Atlantoaxial dislocation in the setting of NMLFS.

BACKGROUND: Nablus mask-like facial syndrome (NMFLS) is an extremely rare genetic syndrome characterized by facial dysmorphia as well as developmental delay. In the present report we describe a potential association between non-traumatic atlanto-occipital dislocation and NMFLS in an 11-year old female lacking typical facial features of NMFLS. CASE DESCRIPTION: An 11-year-old female with autism presented with symptoms of persistent headache and vomiting as well as neck stiffness. Further investigation and CT imaging revealed congenital malformation of the skull base and craniocervical junction with complete posterior subluxation of the left occipital condyle. MRI findings later corroborated the findings on CT. CONCLUSIONS: The patient was successfully treated with occipitocervical fusion. The findings in this case suggest the possibility that atlanto-occipital instability and generalized occipitocervical may be associated with NMFLS.

[Preoperative Simulation for Complication Control in Spinal Surgery].

The craniovertebral junction not only contains anatomically important structures such as the medulla oblongata, upper cervical spinal cord, and vertebral artery, but also controls the dynamic movements of flexion, extension, and rotation of the head and neck. Consequently, instability and spinal deformities can easily occur in the craniovertebral region, and appropriate treatment should be selected based on the specificity of the lesion. Basilar invagination often involves bone and vascular anomalies and fusion surgery is often required. Therefore, careful pre-operative simulations are necessary. The creation and use of three-dimensional bone models, including image navigation, are useful for surgical simulation.

Exploring the Pathogenesis of Atlanto-Occipital Instability in Chiari Malformation With Type II Basilar Invagination: A Systematic Morphological Study.

BACKGROUND: Our previous study suggested that atlanto-occipital instability (AOI) is common in patients with type II basilar invagination (II-BI). OBJECTIVE: To further understand the pathogenesis of AOI in Chiari malformations (CM) and CM + II-BI through systematic measurements of the bone structure surrounding the craniocervical junction. METHODS: Computed tomography data from 185 adults (80 controls, 63 CM, and 42 CM + II-BI) were collected, and geometric models were established for parameter measurement. Canonical correlation analysis was used to evaluate the morphological and positional relationships of the atlanto-occipital joint (AOJ). RESULTS: Among the 3 groups, the length and height of the condyle and superior portion of the lateral masses of the atlas (C1-LM) were smallest in CM + II-BI cases; the AOJ had the shallowest depth and the lowest curvature in the same group. AOJs were divided into 3 morphological types: type I, the typical ball-and-socket joint, mainly in the control group (100%); type II, the shallower joint, mainly in the CM group (92.9%); and type III, the abnormal flat-tilt joint, mainly in the CM + II-BI group (89.3%). Kinematic computed tomography revealed AOI in all III-AOJs (100%) and some II-AOJs (1.5%) but not in type I-AOJs (0%). Morphological parameters of the superior portion of C1-LM positively correlated with those of C0 and the clivus and significantly correlated with AOI. CONCLUSION: Dysplasia of the condyle and superior portion of C1-LM exists in both CM and II-BI cases yet is more obvious in type II-BI. Unstable movement caused by AOJ deformation is another pathogenic factor in patients with CM + II-BI.

Proposed Grading System for Posterior Atlantooccipital Membrane Complex Injuries on Cervical Magnetic Resonance Imaging.

BACKGROUND: The posterior atlantooccipital membrane complex consists of the posterior atlantooccipital membrane and posterior atlantoaxial membrane. Posttraumatic, posterior atlantooccipital membrane complex injuries may have varied appearances on cervical magnetic resonance imaging. The purpose of this study was to identify the different types of posterior atlantooccipital membrane complex injuries that occur in trauma patients. METHODS: Patients who suffered a posterior atlantooccipital membrane complex injury were identified retrospectively using key word searches of cervical magnetic resonance imaging reports between 2013 and 2020 using Nuance mPower software. All relevant imaging studies were reviewed by 2 neuroradiologists. A description of the location and type of posterior atlantooccipital membrane complex injury was recorded, along with additional osteoligamentous trauma of the craniocervical junction and relevant clinical history. RESULTS: Forty-one patients were identified with acute posterior atlantooccipital membrane complex injury. Four distinct patterns of posterior atlantooccipital membrane complex injury were observed. CONCLUSIONS: A grading system for posterior atlantooccipital membrane complex injuries is proposed on the basis of these data: grade 1-edema confined to the posterior atlantooccipital and atlantoaxial membrane; grade 2-edema confined to the posterior atlantooccipital and atlantoaxial membrane and ligamentum nuchae; grade 3-stripping injury of the posterior atlantooccipital membrane with C1-C2 dorsal epidural hematoma; and grade 4-frank disruption of the posterior atlantooccipital membrane at C1 with edema in the remaining posterior atlantooccipital membrane complex.

