Asymmetrical arthrogryposis of the upper extremity associated with congenital spine anomalies

Background: The term arthrogryposis is a descriptive term referring to a variety of clinical conditions characterized by multiple joint contractures. The spine may be involved; however, the presence of associated congenital vertebral abnormalities is rare. Methods: A retrospective chart review of 6 patients with asymmetric upper extremity joint contractures and concomitant congenital scoliosis of the cervical spine was performed. Results: Six patients with congenital anomalies of the cervical spine and asymmetric contractures of the upper extremities were identified. Clinical findings and radiographic studies were reviewed. Upper extremity involvement was contralateral to the convexity of the spinal curvature in 5 of the 6 cases. Reconstruction of the upper extremity was required in 5 of 6 patients; however, no patient required operative intervention for their congenital cervical scoliosis. Conclusions: Congenital cervical spine anomalies can be associated with asymmetric arthrogryposis of the upper extremities. Physicians who take care of pediatric patients with arthrogryposis should carefully evaluate the cervical spine. Level Of Evidence: Level IV, case series.