Craniocervical Instability in Oculoauriculovertebral Spectrum

J Craniofac Surg. 2023 Jun 1;34(4):1283-1286. doi: 10.1097/SCS.0000000000009192. Epub 2023 Jan 20.


Introduction: The oculoauriculovertebral spectrum (OAVS) are anomalies of the first and second pharyngeal arches, causing craniofacial changes, principally facial asymmetry, as well as anomalies in the spine, that can be a cause of instability of the craniocervical junction - manipulation of an unstable spine can result in morbimortality. However, few studies have related OAVS to craniocervical instability.

Objectives: Correlate patients with OAVS through radiography with craniocervical instability and prediction of its occurrence.

Material and methods: Through a radiographic study of the cervical spine, the assessment of vertebral malformations and the presence of craniocervical instability was performed and clinical assessment using the OMENS score as a phenotypic criteria for patients with OAVS, by a specialized multidisciplinary team. Student's t test, Kolmogorov-Smirnova, and χ 2 were performed.

Results: Twenty-six patients with OAVS were evaluated, 7 (26.9%) had craniocervical instability, the OMENS score was 3 times higher, but without statistical significance. All patients with instability also had spinal malformations, and of those without craniocervical instability (19 patients), 57.8% had spinal malformations. Vertebral malformations had a similar incidence in those with and without instability, the most common being scoliosis. There was no statistical significance in the presence or absence of spinal malformations with the presence or absence of craniocervical instability. Extracraniofacial findings were found in all patients with instability; 71.4% of them were radial.

Conclusions: Patients with higher OMENS scores had more craniocervical instability but without statistical significance. It also did not show statistical relevance between the presence of malformations and craniocervical instability.

MeSH terms

  • Cervical Vertebrae / diagnostic imaging
  • Facial Asymmetry
  • Goldenhar Syndrome* / diagnostic imaging
  • Humans
  • Radiography
  • Spinal Diseases*