Atlanto-occipital Dissociation in the Setting of Relatively Normal Radiologic Findings.

BACKGROUND: Craniocervical junction (CCJ) dislocations are often fatal. Atlanto-occipital dissociation can be challenging to diagnose, especially in patients who present with absent or subtle radiologic signs. CASE DESCRIPTION: A neurologically intact 37-year-old patient presented to the hospital following a high-speed motor vehicle accident. Initial computed tomography scans showed normal CCJ anatomy, but magnetic resonance imaging (MRI) of the CCJ was performed to further evaluate perimesencephalic subarachnoid hemorrhage. MRI revealed partial disruption of the anterior atlantoaxial membrane and tectorial membrane as well as complete disruption of the posterior atlanto-occipital membrane, ligamentum flavum, and apical ligament, signifying atlanto-occipital dissociation. Halo spinal immobilization was performed in preparation for stabilization with posterior occipitocervical fusion; however, the CCJ distracted widely during surgery owing to the accident-related dislocation, signifying an unstable fracture. Posterolateral fusion was performed, and the distraction injury was corrected via posterior surgical instrumentation. CONCLUSIONS: Normal occiput-C1 craniometric parameters in the setting of unexplained perimesencephalic subarachnoid hemorrhage does not eliminate the possibility of missed or delayed diagnosis of traumatic atlanto-occipital dissociation injuries. Cervical MRI without contrast should be considered in patients with vertebral artery dissection or perimesencephalic subarachnoid hemorrhage after a blunt injury with neck pain. When MRI shows evidence of disruption of ≥2 atlanto-occipital ligaments, surgical stabilization should be considered, as these are clinically very unstable injuries.

Osteoradionecrosis of the Occipitocervical Junction After Radiation for Head and Neck Cancer: A Report of 8 Cases.

BACKGROUND: Osteoradionecrosis (ORN) refers to the degenerative changes seen in bone after local radiation, particularly in head and neck cancer. ORN can present as neck or facial pain and may be confused with tumor recurrence. Magnetic resonance imaging (MRI) and positron emission tomography (PET) scans are often inconclusive, requiring percutaneous biopsy to differentiate ORN from infection and recurrent disease. We reviewed the utility of preprocedural imaging in guiding the decision to biopsy in cases of ORN. CASE DESCRIPTION: Eight patients with a history of prior head and neck cancer, radiation therapy, and suspected ORN at the skull base, occipitocervical junction, and atlantoaxial spine were identified retrospectively from a single academic medical center. In 4 cases, MRI findings and PET imaging were negative for recurrence. One patient in this group underwent an aborted biopsy. Four patients had MRI concerning for infection or recurrent tumor with PET-positive lesions. Three patients in this group underwent biopsy that was negative for recurrent tumor. One patient developed an arteriovenous fistula after biopsy. The fourth patient was observed and did not demonstrate progression at 5 months. At last follow-up for all patients, there was no evidence of tumor recurrence or metastasis at the index site to indicate a misdiagnosis for recurrent tumor. CONCLUSIONS: This case series highlights that PET scanning may not be useful in predicting which patients will benefit from biopsy for ORN because no patients with PET-positive lesions had histopathologic evidence of tumor recurrence or metastasis on biopsy.

Atlanto-occipital ligament calcification: a novel imaging finding in pediatric rotational vertebral artery occlusion.

We describe a 2-year-old girl with bow hunter syndrome complicated by vertebral artery dissection and multiple ischemic infarcts. Pediatric bow hunter syndrome is a rare and likely under-recognized disorder. Interestingly, our patient had atlanto-occipital ligament calcification on CT scan, an imaging finding that has not been reported in association with bow hunter syndrome and one that might help increase recognition of this dynamic disorder of the posterior circulation.

Calcifying pseudoneoplasm of the neuraxis: A case report and review of the literature.

