Poland syndrome: about a case and review of the literature

Poland syndrome is a rare congenital malformation associated with various degrees of thoracic and homolateral upper limb abnormalities. We report the case of a 7-year old girl who underwent exploration for depression of the left hemithorax associated with homolateral subclavicular mass. CT scan showed that the deformation of the thoracic wall was related to the absence of left pectoralis major muscle sterno-costal heads insertion associated with agenesis of the pectoralis minor muscle and hypoplasia of the anterior arches of the first six corresponding ribs. These abnormalities were related to Poland syndrome. General assessment was based on abdominal ultrasound, X-rays of both hands and did not show any associated malformation. Poland syndrome results from a deficit of blood flow to musculoskeletal elements of the chest wall during fetal life. There are many variants of Poland syndrome that can be best detected by CT scan and which must be performed whenever necessary, without omitting the role of general radiology in the detection of associated malformations. This syndrome is characterized by the agenesis of the sternocostal bundles of the pectoralis major muscle. Its etiology remains unknown and discussed. It can be caused by a vascular anomaly, without the primum movens of this vascular anomaly being known.