Consultations for Poland Syndrome: The Essentials for a Thoracic Surgeon.

Abstract

Poland syndrome (PS) is a rare congenital musculoskeletal entity occurring in approximately 1 in 30,000 newborns that manifests with variable symbrachydactyly, ipsilateral costochondral deformities, an absence of pectoral muscles, and breast underdevelopment. These have potential impacts on social, somatic, and psychological functionality, often leading affected individuals to seek expert opinions on corrective surgery. Due to phenotypic variability, strict management guidelines are lacking, with treatment decisions often based on the specialist's personal experience rather than published evidence. Comprehensive imaging with CT and MRI with 3D reconstruction is crucial for providing a descriptive assessment of musculoskeletal defects. Management is multidisciplinary, involving thoracic, plastic, and pediatric surgeons and hand surgery specialists, as well as psychologists and developmental growth specialists. Surgery should achieve both structural and cosmetic correction to reverse the psychological and social impact and achieve patient satisfaction. We aim to provide thoracic surgeons the essential answers for sharing with affected adult individuals during consultations focusing on chest surgical correction.