Calcifying pseudoneoplasms of the neuroaxis (CAPNON) are rare, slow-growing, fibro-osseous lesions that can occur throughout the entire neuroaxis with few reported cases. We describe a case of craniocervical CAPNON that was treated by our unit and review the available literature. CAPNON are rare benign lesions occurring throughout the neuroaxis. Although rare, these lesions should be considered in cases of histopathological and radiology features of calcified lesions in both brain and spine. Despite a paucity of reports complete safe surgical resection should remain the goal to obtain the best clinical outcomes.

Intraspinal Degenerative Cyst Causing Compression Of Cervicomedullary Junction.

BACKGROUND: Intraspinal degenerative cysts in the cervical region are rare disorders that may cause myelopathy or radiculopathy. Most of the intraspinal degenerative cysts reported are extradural cysts. This case report includes the neuroimaging, intraoperative, pathologic, and postoperative findings obtained in a patient with a degenerative intradural cyst at the craniovertebral (CV) junction. CASE DESCRIPTION: We report a patient presenting with progressive spastic quadriparesis resulting from compressive myelopathy due to intradural degenerative cyst at the CV junction. Preoperative magnetic resonance imaging showed the intradural cyst at the cervicomedullary junction with degenerative changes causing myelopathy. We performed decompression of the CV junction, and histopathology of the cyst revealed degenerative cyst. Postoperatively the symptoms were relieved with no apparent complication. Intraspinal degenerative cysts causing compressive myelopathy are rare. To the best of our knowledge, this is the first case of intradural degenerative cyst at the CV junction. CONCLUSIONS: In this case report, we demonstrated the clinical, imaging, intraoperative, and pathologic features of a degenerative intraspinal cyst at the CV junction that was intradural in location. Compression of the spinal cord resulted in a gradually progressive myelopathy that showed remarkable improvement immediately after decompression by cystectomy.

Can the Etiopathogenesis of Chiari Malformation Be Craniocervical Junction Stabilization Difference? Morphometric Analysis of Craniocervical Junction Ligaments.

OBJECTIVE: The craniocervical junction permits a certain amount of mobility for the cervical spine. The biomechanical properties of occipital bone-atlas joint mainly depend on the bony structure, and atlas-axis joint biomechanical properties mainly depend on ligamentous structure. The underlying etiologic factor of Chiari malformation (CM) is debatable. Nowadays, some researchers argue that stabilization difference is one of the suspicious factors for etiopathogenesis. We aim to analyze the ligamentous morphometric differences of CM. METHODS: Magnetic resonance images of 93 adult healthy subjects (n = 93) without any craniocervical junction development abnormalities and 25 (n = 25) adult patients with craniocervical junction development abnormalities (Arnold CM) were evaluated. Length, width, and length-width ratios of ligaments were evaluated. RESULTS: Length of transverse ligament (mean: 23 ± 3.6 [range: 12.1-31.4]) in the normal population was significantly longer than transverse ligament length in CM patients (mean: 21.3 ± 2.5 [range: 17.2-24.9]). Length of alar ligament (mean: 10.7 ± 2 [range: 5.1-15.4]) in the normal population was significantly longer than alar ligament length in CM patients (mean: 8.8 ± 3.8 [range: 1.1-16.6]) (P = 0.007). CONCLUSIONS: Craniocervical ligaments play an important role in maintaining stability and motion capacity of this region. This study promoted better understanding of craniocervical junction anomalies and provided data that facilitate performing more precise surgical treatment.

Basilar invagination in a child with atlanto-occipital subluxation and suspected prenatal Dandy-Walker malformation.

