| Literature DB >> 30078491 |
Maria Victoria Romanini1, Maria Grazia Calevo2, Aldamaria Puliti3, Carlotta Vaccari4, Maura Valle5, Filippo Senes6, Michele Torre7.
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.Entities:
Keywords: Breast asymmetry; Breast reconstruction; Classification; Pectoralis major muscle; Pectus excavatum; Poland syndrome; Thoracic malformation; Thoracoplasty
Mesh:
Year: 2018 PMID: 30078491 DOI: 10.1053/j.sempedsurg.2018.05.007
Source DB: PubMed Journal: Semin Pediatr Surg ISSN: 1055-8586 Impact factor: 2.754