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Poland’s Syndrome: A Concise Review of the Clinical Features Highlighting Associated Dermatologic Manifestations

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A Letter to the Editor to this article was published on 14 November 2017

Abstract

Poland’s syndrome is a rare congenital condition characterized by absence of the pectoralis major muscle and a wide spectrum of associated ipsilateral chest wall and upper extremity anomalies. Associated dermatologic anomalies classically include pectoral and axillary alopecia, anhidrosis due to the absence of sweat glands, and deficiency in subcutaneous fat. Functional limitations are minimal, and thus surgical correction is primarily indicated for aesthetic purposes. Chest wall reconstruction typically involves transposition of a latissimus dorsi myocutaneous flap with or without a temporary subcutaneous tissue expander prior to surgical reconstruction. Using the PubMed database, a literature review was done on Poland’s syndrome. We summarize the key features of Poland’s syndrome, including the epidemiology, clinical presentation, pathogenesis, and management, and highlight the dermatologic associations reported in the literature.

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References

  1. Poland A. Deficiency of the pectoral muscle. Guys Hosp Rep. 1841;6:191.

    Google Scholar 

  2. Garg R, Saheer S, Gupta V, Mehra S. Poland sequence: series of two cases and brief review of the literature. Ann Thorac Med. 2012;7:110–2.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Cingel V, Bohac M, Mestanova V, Zabojnikova L, Varga I. Poland syndrome: from embryological basis to plastic surgery. Surg Radiol Anat. 2013;35:639–46.

    Article  PubMed  Google Scholar 

  4. Yiyit N, Işıtmangil T, Saygın H. Eight patients with multiple bilateral thoracic anomalies: a new syndrome or bilateral Poland’s syndrome? Ann Thorac Surg. 2014;97:1758–63.

    Article  PubMed  Google Scholar 

  5. Sharma CM, Kumar S, Meghwani MK, Agrawal RP. Poland syndrome. Indian. J Hum Genet. 2014;201:82–4.

    Google Scholar 

  6. Fokin AA, Robicsek F. Poland’s syndrome revisited. Ann Thorac Surg. 2002;74:2218–25.

    Article  PubMed  Google Scholar 

  7. Lin Z, Ang Z, Xiao-jun W, Zhi-fei L, Hai-lin Z. Poland’s syndrome in women: 24 cases study and literature review. Chin Med J (Engl). 2012;125:3283–7.

    Google Scholar 

  8. Lam T, Yao Y, Trockman J. Poland syndrome coexisting with blaschkolinear congenital melanocytic nevi. JAMA Dermatol. 2014;150:1019–20.

    Article  PubMed  Google Scholar 

  9. Eroglu A, Yildiz D, Tunc H. Dextrocardia is a component of left-sided Poland syndrome. J Thorac Cardiovasc Surg. 2005;130:1471–2.

    Article  PubMed  Google Scholar 

  10. Karnak I, Tanyel FC, Tunçbilek E, Unsal M, Büyükpamukçu N. Bilateral Poland anomaly. Am J Med Genet. 1998;75:505–7.

    Article  CAS  PubMed  Google Scholar 

  11. Gashegu J, Byiringiro JC, Nyundo M. Poland syndrome: a case report. East Cent Afr J Surg. 2009;14:112–4.

    Google Scholar 

  12. Shamberger RC, Welch KJ, Upton J III. Surgical treatment of thoracic deformity in Poland’s syndrome. J Pediatr Surg. 1989;24:760–5.

    Article  CAS  PubMed  Google Scholar 

  13. Walker JE, Meijer R, Aranda D. Syndactylism with deformity of the pectoralis muscle—Poland’s syndrome. J Ped Surg. 1969;4:569–72.

    Article  Google Scholar 

  14. Mace JW, Kaplan JM, Schanberger JE, Gotlin RW. Poland’s syndrome. Report of seven cases and review of the literature. Clin Pediatr. 1972;11:98–102.

    Article  CAS  Google Scholar 

  15. Gocmen H, Akkas Y, Doganay S. Poland syndrome: rare presentation in two cases. N Z Med J. 2010;123:71–7.

    PubMed  Google Scholar 

  16. Al Faleh K, Al Saadi M, Khalid-Bantuas S. Poland’s syndrome with absent limb anomalies. J Clin Neonatol. 2014;3(1):44–6.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Moir CR, Johnson CH. Poland’s syndrome. Semin Pediatr Surg. 2008;17(3):161–6.

    Article  PubMed  Google Scholar 

  18. Gausewitz SH, Meals RA, Setoguchi Y. Severe limb deficiency in Poland’s syndrome. Clin Orthop. 1984;185:9–13.

    Google Scholar 

  19. Al-Qattan MM. Classification of hand anomalies in Poland’s syndrome. Br J Plast Surg. 2001;54:132–6.

    Article  CAS  PubMed  Google Scholar 

  20. Perez Aznar JM, Urbano J, Garcia Laborda E, Quevedo Moreno P, Ferrer Vergara L. Breast and pectoralis muscle hypoplasia. A mild degree of Poland’s syndrome. Acta Radiol. 1996;37:759–62.

