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Os Odontoideum: Floating Bone of the Axis

April 1, 2023April 1, 2023virtualmedstudent

In order to understand what an os odontoideum is, we have to first appreciate the anatomy of the first two cervical vertebrae.

The first cervical vertebrae is known as the "atlas". It forms joints with the base of the skull and the second cervical vertebrae, which is also known as the axis. It has a an elongated structure on its ventral aspect called the “odontoid”. The odontoid of the axis connects to the atlas via numerous ligaments. This joint provides most of the flexibility that allows you to move your head in various directions.

An os odontoideum is a failure of the tip of the odontoid (ie: the part closest to the atlas) to fuse with its base on the axis.

Exactly why this occurs is still debated. The first theory is that it represents a congenital failure of the odontoid to fuse properly with the axis. The second, and more supported theory is that it may be caused by a previous fracture in early childhood that failed to heal properly. Regardless of the cause, the end result is a floating mass of bone that represents the superior (ie: top) most portion of the odontoid process.

This mass of bone may be fused to the base of the skull. If this is the case, the term "dystopic" os odontoideum is used. Or it may articulate and move with the atlas; if this is the case, the term "orthotopic" os odontoideum is used.

Signs and Symptoms

Many patients with os odontoideum are asymptomatic. However, because the tip of the odontoid is not technically connected to the base of the axis the patient may have an unstable neck. If the instability is severe, damage to the spinal cord can result causing myelopathy.

Myelopathy can manifest with several symptoms. Patients may have numbness and tingling in the upper and lower extremities. If damage to the nervous tissue responsible for motor movements occurs, patients may complain of weakness (and possibly even paralysis in extreme cases!).

On examination, patients may have both upper and lower motor neuron signs. Upper motor neuron signs refer to exaggerated reflexes – Babinski and Hoffmann signs, and clonus are all examples of this. These findings tend to be seen below the level of the actual spinal cord injury. Lower motor neuron findings typically occur at the level of the spinal cord damage, and consist of flaccid weakness with decreased reflexes.

Diagnosis

Diagnosis of os odontoideum is made by x-rays or CT of the cervical spine. To assess the degree of instability in the joint, some doctors will get flexion and extension x-rays as well.

The image to the right is a CT of the cervical spine that illustrates the missing portion of the odontoid process (marked by arrows in the image). A normal CT of the cervical spine is shown to the left for comparison.

Os Ondontoideum

Some patients may also get an MRI to assess for spinal cord and ligamentous injury, especially when symptoms or physical examination findings are present.

Treatment

Treatment depends on whether or not symptoms are present, and whether or not the cervical spine is unstable. Many patients without symptoms may be followed with serial X-rays or CT scans to assess for progression of instability.

If significant instability exists, or the patient has signs and symptoms consistent with spinal cord injury, then surgical stabilization is performed. There are numerous ways to achieve stabilization in this region surgically, which are outside the scope of this article. Regardless of which method is used, the end result is stabilization of the joint between the first and second cervical vertebrae.

Overview

Os odontoideum is an absence of part of the odontoid process. It may be due to a congenital malformation, or an early childhood fracture that fails to heal properly. Symptoms, when present, are due to spinal cord injury (ie: myelopathy) and consist of weakness, numbness, tingling, and other signs of spinal cord dysfunction. Imaging with x-rays or CT scan can show the bony defect. MRI is occasionally used to assess the spinal cord itself. Treatment depends on whether or not symptoms or significant instability is present. The best treatment options are surgical stabilization of the joint between C1 and C2 using one of several potential methods.

