- 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
- Although there is an association with:
- 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
- There is no inheritance pattern or known genetic cause:
- Poland syndrome is thought to occur due to:
- A 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
- An interruption of the embryonic blood supply to the subclavian artery:
- A vascular malformation resulting in reduced blood flow during embryogenesis:
- 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
- Reconstruction of anaplastic ribs:
- Repair can include:
- Is a rare congenital malformation that includes:
- 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.
- National Human Genome Research Institute website. About Poland anomaly. 2012. http://www.genome.gov/14514230. Accessed January 10, 2020.
#Arrangoiz #BreastSurgeon #CancerSurgeon #SurgicalOncologist #Miami #PolandSyndrome #Mexico
Rodrigo Arrangoiz MS, MD, FACS, FSSO 