    Article  CAS  PubMed  Google Scholar 

  21. Hsieh WB, Leung C. Poland syndrome in a neonate: report of one case. Acta Paediatr Taiwan. 2005;46:239–43.

    PubMed  Google Scholar 

  22. Hedge HR, Leung AKC. Aplasia of pectoralis major muscle and renal anomalies. Am J Med Genet. 1989;32:109–11.

    Article  Google Scholar 

  23. Stevens DB, Fink BA, Prevel C. Poland’s syndrome in one identical twin. J Pediatr Orthop. 2000;20:392–5.

    CAS  PubMed  Google Scholar 

  24. Chen C, Lu J, Lu X, Wu W, Han W. Neurofibroma and pectoralis muscle hypoplasia: a mild degree of Poland’s syndrome. Breast Care (Basel). 2012;7:490–2.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Fistarol SK, Itin PH. Acquired perforating dermatosis in a patient with Poland syndrome. Dermatology. 2003;207:390–4.

    Article  PubMed  Google Scholar 

  26. Riyaz N, Riyaz A. Poland syndrome (anomaly) with congenital hemangioma: a new association. Indian J Dermatol Venereol Leprol. 2006;72:222–3.

    Article  PubMed  Google Scholar 

  27. Sasmaz S, Coban YK, Boran C, Gumusalan Y. Psoriasis vulgaris and Poland’s syndrome in the same patient. J Dermatol. 2002;29:773–5.

    Article  PubMed  Google Scholar 

  28. Kuklik M. Poland–Möbius syndrome and disruption spectrum affecting the face and extremities: a review paper and presentation of five cases. Acta Chir Plast. 2000;42:95–103.

    CAS  PubMed  Google Scholar 

  29. Bavinck JNB, Weaver DD. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel–Feil, and Möbius anomalies. Am J Med Genet. 1986;23:903–18.

    Article  CAS  PubMed  Google Scholar 

  30. Vélez A, Moreno J. Poland’s syndrome and recessive X-linked ichthyosis in two brothers. Clin Exp Dermatol. 2000;25:308–11.

    Article  PubMed  Google Scholar 

  31. Dintiman BJ, Shapiro RS, Hood AF, Guba AM. Parry–Romberg syndrome in association with contralateral Poland syndrome. J Am Acad Dermatol. 1990;22:371–3.

    Article  CAS  PubMed  Google Scholar 

  32. Kadavkolan AS, Bhatia DN, Dasgupta BD, Bhosale PB. Sprengel’s deformity of the shoulder: current perspectives in management. Int J Shoulder Surg. 2011;5:1–8.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Costa R, Afonso E, Benedito M, Maricato L. Poland’s syndrome associated with chronic granulocytic leukemia. Sangre (Barc). 1991;36:417–8.

    CAS  Google Scholar 

  34. Sackey K, Odone V, George SL, Murphy SB. Poland’s syndrome associated with childhood non-Hodgkin’s lymphoma. Am J Dis Child. 1984;138:600–1.

    CAS  PubMed  Google Scholar 

  35. Shaham D, Ramu N, Bar-Ziv J. Leiomyosarcoma in Poland’s syndrome. A case report. Acta Radiol. 1992;33:444–6.

    Article  CAS  PubMed  Google Scholar 

  36. Ahn MI, Park SH, Park YH. Poland’s syndrome with lung cancer. A case report. Acta Radiol. 2000;41:432–4.

    CAS  PubMed  Google Scholar 

  37. Mojallal A, La Marca S, Shipkov C, Sinna R, Braye F. Poland syndrome and breast tumor: a case report and review of the literature. Aesthet Surg J. 2012;32:77–83.

    Article  PubMed  Google Scholar 

  38. Fukushima T, Otake T, Yashima R, et al. A case report. Breast cancer in two patients with Poland’s syndrome. Breast Cancer. 1998;6:127–30.

    Article  Google Scholar 

  39. Zhang F, Qi X, Xu Y, Zhou Y, Zhang Y, Fan L, Zhong L, Yang X, Jiang J. Breast cancer and Poland’s syndrome: a case report and literature review. Breast J. 2011;17:196–200.

    Article  CAS  PubMed  Google Scholar 

  40. Athale UH, Warrier R. Poland’s syndrome and Wilms tumor: an unusual association. Med Pediatr Oncol. 1998;30:67–8.

    Article  CAS  PubMed  Google Scholar 

  41. Caksen H, Patiroglu T, Ozdemir MA, Patiroglu TE, Poyrazoglu MH, Tercan M. Neuroblastoma and Poland syndrome in a 15-year-old boy. Acta Paediatr Jpn. 1997;39:701–4.