Related Articles

References and Resources

Arvin B, Fournier-Gosselin MP, Fehlings MG. Os odontoideum: etiology and surgical management. Neurosurgery. 2010 Mar;66(3 Suppl):22-31.
Klimo P Jr, Kan P, Rao G, et al. Os odontoideum: presentation, diagnosis, and treatment in a series of 78 patients. J Neurosurg Spine. Oct 2008;9(4):332-42
Fielding JW, Griffin PP. Os odontoideum: an acquired lesion. J Bone Joint Surg Am. Jan 1974;56(1):187-90.
Gwinn J, Smith J. Acquired and congenital absence of the odontoid process. AJR. 1962;88:424-31.
reenberg MS. Handbook of Neurosurgery. Sixth Edition. New York: Thieme, 2006. Chapter 25.
Harms J, Melcher RP. Posterior C1-C2 fusion with polyaxial screw and rod fixation. Spine (Phila Pa 1976). 2001 Nov 15;26(22):2467-71.
Arvin B, Fournier-Gosselin MP, Fehlings MG. Os Odontoideum: Etiology and Surgical Management.

Cervical Myelopathy: A Squished Spinal Cord

March 30, 2023March 30, 2023virtualmedstudent

Cervical myelopathy refers to a constellation of signs and symptoms that occur when the spinal cord is squeezed and unable to function properly. Myelopathy is most commonly caused by cervical stenosis, which is a general term used to describe a decrease in the cross sectional area of the spinal canal

Stenosis comes in two flavors: congenital or acquired. In congenital cervical stenosis the spinal canal is developmentally narrower than normal. Congenital stenosis is not considered hereditary, but is present from birth, and is a result of “abnormal” development.

Acquired stenosis is more common, and typically occurs in people over the age of 50. This type of stenosis is caused by a combination of factors and pathologic changes.

Degenerative cervical disease is the most common cause of acquired cervical stenosis and myelopathy. It has several distinct features, which include: loss of disc height, disc herniation, osteophyte formation, and resultant deformity (ie: kyphosis, or a forward leaning cervical spine). Let’s discuss each of these features in more detail…

The first feature in acquired degenerative stenosis is a loss of intervertebral disc elasticity and height. You can think of the intervertebral discs like shock absorbers for the spinal column. In the cervical spine, a healthy disc is slightly taller at its front most portion and slightly shorter at its back most portion. This gives the normal cervical spine a lordotic curvature.

As someone ages, the discs tend to dry out and the height differential between the front and back of the disc is lost. This can cause the cervical spine to bow forward resulting in a loss of lordosis. The new forward lean to the spine is called a kyphotic deformity. This deformity can cause the cord to stretch over the bones of the spine resulting in myelopathy.

The second factor that contributes to acquired degenerative stenosis is when the back most section of the disc weakens and pooches out towards the spinal cord. This is known as a disc herniation. Disc herniations narrow the cross sectional diameter of the spinal canal and contribute to stenosis.

Finally, as the discs age and dry out they lose their elasticity, and therefore lose their ability to absorb shock. As a result, the bones and ligaments take on more of the burden. They attempt to fight back by forming bony growths known as osteophytes. The osteophytes further narrow the spinal canal and contribute to stenosis.

Let’s bring it all together… Acquired degenerative cervical stenosis occurs when a combination of kyphotic deformity, disc herniations, and osteophytic growths narrow the spinal canal and cause compression of the spinal cord.

Other less common causes of cervical stenosis include ossification of the posterior longitudinal ligament. Additionally, traumatic injuries of the cervical spine can cause immediate cervical stenosis and cord injury. And even less commonly, tumors in the cervical spine can push on the cord and cause stenosis.

It is important to note that cervical stenosis does not always cause myelopathy. In fact, some patients can have a very "tight" cervical cord, but have no signs or symptoms of cord compression.

Signs and Symptoms

Patient’s that are myelopathic present with a constellation of signs and symptoms depending on how "squished" their cord is. Myelopathic patients present with a combination of gait dysfunction, clumsiness when using their hands, weakness, abnormally brisk reflexes, spasticity, and Lhermitte sign.

