FACTS
- most common congenital anomaly of CCJ
- Craniovertebral junction (CVJ) abnormality where the odontoid projects abnormally upward into the foramen magnum.
- Can be congenital (e.g., Chiari, Klippel-Feil, Down syndrome) or acquired (RA, bone softening disorders, trauma, degenerative settling).
- Concern is for ventral brainstem compression, craniocervical instability, neuro deficits, and compromised CSF flow
- Surgical need is based on: myelopathy, instability, ventral compression magnitude, and failure of traction realignment.
HPI
Focused, high-yield questions:
- Symptoms of myelopathy: gait imbalance, hand clumsiness, falls, bowel/bladder changes.
- Cranial nerve symptoms: dysphagia, dysarthria, facial numbness, vertigo.
- Neck pain, occipital headaches; worse with neck motion?
- History of RA, congenital syndromes, trauma.
- Progression speed (weeks? months? acute?).
- Signs of cord compression: Lhermitte’s, hyperreflexia, sensory levels.
- Prior cervical spine surgeries.
- Any respiratory difficulty or central sleep apnea (brainstem compression).
🚩Emergent Red Flags: Acute respiratory compromise, Rapid neurological decline, New severe occipital headache with brainstem signs, Loss of protective airway reflexes.
Associated congenital syndrome and acquired pathologies:
- Congenital: Down syndrome, Klippel-Feil syndrome, Chiari, syringomyelia, RA
- Acquired: Paget’s, osteogensis imperfecta, osteomalacia, rickets, hyperparathyroidism
IMAGING
CT C-spine:
- Look for odontoid migration above key lines:
- Chamberlain line (normal ≤ 3mm of odontoid above this line, 6mm+ definitely pathologic): hard palate to opisthinon
- McGregor line (normal ≤ 4.5mm dens above this line): hard palate to most caudal point of occiput
- Basilar impression vs invagination differentiation.
- Congenital anomalies, occipital assimilation, facet alignment.
MRI Cervical spine / brain:
- Evaluate:
- Ventral brainstem compression, cord flattening.
- Myelomalacia.
- Syrinx / CSF flow compromise.
- Ligamentous laxity or pannus (e.g., RA).
- for platybasia (may or may not be associated with BI)
A/P
Initial Management
- Immobilize the neck (C-collar) if symptomatic or unstable.
- Avoid excessive manipulation until full imaging reviewed.
- Operative indications: Myelopathy or progressive neuro deficit, Significant ventral cord/brainstem compression, demonstrated atlantoaxial instability, failure of traction to reduce deformity.
- Decide what surgery is needed based on presence of Chiari
ㅤ | Type 1 Basilar Invagination | Type 2 Basilar Invagination |
Definition | no Chiari malformation | with Chiari malformation |
Notes | Tip of odontoid tends to be above Chamberlain’s and McRaes | Tip of odontoid tends to be above Chamberlain’s but not McR |
Treatment | transoral surgery + PSF | foramen magnum decompression |
If not surgical:
- Rigid cervical immobilization.
- RA-associated pannus → aggressive rheumatologic management.
- Close follow-up with serial neuro exams and imaging.