255 lines
24 KiB
Markdown
255 lines
24 KiB
Markdown
---
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title: "Craniovertebral Junction Abnormality, General"
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docid: "e73f8637-ed99-4ff8-96c3-3afcc0e01f21"
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authors:
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- key: "bee1f359-33fb-4cba-9e6b-ed1ca1842439"
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value: "Jeffrey S. Ross, MD"
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- key: "86b8c311-8667-4afd-9b2b-0c2036a02b8a"
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value: "Julia R. Crim, MD"
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breadcrumbs:
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-
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name: "Spine"
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slug: "spine"
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treeNodeId: "b337a156-914a-4696-a77c-af206720fab5"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "ef5fd925-2033-4f3b-aa7c-640fef9aa956"
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-
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name: "Craniovertebral Junction"
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slug: "craniovertebral-junction"
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treeNodeId: "1b500928-e185-4268-95e4-d472fc416f2c"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "13201c88-825d-423b-a917-f0e7cce4a599"
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-
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name: "Craniovertebral Junction Abnormality, General"
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slug: "craniovertebral-junction-abnormali-"
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treeNodeId: null
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category: "Spine"
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documentVersionId: "c5910b07-7b82-4489-b403-86b692c9cbd5"
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imageCount: 28
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lastUpdated: "01/18/23"
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pageDescription: "Craniovertebral Junction Abnormality, General"
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pageKeywords: "Spine, Differential Diagnosis, Craniovertebral Junction, Anatomically Based Differentials, Craniovertebral Junction Abnormality, General"
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pageTitle: "Craniovertebral Junction Abnormality, General | STATdx"
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enhancedTitle: "Craniovertebral Junction Abnormality, General"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Spine"
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- "Differential Diagnosis"
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- "Craniovertebral Junction"
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- "Anatomically Based Differentials"
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- "Craniovertebral Junction Abnormality, General"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- **Hint**: Differentiate trauma vs. bony congenital variant
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- Soft tissue swelling usually evident in trauma
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- Cortication of bone indicates nonacute trauma
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- Os odontoideum thought to be nonunited fracture, not necessarily congenital variant
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- **Hint**: Watch for mass adjacent to dens
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- Pannus from rheumatoid arthritis (RA): Dens eroded, no calcification
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- Seronegative spondyloarthropathy: Like RA, plus enthesophytes, joint fusion
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- Juvenile inflammatory arthropathy: Like adult RA or seronegative spondyloarthropathy
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- Usually involves multiple levels in cervical spine
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- Growth disturbance characteristic
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- Calcium pyrophosphate deposition disease (CPPD): Calcifications, cysts in bone
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- Infection: Usually involves disc space
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- Tuberculosis involves disc space later in course of infection
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- Tumor: Origin in bone, meninges, or cord
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- **Hint**: Watch for heterogeneous high signal in bone marrow without cortical breakthrough
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- Myeloma
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- Lymphoma
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- Metastases
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- ## Helpful Clues for Common Diagnoses
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- **Bone Trauma**
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- **Odontoid fracture, C2**
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- Type I: Obliquely oriented through tip
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- Type II: Horizontally oriented through base
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- Type III: Really fracture of body; horizontally oriented, through body and below base of dens
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- **Hangman's fracture, C2**
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- Hyperflexion or hyperextension, usually from motor vehicle accident
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- Traumatic spondylolisthesis of C2
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- Fracture through C2 pedicles
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- Usually see focal kyphosis and anterolisthesis at C2-C3
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- Effendi type I: Traumatic spondylolisthesis isolated
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- Effendi type II: Also disruption of C2-C3 disc
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- Effendi type III: Also disruption of C2-C3 facet joints
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- **Burst fracture, C2**
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- Axial load injury
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- Extends through posterior cortex of vertebral body
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- **Os odontoideum**
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- Chronic nonunited fracture
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- **Congenital Bone and Ligament Abnormalities**
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- May be multiple
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- May be isolated, detected as incidental finding in adulthood
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- Often cause adjacent premature degeneration
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- **Trisomy 21**
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- Spinal stenosis
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- Instability occiput-C1 and C1-C2
