Article
Incidence of myelopathy and Chiari-malformation in children with cranio-cervical instability
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Authors
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Andreas Röhrig
- Abteilung für Kinderneurochirurgie, Asklepios Kinderklinik Sankt Augustin
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Sergey Persits
- Abteilung für Kinderneurochirurgie, Asklepios Kinderklinik Sankt Augustin
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Martina Messing-Jünger
- Abteilung für Kinderneurochirurgie, Asklepios Kinderklinik Sankt Augustin
| Published: | June 2, 2015 |
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Outline
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Objective: Due to a complex anatomy diagnostic assessment and treatment of cranio-cervical lesions in children are challenging. Immature osseous and ligamentary structures and a large head lead to a high biomechanical demand (stability versus mobility). The embryogenesis of posterior fossa, atlas and axis differs substantially from other spinal regions. Chiari malformation and varying width of the foramen magnum are common findings in these patients.
Method: Since 2007 we performed cranio-cervical instrumentation in 15 children age 3 to 17 years (mean 9,8 years). Patients with acute or post-traumatic lesions, tumors or inflammatory diseases were excluded. Diagnoses, CT-scans with 3D-reconstructions pre- and postoperative and MRI of the CVJ are demonstrated. The incidence of associated Chiari malformation and signs of myelopathy was assessed.
Results: 8 of 15 patients had syndromal diseases (5 Klippel-Feil, 1 Ehler-Danlos, 1 Down syndrom, 1 Neurofibromatosis type 1). 1 child suffered from craniocervical meningocele with C1 hypoplasia and consecutive instability. Myelopathy was confirmed in 5 children (33%) and was the leading symptom due to a significantly reduced spinal canal width in syndromic patients (10.7 mm versus 21.6 mm). In contrast non syndromic cases predominantly showed neck pain. Chiari malformation was seen in 5 patients (33%; 2 syndromic and 3 non-syndromic ). In 2 children presented an associated atlas assimilation. In our series no Chiari patient developed myelopathy. Additional Gardner-decompression was performed in 5 cases (4 with Chiari malformation type 1, 1 with severe myelopathy before reposition). The mean diameter of the spinal canal at the CVJ before surgery was 17.3 mm, after surgery 28.9 mm.
Conclusions: Craniocervical instability in childhood leads to a high risk of ventral protrusion and consecutive compression of neural structures (13/15). The myelopathy risk was higher (53%) in syndromal patients (overall 33%). Chiari malformation was seen in 33% (25% of syndromic cases).
