Approach to the Pediatric Poland Syndrome Patient: A 20-Year Academic Experience and Update of the Literature

Abstract

Background: Poland syndrome is a spectrum of deformities involving the chest/rib cage, breast bud, nipple-areola complex, upper limb, and surrounding tissue types, with pectoralis major muscle agenesis/hypoplasia as the cardinal feature. Although various surgical approaches exist, there remains a gap in the literature reporting long-term outcomes and patient-reported results for the pediatric population.

Methods: A retrospective review identified 47 pediatric patients who underwent surgical reconstruction for Poland syndrome between 2003 and 2024 and subsequently underwent intervention under 18 years of age. Patients were stratified using Foucras and Thorax, Breast, Nipple-areola complex (TBN) classification systems. Data collected included demographics, surgical procedures, complications, and behavioral health notes. Statistical analysis examined relationships between classification systems, surgical interventions, and outcomes.

Results: The cohort included 12 males and 35 females. Twenty-four patients (51%) were Foucras class I, 20 (43%) were class II, and 3 (6%) were class III. Initial surgeries offered were fat grafting (45%) and latissimus dorsi muscle reconstruction (55%). Higher Foucras classification correlated with higher number of surgical procedures (P = 0.029, ρ = 0.388). Seven complications occurred: two minor (seroma, wound dehiscence) and five major (infection or implant complications requiring reoperation). Behavioral health assessment revealed appearance concerns (87%), clothing challenges (47%), and pain (20%) as the primary psychosocial stressors. The mean follow-up was 834 days (2.3 years). We present several of our reconstructive principles and techniques relevant to pediatric care, as well as highlight representative clinical cases.

Conclusions: This 20-year institutional experience demonstrates that pediatric Poland syndrome reconstruction requires consideration of both immediate and long-term goals while accounting for physical development and psychological maturity. Behavioral health assessment revealed appearance as the predominant concern, emphasizing the importance of addressing psychosocial aspects in treatment. A multidisciplinary approach focusing on patient education, expectation management, and psychological support is crucial for improved outcomes in pediatric Poland syndrome breast and chest wall reconstruction.