Congenital cervicothoracic dissociation: report of two cases.

PURPOSE: Congenital cervicothoracic dissociation (CCTD) of the spine is a rare condition while having major impacts on stability and neurological function. Surgical treatment includes decompression and instrumented fusion. Only few cases of CCTD have been reported in children. This report intends to demonstrate the complexity of this condition and its surgical management options based on two cases.

METHODS

Retrospective illustration of two cases with CCTD treated with instrumented occipito-thoracic fusion. Timing and options of surgical management are discussed.

RESULTS

Two patients aged 9 and 12 were treated. Patient 1 presented with asymmetric chronic weakness of the upper extremity and unstable neurogenic bladder. Imaging showed a CCTD with severe distortion of the posterior facets and a dysplastic spinal cord. Patient 2 presented with mild spasticity and unilateral weakness of the upper extremity. A low energy trauma resulted in tetraplegia by increasing the amount of antero-posterior displacement. Both patients were treated with Halo vest application, followed by posterior decompression, and instrumented occipito-thoracic fusion. Additional upfront Halo traction was applied in patient 2 for distraction and sagittal alignment improvement. The last follow-up confirmed solid fusion and minor residual neurological impairment in both patients at 5, and 6 years, respectively.

CONCLUSION

CCTD is a rare congenital condition, which can be successfully managed by posterior instrumented fusion. A sudden cervicothoracic displacement with neurological deterioration may respond to careful Halo traction with preoperative sagittal alignment correction. Timing of surgery depends on the actual instability in neurologically stable patients.