Module 5 Cervical Spine Syndromes Flashcards
List 5 difference between the paediatric versus Adult cervical spine KNOW
- Cervical spine injuries in children usually occur in the upper cervical spine from the occiput to C3.
- This fact may be explained by the unique biomechanics and anatomy of the pediatric cervical spine.
- The fulcrum of motion in the cervical spine in children is at the C2-C3 level; in the adult cervical spine, the fulcrum is at the C5-C6 level
- The immature spine is hypermobile because of ligamentous laxity, shallow and angled facet joints, underdeveloped spinous processes, and physiologic anterior wedging of vertebral bodies, all of which contribute to high torque and shear forces acting on the C1-C2 region.
- Incomplete ossification of the odontoid process, a relatively large head, and weak neck muscles are other factors that predispose to instability of the pediatric cervical spine
What are the normal radiographic parameters and variants of the pediatric cervical spine
The atlantodens interval (ADI) is defined as the distance between the anterior aspect of the dens and the posterior aspect of the anterior ring of the atlas.
This distance should be 4 mm or less. In the adult population, the normal ADI is 3 mm.
Up to 6 mm of displacement of the lateral masses relative to the dens is common in patients up to 4 years old and may be seen in patients up to 7 years old
- The absence of lordosis, although potentially pathologic in an adult, can be seen
in children up to 16 years of age when the neck is in a neutral position - Ossification centers such as secondary centers of ossification of spinous processes and unfused ring apophyses of vertebral bodies can be confused with fractures.
- Normal physeal plates should be recognized as smooth, regular structures with subchondral sclerotic lines.
- Acute fractures are irregular, are not sclerotic, and can occur at any location.
- The posterior ring of C1 can remain cartilaginous
- The apical odontoid epiphysis is visualized at radiography in 26% of children between 6 and 8 years of age and should not be confused with fracture
- In early infancy, cervical vertebral bodies have an oval appearance.
These vertebrae take on a more rectangular appearance with advancing age.
Anterior wedging of up to 3 mm of the vertebral bodies should not be confused with compression fracture
* A prevertebral space of less than 6 mm at the level of C3 is considered normal in children
Pseudosubluxation
In children, the C2-3 space and, to a lesser extent, the C3-4 space have a normal physiologic displacement (pseudosubluxation)
46% of children less than 8 years old had pseudosubluxation of C2 on C3 on lateral flexion and extension radiographs of the cervical spine
describe what’s happening here.
In children, the C2-3 space and, to a lesser extent, the C3-4 space have a normal physiologic displacement (pseudosubluxation)
In a study involving 160 pediatric patients with no history of cervical spine trauma, 46% of children less than 8 years old had pseudosubluxation of C2 on C3 on lateral flexion and extension radiographs of the cervical spine
* An abnormal posterior cervical line measurement often indicates the presence of a bilateral pars interarticularis (“hangman fracture”) of C2.
Lateral radiograph of the cervical spine in
a pediatric patient shows pseudosubluxation at the C2-C3 level (arrow).
Occiput - C1 injury
How would a Jefferson fracture show up on an Xray
A Jefferson fracture usually manifests as asymmetry between the odontoid process and the lateral masses on an open-mouth odontoid image.
These fractures are stable if the transverse ligament is intact
A distance of 6 mm or more between the lateral mass of C1 and the odontoid process is suggestive of ligamentous disruption
Odontoid Fractures
- In children less than 7 years old most commonly occur through the cartilaginous synchondrosis
- Fractures to this area usually heal without the complications typically seen in adults, such as nonunion and pseudoarthrosis
- The odontoid displacement is usually anterior, with the dens tilted posteriorly
- Os odontoideum may occur in a normal anatomic location or may be displaced
It is associated with instability of the atlantoaxial joint and cervical spinal cord injury
Traumatic Spondylolisthesis of C2 (Hangman Fracture)
- Hangman fracture is a hyperextension injury of the cervical spine.
- This fracture can occur in younger children but is less common than fractures of C1 and the odontoid process
- Anterior subluxation of C2 on C3 with associated horizontal tearing of the C2-3 disk space may also be seen
- This lesion can be diagnosed with lateral radiography, although CT and MR imaging may be useful for more detailed evaluation
Atlantoaxial Instability
What are the consequences?
