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Severely symptomatic craniovertebral junction abnormalities in children: long-term reliability of aggressive
Carlo Giussani1, Franck-Emmanuel Roux, Paolo Guerra
1Neurosurgery Section, Department of Neuroscience and Biomedical Technology, Università degli Studi Milano-Bicocca, Monza, Italy. carlo.giussani@libero.it
Insights
Aggressive, multidisciplinary treatment of cranio-vertebral junction (CVJ) instability in children leads to significant neurological improvement and spinal stability. This approach ensures effective long-term outcomes for severe pediatric CVJ abnormalities.
Area of Science:
- Pediatric Neurosurgery
- Spinal Surgery
- Developmental Pediatrics
Background:
- Cranio-vertebral junction (CVJ) instability in children presents a significant treatment challenge.
- Understanding the long-term effectiveness of aggressive management for CVJ abnormalities is crucial.
Observation:
- Severely symptomatic pediatric patients with CVJ abnormalities underwent tailored, aggressive treatment.
- Patients included those with Down syndrome, mucopolysaccharidosis, and os odontoideum, presenting with severe neurological deficits (Ranawat III A/III B).
Findings:
- All patients achieved remarkable neurological improvements post-treatment.
- Surgical techniques, perioperative halo-orthosis use, and rehabilitation were individualized.
- Achieved construct stability and bone fusion without impacting spinal growth.
Implications:
- Recommends a multidisciplinary, aggressive, and tailored treatment strategy for pediatric CVJ instability.
- Highlights the efficacy of rigid internal fixation for long-term positive outcomes.
- Suggests that aggressive management is beneficial even in severely symptomatic children.
Objectives:
The treatment of symptomatic cranio-vertebral junction (CVJ) instability in children affected by CVJ abnormalities is a challenge. A series of severely symptomatic children has been reviewed to understand the controversial long-term effectiveness of the aggressive management of CVJ abnormalities, in terms of clinical improvement, spinal stability and growth.
Methods:
Three Down syndrome patients, 1 with mucopolysaccharidosis and 1 with os odontoideum (range 3-6 years old) with a CVJ instability determining spinal cord compression with severe neurological deficits (the patients presented at admission a Ranawat III A/III B neurological condition), were consecutively treated at our institution. Medical records, imaging studies, adopted surgical techniques and long-term results were reviewed. Details of the presenting symptoms, clinical and radiological signs were compared to the signs and symptoms at follow-up.
Results:
The perioperative use of an halo-orthosis, the operative techniques and the timing of rehabilitation were always tailored to the patient's anatomical features. All the patients showed remarkable neurological improvements, along with construct stability and bone fusion without abnormalities of the developing spine.
Conclusions:
Considering the effective long-term results, we recommend, even in severely symptomatic children with CVJ abnormalities, a multidisciplinary aggressive tailored treatment of instability with rigid internal fixation.
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