What is the treatment for chest pain in individuals with Poland syndrome?

Treatment of Chest Pain in Poland Syndrome

Chest pain in Poland syndrome is primarily musculoskeletal in origin and does not require cardiac-specific treatment unless concurrent cardiac disease is present; management focuses on addressing the underlying chest wall deformity through surgical reconstruction when symptomatic.

Understanding the Source of Chest Pain

Poland syndrome is a congenital anomaly characterized by partial or complete absence of the pectoralis major muscle (particularly the sternocostal head), often with associated chest wall deformities including absence of costal cartilages or ribs 2-5 1, 2. The chest wall defect can be associated with lung herniation and variable thoracic skeletal abnormalities 2, 3. Chest pain in these patients typically arises from:

• Musculoskeletal instability due to absent pectoral muscles and rib/cartilage deficiencies 1, 4
• Chest wall asymmetry causing mechanical strain on surrounding structures 4, 3
• Lung herniation through the deficient chest wall 2

Initial Evaluation: Excluding Cardiac Causes

Before attributing chest pain solely to Poland syndrome anatomy, cardiac causes must be excluded, particularly in patients with risk factors for coronary disease 5, 6:

• Obtain 12-lead ECG within 10 minutes if acute presentation 6
• Measure cardiac troponin levels 6
• Consider stress testing if anginal features present in older patients or those with multiple cardiac risk factors 5
• Echocardiography to assess for any structural cardiac abnormalities 5

Critical pitfall: Do not assume all chest pain is musculoskeletal simply because Poland syndrome is present. Standard cardiac evaluation protocols apply 5, 6.

Non-Surgical Management

For mild, intermittent chest pain without significant functional impairment:

• Neuropathic pain features (burning, shooting, electrical sensations): Gabapentin starting at 300-600 mg/day, titrating to 2400 mg/day in divided doses may be beneficial 7
• Musculoskeletal pain: Standard analgesics and NSAIDs for symptomatic relief
• Reassurance regarding the benign nature of the anatomical variant when cardiac causes excluded 5

Surgical Treatment: The Definitive Approach

Surgical reconstruction is the primary treatment for symptomatic chest wall deformity causing persistent pain or functional limitation 1, 4, 2. The approach depends on anatomical classification:

Simple Deformity (More Common)

• Latissimus dorsi muscle transfer provides chest wall stabilization and symmetry 1, 4
• In females, combine with sublatissimus mammary prosthesis for cosmetic restoration 4
• Single-stage reconstruction achieves superior outcomes compared to multiple procedures 2

Complex Deformity (Original Poland Description)

• Latissimus dorsi transfer plus musculoskeletal chest wall realignment 4
• May require sternal/rib reconstruction for severe skeletal deficiencies 4
• Custom-made chest wall prostheses carry higher complication rates and should be avoided when possible 4

Surgical Benefits

• Stabilizes the chest wall mechanically 1
• Eliminates lung herniation when present 2
• Provides symmetric body appearance reducing psychological distress 1
• Long-term follow-up (1-21 years) demonstrates sustained improvement 4

Preoperative Considerations

3-D CT imaging of the thorax should be obtained before surgical planning to:

• Define the exact extent of muscle absence 3
• Identify rib and cartilage abnormalities 3
• Guide surgical approach selection 3
• Assess for associated anomalies (Sprengel deformity is most common, seen in 16% of cases) 1

Anesthetic Management for Surgery

When proceeding to chest wall reconstruction 8:

• Thorough preoperative cardiopulmonary assessment
• Intraoperative monitoring for potential lung herniation
• Multimodal pain management planning given chest wall manipulation
• Consider epidural analgesia for postoperative pain control

When to Refer for Surgery

Indications for surgical referral:

• Persistent chest pain unresponsive to conservative measures 1, 4
• Functional limitation from chest wall instability 4
• Visible lung herniation 2
• Patient desire for cosmetic improvement with symptomatic benefit 4

Timing: Surgery is typically performed after skeletal maturity in adolescents, though can be done earlier for severe functional impairment 4, 8.