TY - JOUR
T1 - Poland syndrome
T2 - A proposed classification system and perspectives on diagnosis and treatment
AU - Romanini, Maria Victoria
AU - Calevo, Maria Grazia
AU - Puliti, Aldamaria
AU - Vaccari, Carlotta
AU - Valle, Maura
AU - Senes, Filippo
AU - Torre, Michele
PY - 2018/6/1
Y1 - 2018/6/1
N2 - 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.
AB - 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.
KW - Breast asymmetry
KW - Breast reconstruction
KW - Classification
KW - Pectoralis major muscle
KW - Pectus excavatum
KW - Poland syndrome
KW - Thoracic malformation
KW - Thoracoplasty
UR - http://www.scopus.com/inward/record.url?scp=85048543278&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85048543278&partnerID=8YFLogxK
U2 - 10.1053/j.sempedsurg.2018.05.007
DO - 10.1053/j.sempedsurg.2018.05.007
M3 - Article
C2 - 30078491
AN - SCOPUS:85048543278
VL - 27
SP - 189
EP - 199
JO - Seminars in Pediatric Surgery
JF - Seminars in Pediatric Surgery
SN - 1055-8586
IS - 3
ER -