Classic history and presentation: Poland syndrome is a rare congenital malformation classically described as hypoplasia / absence of the pectoralis major on one side of the chest (more commonly the right side) with ipsilateral hand abnormalities. Currently, diagnosis requires hypoplasia / absence of pectoralis major with at least 2 minor criteria:
- Hypoplasia or absence of the breast
- Absence of the nipple
- Absence of axillary hair
- Absence of adjacent muscles (eg, serratus anterior, latissimus dorsi, external oblique, deltoid, pectoralis minor, supraspinatus, and infraspinatus)
- Absence of costal cartilage and anterior ribs
- Axillary webbing
- Ipsilateral brachydactyly, brachysyndactyly, or amelia
- Age – Present from birth. In mild variants involving females, Poland syndrome may only be noticed when there is a unilateral absence of breast growth during puberty.
- Sex / gender – There is a male-to-female ratio of 3:1.
Grade / classification system: Due to the variable presentation of Poland syndrome, there is not one universally used classification system. The syndrome can be defined as mild, moderate, or severe based on breast and anterior thoracic abnormalities that affect reconstruction.
Q79.8 – Other congenital malformations of musculoskeletal system
38371006 – Poland anomaly