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.