Signs and Symptoms
of Myelopathy:

– Gait dysfunction
– Clumsiness/weakness
– Brisk reflexes
– Spasticity
– Sensation problems
– Lhermitte’s sign

Reflexes are typically brisk, especially in the legs. Examples of brisk reflexes include clonus (a sustained contraction that causes the foot to "beat" several times), upgoing plantar response (ie: the big toes extend upwards, as opposed to curling down when the sole of the foot is stroked), and Hoffmann’s sign (ie: the thumb curls in when the tip of the index or middle finger is flicked).

Patients who have myelopathy tend to walk with a broad based gait as if steadying themselves. They often complain of feeling like they are walking “drunk”. Clumsiness is also a common symptom of myelopathy. Patients will typically complain of problems writing, using a utensil, holding a coffee cup, or performing other fine motor movements.

Finally, a phenomenon known as Lhermitte’s sign can occur. Lhermitte’s sign occurs when someone flexes their head forward and a shock like sensation runs down the neck and back into the extremities. It is a result of compression on the back most portion of the spinal cord, which is an area that relays sensation to the brain.

The Nurick grading system (table below) was designed to assess the severity of myelopathy. It is useful when guiding treatment decisions. The Japanese Orthopedic Association (JOA) score, European Myelopathy Score, Myelopathic Disability Index, and Ranawat Classification are other myelopathy grading scales that are commonly used in clinical practice.

The Nurick Grading System for Myelopathy
Grade 0	Nerve root impingement may be present (ie: radiculopathy), but no cord symptoms are present
Grade 1	Able to walk normally, but may have signs (but not symptoms) of cord compression
Grade 2	Some difficulty with walking, but still able to work
Grade 3	Unable to walk to the point of not being able to work
Grade 4	Can only walk when assisted
Grade 5	Confined to a wheelchair or bed bound

Diagnosis and Classification

Diagnosis of cervical stenosis is based on imaging (ie: CT, MRI, etc). Remember that stenosis does not necessarily translate into myelopathy! Patient’s should be symptomatic as well! A stenotic spine is radiographically present when the diameter of the spinal canal in the antero-posterior plane is less than 10mm.

An alternative way of diagnosing cervical stenosis is using Pavlov’s ratio. This is defined as the ratio of the canal diameter to the diameter of the adjacent vertebral body; any ratio less than 0.8 is worrisome.

Treatment

Treatment of cervical stenosis depends on several factors. First and foremost are symptoms. If a patient is not myelopathic, then frequently the best course of action is to monitor the patient until signs or symptoms of myelopathy appear.

If symptoms are present, the discussion becomes whether or not to reconstruct and decompress the front part of the spine (ie: an anterior approach), the back part of the spine (ie: a posterior approach), or both.

Anterior approaches come in one of two flavors. The first is removal of the disc with placement of a bone graft; this is formally known as a cervical discectomy and fusion. When the bones of the spine are causing the compression a more extensive surgery known as a corpectomy is performed; in this procedure the entire vertebral body is removed and a cage is placed in its place to reconstruct the spine.

Posterior approaches include laminoplasty and laminectomies with or without instrumented fusion. Laminoplasty involves splaying open the lamina so that the spinal cord can drift back. Laminectomies involve un-roofing the bone behind the spinal cord. Laminectomies are frequently done with an instrumented fusion in which rods and screws are placed to help the patient maintain a normal cervical alignment (ie: lordosis).

Surgical Treatment of Myelopathy
Deformity	Compression	Surgical Approach
Kyphotic	Anterior and only 3 spinal levels or less involved	Anterior (anterior cervical discectomies and/or corpectomies)
Kyphotic	Anterior and posterior	Combined anterior (anterior cervical discectomies and/or corpectomies) and posterior (laminectomies with or without instrumented fusion)
Lordotic	Anterior and only 3 spinal levels or less involved	Anterior (anterior cervical discectomies and/or corpectomies)
Lordotic	Posterior	Posterior (laminoplasty or laminectomies with or without posterior instrumented fusion)
Lordotic	Anterior and posterior	Combined anterior (anterior cervical discectomies and/or corpectomies) and posterior (laminectomies with or without instrumented fusion)

The choice of surgical options is highly dependent on the degree of deformity, instability, and where specifically (ie: what levels) in the spine the cord compression is located.