BACKGROUND AND PURPOSE: Although advances in imaging have allowed earlier and more accurate diagnosis of various fetal anomalies, Dandy-Walker malformation (DWM) remains one of the more challenging central nervous system anomalies to diagnose accurately before birth. Basilar invagination (BI), which is a dislocation of the dens in an upward direction, is occasionally accompanied by Klippel-Feil syndrome (KFS). We report a pediatric case of BI caused by atlanto-occipital subluxation (AOS) in KFS, suspected of having DWM prenatally but head magnetic resonance images (MRI) showed no evidence of that at 7 months of age. CASE: At 28 weeks of gestation, fetal MRI study revealed a small cerebellar vermis, leading us to suspect a DWM. The patient was born at 40 weeks of gestation. Head CT showed inferior vermian hypoplasia without findings of hydrocephalus. Cervicothoracic CT showed cervical lamina assimilations, thoracic hemivertebrae, and cervicothoracic scoliosis. He was diagnosed with Dandy-Walker variant and KFS. At 7 months of age, head MRI showed near normal cerebellum and vermis and there was no evidence of the DWM. He did not have intellectual or developmental delay and imaging studies were performed periodically. At 9 years of age, an already existing cough headache deteriorated. Three-dimensional reconstructed images from CT scan showed C1 hypoplasia, fusion of C1 and C2, BI, and AOS. Sagittal T2-weighted MRI showed protrusion of cerebellar tonsils inferiorly to the level of the posterior arch of C2. Serum calcium, phosphate, and parathyroid hormone levels were normal. The diagnosis was tonsillar herniation related to BI following AOS in KFS. Posterior occipitocervical fixation was performed under traction. CONCLUSIONS: We found out two important clinical issues: DWM findings after birth can be disappearing and BI can present sequential deterioration because of AOS in KFS. Our observation indicated the possible prognosis of pediatric BI with long follow-up and can help us decide on its surgical treatment timing when associated with AOS.

Management of Unique Basilar Invagination Combined with C1 Prolapsing into the Foramen Magnum in Children: Report of 2 Cases.

BACKGROUND: Basilar invagination (BI) is a developmental anomaly and commonly presents with neurologic findings. The incidence of BI associated with other osseous anomalies of the craniovertebral junction is high, including incomplete ring of C1 with spreading of the lateral masses, atlanto-occipital assimilation, hypoplasia of the atlas, basiocciput hypoplasia, and occipital condylar hypoplasia. However, BI combined with C1 prolapsing into the foramen magnum (FM) is an extremely rare condition. CASE DESCRIPTION: A previously healthy 6-year-old girl (case 1) presented with extremities numbness and left limb weakness over a period of 3 months. Radiograph and computed tomography (CT) scan demonstrated basilar invagination with C1 and dens upward into the FM and C2-3 congenital fusion. Magnetic resonance imaging (MRI) showed ventral brainstem and medulla compression, and the medulla-cervical angle was about 100°. The patient underwent transoral anterior decompression, reduction, and fusion by transoral atlantoaxial reduction plate surgery. The symptoms of extremities numbness and limb weakness were all alleviated after surgery. Postoperative MRI showed that the medulla-cervical angle improved from 100° to 143°. An 11-year-old boy (case 2) presented with a 2-month history of limbs numbness and weakness. CT scan and MRI demonstrated BI and compression of the spinal cord, with a craniospinal angle of only 63°. The 3-dimensional (3D) printed model showed that the anterior arch and lateral of C1 was 90° flipping and vertically upward prolapsing into the FM together with the dens, and the width of the atlas was greater than the maximum diameter of the FM, which resulted in structural incarceration. The patient received posterior occipitocervical fixation and fusion surgery with hyperextension skull traction. Postoperative CT scan revealed the craniospinal angle increased to 102°. CONCLUSIONS: We present 2 rare cases of BI combined with C1 prolapsing into the FM. We adopted different surgical strategies with satisfying outcome for these patients. We deem that the treatment of unique BI should be individualized according to the different image characteristics. The image-based modern rapid prototyping and 3D printed techniques can provide invaluable information in presurgical planning for complex craniovertebral junction anomalies.

Biomechanical Rationale for the Development of Atlantoaxial Instability and Basilar Invagination in Patients with Occipitalization of the Atlas: A Finite Element Analysis.

OBJECTIVE: Occipitalization of the atlas (OA) often is associated with atlantoaxial dislocation and basilar invagination. The purpose of this study is to determine the biomechanical difference between normal and OA conditions in the craniovertebral junction and to further explore the rationale for development of atlantoaxial dislocation and basilar invagination using the finite element model (FEM). METHODS: A ligamentous, nonlinear, sliding-contact, 3-dimensional FEM of the occipitoatlantoaxial complex was generated. Validation of the model was accomplished by comparing kinematic predictions with experimental data. We defined the atlantooccipital joint as a tie contact to simulate the OA deformity. The range of motion and the value of the maximum Von Mises stress were compared between the intact and OA models. RESULTS: We found all of the predicted data in the intact FEM fell within 1 standard deviation of the cadaver data for all 6 loadings. The OA simulation significantly reduced the overall range of motion of the occipitoatlantoaxial complex at all loadings. The maximum Von Mises stress was predicted to increase at the transverse ligament and the superior articular facet of the axis for all the flexion, extension, lateral bending, and axial rotation loadings. CONCLUSIONS: The OA could result in hypermobility of the atlantoaxial segment and cause overstress in the transverse ligament and the lateral atlantoaxial joints. These changes explain the pathogenesis of atlantoaxial dislocation and basilar invagination associated with OA. Follow-up should be scheduled regularly due to the nature of the dynamic development of atlantoaxial dislocation and basilar invagination.

