A novel surgical protocol for safe and accurate placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability: technical details, accuracy assessment and perioperative complications.

PURPOSE: To introduce a novel surgical protocol for safe and accurate placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability, and to categorize the screw accuracy and perioperative complications regarding this technique in a large case series. METHODS: Between January 2015 and January 2020, patients who had atlas assimilation, basilar invagination and atlantoaxial instability, and underwent atlantoaxial fixation using C1 lateral mass screws were reviewed. C1 lateral mass screws were placed with a novel surgical protocol following a series key steps, including posterior para-odontoid ligament release, panoramic exposure of the invaginated lateral mass, and diligent protection of the abnormal VA. Screw accuracy and related complications were specifically evaluated. RESULTS: A total of 434 C1 lateral mass screws were placed. Fifteen screws (3.5%) were classified as unacceptable, 54 screws (12.4%) were classified as acceptable, and 365 screws (84.1%) were classified as ideal. Overall, 96.5% of screws were deemed safe. There were no cases of vascular injury or permanent neurological defects. One patient with an unacceptable screw presented with hypoglossal nerve paralysis and recovered after an immediate revision surgery. Thirty-seven patients complained about occipital neuralgia and were successfully managed with medication. CONCLUSION: Placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability following this surgical protocol is safe and accurate. Thorough para-odontoid ligamental release, wide exposure of the invaginated lateral mass, and diligent protection of the vertebral artery are critical to maximize the chances of successful screw placement.

Stability-Sparing Endoscopic Endonasal Odontoidectomy in a Malformative Craniovertebral Junction: Case Report and Biomechanical Considerations.

BACKGROUND: The craniovertebral junction (CVJ) is often involved in a wide range of congenital, developmental and acquired pathologies that can create bony and ligamentous instability or cause direct compression on the medulla and cervical spine cord, resulting in significant impairment. Atlas assimilation is the most common malformation in the CVJ and can be frequently associated with basilar invagination (BI) and Chiari malformation (CM) type I. Posterior atlas assimilation more frequently leads to BI type II with a mass effect on neural structures but usually no signs of biomechanical instability. Operative approaches to the CVJ have undergone a remarkable evolution and can be divided into ventral, lateral and dorsal ones. In this kind of surgery, it is vital to detect and eventually treat any CVJ instability. CASE DESCRIPTION: We present a case of CVJ malformation comprising assimilation of the posterior arch of the atlas, BI type II and CM, treated by endoscopic endonasal odontoidectomy and partial clivus removal to spare CVJ stability. CONCLUSION: Neurological and biomechanical analysis of all CVJ malformations permits stratification and selection of those cases that can be managed by simple, direct, minimally invasive decompression with no need for surgical fusion.

A morphometric study of the atlas occipitalization and coexisted congenital anomalies of the vertebrae and posterior cranial fossa with neurological importance.

PURPOSE: Our study highlights the morphometry of the partial and complete atlas occipitalization (AOZ), its coexistence with fusions of the 2nd and 3rd cervical vertebrae and morphological and morphometric abnormalities of the posterior cranial fossa that are of paramount neurological importance. METHODS: One hundred and eighty adult dry skulls, the atlas and axis vertebrae were examined. RESULTS: Four skulls (2.2 %) showed AOZ. Two of them (1.1 %) presented a partial AOZ, one male skull (0.6 %) a complete AOZ and a female skull (0.6 %) had a fused left hemiatlas with the occipital bone and a fusion of the 2nd and 3rd cervical vertebrae. The inner anteroposterior and transverse diameters of the foramen magnum (FM) in the control group were 34.6 ± 3.46 and 29.3 ± 3.47 mm. Only the skull with the complete AOZ had a reduced outer anteroposterior diameter of the FM (29.8 mm), while no specimen was found with a reduced transverse diameter. A wide total decrease (range 13.1-50.9 %) in the surface area of the FM in skulls with AOZ was detected. Extracranial, the clivus length in two skulls with AOZ was smaller than the normal range. No skull was detected with a reduction in the intracranial length of the clivus. All skulls with the AOZ had a vermian fossa. CONCLUSIONS: The study adds important morphometric details about the partial and complete AOZ and correlates the phenomenon of synostosis with the narrowing of the FM, particularly in the case of complete AOZ. Awareness of the AOZ and other fusions of the upper cervical vertebrae and their topographical relations and attendant problems are of paramount importance to surgeons, when operate to the craniocervical junction, or interpret imaging studies to plan a safe surgery for nerve or spinal tissue decompression.