    Article  CAS  PubMed  Google Scholar 

  42. Freire-Maia N, Chautard EA, Opitz JM, Freire-Maia A, Quelce-Salgado A. The Poland syndrome—clinical and genealogical data, dermatoglyphic analysis, and incidence. Hum Hered. 1973;23:97–104.

    Article  CAS  PubMed  Google Scholar 

  43. David TJ. Nature and etiology of the Poland anomaly. N Engl J Med. 1972;287:487–9.

    Article  CAS  PubMed  Google Scholar 

  44. Vaccari CM, Romanini MV, Musante I, Tassano E, Gimelli S, Divizia MT, Torre M, Morovic CG, Lerone M, Ravazzolo R, Puliti A. De novo deletion of chromosome 11q12.3 in monozygotic twins affected by Poland Syndrome. BMC Med Genet. 2014;15:63.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Sunitha VC, Narayanan S, Nair PP, Prakash ML. Left-sided Poland’s syndrome in a girl with rare associations like spina bifida and diaphragmatic hernia. BMJ Case Rep. 2013;2013. doi:10.1136/bcr-2013-200930.

  46. Allam SR, Yadav R, Meziane M, Mehta AC. A middle-aged man with asymptomatic chest wall asymmetry. Cleve Clin J Med. 2006;73:754–6.

    Article  PubMed  Google Scholar 

  47. Fodor PB, Khoury F. Latissimus dorsi muscle flap in reconstruction of congenital absent breast and pectoralis muscle. Ann Plast Surg. 1980;4:422.

    Article  CAS  PubMed  Google Scholar 

  48. Ravitch MM, Handelsman JC. Lesions of the thoracic parietes in infants and children. Deformities and tumors of the chest wall, abnormalities of diaphragm. Surg Gynecol Obstet. 1952;95:1397–1424.

    CAS  PubMed  Google Scholar 

  49. Seyfer AE, Flx JP, Hamilton CG. Poland syndrome: evaluation and treatment of the chest wall in 63 patients. Plast Reconstr Surg. 2010;126:902–11.

    Article  CAS  PubMed  Google Scholar 

  50. Avci G, Misirlioğlu A, Eker G, Aköz T. Mild degree of Poland’s Syndrome reconstruction with customized silicone prosthesis. Aesthet Plast Surg. 2003;27:112–5.

    Article  CAS  Google Scholar 

  51. Glicenstein J. Corrective surgery of thoracic anomalies in Poland syndrome. General review of 20 patients. Ann Chir Plast Esthet. 2001;46:640–51.

    Article  CAS  PubMed  Google Scholar 

  52. Hodgkinson DJ. Chest wall implants: their use for pectus excavatum, pectoralis muscle tears, Poland’s syndrome, and muscular insufficiency. Aesthet Plast Surg. 1997;21:7–15.

    Article  CAS  Google Scholar 

  53. Marks MW, Iacobucci J. Reconstruction of congenital chest wall deformities using solid silicone onlay prostheses. Chest Surg Clin N Am. 2000;10:341–55.

    CAS  PubMed  Google Scholar 

  54. Ohjimi Y, Shioya N, Ohjimi H, Kamiishi H. Correction of a chest wall deformity utilizing latissimus dorsi with a turnover procedure. Aesthet Plast Surg. 1989;13:199–202.

    Article  CAS  Google Scholar 

  55. Freitas Rda S, o Tolazzi AR, Martins VD, Knop BA, Graf RM, Cruz GA. Poland’s syndrome: different clinical presentations and surgical reconstructions in 18 cases. Aesthet Plast Surg. 2007;31:140–6.

    Article  Google Scholar 

  56. Gatti JE. Poland’s deformity reconstructions with a customized, extrasoft silicone prosthesis. Ann Plast Surg. 1997;39:122–30.

    Article  CAS  PubMed  Google Scholar 

  57. Longaker MT, Glat PM, Colen LB, Siebert JW. Reconstruction of breast asymmetry in Poland’s chest-wall deformity using microvascular free flaps. Plast Reconstr Surg. 1997;99:429–36.

    Article  CAS  PubMed  Google Scholar 

  58. Pinsolle V, Chichery A, Grolleau JL, Chavoin JP. Autologous fat injection in Poland’s syndrome. J Plast Reconstr Aesthet Surg. 2008;61:784–91.

    Article  CAS  PubMed  Google Scholar 

  59. Costa SS, Blotta RM, Mariano MB, Meurer L, Edelweiss MI. Laparoscopic treatment of Poland’s syndrome using the omentum flap technique. Clinics (Sao Paulo). 2010;65:401–6.

    Article  Google Scholar 

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No funding was received for the preparation of this manuscript. Aria Vazirnia and Philip R. Cohen have no conflicts of interest to declare.

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Vazirnia, A., Cohen, P.R. Poland’s Syndrome: A Concise Review of the Clinical Features Highlighting Associated Dermatologic Manifestations. Am J Clin Dermatol 16, 295–301 (2015). https://doi.org/10.1007/s40257-015-0132-x

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