- Not all cases require surgery: The radiological findings in metabolic bone disorders must be interpreted in the context of the clinical progress of the child and the likely natural history of the underlying condition. Not all patients require surgical intervention but should remain under clinical and radiological surveillance.
Principles of Management
The principles of surgical management of cervical spine anomalies in metabolic bone disease can be summarized as follows:
- Reduction of spinal deformity: Reduction of spinal deformity restores alignment.
- Decompression of the neuraxis: Any compression of the brainstem or spinal cord by bone or ligamentous thickening needs to be addressed. The predominant direction of compression in relation to the neuraxis will indicate the surgical approach to achieve decompression. This might require anterior (transnasal or transoral), lateral (posterolateral, far lateral, extreme lateral) or posterior approaches.
- Immobilization: Contemporary instrumentation techniques have a high rate of success in providing durable internal fixation.
- Control of instability: In cases of severe instability or rapidly evolving neurological deficit, immobilization in a halo-body orthosis can improve symptoms and gain time to appropriately investigate and plan surgery.
- Avoidance of extensive fusing: The extent of fixation should be sufficient to immobilize the affected spinal segment but not so long as to compromise residual motion.