How does the various types occur?
Instability of the upper cervical spine in children is uncommon but potentially devastating.
- In the normal upper cervical spine, flexion and extension take place at the occiput–first cervical vertebra (C1) articulation, and rotation occurs at the C1–2 joint.
- Neither joint is intrinsically stable, and both depend on the integrity of the ligaments and joint capsules that surround the joints to constrain motion.
- Developmental, traumatic, inflammatory, or metabolic lesions that affect the stability of the occiput-C1 or C1–2 joint have serious implications.
- Progressive myelopathy may develop in patients with chronic instability; acute impingement and death are possible in patients with severe instability
CONGENITAL
Vertebral (Bony Anomalies)
Cranio-occipital defects (occipital vertebrae, basilar impression, occipital dysplasias, condylar hypoplasia, occipitalized atlas)
Atlantoaxial defects (aplasia of atlas arch, aplasia of odontoid process)
Subaxial anomalies (failure of segmentation and/or fusion, spina bifida, spondylolisthesis)
Ligamentous or Combined Anomalies
Found at birth as an element of somatogenic aberration
Syndromic Disorders
Down syndrome Klippel-Feil syndrome 22q11.2 deletion syndrome Larsen syndrome
Marfan syndrome Ehlers-Danlos syndrome
ACQUIRED
Trauma
Infection (pyogenic, granulomatous)
Tumor (including neurofibromatosis)
Inflammatory conditions (e.g., juvenile rheumatoid arthritis)
Osteochondrodysplasias (e.g., achondroplasia, diastrophic dysplasia, metatropic dysplasia, spondyloepiphyseal dysplasia)
Storage disorders (e.g., mucopolysaccharidoses, Morquoi syndrome) Metabolic disorders (rickets)
Miscellaneous (including osteogenesis imperfecta, sequela of surgery)
Atlantoaxial instability can be the cause of torticollis which can occur spontaneously, or as a result of trauma ma, or in association with underlying congenital abnormalities.
What are other disorders that may cause childhood torticollis include?
- infections,
- spinal cord tumors
- an underlying abnormality within the sternocleidomastoid muscle
Atlanto -axial subluxation
there is an increased incidence in
- Down’s Syndrome,
- Skeletal dysplasia (Morquio),
- Juvenile rheumatoid arthritis,
- Os odontoidism,and
- Grisel’s syndrome.
(Grisel’s syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck region. It can be observed after surgery of head and neck region_ Google)
Down’s syndrome = 10-20% incidence of asymtomatic atlanto-axial instability due to laxity of transverse ligaments, which holds the dens against the posterior border of the anterior arch. 1-2% will develop subluxation.
Down syndrome
What are the reported abnormalities in the spine?
How should we examine a Child with Down syndrome? Radiographically.
The association of trisomy 21 (Down syndrome) with instability of the upper cervical spine is well known; estimates of the incidence of instability range from 10–25%
.
➢Reported abnormalities include occipito-atlantal instability, atlanto-axial instability, occipitalization of the atlas, and os odontoideum.
➢The number of patients with symptoms related to C1–2 instability or other anomalies of the cervical spine is much lower.
➢A minority of Down patients with cervical instability have neurologic symptoms.
RADIOGRAPHIC:
➢ The clinical diagnosis of neurologic dysfunction may be challenging
➢ Subtle findings such as decreased exercise tolerance and gait abnormalities (increased tripping or falling) may be the earlie st signs of myelopathy.
➢ The atlanto-dens interval (ADI) is measured as the space between the dens and the anterior ring of C1 (ADI) on lateral radiographs in neutral, flexion, and extension.
➢ A normal ADI in children with Down syndrome is <4.5 mm.
➢ Hypermobility is diagnosed with an ADI between 4.5 and 10 mm
➢ An ADI >10 mm represents instability and carries a significant risk of neurologic injury.
➢ An MRI with flexion and extension, usually performed under supervision, helps to further evaluate instability and neurologic compression.
➢ Although hypermobility at the occipitoatlantal joint is present in >50% of children with Down syndrome, most patients do not develop instability or neurologic symptoms.
