Reduction of atlantoaxial dislocation prevented by pathological position of the transverse ligament in fixed, irreducible os odontoideum: operative illustrations and radiographic correlates in 41 patients

Abstract

OBJECTIVE Os odontoideum (OO) is a craniovertebral junction (CVJ) abnormality in which an ossicle (small bone) is cranial to a hypoplastic dens by a variable gap. This abnormality can result in instability, which may be reducible or irreducible. What leads to irreducibility in OO is unclear. Therefore, the authors sought to better understand the causes of irreducibility in OO. METHODS A retrospective review was conducted, which identified more than 200 patients who had undergone surgical treatment for OO between 1978 and 2015 at the University of Iowa Hospitals and Clinics. Only the 41 patients who had irreducible OO were included in this study. All inpatient and outpatient records were retrospectively reviewed, and patient demographics, clinical presentation, radiographic findings, surgical treatment, and operative findings were recorded and analyzed. RESULTS The cohort of 41 patients who were found to have irreducible OO included both children and adults. A majority of patients were adults (61% were 18 years or older). Clinical presentation included neck pain and headache in the majority of patients (93%). Weakness, sensory disturbances, and myelopathy were invariably present in all 41 patients (100%). Down syndrome was much more common in the pediatric cohort than in the adult cohort; of the 16 pediatric patients, 6 had Down syndrome (38%), and none of the adults did. Of the 16 pediatric patients, 5 had segmentation failure (31%) in the subaxial spine, and none of the adults did. A form of atlantoaxial dislocation was seen in all cases. On CT imaging, atlantoaxial facets were dislocated in all 41 cases but did not have osseous changes that would have prevented reduction. On MRI, the transverse ligament was identified anterior and inferior to the ossicle and superior to the hypoplastic odontoid process in all cases in which these studies were available (i.e., post-MRI era; 36 of 36 cases). The ligament was hypointense on T2-weighted images but also had an associated hyperintense signal on T2 images. Intraoperatively, the transverse ligament was identified anterior and inferior to the ossicle and superior to the hypoplastic odontoid process in all 41 cases. CONCLUSIONS In the largest series to date of irreducible OO and the only study to examine variable factors that lead to irreducibility in OO, the authors found that the position of the transverse ligament anterior and inferior to the ossicle is the most common factor in the irreducibility of OO. The presence of granulation tissue and of the dystopic variant of OO is also associated with irreducibility. The presence of Down syndrome and segmentation failure probably leads to faster progression of ligamentous incompetence and therefore earlier presentation of instability and irreducibility. This is the first study in which intraoperative findings regarding the transverse ligament have been correlated with MRI.

Keywords: CMJ = cervicomedullary junction; CVJ = craniovertebral junction; OO = os odontoideum; OP = odontoid process; congenital; craniocervical junction; craniovertebral junction; cruciate ligament; irreducible; os odontoideum; reducible; transoral approach; transverse ligament.