Instrumented arthrodesis for non-traumatic craniocervical instability in very young children

Purpose: Occipitocervical instrumentation is infrequently required for stabilization of the axial and subaxial cervical spine in very young children. However, when it is necessary, unique surgical considerations arise in children when compared with similar procedures in adults. Methods: The authors reviewed literature describing fusion of the occipitocervical junction (OCJ) in toddlers and share their experience with eight cases of young children (age less than or equal to 4 years) receiving occiput to axial or subaxial spine instrumentation and fixation. Diagnoses and indications included severe or secondary Chiari malformation, skeletal dysplastic syndromes, Klippel-Feil syndrome, Pierre Robin syndrome, Gordon syndrome, hemivertebra and atlantal occipitalization, basilar impression, and iatrogenic causes. Results: All patients underwent occipital bone to cervical spine instrumentation and fixation at different levels. Constructs extended from the occiput to C2 and T1 utilizing various permutations of titanium rods, autologous rib autografts, Mersilene sutures, and combinations of autografts with bone matrix materials. All patients were placed in rigid cervical bracing or halo fixation postoperatively. No postoperative neurological deficits or intraoperative vascular injuries occurred. Conclusion: Instrumented arthrodesis can be a treatment option in very young children to address the non-traumatic craniocervical instability while reducing the need for prolonged external halo vest immobilization. Factors affecting fusion are addressed with respect to preoperative, intraoperative, and postoperative decision-making that may be unique to the toddler population.