➢ The relationships at this articulation are difficult to measure reliably on plain radiographs, and a dynamic MRI can help to clarify the significance of any questionable radiographic findings.
➢ Degenerative changes and/or instability can result in pain, radiculopathy, and myelopathy.
- Recommendations for surveillance of potential cervical instability in children with Down syndrome vary, and no formal guidelines have been established.
- An annual neurologic examination should be performed.
- It is reasonable to obtain plain radiographs of the cervical spine, including flexion-extension views, in all patients with
Down syndrome. - Flexion and extension radiographs are obtained every other year in those with a normal clinical exam.
- Those with abnormal findings or symptoms, or when neurologic compression is suspected, are sent for an MRI in flexion and extension.
- Patients with normal radiographs who are also neurologically normal may be allowed to participate in a full level of activities.
- Those in whom hypermobility is diagnosed should be restricted from contact sports and other high-risk activities that might increase the risk of trauma to the cervical spine.
- Only a small subset of patients with myelopathy or instability (ADI >10 mm) with impending neurologic injury are candidates for posterior atlantoaxial (or occipitocervical) fusion, because the risks of a major complication (death, neurologic dysfunction, nonunion of fusion) is significant in patients with Down syndrome.
TORTICOLLIS
➢Torticollis is a sign, not a disease, and may be the result of a wide range of underlying pathophysiologic processes.
LIST OF some differential diagnosis of torticollis in infants and children
Congenital:
-Osseous anomalies (Hemivertebra, unilateral atlanto-occipital fusion, Klippel-Feil syndrome)
-Soft tissue abnormalities (unilateral absence of sternocleidomastoid, pterygium colli)
(Malformation or congenital cervical scoliosis result in deformity of the neck that will not respond to stretching exercise programs or adjusting)
Acquired:
-Positional deformation or congenital muscular torticollis
-Trauma (muscular injury, fractures)
-Cervical instability (atlanto-occipital subluxation,
atlantoaxial subluxation, subaxial subluxation)
Atlanto- axial rotatory displacement
Inflammation:
-Cervicallymphadenitis
-Retropharyngeal abscess
-Diskitis or vertebral osteomyelitis Rheumatoid arthritis
Neurologic:
-Visual disturbances (nystagmus, superior oblique paresis)
-Dystonic drug reactions (phenothiazines, haloperidol, metoclopramide)
-Neoplasia (cervical spinal cord or posterior fossa tumor)
-Chiari I malformation and/or syringomyelia
-Wilson disease
-Dystonia
-Spasmus nutans (nystagmus, head bobbing, head tilting)
Whats happening here?
A rare cause of congenital torticollis
* Not responsive to chiropractic treatment
Atlanto- occipital fusion
Which area is fused in most cases?
What else is Atlanta-occipital assimilation associated with?
- Atlanto-occipital assimilation occurs in 0.5% (range 0.25- 1%) of the population
- Atlanto-occipital assimilation is typically asymptomatic but symptoms from nerve or vascular compression can occur
- Atlanto-occipital assimilation is associated with:
- Fusion ofC2 and C3 (occurs in 50% of cases)
- Basilar invagination
- Cleft palate
- Cervical ribs
- Urinary tract anomalies
Klippel- Feil Syndrome
May present with?
What is needed for a diagnosis?
May present with?
1. Short neck
2. Low posterior hairline
3. Limited range of motion
Congenital elevation os the scapula, is the most common congenital shoulder abnormality.
2 or more non-segmented cervical vertebrae are usually sufficient for diagnosis.
What is this and what are the associated risks or complications with it>
The importance of the arcuate foramen lies in the external pressure it may cause on the vertebral artery as it passes from the foramen transversarium of the first cervical vertebra to the foramen magnum of the skull.
Tethering of the vertebral artery in the congenital arcuate foramen of the atlas vertebra: a possible cause of vertebral artery dissection in
children
- Vertebral artery dissection due to arcuate foramen commonly present with:
- Occipital headache
- Nausea
- Vomiting
- Dizziness or vertigo
Neurological causes of Torticollis:
Chiari Malformations:
What is the clinical presentation of Chiari malformations
An infant with CM (Type II, I) may present with
❖ feeding problems due to difficulties swallowing,
❖ poor sleeping patterns,
❖ periods of apnea, weak or hoarse sounding cry and
❖ possible delayed attainment of milestones due weakness in extremities and affected fine motor skills.
