Multimodality imaging of Poland syndrome with dextrocardia and limb anomalies.
Poland syndrome is a rare disorder featuring unilateral anterior thoracic and upper extremity anomalies. These include hypoplasia of the pectoralis major costosternal head, absent pectoralis minor, breast hypoplasia, and absent upper rib cartilage. Pulmonary hypoplasia may occur secondarily. The postulated etiology is underdevelopment of the subclavian artery. Less than 50 cases of Poland syndrome with dextrocardia have been reported. Rib anomalies cause rightward cardiac displacement in fetal life. This case of left-sided Poland syndrome in a young girl is unique not only because of dextrocardia, but also because the condition preferentially afflicts the right side and males.
Department of Radiology, Seattle Children's Hospital, Seattle, WA 98105, USA. riyer@uw.edu
Ramesh S
RS
Iyer
Marguerite T
MT
Parisi
D002675:Child, Preschool; D003914:Dextrocardia / Q000150:complications* / Q000000981:diagnostic imaging*; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D011045:Poland Syndrome / Q000150:complications* / Q000000981:diagnostic imaging*; D013902:Radiography, Thoracic; D011877:Radionuclide Imaging; D038062:Upper Extremity Deformities, Congenital / Q000150:complications* / Q000000981:diagnostic imaging*