Craniocervical fusion in the paediatric population - case series of 21 patients.

PURPOSE: Craniocervical junction instability in a paediatric population presents a formidable challenge to the neurosurgeon. With a variety of anatomical variations, diminutive bony and ligamentous structures, possible superimposed syndromic abnormalities, the craniocervical junction is a technically challenging region to operate within. We aimed to review our series of patients to identify the common pathologies necessitating craniocervical fusion along with the use of intraoperative image guidance.

METHODS: We performed a retrospective review of twenty-one patients, with a mean age of 8.1 years, undergoing craniocervical fixations, involving either occipitocervical fusion or atlantoaxial fixation, from a single institution over a twelve-year period. The presentation, preoperative investigations, indication for surgery, surgical procedure, use of intraoperative navigation and clinical and radiological results were examined.

RESULTS: Twelve patients underwent primary C1-2 fixation, with the remaining 9 undergoing occipitocervical fusion. Five patients underwent surgery for traumatic instability, seven for os odontoideum, six for congenital anomalies and three for post-infectious instability. Follow up for 20 patients averaged 34 months (range 2-93 months). Five patients had Trisomy 21 and all underwent C1-2 fixation. Frameless stereotactic image guidance was utilised in five patients. No patients suffered immediate complications resulting from craniocervical junction fusion. Patients were observed to either neurologically improve ( n  = 6), or arrest their neurological deterioration following surgical intervention ( n  = 14), with concomitant radiological evidence of osseous fusion at follow up in 18 of 20 patients (90%). Two patients (10%) had radiological evidence of screw lucency, but neither required intervention because of being asymptomatic.

CONCLUSIONS: Craniocervical fixation in a paediatric population is a viable and safe treatment modality for the management of disorders requiring stabilisation at the craniocervical junction. Our experience of utilising frameless stereotaxy in the setting of grossly distorted anatomical landmarks is also reported.