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

Maria Victoria Romanini, Maria Grazia Calevo, Aldamaria Puliti, Carlotta Vaccari, Maura Valle, Filippo Senes, Michele Torre

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)189-199
Number of pages11
JournalSeminars in Pediatric Surgery
Volume27
Issue number3
DOIs
Publication statusPublished - Jun 1 2018

Keywords

  • Breast asymmetry
  • Breast reconstruction
  • Classification
  • Pectoralis major muscle
  • Pectus excavatum
  • Poland syndrome
  • Thoracic malformation
  • Thoracoplasty

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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