What is the approach to charting Poland syndrome?

Charting Poland Syndrome

When documenting Poland syndrome, use the TBN (Thorax, Breast, Nipple-areola complex) classification system to accurately record the specific anatomical defects present in the patient. 1

Thoracic Anomalies Classification (T)

• T1: Muscle defect only (most common, occurring in 47% of cases) 1
• T2: Muscle defect with mild chest wall deformity 1
• T3: Moderate chest wall deformity 1
• T4: Complex deformity with rib and sternal involvement 1

Breast Anomalies Classification (B)

• B1: Hypoplasia (more common in female patients) 1
• B2: Amastia (complete absence of breast tissue) 1

Nipple-Areola Complex Classification (N)

• N1: Dislocation <2 cm 1
• N2: Dislocation >2 cm (most common, occurring in 74% of cases) 1
• N3: Athelia (absence of nipple-areola complex) 1

Essential Clinical Features to Document

• Laterality: Document whether the condition affects the right side (more common, 55.7%), left side (37.1%), or is bilateral (rare, 7%) 2
• Pectoralis major muscle involvement: Complete absence (71.6%) or partial absence (typically sternocostal head only, 28.3%) 2
• Pectoralis minor muscle: Presence or absence 3
• Associated chest wall defects: Absence of costal cartilages or ribs (typically 2-3-4 or 3-4-5) 3
• Presence of lung hernia if applicable 3

Associated Anomalies to Document

• Hand anomalies: Document presence of syndactyly, brachydactyly, or other hand deformities 3
• Sprengel deformity (most common associated anomaly) 2
• Other muscle abnormalities beyond the pectoral muscles 2
• Subcutaneous tissue hypoplasia 3
• Mammary hypoplasia in female patients 4

Imaging Findings to Include in Documentation

• Document results of relevant imaging studies:
  • Sonography findings for muscle and mammary abnormalities 4
  • MRI findings for detailed muscle assessment 4
  • Vascular status evaluation (color-coded duplex sonography for peripheral arteries) 4
  • MR-angiography results for supra-aortic arteries if performed 4

Surgical Planning Documentation

• For patients requiring surgical correction, document the planned approach based on TBN classification:
  • Simple deformities: Latissimus dorsi muscle transfer (with sublatissimus mammary prosthesis in females) 5
  • Complex deformities: Latissimus dorsi transfer plus musculoskeletal chest wall realignment 5
  • N2 and N3 cases typically require two-step approach with tissue expanders 1
  • N1 cases may be corrected in a single surgical step 1

Documentation Pitfalls to Avoid

• Failing to document the specific anatomical variant using a standardized classification system 1
• Overlooking associated anomalies beyond the chest wall 2
• Incomplete documentation of vascular status, which may impact surgical planning 4
• Not documenting the full extent of muscle involvement beyond just the pectoralis major 2