Surgical and Radiologic Anatomy

, Volume 35, Issue 8, pp 639–646

Poland syndrome: from embryological basis to plastic surgery

  • Vladimir Cingel
  • Martin Bohac
  • Veronika Mestanova
  • Lenka Zabojnikova
  • Ivan Varga
Review

Abstract

Poland syndrome is a rare congenital anomaly described by Sir Alfred Poland over 170 years ago. Combination of unilateral aplasia of the sternocostal head of musculus pectoralis major, and an ipsilateral hypoplastic hand with simple syndactyly and short fingers is typical for this condition. It occurs more frequent among males, and is usually situated on the right hemithorax in the unilateral form. The pathogenesis of Poland syndrome is not clear. Most of the authors assume that the etiologic insult is vascular in nature. During the sixth week of gestation, not only the pectoral mass splits (future muscles of the thorax) and intervening tissue between the finger rays of hands starts to disappear but also the vascular differentiation from six aortic arches begins. In our paper we report two cases of children with Poland syndrome, who underwent surgical procedure in the Department of Pediatric Surgery, Comenius University in Bratislava, Slovakia. Our case reports are focused on pre-operatively and also post-operatively imaging (RTG, CT, and 3D CT imaging) of the affected thorax and arm, as well as the operative reconstruction technique of abnormal ribs. We also discussed the possible embryonic backgrounds of this anomaly as well as the importance of plastic surgery resulting in patients’ normal life.

