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Hellenic Journal of Surgery

, Volume 83, Issue 1, pp 33–38 | Cite as

A very unusual case of poland syndrome with amastia-athelia and dextrocardia

  • N. Baltayiannis
  • F. Ifantidis
  • Th. Gavressea
  • H. Papatheodorou
  • E. Kayianni
  • Sp. Rizos
Report of a Case

Abstract

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 Words

Poland Syndrome Amastia Athelia Dextrocardia Brachydactyly Syndactyly 

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References

  1. 1.
    Briner V, Thiel G: Hereditäres Poland-Syndrom mit Megacalicose der rechten Niere. Schweiz Med Wochenschr,1988;118:898.PubMedGoogle Scholar
  2. 2.
    Lord MJ, Laurenzano KR, Hartmann Jr RW: Poland’s syndrome. Clin Pediatr (Phila) 1990;29:606–609.CrossRefGoogle Scholar
  3. 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
  4. 4.
    Versaci AD, Balkovich ME, Goldstein SA: Breast reconstruction by tissue expansion for congenital and burn deformities. Ann Plast Surg 1986; 16:20–31.PubMedCrossRefGoogle Scholar
  5. 5.
    Ravitch MM:Poland’s syndrome — a study of an eponym. Plast Reconstr Surg. 1977; 59:508–512.Google Scholar
  6. 6.
    Ribeiro RC, Saltz R, Mangles MG, Koch H: Clinical and radiographic poland syndrome classification: a proposal. Aesthet Surg J. 2009;29(6):494–504.PubMedCrossRefGoogle Scholar
  7. 7.
    David TJ: Familial Poland anomaly. J Med Genet. 1982;19(4):293–296.CrossRefGoogle Scholar
  8. 8.
    Martinez-Frias ML, Czeizel AE, Rodriguez-Pinilla E, Bermejo E: Smoking during pregnancy and Poland sequence: results of a population-based registry and a case-control registry. Teratology. 1999;59(1):35–38.PubMedCrossRefGoogle Scholar
  9. 9.
    Bavinck JN, Weaver DD: Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies. Am J Med Genet. 1986;23(4):903–918.PubMedCrossRefGoogle Scholar
  10. 10.
    Bohun CM, Potts JE, Casey BM, Sandor GG: A populationbased study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol. 2007;100(2):305–309.PubMedCrossRefGoogle Scholar
  11. 11.
    Sepulveda W: Poland syndrome: a rare cause of cardiac dextroposition in the fetus. Prenat Diagn. 2009;29(9):903–905.PubMedCrossRefGoogle Scholar
  12. 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
  13. 13.
    Booth DH, Persaud TV: Congenital absence of the breast. Anat Anz. 1984;155(1–5):29–30.PubMedGoogle Scholar
  14. 14.
    Briner V, Thiel G: Hereditary Poland syndrome with megacalycosis of the right kidney. Schweiz Med Wochenschr. 1988;118(23):898–903.PubMedGoogle Scholar
  15. 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
  16. 16.
    Balachandran G: Klippel-Feil syndrome and anterior cervical meningomyelocele: a rare case report. AJNR Am J Neuroradiol. 2009; 30(9):E130.PubMedCrossRefGoogle Scholar
  17. 17.
    Mishra D, Sinha BP, Kumar R: Goldenhar syndrome with unusual association of pre-axial polydactyly. Eur J Ophthalmol. 2009;19(6):1063–1064.PubMedGoogle Scholar
  18. 18.
    Curcio A, Giuricin M, Lelli D, Falcini F, Nava MB, Folli S: Poland’s syndrome and thoracic duct anomaly. Eur J Plast Surg 2009; 32:155–156.CrossRefGoogle Scholar
  19. 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
  20. 20.
    Haller JA Jr, Colombani PM, Miller D, Manson P: Early reconstruction of Poland’s syndrome using autologous rib grafts combined with latissimus muscle flap. J Pediatr Surg 1984;19:423–429.PubMedCrossRefGoogle Scholar
  21. 21.
    Kim EK, Eom JS, Ahn SH, Son BH, Lee TJ: Evolution of the pedicled TRAM flap: a prospective study of 500 consecutive cases by a single surgeon in Asian patients. Ann Plast Surg. 2009;63(4):378–382.CrossRefGoogle Scholar
  22. 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. 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
  24. 24.
    Baban A, Torre M, Bianca S, Buluggiu A, Rossello MI, Calevo MG, Valle M, Ravazzolo R, Jasonni V, Lerone M: Poland syndrome with bilateral features: case description with review of the literature. Am J Med Genet A. 2009;149A(7):1597–1602.PubMedCrossRefGoogle Scholar

Copyright information

© Hellenic Surgical Society 2011

Authors and Affiliations

  • N. Baltayiannis
    • 1
    • 2
  • F. Ifantidis
    • 1
    • 2
  • Th. Gavressea
    • 1
    • 2
  • H. Papatheodorou
    • 1
    • 2
  • E. Kayianni
    • 1
    • 2
  • Sp. Rizos
    • 1
    • 2
  1. 1.Metaxa Cancer HospitalPiraeusGreece
  2. 2.Tzanion General Hospital of PiraeusPiraeusGreece

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