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- Unlike RA, no erosion of dens
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- **Arthritis**
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- **Osteoarthritis**
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- Common at craniocervical junction
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- Involves synovial articulations: Facet joints, dens/C1 articulation
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- Dens and anterior arch of C1 develop osteophytes, sclerosis best seen on CT
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- May have prominent soft tissues posterior to dens but no erosions
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- Facet osteoarthritis at occiput-C1 or C1-C2 may develop large osteophytes, synovial cysts
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- **Calcium pyrophosphate deposition disease**
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- Mimics RA on MR, but subchondral bone plate not eroded
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- Calcifications visible on CT, radiographs
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- **Rheumatoid arthritis**
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- Calcification never present
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- Pannus heterogeneous signal intensity on MR
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- Low signal intensity areas on T2WI mimic crystals, calcification
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- Almost always see erosion of dens
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- Early erosion: Loss of subchondral bone plate
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- Late erosion: Pencilling of dens
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- Facet erosion: Atlantoaxial impaction
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- Craniocervical disease does not occur without peripheral disease (hands/feet)
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## References
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# Selected References
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1. [Patel R et al: Surgical outcomes of posterior occipito-cervical decompression and fusion for basilar invagination: a prospective study. J Clin Orthop Trauma. 13:127-33, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33680811%5Bpmid%5D)
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1. [Berritto D et al: Trauma imaging of the acute cervical spine. Semin Musculoskelet Radiol. 21(3):184-98, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28571084%5Bpmid%5D)
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1. [Hadley MN et al: Introduction to the uidelines for the management of acute cervical spine and spinal cord injuries. Neurosurgery. 72 Suppl 2:5-16, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417174%5Bpmid%5D)
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1. [Ryken TC et al: Radiographic assessment. Neurosurgery. 72 Suppl 2:54-72, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417179%5Bpmid%5D)
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1. [Theodore N et al: The diagnosis and management of traumatic atlanto-occipital dislocation injuries. Neurosurgery. 72 Suppl 2():114-26, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23417184%5Bpmid%5D)
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1. [Munera F et al: Imaging evaluation of adult spinal injuries: emphasis on multidetector CT in cervical spine trauma. Radiology. 263(3):645-60, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22623691%5Bpmid%5D)
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## Images
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### Selected Images
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**Occipital Condyle Fracture**
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*Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.*
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**Occipital Condyle Fracture**
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*Axial bone CT shows small, bony density without cortical margins adjacent to the right condyle, consistent with a nondisplaced right occipital condyle avulsion fracture <img src='img/arrows/WS.png'/>.*
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**Jefferson C1 Fracture**
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*Axial bone CT shows severe comminuted fractures involving both the posterior and anterior arches of C1 <img src='img/arrows/WS.png'/>. There is also an avulsion off of the medial aspect of the left C1 lateral mass at the insertion of the transverse ligament <img src='img/arrows/WO.png'/>. The atlantodental interval is normal.*
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**Odontoid Fracture, C2**
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*Sagittal NECT shows a type II odontoid fracture <img src='img/arrows/WS.png'/>. There is slight posterior angulation and displacement of the anterior aspect of the fracture. This fracture usually occurs in older adult patients, often from a ground-level fall, and may be missed on radiographs due to osteopenia.*
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**Burst Fracture, C2**
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*Sagittal NECT shows horizontal and vertical fractures of C2 <img src='img/arrows/WS.png'/> due to axial load injury. The posterior fragment can displace posteriorly and cause cord compression <img src='img/arrows/WO.png'/>.*
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**Chiari 1 Malformation**
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*Sagittal T2WI MR shows changes of Chiari 1 malformation with pronounced inferior position of the cerebellar tonsils <img src='img/arrows/WS.png'/>. There is assimilation of C1 to the occiput with abnormal position of the C1 arch <img src='img/arrows/WO.png'/>. Note the associated syrinx <img src='img/arrows/WC.png'/>.*
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**Chiari 2 Malformation**
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*Sagittal T1WI MR shows the typical changes of Chiari 2 malformation with hindbrain herniation <img src='img/arrows/WS.png'/>, small posterior fossa, beaked tectum <img src='img/arrows/WO.png'/>, and dysmorphic clivus <img src='img/arrows/WC.png'/>. Note also the associated callosal hypoplasia <img src='img/arrows/CS.png'/>.*
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**Craniovertebral Junction Variants**
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*Lateral radiograph shows the C1 <img src='img/arrows/WS.png'/> fused to the occiput, resulting in dysmorphic C1-C2 articulations and dysmorphic C2 body. The odontoid is triangular in shape <img src='img/arrows/WC.png'/>.*
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**Trisomy 21**
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*Sagittal bone CT shows C1/C2 subluxation <img src='img/arrows/WO.png'/> without erosions. There was no history of trauma. Marked narrowing of the spinal canal was symptomatic.*
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**Osteoarthritis**
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*Coronal NECT shows marked degenerative change about the right C1-C2 joint <img src='img/arrows/WS.png'/> with joint space loss, bony sclerosis, and irregularity. Compare to the more normal left C1-C2 joint <img src='img/arrows/WO.png'/>.*
