Cervicovertebral anomalies and basilar impression in Goldenhar syndrome

Although previously unreported, neurologic compromise may occur secondary to anomalies of the cervical spine in the oculoauriculovertebral spectrum. Medical records and cephalograms were reviewed from 18 patients with classic Goldenhar syndrome and from 18 normal adults. One patient, a 22-year-old man, had experienced a 5-year history of progressive neurologic compromise from basilar impression; the remaining 17 patients were asymptomatic. Radiographic evidence of cervicovertebral fusion was documented in 11 of 18 patients (61 percent). Fusion occurred at every cervical level other than the atlantoaxial joint. Radiographic indices that characterize the cranial base were not significantly different between asymptomatic Goldenhar patients and normal adults. Posterior inclination of the odontoid with respect to the foramen magnum appears to be the best indicator that a patient is at risk for basilar impression. Careful radiographic evaluation may indicate which patients require more careful surveillance with periodic neurologic examination and CT and/or MRI scans.