We examined the shoulder function in patients with congenital clavicle and congenital muscle defect around the shoulder. 26 shoulders of 19 patients with congenital clavicle and congenital muscle defect around the shoulder were retrospectively investigated for this study. Diagnoses were congenital pseudoarthrosis in 3 patients, major defect due to cleidocranial dysplasia in 3 patients, congenital midshaft defect of clavicle in 1 patient, distal defect related to congenital progeroid syndrome in 1 patient, proximal half defect related to Goltz syndrome in 1 patient, congenital sternum defect in 1 patient, total absence of the trapezius in 2 patients, partial absence of trapezius in 1 patient, and Poland syndrome in 7 patients. The mean follow-up term was 5.4 years. We evaluated their range of motion (ROM) of shoulder and activities of daily living. In the case of congenital midshaft defect of clavicle and 2 pediatric cases of cleidocranial dysplasia, slight limitation of anterior elevation of shoulder was admire. This limitation has been improved with growth. In the remaining cases, there was no limitation. All patients' daily activities were not disturbed. In the 2 cases of absence of trapezius, ROM limitation, especially lateral elevation, has appeared with growth. But there were no limitation in patients with Poland syndrome. In congenital defect, the dysfunction due to the bone defect was able to be compensated for with muscles. But the dysfunction due to the muscle defect was not be able to compensated with muscles and the limitation showed the tendency to deteriorate for the weight of the arm.
