nov 10.Google Scholar
- 15.Mooney JF 3rd, White DR, Glazier S: Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. 2009;29(1):26–28.Google Scholar
- 19.Sone Y, Wada J, Yokoyama M, Kasagi Y, Saito M, Itaoka T: Surgical treatment of Poland’s syndrome. In: Wada J, Yokoyama M, eds. Chest wall deformities and their operative treatment. Tokyo: AD Printing Inc, 1990:197–208.Google Scholar
- 22.Damen TH, Timman R, Kunst EH, Gopie JP, Bresser PJ, Seynaeve C, Menke-Pluijmers MB, Mureau MA, Hofer SO, Tibben A: High satisfaction rates in women after DIEP flap breast reconstruction. J Plast Reconstr Aesthet Surg. 2008.Google Scholar
- 23.Masia J, Kosutic D, Clavero JA, Larranaga J, Vives L, Pons G: Preoperative Computed Tomographic Angiogram for Deep Inferior Epigastric Artery Perforator Flap Breast Reconstruction. J Reconstr Microsurg. 2009.Google Scholar