Poland syndrome: A proposed classification system and perspectives on diagnosis and treatment

Abstract

Poland Syndrome (PS) is a rare condition, with an estimated incidence of approximately 1 per 30,000 births and encompasses a wide range of severities of chest and upper arm anomalies. The etiology remains unknown, but genetic involvement is suspected. Few radiological investigations have proven useful in the study PS phenotypes and we propose a reference algorithm for guiding pediatricians. Our experience with 245 PS patients in the last 10 years stimulated a phenotypical classification of PS. The management of the different PS types and a therapeutic algorithm according to the phenotypical features of each PS patient are also proposed.

Keywords: Breast asymmetry; Breast reconstruction; Classification; Pectoralis major muscle; Pectus excavatum; Poland syndrome; Thoracic malformation; Thoracoplasty.