Atlantooccipital Synovial Cyst with Isolated Hypoglossal Nerve Palsy: Case Report of Nonfusion Surgical Approach and Review of Literature.

BACKGROUND: Isolated hypoglossal nerve palsy caused by an atlantooccipital synovial cyst is a rare lesion, with fewer than 5 cases reported in the literature. CASE DESCRIPTION: Our patient presented with acute hypoglossal nerve dysfunction. Our differential included neoplasm, trauma, stroke, multiple sclerosis, or other inflammatory/infectious etiology. Imaging revealed a peripherally enhancing, extradural focus in the left premedullary cistern, most likely consistent with a synovial cyst. CONCLUSIONS: A left suboccipital craniectomy was performed in the region of the left hypoglossal canal, in which a cystic structure was noted at the occipital condyle and C1 vertebral junction. The nerve was adequately decompressed via aspiration of the cyst. Postoperatively, the patient substantially improved. Although rare, synovial cysts must be included in the differential diagnosis of atlantooccipital lesions.

Extreme lateral transodontoid approach to the ventral craniocervical junction: cadaveric dissection and case illustrations.

OBJECTIVE: Surgical treatment of pathological processes involving the ventral craniocervical junction (CCJ) traditionally involves anterior and posterolateral skull base approaches. In cases of bilateral extension, when lesions extend beyond the midline to the contralateral side, a unilateral corridor may result in suboptimal resection. In these cases, the lateral extent of the tumor will prevent extirpation of the lesion via anterior surgical approaches. The authors describe a unilateral operative corridor developed along an extreme lateral trajectory to the anterior aspect of the clival and upper cervical dura, allowing exposure and resection of tumor on the contralateral side. This approach is used when the disease involves the bone structures inherent to stability at the anterior CCJ. METHODS: To achieve exposure of the ventral CCJ, an extreme lateral transcondylar transodontoid (ELTO) approach was performed with transposition of the ipsilateral vertebral artery, followed by drilling of the C1 anterior arch. Resection of the odontoid process allowed access to the contralateral component of lesions across the midline to the region of the extracranial contralateral vertebral artery, maximizing resection. RESULTS: Exposure and details of the surgical procedure were derived from anatomical cadavers. At the completion of cadaveric dissection, morphometric measurements of the relevant anatomical landmarks were obtained. Illustrative case examples for approaching ventral CCJ chordomas via the ELTO approach are presented. CONCLUSIONS: The ELTO approach provides a safe and direct surgical corridor to treat complex lesions at the ventral CCJ with bilateral extension through a single operative corridor. This approach can be combined with other lateral approaches or posterior infratemporal approaches to remove more extensive lesions involving the rostral clivus, jugular foramen, and temporal bone.

Instrumented fusion in a 12-month-old with atlanto-occipital dislocation: case report and literature review of infant occipitocervical fusion.

BACKGROUND: The treatment of atlantoaxial dislocation in very young children is challenging and lacks a consensus management strategy. DISCUSSION: We review the literature on infantile occipitocervical (OC) fusion is appraised and technical considerations are organized for ease of reference. Surgical decisions such as graft type and instrumentation details are summarized, along with the use of bone morphogenic protein and post-operative orthoses. ILLUSTRATIVE CASE: We present the case of a 12-month-old who underwent instrumented occipitocervical (OC) fusion in the setting of traumatic atlanto-occipital dislocation (AOD). CONCLUSION: Occipitocervical (OC) arthrodesis is obtainable in very young infants and children. Surgical approaches are variable and use a combination of autologous grafting and creative screw and/or wire constructs. The heterogeneity of pathologic etiology leading to OC fusion makes it difficult to make definitive recommendations for surgical management.

Occipitocervical Instability After Far-Lateral Transcondylar Surgery: A Biomechanical Analysis.