Evolution of transoral approaches, endoscopic endonasal approaches, and reduction strategies for treatment of craniovertebral junction pathology: a treatment algorithm update.

The craniovertebral junction (CVJ), or the craniocervical junction (CCJ) as it is otherwise known, houses the crossroads of the CNS and is composed of the occipital bone that surrounds the foramen magnum, the atlas vertebrae, the axis vertebrae, and their associated ligaments and musculature. The musculoskeletal organization of the CVJ is unique and complex, resulting in a wide range of congenital, developmental, and acquired pathology. The refinements of the transoral approach to the CVJ by the senior author (A.H.M.) in the late 1970s revolutionized the treatment of CVJ pathology. At the same time, a physiological approach to CVJ management was adopted at the University of Iowa Hospitals and Clinics in 1977 based on the stability and motion dynamics of the CVJ and the site of encroachment, incorporating the transoral approach for irreducible ventral CVJ pathology. Since then, approaches and techniques to treat ventral CVJ lesions have evolved. In the last 40 years at University of Iowa Hospitals and Clinics, multiple approaches to the CVJ have evolved and a better understanding of CVJ pathology has been established. In addition, new reduction strategies that have diminished the need to perform ventral decompressive approaches have been developed and implemented. In this era of surgical subspecialization, to properly treat complex CVJ pathology, the CVJ specialist must be trained in skull base transoral and endoscopic endonasal approaches, pediatric and adult CVJ spine surgery, and must understand and be able to treat the complex CSF dynamics present in CVJ pathology to provide the appropriate, optimal, and tailored treatment strategy for each individual patient, both child and adult. This is a comprehensive review of the history and evolution of the transoral approaches, extended transoral approaches, endoscopie assisted transoral approaches, endoscopie endonasal approaches, and CVJ reduction strategies. Incorporating these advancements, the authors update the initial algorithm for the treatment of CVJ abnormalities first published in 1980 by the senior author.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Metopic skull with occipitalisation of the atlas.

In this study we report a combination of anatomic variations in the neurocranium of an adult male skull. The skull is scanned using an industrial µCT system Nikon XT H 225. The cranial vault shows a persistent metopic suture, a remnant from the mendosal suture and supernumerary bones. Cranial base inspection reveals atlas occipitalisation (AO), basilar kyphosis, unusually shaped dorsum sellae and bilateral interclinoid bridging. AO is extensive without signs of atlantooccipital articulation. The anterior and posterior arches of the atlas and the right transverse process are fussed to the occipital bone above. The complete fusion of the posterior arch causes a formation of bone canals for the vertebral arteries passage into the cranium. AO in this case is not related to a reduction of the foramen magnum dimensions and clivus length.

Role of Atlas Assimilation in the Context of Craniocervical Junction Anomalies.

Atlas assimilation (AA) may be associated with atlantoaxial dislocation, Chiari malformation (CM), and basilar invagination. The importance of AA in the context of craniocervical junction (CVJ) anomalies is unclear. Considering this context, this study's objective is to discuss the role of AA in the management of CVJ anomalies, especially in CM. A comprehensive literature review was performed. In addition, some illustrative cases were discussed on the basis of our review. Finally, we propose a theoretic algorithm to evaluate patients with AA and CM. AA is a proatlas segmentation anomaly that may be complete or incomplete. It may be totally asymptomatic or symptomatic as the result of transferred shifted forces onto the C1-2 joints, leading to clear instability (atlantoaxial dislocation) or mild C1-2 instability. Cautious surgical planning may be required due to associated vertebral artery anomalies. AA with concomitant C2-C3 segmentation failure is highly associated with late C1-C2 instability. CVJ decompression failure was reported in patients with CM and a low clivus canal angle (<130-135 degrees). Patients with assimilated anterior C1 arches usually have evident AAD. CM patients with AA generally have type 1 BI or type 2 BI and are reported with higher rates of CVJ instabilities when compared with those "pure" CM. Dynamic examinations may provide additional evidence of atlantoaxial instability. Although AA per se is not considered an unstable configuration, further and detailed evaluations of patients with CM associated with AA are necessary. Some associated unstable configurations required concomitant CVJ fixation.

