CranioCervical Disorders

Predisposition to disorders with

Chiari / brainstem deformity
CC Instability due to lax ligaments, as assessed by cervical flexion/extension MRI/XR

Elher Danlos Syndrome (17 types, hypermobile are most common >1:100)
Osteogenesis Imperfecta (brittle bone disease, fractures+, shorter height, blue sclera, seizures, hearing loss, communicating hydrocephalus, basilar invagination)

Marfan Syndrome (arachnodactyly, dilation or dissection of aorta, pectus excavatum or carinatum, dislocation lenses, retinal detachment, scoliosis
(maybe George Washington and Abraham Lincoln had Marfans).

Loeys Dietz Syndrome (similar to Marfan with strabismus, club foot, scoliosis, heart path, bifid uvula)

Stickler Syndrome (flattened nasal bridge, small jaw, eyes glaucoma cataracts myopia, hearing issues, scoliosis, cleft palate, arthritis, scoliosis

Degenerative CC disorders from movement with lax ligaments.

2103 Consensus
Pathology Criteria for CCH (hypermobility)
and Basilar Invagination/Impression (dens projects above foramen magnum)

3 criteria
1. Clivo-axial angle
2. Grabb-Mapstone-Oakes Measurement
3. Harris measurement

Clivo-axial angle  CXA   (figure 1 below)
Wackenheim line angle with plane of dens
should be >150 deg (150-165deg), if <135 = pathological kyphotic CXA because has FULCRUM effect on brainstem

G-M-O measurement  (figure 2 above)
a line is drawn from the basion (the midpoint of the anterior margin of the foramen magnum) to the inferior posterior C2. A perpendicular line is then drawn from the center of this line to the dura of the brainstem. A Grabb-Oakes measurement greater than 9 mm denotes a form of basilar invagination. This is a very helpful measurement for determining how much a retroflexed odontoid is compressing the brainstem.

Harris Measurement (figure 3)
distance between the basion and the Posterior Axial Line. This distance should not be more than 12 mm. A measurement of more than 12 mm also denotes instability. This measurement can also be used to measure the translation between flexion and extension in dynamic imaging

between clivus (anterior to foramen magnum (abducens passes over and can be compromised causing
Wackenheim Line posterior plane of Clivus, association with tip of dens which should not pass through line. if it does then posterior subluxation?

Symptoms of Ventral Brain Stem Compression (Cervicomedullary Syndrome)

  • A heavy headache (often referred to as feeling like a “bobblehead” or feeling like the head is a “bowling ball”)
  • A Chiari-type pressure headache aggravated by Valsalva maneuvers (because these conditions, like Chiari, can also cause flow issues)
  • Dysautonomia (including tachycardia, heat intolerance, orthostatic intolerance, syncope (fainting), polydipsia (extreme thirst), delayed gastric emptying, chronic fatigue)
  • Neck pain (often severe)
  • Central or mixed sleep apnea
  • Facial pain or numbness – Occasionally, including Trigeminal Neuralgia
  • Balance and coordination impairment
  • Muscle weakness
  • Dizziness and vertigo
  • Vision problems, including double vision and downward nystagmus
  • Reduced gag reflex and dysphagia (difficulty swallowing)
  • Tinnitus (ringing in the ears) and hearing loss
  • Nausea and vomiting
  • Paralysis
  • In more severe cases, non-epiform seizures have also been documented

Surgical Treatments

Basilar Invagination  =  reduction
Brainstem Compression due to Chiari mal = decompression
CCI = stabilization and fusion