Three-Dimensional Morphology and Configuration Analyses of the Craniovertebral Junction in Children with Hemifacial Microsomia

Spine (Phila Pa 1976). 2022 Jun 1;47(11):817-824. doi: 10.1097/BRS.0000000000004346. Epub 2022 Feb 22.

Abstract

Study design: Observational and morphological study with three-dimensional (3D) computed tomography (CT) analysis.

Objective: To discover the morphology and configuration deformities of craniovertebral junction (CVJ) and upper cervical spine in children with unilateral hemifacial microsomia (HFM). To determine whether there are specific HFM patients who are at higher risk of certain cervical vertebral anomaly.

Summary of background data: The evaluation for cervical vertebrae anomaly in HFM children, especially in CVJ region, is underreported.

Methods: Eighty-eight unilateral HFM children (64 males, 24 females) with four Pruzansky-Kaban types (I, Ila, Ilb, and III) underwent cranial and cervical CT scanning from skull to C5 in neutral position. The 3D morphology and configuration of the occipital condyle, atlas, and axis, etc. were evaluated on the presence of deformed detailed structures of CVJ region.

Results: No C1 deformation was found in type I group. Six (14.3%) type Ila cases, seven (33.3%) type IIb cases, and six (37.5%) type Ill cases had lateral masses asymmetry of C1 (P < 0.05). Five (55.6%) type I cases, 17 (40.5%) type Ila cases, 12 (57.1%) type Ilb cases, and 10 (62.5%) type Ill cases had C2 anomaly (P > 0.05). The incidence rate of C1-C2 instability for four groups were 33.3% (type I), 33.3% (type IIa), 33.3% (type IIb), and 31.3% (type Ill), respectively (P > 0.05).

Conclusion: For HFM children, the incidence of C1 deformation increased from type I to type Ill. The probability of C2 anomaly and C1-C2 instability in children with different types of HFM is nearly the same. The craniovertebral junction of every HFM child must be monitored carefully for C1-C2 instability before any surgical procedure to avoid atlantoaxial dislocation and spinal cord injury.Level of Evidence: N/A.

MeSH terms

  • Atlanto-Axial Joint* / diagnostic imaging
  • Atlanto-Axial Joint* / surgery
  • Cervical Vertebrae / diagnostic imaging
  • Cervical Vertebrae / surgery
  • Child
  • Female
  • Goldenhar Syndrome* / diagnostic imaging
  • Humans
  • Joint Dislocations*
  • Male
  • Occipital Bone / diagnostic imaging
  • Spinal Diseases*