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**Juvenile Idiopathic Arthritis**
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*Sagittal T2WI MR shows the cortical margin of the odontoid lost anteriorly due to erosions; the soft tissue mass <img src='img/arrows/WO.png'/> is due to pannus. Cranial settling is present with the odontoid at the level of clivus.*
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**Calcium Pyrophosphate Deposition Disease**
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*Sagittal T2WI MR shows a large, predominately low signal intensity mass involving the dorsal retrodental soft tissues <img src='img/arrows/WS.png'/>. There is severe cord compression from the mass with cord signal abnormality <img src='img/arrows/WO.png'/>.*
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**Tumoral Calcinosis**
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*Axial NECT at the level of C2 shows a well-defined, lobulated mass with increased attenuation surrounding the lateral C1-C2 articulation. The lesion shows the appearance of milk of calcium with diffuse increased density.*
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**Osteomyelitis, C1-C2**
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*Sagittal T2WI MR shows a large prevertebral abscess spanning C1 to C4 <img src='img/arrows/WS.png'/> and extension posteriorly involving the interspinous region <img src='img/arrows/WO.png'/>. There is involvement of the C1-C2 articulation with widening of the atlantodental interval <img src='img/arrows/WC.png'/>.*
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**Chordoma**
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*Sagittal T2WI MR shows a mass involving the craniocervical junction with marked T2 hyperintensity and spiculated internal morphology <img src='img/arrows/WS.png'/>. Morphology would be consistent with either chordoma or chondrosarcoma. The location is much more typical for chordoma.*
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**Atlantooccipital Assimilation**
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*Sagittal NECT shows findings typical for congenital assimilation of C1 to the occiput with a high-riding position of the C1 anterior arch parked underneath the inferior clivus <img src='img/arrows/WS.png'/>, upward translocation of the odontoid <img src='img/arrows/WO.png'/>, and lack of visible posterior C1 arch <img src='img/arrows/WC.png'/>.*
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**Paget Disease**
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*Sagittal T1WI MR in this patient with extensive Paget disease involving the calvarium <img src='img/arrows/WS.png'/> shows basilar impression with upward translocation of the odontoid into the foramen magnum and draping deformity <img src='img/arrows/WO.png'/> of the brainstem.*
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### Additional Images
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**Odontoid Fracture, C2**
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*Sagittal bone CT shows atrophic odontoid fracture nonunion <img src='img/arrows/WO.png'/> after open reduction and internal fixation (ORIF). This usually is due not to infection but poor vascularity.*
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**Hangman's Fracture, C2**
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*Sagittal STIR MR shows a classic example of a type 2 Hangman's fracture (Effendi classification) with bilateral pars fractures and disruption of the C2/C3 disc but intact facet joints. There is disruption of the anterior longitudinal ligament <img src='img/arrows/WS.png'/>, posterior longitudinal ligament <img src='img/arrows/WO.png'/>, and interspinous ligaments <img src='img/arrows/WC.png'/>, indicating both column disruption.*
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**Os Odontoideum**
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*Sagittal T1WI MR shows a nonunited fracture of dens <img src='img/arrows/WS.png'/>, so-called os odontoideum. Posterior displacement of the ossicle has resulted in cord impingement.*
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**Craniovertebral Junction Variants**
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*Sagittal T1WI MR shows atlantooccipital assimilation <img src='img/arrows/WS.png'/> and a small os odontoideum <img src='img/arrows/WO.png'/>. Vertebral anomalies are also seen in the subaxial region <img src='img/arrows/WC.png'/>.*
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**Aneurysmal Bone Cyst**
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*Sagittal NECT shows ballooning of the posterior process of C2 <img src='img/arrows/WS.png'/>, typical of aneurysmal bone cyst. Tumor also involves vertebral body and has resulted in a pathologic fracture <img src='img/arrows/WO.png'/>.*
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**Chiari 1 Malformation**
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*Sagittal T2WI MR shows the typical peg-shaped appearance of cerebellar tonsils <img src='img/arrows/WC.png'/>, which descend to the level of the C1 arch. The 4th ventricle is normal. There is a small syrinx <img src='img/arrows/WS.png'/>.*
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**Chiari 2 Malformation**
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*Sagittal T2WI MR shows characteristic Chiari 2 features of a small posterior fossa and 4th ventricle, medullary kink <img src='img/arrows/WS.png'/>, and verminal ectopia through the foramen magnum <img src='img/arrows/WO.png'/>.*
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**Calcium Pyrophosphate Deposition Disease**
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*Sagittal bone CT shows calcifications and soft tissue fullness <img src='img/arrows/WS.png'/> at the craniocervical junction due to calcium pyrophosphate deposition disease (CPPD). CPPD of the craniocervical junction is not uncommon in older adult patients and may cause instability.*
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**Pannus From Rheumatoid Arthritis**
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*Sagittal STIR MR shows extensive erosion of the odontoid process and a large soft tissue mass <img src='img/arrows/WS.png'/> from rheumatoid arthritis (RA). RA may mimic infection or tumor.*
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**Abscess, Epidural, Paravertebral**
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*Sagittal T2WI MR shows epidural abscess <img src='img/arrows/WS.png'/> compressing the spinal cord.*
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**Meningioma**
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*Sagittal bone CT shows a calcified mass <img src='img/arrows/WS.png'/> with a dural tail arising from the ventral dura at C2, providing a clue to the dural origin. There is mass effect on adjacent spinal cord.*
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**Multiple Myeloma**
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*Sagittal T2WI FS MR shows multiple small foci <img src='img/arrows/WS.png'/> of abnormal signal intensity in the bone marrow of C-spine, clivus, and occiput. Note posterior element involvement, which is a common MR finding with myeloma.*
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