Consultations for Poland Syndrome: The Essentials for a Thoracic Surgeon.
Małgorzata Edyta WojtyśDawid KordykiewiczJanusz WójcikPeriklis TomosKonstantinos KostopanagiotouPublished in: Medicina (Kaunas, Lithuania) (2024)
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.
Keyphrases
- minimally invasive
- coronary artery bypass
- patient satisfaction
- spinal cord
- quality improvement
- healthcare
- contrast enhanced
- surgical site infection
- mental health
- magnetic resonance imaging
- pregnant women
- computed tomography
- palliative care
- robot assisted
- case report
- randomized controlled trial
- primary care
- social media
- general practice
- gene expression
- systematic review
- copy number
- atrial fibrillation
- magnetic resonance
- preterm birth
- cord blood
- positron emission tomography
- meta analyses