Article
Severely symptomatic cranio-vertebral-junction abnormalities in paediatric patients: Long-term reliability of aggressive management
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Published: | May 30, 2008 |
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Objective: The treatment of severe symptomatic CVJ instability is a challenge in paediatric patients affected by CVJ abnormalities. To better understand the controversial long-term effectiveness of rigid internal fixation in paediatric patients, in terms of clinical improvement and spinal stability and growth, a series of six consecutive cases has been reviewed.
Methods: Three Down syndrome, one Mucopolysaccharidosis and two os odontoideum patients (range 3-13 years old, median age 5 years) with a symptomatic CVJ instability have been treated by a multidisciplinary team. Presenting symptoms, clinical and radiological signs have been compared to the signs and symptoms at follow-up classifying the neurologic conditions by the Ranawat Scale. A thorough neuroimaging evaluation has been always obtained to describe the bone abnormalities, the dimensions and dynamic modifications of the spinal canal and the entity of spinal cord damage. An atlanto-dens interval (ADI: distance between the posterior surface of the anterior arch of C-1 and the anterior surface of the dens) of more than 5 mm was considered to be abnormal.
Results: Five patients out of six presented at admission severe neurologic deficits (Ranawat III A or B). An Halo-vest was positioned preoperatively in all the severely compromised patients with immediate reduction and stabilization of the CVJ dislocations. An occipito-cervical and/or atlanto-axial stable fusion, as judged on flexion-extension radiographs, was achieved in all the cases by the use of different rigid internal fixation techniques always tailored to the patient anatomical features and age. All the patients presented at long-term follow-ups a stable and remarkable neurologic improvement. No cervical developmental deformity was observed at long-term neuroimaging controls.
Conclusions: In the light of the effective clinical/radiological long-term results, we recommend, even in severely symptomatic children with CVJ abnormalities, an aggressive approach to the instability, tailoring the per-operative management and the rigid internal fixation technique to the patient’s age and anatomical features.