BACKGROUND: After a far-lateral transcondylar approach, patients may maintain neutral alignment in the immediate postoperative period, but severe occipitoatlantal subluxation may occur gradually with cranial settling and possible neurological injury. Previous research is based on assumptions regarding the extent of condylar resection and the change in biomechanics that produces instability. OBJECTIVE: To quantify the extent of bone removal during a far-lateral transcondylar approach, determine the changes in range of motion (ROM) and stiffness that occur after condylar resection, and identify the threshold of condylar resection that predicts alterations in occipitocervical biomechanics. METHODS: Nine human cadaveric specimens were biomechanically tested before and after far-lateral transcondylar resection extending into the hypoglossal canal (HC). The extent of condylar resection was quantified using volumetric comparison between pre- and postresection computed tomography scans. ROM and stiffness testing were performed in intact and resected states. The extent of resection that produced alterations in occipitocervical biomechanics was assessed with sensitivity analysis. RESULTS: Bone removal during condylar resection into the HC was 15.4%-63.7% (mean 35.7%). Sensitivity analysis demonstrated that changes in biomechanics may occur when just 29% of the occipital condyle was resected (area under the curve 0.80-1.00). CONCLUSION: Changes in occipitocervical biomechanics may be observed if one-third of the occipital condyle is resected. During surgery, the HC may not be a reliable landmark to guide the extent of resection. Patients who undergo condylar resections extending into or beyond the HC require close surveillance for occipitocervical instability.

Surgical Treatment of Skull Base Tumors, Extending to Craniovertebral Junction.

OBJECTIVE: To conduct a comparative analysis of 2 groups of patients with skull base chordomas extending onto the craniovertebral junction, who underwent surgical treatment using extracranial approaches with and without craniocervical fusion. METHODS: The study group included 29 patients with skull base chordomas, extending to the craniovertebral junction, who were operated on from 2000 to 2015. The patients underwent the following surgical treatment: posterior craniocervical fusion followed by tumor removal using transoral and combined transoral and transnasal approaches. The reference group included 21 patients with the same disease, who underwent tumor removal surgery using the transoral approach without craniocervical fusion. RESULTS: In the study group, in 27.5% of all cases (8 patients), the tumors were removed radically; in 65.5% of all cases (19 patients), the tumors were removed subtotally; and in 7% of all cases (2 patients), the tumors were removed partially. In the reference group, the extent of surgical radicality was as follows: radical, 0%; subtotal, 19% (4 cases); partial, 81% (17 cases). CONCLUSIONS: Use of the proposed surgical technique in clinical practice may help increase the radicality of tumor excisions, decrease the number of postoperative complications, accelerate the rehabilitation process, and increase the quality of life in patients with skull base tumors extending to the craniovertebral junction.

Traumatic atlanto-occipital dislocation: do children and adolescents have better or worse outcomes than adults? A narrative review.

BACKGROUND: Traumatic atlanto-occipital dislocation is an uncommon, severely unstable pathology, which can lead to detrimental or even fatal neurological impairment. Specifically, children have consistently been reported to be more susceptible to this type of injury because of their disproportionately larger head, ligament laxity, and injury mechanisms. However, to date, rates of missed injury and outcomes including neurologic recovery of pediatric and adult populations following this insult have not been comparatively evaluated. METHODS: Standard search engines were used to investigate outcomes of traumatic atlanto-occipital dislocation in children and adolescents compared to adults. CONCLUSIONS: Based on case reports and small series from the literature, it seems that children and adolescents tend to have a better likelihood of survival with the possibility of long-term neurological complications. Comparatively, adults who suffer traumatic atlanto-occipital dislocation either succumb to their injuries or survive with very little if any neurological complications.

[Occipitocervical junction: Aanatomy, craniometry and pathology]. / Okzipitozervikaler Übergang: Anatomie, Kraniometrie und Pathologien.

The occipitocervical junction comprises of the occiput condyles, the atlas, and the axis. The radiological evaluation of this region is supported by craniometric measurement methods which are based on predefined anatomical landmarks. The main pathologies of the occipitocervical junction are traumatic injuries, congenital anomalies or normal variants, infections, arthropathies, and tumors. In this article, the anatomy of the occipitocervical junction as well as the most important craniometric measurement methods are explained. Moreover various pathologies and similar appearing normal variants are presented.