Poland Syndrome

  • Poland Syndrome:
    • Is a rare congenital malformation that includes:
      • Aplasia or hypoplasia of the chest wall
      • Hypoplasia of the breast parenchyma
      • And is associated with thoracic and upper extremity anomalies
    • Is a rare chest wall deformity consisting of:
      • Unilateral chest wall hypoplasia and unilateral upper limb deformity
    • There can be:
      • Absence or hypoplasia of the breast
      • Absent pectoralis major or minor muscle
      • Absent nipple
      • Absent costal cartilages
      • Rib abnormalities
      • Upper limb deformities including:
        • Syndactyly
        • Micromelia
        • Brachydactyly
    • This rare disease affects men three times more commonly than women
    • There is no increased risk of breast cancer with Poland syndrome:
      • Although there is an association with:
        • Hodgkins lymphoma and leukemia
    • Most cases are sporadic:
      • There is no inheritance pattern or known genetic cause:
        • Genetic testing or high-risk screening for breast cancer is not warranted
    • Poland syndrome is thought to occur due to:
      • vascular malformation resulting in reduced blood flow during embryogenesis:
        • An interruption of the embryonic blood supply to the subclavian artery:
          • At the 6th week of embryonic development
    • The defects can be corrected surgically:
      • Repair can include:
        • Reconstruction of anaplastic ribs:
          • Using bone grafts or prosthetic mesh
        • Muscle flaps such as latissimus dorsi flap to correct muscle hypoplasia
        • Breast implants or autologous fat grafting for breast hypoplasia
  • References:
    • Kulkarni D, Dixon JM. Congenital abnormalities of the breast. Women’s Health. 2012;8(1):75–88.
    • Baldelli I, Santi P, Dova L, Cardoni G, Ciliberti R, Franchelli S, Merlo DF, et al. Body image disorders and surgical timing in patients affected by Poland Syndrome: data analysis of 58 case studies. Plast Reconstr Surg. 2016;137(4):1273-1282.
    • Fokin AA, Robicsek F. Poland’s syndrome revisited. Ann Thorac Surg. 2002:74(6),2218–2225.
    • 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(1):77–83.

#Arrangoiz #BreastSurgeon #CancerSurgeon #SurgicalOncologist #Miami #PolandSyndrome #Mexico

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