枕颈畸形的诊断与治疗进展
主讲人:田野 教授
时间:2019年10月24日7:00-7:30
地点:外科楼骨科8层会议室
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By
Michael Rubin, MDCM, Weill Cornell Medical College
Craniocervical junction abnormalities are congenital or acquired abnormalities of the occipital bone, foramen magnum, or first two cervical vertebrae that decrease the space for the lower brain stem and cervical cord. These abnormalities can result in neck pain; syringomyelia; cerebellar, lower cranial nerve, and spinal cord deficits; and vertebrobasilar ischemia. Diagnosis is by magnetic resonance imaging (MRI) or computed tomography (CT). Treatment often involves reduction, followed by stabilization via surgery or an external device.
Neural tissue is flexible and susceptible to compression. Craniocervical junction abnormalities can cause or contribute to cervical spinal cord or brain stem compression; some abnormalities and their clinical consequences include the following:
Fusion of the atlas (C1) and occipital bone: Spinal cord compression if the anteroposterior diameter of the foramen magnum behind the odontoid process is < 19 mm
Basilar invagination (upward bulging of the occipital condyles): A short neck and compression that can affect the cerebellum, brain stem, lower cranial nerves, and spinal cord
Atlantoaxial subluxation or dislocation (displacement of the atlas anteriorly in relation to the axis): Acute or chronic spinal cord compression
Klippel-Feil malformation (fusion of cervical vertebrae): Deformity and limited motion of the neck but usually no neurologic consequences
Platybasia (flattening of the skull base so that the angle formed by the intersection of the clival and anterior fossa planes is > 135°), seen on lateral skull imaging: Usually, no symptoms or cerebellar or spinal cord deficits
Congenital abnormalities may be specific structural abnormalities or general or systemic disorders that affect skeletal growth and development. Many patients have multiple abnormalities.
Structural skeletal abnormalities include the following:
Os odontoideum (anomalous bone that replaces all or part of the odontoid process)
Atlas assimilation (congenital fusion of the atlas and occipital bone)
Congenital Klippel-Feil malformation (eg, with Turner syndrome or Noonan syndrome), often associated with atlanto-occipital anomalies
Atlas hypoplasia
Platybasia, often with basilar invagination, Chiari malformations (descent of the cerebellar tonsils or vermis into the cervical spinal canal), and other abnormalities
Systemic disorders that affect skeletal growth and development and involve the craniocervical junction include the following:
Achondroplasia (impaired epiphyseal bone growth, resulting in shortened, malformed bones) sometimes causes the foramen magnum to narrow or fuse with the atlas and thus may compress the spinal cord or brain stem.
Down syndrome, Morquio syndrome (mucopolysaccharidosis IV), or osteogenesis imperfecta can cause atlantoaxial subluxation or dislocation.
Acquired causes include injuries and disorders.
Injuries may involve bone, ligaments, or both and are usually caused by vehicle or bicycle accidents, falls, and particularly diving; some injuries are immediately fatal.
Rheumatoid arthritis (RA, the most common disease cause) and Paget disease of the cervical spine can cause atlantoaxial dislocation or subluxation, basilar invagination, or platybasia.
Metastatic tumors that affect bone can cause atlantoaxial dislocation or subluxation.
Slowly growing craniocervical junction tumors (eg, meningioma, chordoma) can impinge on the brain stem or spinal cord.
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