Surgical Algorithm of Poland Syndrome Based on Thorax, Breast, and Nipple-areola Complex Classification.

Abstract

Background: Poland syndrome (PS) is a rare congenital syndrome characterized by unilateral pectoralis major muscle defect. In 2016, we proposed the thorax, breast, and nipple-areola complex (TBN) classification and a subsequent treatment algorithm, which included conservative and surgical procedures. Our aim is to report the results we obtained treating all thoracic anomalies according to the proposed algorithm in a cohort of adolescents affected by PS.

Methods: Between January 2016 and June 2023, 52 patients with PS were treated using the TBN classification in our institute. Each patient was evaluated by a multidisciplinary team composed of pediatric and plastic surgeons and treated according to the algorithm. Surgical procedures included were minimally invasive pectus excavatum repair, open sternochondroplasty, patch, metal plate, 3-dimensional chest wall prosthesis, fat grafting, tissue expanders, and breast and pectoral implants. Conservative treatments included vacuum bell and corset.

Results: Half of the patients had no thoracic skeletal defect (T1), but all had soft-tissues defects. Nineteen patients required thoracoplasty, all of whom experienced T downstaging after treatment, and 90% were postoperatively reclassified as T1. Conservative treatment for T correction was proposed in 8 patients; 83% underwent fat grafting, and 88% had a breast/pectoral prosthesis implanted. There were no major complications registered. The mean follow-up was 5.9 years.

Conclusions: The proposed algorithm can be considered a useful tool for standardized surgical decision-making in PS. Fat grafting can play a major role in a pediatric setting. A multidisciplinary and minimally invasive approach, whenever possible, should be prioritized in patients younger than 18 years.