Poland Syndrome

  • Poland Syndrome:
    • 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
      • 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
    • Poland syndrome is thought to occur due to:
      • 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.

#Arrangoiz #BreastSurgeon #CancerSurgeon #SurgicalOncologist #PolandSyndrome

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