Anomalous vertebral arteries in Klippel-Feil syndrome with occipitalized atlas: CT angiography.

Klippel-Feil syndrome is an uncommon anomaly that may be asymptomatic. Early clinical signs such as restricted neck motion or short neck can be subtle and incorrectly treated as spasms. High incidence of associated craniovertebral junction (CVJ) anomalies such as occipitalized atlas predisposes them to serious neurologic complications requiring invasive procedures and surgeries. However, these often have anomalous vertebral artery course which is more prone to injury during CVJ procedures, and also sparsely known in radiology literature. We demonstrate the importance of computed tomography angiography in preprocedural planning to avoid catastrophic injury to anomalous vertebral artery at CVJ in such case.

C2 segmental type of vertebral artery on the unfused side of partially occipitalized atlas.

Patients with craniovertebral junction anomalies are more likely to undergo craniovertebral junction procedures because of neurologic complications. Vertebral arteries (VAs) are more prone to injury in these cases because of an often anomalous course, with potentially disabling or grave sequel. In our patient with partially occipitalized atlas, CT angiogram revealed that 1 of the VAs passed through the bony canal in the occipitalized and/or fused bone, whereas the VA on unfused side was also unexpectedly anomalous and of C2 segmental type. To the best of our knowledge, such anomaly has never been reported in studies of patients with occipitalized atlas in the literature.

Quantitative Measurements of the Skull Base and Craniovertebral Junction in Congenital Occipitalization of the Atlas: A Computed Tomography-Based Anatomic Study.

OBJECTIVE: To study the craniovertebral junction and determine the anatomic characteristics of occipitalization of the atlas (OC) by computed tomography (CT) imaging. METHODS: We retrospectively reviewed 80 cases of patients with OC who underwent cervical CT scanning between March 2012 and March 2014. Forty healthy subjects were recruited as a control cohort. Fusion pattern and associated osseous anomalies were recorded. Fifteen linear quantitative parameters were measured to study the outlet of the foramen magnum, angular dimension of the skull base, posterior cranial fossa, and height of the odontoid process. RESULTS: The most common fusion pattern was the complete osseous fusion (83.75%). Fifty-four patients (67.5%) presented with other osseous anomalies. Measurements of the OC group, such as the length of the clivus, cranial canal angle, and height of the odontoid process, were significantly different than those of the control group. The correlation analysis showed that the C1 lateral facet inclination was significantly correlated with the age of onset. CONCLUSIONS: The outlet of the foramen magnum is severely impaired in patients with OC, and the presence of other osseous anomalies is common. Deformity is not confined to the region of the assimilated atlas; the clivus and odontoid process are also shorter than normal. The lateral facet inclination likely influences disease progression.

Surgical Treatment of Occipitocervical Dislocation with Atlas Assimilation and Klippel-Feil Syndrome Using Occipitalized C1 Lateral Mass and C2 Fixation and Reduction Technique.

OBJECTIVE: To introduce and assess a surgical treatment of occipitocervical (OC) dislocation with atlas assimilation and Klippel-Feil syndrome (KFS) using occipitalized C1 lateral mass and C2 fixation and reduction technique. METHODS: From January 2007 to August 2013, 58 symptomatic patients with OC dislocation and KFS of C2-3 congenital fusion and atlas assimilation were surgically treated in our institution via this technique. After opening the C1-2 facet joints via a posterior approach, OC reduction was conducted by intraoperative manipulation and C1 lateral mass and C2 pedicle screw and rod fixation. The instrument position, fusion status, and clinical outcome were analyzed. RESULTS: The average follow-up was 36 months (range, 18-52 months). Radiologically, effective reduction was achieved in 56 patients (96.6%) and <50% reduction in 2 (3.4%) who had additional transoral decompression. Neurologic improvement and solid bone fusion were achieved in all patients. The clinical symptoms improved for all patients, with the averaged Japanese Orthopedic Association myelopathy scores increasing from 11.5 to 15.6 (P < 0.01). CONCLUSIONS: In patients with OC dislocation and KFS of C2-3 fusion and atlas assimilation, posterior manipulative reduction combined with occipitalized C1 lateral mass and C2 fixation provides a reliable and effective treatment.