Ultimately, the surgical treatment of cervical stenosis and myelopathy must be tailored to each individual patient based on many factors.

Overview

Cervical stenosis is abnormal narrowing of the spinal canal. It has numerous causes, of which the most common one is acquired degenerative stenosis. Stenosis can cause compression of the spinal cord, which can cause signs and symptoms of myelopathy. Treatment for symptomatic patients involves decompressing the spinal cord via either an anterior, posterior, or combined approach.

Related Articles

References and Resources

Revanappa KK, Rajshekhar V. Comparison of Nurick grading system and modified Japanese Orthopaedic Association scoring system in evaluation of patients with cervical spondylotic myelopathy. Eur Spine J. 2011 Sep;20(9):1545-51.
Scardino FB, Rocha LP, Barcelos AC, et al. Is there a benefit to operating on patients (bedridden or in wheelchairs) with advanced stage cervical spondylotic myelopathy? Eur Spine J. 2010 May;19(5):699-705.
Emery SE, Bohlman HH, Bolesta MJ, et al. Anterior cervical decompression and arthrodesis for the treatment of cervical spondylotic myelopathy. Two to seventeen-year follow-up. J Bone Joint Surg Am. 1998 Jul;80(7):941-51.
Wada E, Suzuki S, Kanazawa A, et al. Subtotal corpectomy versus laminoplasty for multilevel cervical spondylotic myelopathy: a long-term follow-up study over 10 years. Spine (Phila Pa 1976). 2001 Jul 1;26(13):1443-7; discussion 1448.
Manzano GR, Casella G, Wang MY, et al. A prospective, randomized trial comparing expansile cervical laminoplasty and cervical laminectomy and fusion for multilevel cervical myelopathy. Neurosurgery. 2012 Feb;70(2):264-77.
Ranawat CS, O’Leary P, Pellicci P, et al. Cervical spine fusion in rheumatoid arthritis. J Bone Joint Surg Am. 1979 Oct;61(7):1003-10.
Nurick S. The pathogenesis of the spinal cord disorder associated with cervical spondylosis. Brain. 1972;95(1):87-100.
Vitzthum HE, Dalitz K. Analysis of five specific scores for cervical spondylogenic myelopathy. Eur Spine J 2007;16:2096–103.
Herdmann J, Linzbach M, Krzan M, et al. The European myelopathy score. In: Baucher BL, Brock M, Klinger M, eds. Advances in Neurosurgery. Vol. 22. Berlin, Germany: Springer; 1994:266–8.
Clark E, Robinson PK. Cervical myelopathy: a complication of cervical spondylosis. Brain. 1956 Sep;79(3):483-510.

Chiari Malformation: Type1, Tonsils and Syrinx

March 26, 2023March 26, 2023virtualmedstudent

The Chiari malformations are a group of disorders characterized, at least in part, by herniation of hindbrain structures through the foramen magnum at the base of the skull. They are categorized as type 1, type 2, and type 3 depending on clinical and radiographic findings.

This article will focus on type 1 Chiari malformations. The definition of this malformation has been debated, but most agree that the combination of herniated cerebellar tonsils (usually defined as greater than 5mm below the foramen magnum), with or without a syrinx, in the setting of referable symptoms is sufficient to make the diagnosis.

So why do the cerebellar tonsils herniate? Nobody knows for sure! We do know that type 1 malformations can be congenital or acquired. One theory is that tonsillar herniation is a result of an abnormally small posterior fossa (ie: the bones that compose the base of the skull). A small posterior fossa may be caused by under-development of the occipital somites in-utero (the fetal precursors that form bone and connective tissues), premature fusion of the cranial bones (ie: craniosynostosis), or medical conditions that promote abnormal bony growth.

