Cervical spine instability in children with Goldenhar's syndrome.

OBJECTIVE To study the vertebral involvement of the cervical spine, in particular the stability of C1-C2, in children with proven Goldenhar's syndrome. DESIGN A case review. SETTING The Children's Hospital of Eastern Ontario, Ottawa. PATIENTS Eight children who had a minimum of 2 out of 3 Goldenhar criteria plus other strong associations with the syndrome and for whom detailed radiographic spinal assessment, including flexion-extension views of the cervical spine and computed tomography of the congenital anomalies, were available. OUTCOME MEASURES Radiographic findings. RESULTS Seven children demonstrated cervical spine anomalies. Of particular concern was the high incidence of C1-C2 instability in 3 children, 2 of whom required occiput to C2 fusion. The presence of hemivertebrae and failures of segmentation were most common and resulted in thoracic scoliosis, leading to spinal fusion in 2 children. CONCLUSIONS In patients with Goldenhar's syndrome the cervical spines must be monitored carefully for C1-C2 subluxation before any proposed surgery for other malformations associated with the syndrome, so that any instability can be identified to avoid cord impingement during a general anesthetic.