C1-C2 Fixation for Chiari with Syringomyelia Associated with Atlantoaxial Instability, Atlantooccipital Assimilation and an Anomalous Vertebral Artery Course.

Abstract

Chiari malformations, characterized by the herniation of cerebellar tonsils through the foramen magnum, are complex neurosurgical conditions that pose significant diagnostic and therapeutic challenges.¹ Traditional interventions, such as foramen magnum decompression, have been the mainstay of treatment but are not universally effective, especially in cases with underlying atlantoaxial instability. However, recent studies highlight the importance of atlantoaxial instability in their pathogenesis, shifting the therapeutic focus towards atlantoaxial fixation.² Research shows that atlantoaxial fixation can effectively address the underlying instability, leading to better symptomatic relief and neurological outcomes compared to traditional decompression methods.³^,^4-17 The findings from these studies consistently demonstrate that atlantoaxial instability is a critical factor in the development of Chiari malformations. Atlantoaxial fixation not only resolves the mechanical instability but also leads to significant symptomatic relief and improved neurological outcomes. Comparative analysis indicates superior efficacy of atlantoaxial fixation over traditional decompression techniques, especially in patients with concurrent basilar invagination and syringomyelia. This surgical Video 1 illustrates a case of a 20-year-old male patient with tonsillar herniation, syringomyelia, atlantooccipital assimilation, atlantoaxial instability, basilar invagination and dorsolumbar scoliosis, who was successfully managed with cervical fixation using a bilateral C1 lateral mass and C2 transpedicular screw construct. This surgery was able to achieve clinical and radiologic improvement without the need for a foramen magnum decompression. The patient consented to the procedure and to the publication of his images.