Other experts advocate that abnormal cerebrospinal fluid pressures between the brain and spine may cause the tonsils to herniate downwards.

Regardless of how you slice it, we can say with certainty that there are multiple potential etiologies for type 1 Chiari malformations.

Type 1 Chiari malformations are commonly associated with a finding known as a “syrinx”. A syrinx is an abnormal fluid filled cavity that is seen in the cervical and/or thoracic spinal cord. It may represent an enlargement and extension of the central canal of the cord, in which case it is termed hydromyelia; it may also represent a complex glial lined cavity, which is referred to as syringomyelia. Regardless, syrinxes are found in 30% to 70% of type 1 Chiari malformations.

For unclear reasons, type 1 Chiari malformations with a syrinx are associated with scoliotic curves of the spine (especially left sided curves). It is believed that the syrinx puts pressure on the motor pathways of the spinal cord. This results in weakness of the paraspinal muscles causing the vertebral column to curve.

Signs and Symptoms

The most common presenting symptom of a type 1 Chiari malformation is pain. The pain is usually located at the back of the head and upper neck. Additionally, a cape-like sensation loss, as well as problems with vision and/or hearing may also be present.

Myelopathic signs or symptoms may be present if there is a syrinx. Myelopathic patients present with a combination of gait dysfunction, hand clumsiness, weakness, abnormally brisk reflexes, spasticity, and Lhermitte’s sign.

Diagnosis

Diagnosis of a type 1 Chiari malformation is made when an MRI shows abnormal herniation of the cerebellar tonsils, with or without an associated syrinx, in the context of appropriate signs and/or symptoms.

Treatment

The treatment of Chiari malformation is with surgical decompression. Most commonly this involves "shaving" off part of the occipital bone and removing the C1 lamina. This effectively decompresses the spinal cord and cerebellar tonsils.

If an associated syrinx is present, many neurosurgeons will open the dura (ie: the lining of the spinal cord) and perform a "duraplasty"; during the duraplasty a patch is sewn into place to give the spinal cord and cerebellar tonsils more room. Duraplasty generally improves the size and severity of the syrinx over time, but adds risk and complications to the procedure.

Some neurosurgeons will surgically shrink the cerebellar tonsils after opening the dura. This is done with bipolar electrocautery and serves to further decompress the area.

Overview

Type 1 Chiari malformations are hindbrain abnormalities characterized by herniation of the cerebellar tonsils below the foramen magnum. They are associated with cervicothoracic syrinxes as well as neuromuscular scoliosis. Symptoms can range from pain to neurological deficits. Treatment is with surgical decompression, although the exact type of decompression has been the subject of intense research.

References and Resources

Colombo LF, Motta F. Consensus conference on Chiari: a malformation or an anomaly? Scoliosis and others orthopaedic deformities related to Chiari 1 malformation. Neurol Sci. 2011 Dec;32 Suppl 3:S341-3.
Eule JM, Erickson MA, O’Brien MF, et al. Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population. Spine (Phila Pa 1976). 2002 Jul 1;27(13):1451-5.
Cardoso M, Keating RF. Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976). 2009 Aug 1;34(17):1775-82.
Milhorat TH, Nishikawa M, Kula RW, et al. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as guide to clinical management. Acta Neurochir (Wien). 2010 Jul;152(7):1117-27.
Batzdorf U, McArthur DL, Bentson JR. Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients. J Neurosurg. 2013 Feb;118(2):232-42.
Klekamp J. Surgical treatment of Chiari I malformation–analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery. 2012 Aug;71(2):365-80; discussion 380.
Yilmaz A, Kanat A, Musluman AM, et al. When is duraplasty required in the surgical treatment of Chiari malformation type I based on tonsillar descending grading scale? World Neurosurg. 2011 Feb;75(2):307-13.
Durham SR, Fjeld-Olenec K. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr. 2008 Jul;2(1):42-9.