Keywords

Poland syndrome Case reports Embryology Surgical treatment 3D CT imaging 

References

  1. 1.
    Al-Qattan MM (2001) Classification of hand anomalies in Poland’s syndrome. Br J Plast Surg 54:132–136PubMedCrossRefGoogle Scholar
  2. 2.
    Atasoy HI, Yavuz T, Altunrende S, Guven M, Kılıcgun A, Polat O, Yesiller E, Duzenli S (2012) A unique case of right-sided Poland syndrome with true dextrocardia and total situs inversus. Eur J Pediatr (Ahead of print)Google Scholar
  3. 3.
    Avci G, Misirlioğlu A, Eker G, Aköz T (2003) Mild degree of Poland’s Syndrome reconstruction with customized silicone prosthesis. Aesthetic Plast Surg 27:112–115PubMedCrossRefGoogle Scholar
  4. 4.
    Baban A, Torre M, Bianca S, Buluggiu A, Rossello MI, Calevo MG, Valle M, Ravazzolo R, Jassoni V, Lerone M (2009) Poland syndrome with bilateral features: case description with review of the literature. Am J Med Gen Part A 149A:1597–1602CrossRefGoogle Scholar
  5. 5.
    Bamforth JS, Fabian C, MAchin G, Honore L (1992) Poland anomaly with a limb body wall disruption defect: case report and review. Am J Med Genet 43:780–784PubMedCrossRefGoogle Scholar
  6. 6.
    Bavinck JN, Weaver DD (1986) Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies. Am J Med Genet 23:903–918PubMedCrossRefGoogle Scholar
  7. 7.
    Beer GM, Kompatscher P, Hergan K (1996) Poland’s syndrome and vascular malformations. Br J Plast Surg 49:482–484PubMedCrossRefGoogle Scholar
  8. 8.
    Bouvet JP, Leveque D, Bernetieres F, Gros JJ (1978) Vascular origin of Poland syndrome? A comparative rheographic study of the vascularisation of the arms in eight patients. Eur J Pediatr 128:17–26PubMedCrossRefGoogle Scholar
  9. 9.
    Catena N, Divizia MT, Calevo MG, Baban A, Torre M, Ravazzolo R, Lerone M, Sénès FM (2012) Hand and upper limb anomalies in Poland syndrome: a new proposal of classification. J Pediatr Orthop 32:727–731PubMedCrossRefGoogle Scholar
  10. 10.
    Coleman SR (1995) Long-term survival of fat transplants: controlled demonstrations. Aesthetic Plast Surg 19:421–425PubMedCrossRefGoogle Scholar
  11. 11.
    Coleman SR (1997) Facial recontouring with lipostructure. Clin Plast Surg 24:347–367PubMedGoogle Scholar
  12. 12.
    Costa SS, Blotta RS, Mariano MB, Meurer L, Edelweiss MIA (2010) Laparoscopic treatment of Poland’s syndrome using the omentum flap technique. Clinics 65:401–406PubMedCrossRefGoogle Scholar
  13. 13.
    Czeizel A, Vitez M, Lenz W (1990) Birth prevalence of Poland sequence and proportion of its familial cases. Am J Med Genet 36:524PubMedCrossRefGoogle Scholar
  14. 14.
    Darian VB, Argenta LC, Pasyk KA (1989) Familial Poland’s syndrome. Ann Plast Surg 23:531–538PubMedCrossRefGoogle Scholar
  15. 15.
    Fijałkowska M, Antoszewski B (2011) Surgical treatment of patients with Poland’s syndrome—own experience. Pol Przegl Chir 83:662–667PubMedGoogle Scholar
  16. 16.
    Fokin AA, Robicsek F (2002) Poland’s syndrome revisited. Ann Thorac Surg 74:2218–2225PubMedCrossRefGoogle Scholar
  17. 17.
    Freitas Rda S, o Tolazzi AR, Martins VD, Knop BA, Graf RM, Cruz GA (2007) Poland’s syndrome: different clinical presentations and surgical reconstructions in 18 cases. Aesthetic Plast Surg 31:140–146PubMedCrossRefGoogle Scholar
  18. 18.
    Fuhrmann A (1971) Klinik und genetic des Poland-syndromes. Deutsch Med Wochenschr 96:1076–1078CrossRefGoogle Scholar
  19. 19.
    Garcia VF, Seyfer AE, Graeber GM (1989) Reconstruction of congenital chest-wall deformities. Surg Clin North Am 69:1103–1118PubMedGoogle Scholar
  20. 20.
    Gausewitz SH, Meals RA, Setoguchi Y (1984) Severe limb deficiency in Poland’s syndrome. Clin Orthop 185:9–13PubMedGoogle Scholar
  21. 21.
    Gatti JE (1997) Poland’s deformity reconstructions with a customized, extrasoft silicone prosthesis. Ann Plast Surg 39:122–130PubMedCrossRefGoogle Scholar
  22. 22.
    Glicenstein J (2001) Corrective surgery of thoracic anomalies in Poland syndrome. General review of 20 patients. Ann Chir Plast Esthet 46:640–651PubMedCrossRefGoogle Scholar
  23. 23.
    Hodgkinson DJ (1997) Chest wall implants: their use for pectus excavatum, pectoralis muscle tears, Poland’s syndrome, and muscular insufficiency. Aesthetic Plast Surg 21:7–15PubMedCrossRefGoogle Scholar
  24. 24.
    Ireland DCR, Takayama N, Flatt AE (1976) Poland’s syndrome: a review of forty-three cases. J Bone Joint Surg 58A:52–58Google Scholar
  25. 25.
    La Marca S, Delay E, Toussoun G, Ho Quoc C, Sinna R (2012) Treatment of Poland syndrome thorax deformity with the lipomodeling technique: about ten cases. Ann Chir Plast Esthet. doi:10.1016/j.anplas.2012.10.003 PubMedGoogle Scholar
  26. 26.
    Lacorte D, Marsella M, Guerrini P (2010) A case of Poland syndrome associated with dextroposition. Ital J Pediatr 36:21PubMedCrossRefGoogle Scholar
  27. 27.
    Lee SH, Kim JB, Park NH, Keum DY, Kim YH (2012) Rare combination of dextrocardia with right-sided Poland syndrome. Ann Thorac Surg 94:e103–e104PubMedCrossRefGoogle Scholar
  28. 28.
    Liao HT, Cheng MH, Ulusal BG, Wei FC (2005) Deep inferior epigastric perforator flap for successful simultaneous breast and chest wall reconstruction in a Poland anomaly patient. Ann Plast Surg 55:422–426PubMedCrossRefGoogle Scholar
