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.

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

Romanini, Maria Victoria;Puliti, Aldamaria;Vaccari, Carlotta;Torre, Michele
2018-01-01

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.
File in questo prodotto:
File Dimensione Formato  
Romanini et al 2018.pdf

accesso chiuso

Tipologia: Documento in versione editoriale
Dimensione 2.98 MB
Formato Adobe PDF
2.98 MB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11567/920871
Citazioni
  • ???jsp.display-item.citation.pmc??? 11
  • Scopus 43
  • ???jsp.display-item.citation.isi??? 34
social impact