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

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.