  29. 29.
    Lord MJ, Laurenzano KR, Hartmann RW Jr (1990) Poland’s syndrome. Clin Pediatr 29:606–609CrossRefGoogle Scholar
  30. 30.
    Mace JW, Kaplan JM, Schanberger JE, Gotlin RW (1972) Poland’s syndrome. Report of seven cases and review of the literature. Clin Pediatr 11:98–102CrossRefGoogle Scholar
  31. 31.
    Marks MW, Argenta LC, Izenberg PH, Mes LG (1991) Management of the chest-wall deformity in male patients with Poland’s syndrome. Plast Reconstr Surg 87:674–678PubMedCrossRefGoogle Scholar
  32. 32.
    Marks MW, Iacobucci J (2000) Reconstruction of congenital chest wall deformities using solid silicone onlay prostheses. Chest Surg Clin N Am 10:341–355PubMedGoogle Scholar
  33. 33.
    Mestak J, Zadorozna M, Cakrtova M (1991) Breast reconstruction in women with Poland’s syndrome. Acta Chir Plast 33:137–144PubMedGoogle Scholar
  34. 34.
    Mestak O, Zimovjanova M (2012) Breast reconstruction by autologous fat transfer. Rozhl Chir 91:373–377PubMedGoogle Scholar
  35. 35.
    Moir CR, Johnson CH (2008) Poland’s syndrome. Semin Pediatr Surg 17:161–166PubMedCrossRefGoogle Scholar
  36. 36.
    Ohjimi Y, Shioya N, Ohjimi H, Kamiishi H (1989) Correction of a chest wall deformity utilizing latissimus dorsi with a turnover procedure. Aesthetic Plast Surg 13:199–202PubMedCrossRefGoogle Scholar
  37. 37.
    Papadopulos NA, Eder M, Stergioula S, Teymouri HR, Mavroudis MC, Herschbach P, Henrich G, Papadopoulos ON, Biemer E, Kovacs L (2011) Women’s quality of life and surgical long-term outcome after breast reconstruction in Poland syndrome patients. J Womens Health (Larchmt) 20:749–756CrossRefGoogle Scholar
  38. 38.
    Pierrefeu-Lagrange AC, Delay E, Guerin N, Chekaroua K, Delaporte T (2006) Radiological evaluation of breasts reconstructed with lipomodeling. Ann Chir Plast Esthet 51:18–28PubMedCrossRefGoogle Scholar
  39. 39.
    Perez Aznar JM, Urbano J, Garcia Laborda E, Quevedo Moreno P, Ferrer Vergara L (1996) Breast and pectoralis muscle hypoplasia. A mild degree of Poland’s syndrome. Acta Radiol 37:759–762PubMedCrossRefGoogle Scholar
  40. 40.
    Pinsolle V, Chichery A, Grolleau JL, Chavoin JP (2008) Autologous fat injection in Poland’s syndrome. J Plast Reconstr Aesthet Surg 61:784–791PubMedCrossRefGoogle Scholar
  41. 41.
    Powell CVE, Coombs RC, David TJ (1993) Poland anomaly with contralateral ulnar ray defect. J Med Genet 30:423–424PubMedCrossRefGoogle Scholar
  42. 42.
    Rajaram S, Mehta S, Gupta B, Rathi V, Goel N (2012) Poland syndrome with Mullerian agenesis: a rare occurrence. Eur J Obstet Gynecol Reprod Biol 161:232–233PubMedCrossRefGoogle Scholar
  43. 43.
    Rintala AE, Nordstrom RE (1989) Treatment of severe developmental asymmetry of the female breast. Scand J Plast Reconstr Surg Hand Surg 23:231–235PubMedCrossRefGoogle Scholar
  44. 44.
    Sadler TW (2010) Langman’s Medical Embryology. Eleventh Edition. Baltimore, Lippincott Williams & Wilkins, p 1-385Google Scholar
  45. 45.
    Schoenwolf GC, Bleyl SB, Brauer PR, Francis-West PH (2009) Larsen’s Human Embryology. Fourth Edition. Philadelphia; Churchill Livingstone Elsevier Inc. 1-688Google Scholar
  46. 46.
    Shamberger RC, Welch KJ, Upton J (1989) Surgical treatment of thoracic deformity in Poland’s syndrome. J Pediatr Surg 24:760–765PubMedCrossRefGoogle Scholar
  47. 47.
    Sierra Santos L, González Rodríguez MP (2008) Poland syndrome: description of two patients in the same family. An Pediatr (Barc) 69:49–51CrossRefGoogle Scholar
  48. 48.
    Slezak R, Sasiadek M (2000) Poland’s syndrome. Pol Merkur Lekarski 9:568–571PubMedGoogle Scholar
  49. 49.
    Sugiura Y (1976) Poland’s syndrome. Clinico-roentgenographic study on 45 cases. Cong Anom 16:17–28Google Scholar
  50. 50.
    Stylianos K, Constantinos P, Alexandros T, Aliki F, Nikolaos A, Demetriou M, Petros P (2012) Muscle abnormalities of the chest in Poland’s syndrome: variations and proposal for a classification. Surg Radiol Anat 34:57–63PubMedCrossRefGoogle Scholar
  51. 51.
    Urschel HC Jr (2000) Poland’s syndrome. Chest Surg Clin N Am 10:393–403PubMedGoogle Scholar
  52. 52.
    Tomo I, Vršanský V (1975) Contribution to the elucidation of potential teratogenic influences on the development of malformations of the upper extremities. Bratisl Lek Listy 63:531–537PubMedGoogle Scholar

Copyright information

© Springer-Verlag France 2013

Authors and Affiliations

  • Vladimir Cingel
    • 1
  • Martin Bohac
    • 2
  • Veronika Mestanova
    • 3
  • Lenka Zabojnikova
    • 1
  • Ivan Varga
    • 4
  1. 1.Department of Pediatric Surgery, Faculty of Medicine, Children’s Medical HospitalComenius University in BratislavaBratislavaSlovakia
  2. 2.Department of Plastic Surgery, Faculty of Medicine, University HospitalComenius University in BratislavaBratislavaSlovakia
  3. 3.Institute of Histology and Embryology, Jessenius Faculty of MedicineComenius UniversityMartinSlovakia
  4. 4.Institute of Histology and Embryology, Faculty of MedicineComenius University in BratislavaBratislavaSlovakia

Personalised recommendations