* Hydrocephalus may develop and cause macrocephaly, vomiting, irritability, seizures, and further delays in attaining developmental milestones.
- Severe cases of CM can be life threatening
Imaging findings might include:
1. Abnormalities of the skull
2. Basilar invagination
3. Occipitalisation of atlas
4. Spina Bifida of C1 posterior arch
5. Foramen magnum variant anatomy
6. Spina Bifida
While older children (usually Type I) may present with history of the above,
* occipital headache is the most common symptom.
* Vision problems, tinnitus, vertigo and dizziness may
also be reported.
* Problems with balance, paraesthesia in limbs, and syncope are sometimes present.
* If syringomyelia develops loss of muscle, muscle weakness, numbness, scoliosis, loss of bowel and bladder control, chronic pain, cramps, ataxia, and spasticity may be occur.
* A clinician should have increased suspicion of CM in presence of Spina Bifida.
* Prenatal maternal depression and consumption of SSRI have been shown to be risk factors
Neurological causes of Torticollis:
Brain tumours in children: a potential cause of torticollis
Clinical presentation of brain tumours
- Signs of increased intracranial pressure (Hydrocephalus)
- Increased head circumference
- Nausea
- Vomiting
- Lethargy
Morning headache
Headache wakes child from sleep Swallowing issues
Unsteadiness
Facial weakness
HA’s due to increased I.C.P tend to:
– occur in mornings or wake the child late at night/early hours of morning
– be relieved with standing
– be described as dull, generalised and steady
– may be intermittent and worse with cough, sneeze, defecation
– often associated with vomiting & the vomiting relieves the headache
* Headache can be a symptom of tumour irrespective of location
* Headaches are a very common symptoms of brain tumour
– 70% occurrence in infratentorial tumours
– 58% occurrence in supratentorial tumours
– Note HA is rarely in isolation, usually other neuro symptoms &/or vomiting as well
* Note that occipital HA or neck pain can also be a symptom of a posterior fossa tumour due to irritation of posterior roots of the cervical cord.
* When this occurs the pt. may also show signs of tonsilar herniation (e.g. neck stiffness & opisthotonus)
* Therefore, a headache with neurological deficits and signs of increased I.C.P is of concern
Vomiting
* One of the most common symptoms of brain tumour in children
* Commonly misdiagnosed as migraine, viral or G.I.T illness
* Bailey et al reports vomiting history in 84% of kids with brain tumours (quite an old study 1939)
* Baren reports 60-80% of children with brain tumour have a history of vomiting (2008)
* Can present in many ways: - alone ,mild
- intermittent, early hrs of morning -abrupt, daily with HA
* Vomiting with no sign of fever, no diarrhoea, lethargy, no improvement with rehydration, head tilt and abnormal neuro exam is of concern
Therefore, thorough history and examination (esp. neuro) with children presenting with headache &/or vomiting is imperative.
Infection causing torticollis
Grisel’s Syndrome
History + Exam findings:
treatment involves?
Causes?
History of ENT surgery or ENT infection
*Torticollis some days after onset of infection/operation
*Rotation and slight flexion of the head with chin rotated contralaterally
*Painful active and passive rotation of the head
*In the first days of torticollis, elevated CRP and leucocytes, and later, normalization of these parameters and usually no fever
*Radiograph of the cervical spine: space between atlas and dens
axis >5 mm
*CT scan: atlantoaxial subluxation and rotation
treatment:
1. Lymohatic drainage
2. reducing inflammation
3. adjustments during acute phase not usually necessary,
▪ Neurosurgical consultation is mandatory to prevent significant neurological deficit.
▪ 15% of patients may develop permanent neurological sequel.
▪ Early broad spectrum antibiotics
▪ Muscle relaxants
▪ Bedrest
▪ Cervical traction and soft or hard collar
▪ Cases treated inappropriately may result in a fixed and painful neck deformity that may even require surgical fusion.
