PURPOSES: Poland's syndrome (PS) is a rare congenital malformation, which combines anomalies of the chest and the homolateral upper limb. The purposes of the paper are to study the chest musculoskeletal malformations of the syndrome and propose a classification for the thoracic anomalies through our experience and taking into account the literature. METHODS: We reviewed ten patients diagnosed with PS. All of them suffered from anomalies of the thorax and the ipsilateral upper extremity. We proceeded to scan the thorax with 3-D CT for better imaging of the structures and examination of the variations. RESULTS: All patients were young to middle aged: seven women and three men. Both sides of the body were affected to the same extent. Six patients presented with total absence of the pectoralis muscles and variable anomalies of the thoracic skeletal structures. Hypoplasia of the minor pectoralis and retraction of the ribs were observed in two patients, while the two other patients presented with major and minor pectoralis absence as well, except for the clavicular head. CONCLUSIONS: There are many variations of PS with regard to the chest that can be best detected with 3-D CT imaging, which should be used whenever it is available. We propose a simple classification of the musculoskeletal anomalies of the chest in Poland's syndrome that would be a useful tool for the clinicians and especially plastic surgeons to make an easy diagnosis. In combination with 3-D CT imaging, surgeons will be able to achieve the best treatment for each patient.
PURPOSES: Poland's syndrome (PS) is a rare congenital malformation, which combines anomalies of the chest and the homolateral upper limb. The purposes of the paper are to study the chest musculoskeletal malformations of the syndrome and propose a classification for the thoracic anomalies through our experience and taking into account the literature. METHODS: We reviewed ten patients diagnosed with PS. All of them suffered from anomalies of the thorax and the ipsilateral upper extremity. We proceeded to scan the thorax with 3-D CT for better imaging of the structures and examination of the variations. RESULTS: All patients were young to middle aged: seven women and three men. Both sides of the body were affected to the same extent. Six patients presented with total absence of the pectoralis muscles and variable anomalies of the thoracic skeletal structures. Hypoplasia of the minor pectoralis and retraction of the ribs were observed in two patients, while the two other patients presented with major and minor pectoralis absence as well, except for the clavicular head. CONCLUSIONS: There are many variations of PS with regard to the chest that can be best detected with 3-D CT imaging, which should be used whenever it is available. We propose a simple classification of the musculoskeletal anomalies of the chest in Poland's syndrome that would be a useful tool for the clinicians and especially plastic surgeons to make an easy diagnosis. In combination with 3-D CT imaging, surgeons will be able to achieve the best treatment for each patient.
Authors: Anwar Baban; Michele Torre; Sebastiano Bianca; Anna Buluggiu; Mario Igor Rossello; Maria Grazia Calevo; Maura Valle; Roberto Ravazzolo; Vincenzo Jasonni; Margherita Lerone Journal: Am J Med Genet A Date: 2009-07 Impact factor: 2.802