Poland Syndrome Management
Poland syndrome requires a multidisciplinary surgical approach tailored to the specific anatomical defects present, with timing of interventions based on the severity of chest wall and hand deformities, prioritizing functional reconstruction of syndactyly in early childhood and chest wall reconstruction during adolescence or later.
Clinical Recognition and Classification
Poland syndrome is a sporadic congenital disorder characterized by unilateral absence or hypoplasia of the pectoralis major muscle (specifically the sternocostal head) with variable ipsilateral chest wall and upper limb anomalies 1, 2.
Key Diagnostic Features:
- Laterality: Right side affected more frequently than left 1, 2
- Sex distribution: Males affected more than females 1, 2
- Non-hereditary: Sporadic occurrence with unknown etiology 1
Anatomical Classification Systems:
Chest Wall (TBN Classification) 3:
- T1: Muscle defect only (most common at 47%)
- T2-T3: Progressive chest wall involvement
- T4: Complex deformity with rib and sternal involvement
- B1/B2: Breast hypoplasia or amastia
- N1-N3: Nipple-areola complex dislocation or athelia
Hand and Upper Limb Involvement 1, 4:
- Middle phalanx hypoplasia or absence (effectively one interphalangeal joint)
- Syndactyly (usually incomplete and simple, may involve all digits including thumb)
- Thumb positioned in same plane as fingers when involved
- Forearm and arm hypoplasia (forearm more frequently affected)
Surgical Management Algorithm
Hand and Upper Limb Reconstruction
Timing: Initiate by age 1 year, complete before school entry 1
Indications for Surgery 5:
- Syndactyly reconstruction recommended in all but mildest cases
- Digital separation improves functional length given notable brachydactyly
- Operative management determined by severity and resulting anatomical dysfunction
Surgical Approach 1:
- Syndactyly separation as primary intervention
- Selective digit removal to create three-fingered hand in severe cases
- Periodic revisions may be necessary throughout growth
Expected Outcomes 1:
- Hand remains hypoplastic with limited functional capacity due to inherent skeletal anomalies
- Improved functional capacity and cosmetic appearance in majority of patients
Chest Wall Reconstruction
Timing: Based on anatomical type, gender, and patient preference 2, 3
Simple Deformity (T1-T2) 2:
- Males: Latissimus dorsi muscle transfer
- Females: Latissimus dorsi muscle transfer plus sublatissimus mammary prosthesis
Complex Deformity (T3-T4) 2:
- Latissimus dorsi transfer as foundation
- Selective musculoskeletal chest wall realignment
- Sternal/rib reconstruction when indicated
- Custom-made chest wall prostheses (higher complication risk, use selectively)
Nipple-Areola Complex Considerations 3:
- N1 (dislocation <2 cm): Single-step reconstruction sufficient
- N2-N3 (greater dislocation or athelia): Two-step approach with tissue expanders required
Alternative Techniques 2:
- Transverse rectus abdominis musculocutaneous (TRAM) flaps
- Mammary prostheses
- Combination approaches based on defect complexity
Associated Anomalies Requiring Evaluation
Thoracic 1:
- Hypoplastic upper ribs
- Lung herniation
- Anterior axillary web contracture
Musculoskeletal 1:
- Elevated scapula
- Arm and forearm hypoplasia
- Nipple and breast hypoplasia
- Primarily cosmetic deficiency
Critical Management Principles
Pitfalls to Avoid:
- Do not delay hand surgery: Functional improvement requires early intervention before school age 1
- Avoid custom chest wall prostheses as first-line: Higher complication rates compared to autologous tissue reconstruction 2
- Do not use single-step reconstruction for N2-N3 deformities: Tissue expansion required for adequate nipple-areola complex positioning 3
Long-term Considerations:
- Follow-up ranges from 1-21 years demonstrate durability of repairs 2
- Functional capacity remains limited by inherent skeletal anomalies despite optimal surgical intervention 1
- Periodic revisions may be necessary throughout growth and development 1