Case Example:
We report a 5 year old boy with recurring episodes of head tilt and painful and restricted neck movements that developed after repeated bouts of sinusitis.
Radiographs showed a subluxation of the C2–3 joint.
This is the first report of a symptomatic case of Grisel’s syndrome occurring at the C2–3 segment.
CAUSES:
Infection:
-URTI, mumps etc
Postoperative causes: Grommets, tonsils out surgery, etc
What is this diagnosis?
Grisel’s syndrome
Whats the diagnosis- explain why?
Retropharyngeal abscess
- There is pre-vertebral soft tissue swelling noted. This radiograph is consistent with a retropharyngeal abscess, not croup.
- The retropharyngeal space is a pocket of connective tissue that extends from the base of the skull approximately to the tracheal carina.
- It harbors two chains of lymphoid tissue that drain the nasopharynx, adenoids, and posterior paranasal sinuses.
- Bacterial infections of the areas drained may result in suppuration of the nodes and abscess formation.
- These lymphatic chains begin to atrophy about the third or fourth year of life. Thus, 50% of the cases of retropharyngeal abscess occur between 6 and 12 months of age, and 96% of cases occur in children under 6 years of age (prior to lymphatic atrophy).
What is treatment of muscular torticollis?
Stretching
A program of gentle passive stretching exercises started within the first month of life often results in resolution of the SCM contracture within 4-8 weeks.
The parents should be instructed to rotate the chin gently toward the side of head tilt while simultaneously bringing the head to the upright position.
As range of motion improves, the chin can be rotated past neutral, and the head tilted toward the opposite side.
Chiropractic adjustment
Always associated with an ASRI/LI C0 on the side of SCM contracture
What is the 9 step process for examination of the Cervical spine
Step 1:
Identify kinesiopathology at the upper cervical complex in lateral flexion
Interpretation
Loss of lateral flexion unilaterally implies a subluxation on that side
except for the occiput in infants and toddlers where the involved condyle is typically on the side opposite decreased lateral flexion
Loss of lateral flexion bilaterally implies an atypical upper cervical subluxation or a compensation response
Step 2:
Examine long axis condylar movement in both flexion & extension
Interpretation
When flexion is lost: AS occiput When extension is lost: PS occiput
The most common pattern found is restricted UCS lateral flexion opposite side to restricted occipital flexion
Step 3:
Examine the axis/C3 motion segment in flexion (X-axis)
Interpretation
Flexion will be decreased when the axis is subluxated and yields the posterior (P) component of the listing.
The S/-I component is determined by Step 1.
Step 4:
If flexion is impaired at axis/C3, examine the motion segment in rotation (Y-axis)
Interpretation
Impaired movement with right head rotation implies a spinous right fixation and with left head rotation a spinous left fixation
Easiest to assess spinous rotation whilst laterally flexing the cervical spine
Step 5:
Determine the degree of gross cervical rotation
Interpretation
The atlas will be deemed to be anterior on the side to which rotation is lost or posterior on the opposite side
Anterior atlas subluxations are unusual and are often misdiagnosed AS CO on same side.
Step 6:
Determine the side of the short leg in the supine position
Interpretation
The short side corresponds to the side of occiput subluxation, axis wedging and atlas subluxation provided laterality is the dominant kinesiopathological factor.
The short side will be contralateral to the side of atlas subluxation when rotation is the dominant kinesiopathological factor
Step 7:
Examine the head posture in the erect position
Interpretation
The head will tilt away from the side of subluxation in all listings
except the posterior atlas subluxation in which rotation is the dominant kinesiopathological factor and the AS occiput in infants
Step 8:
Examine the point specific connective tissue and muscle locations related to the vectors of kinesiopathology identified in steps 1-7.
Pain is indicated by an involuntary extensor response, spontaneous pupillary constriction, facial wincing, crying or a change in the pitch of the cry.
Step 9:
Elicit the scapulohumeral reflex and examine the muscle stretch reflexes at C5, C6, C7, L4 and S1
Test the sclerotomal response
Measure skin temperature over greater wing of sphenoid and above supraorbital notch bilaterally
