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3 Atlanto-Axial (C1–C2) Subluxation andDislocation

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Fig. 3.2 Gradual correction (CT scan; transverse section; (a–e))

Fig. 3.3 End of treatment ((a) CT scan; transverse section; (b) open mouth radiograph)

increased ADI and decreased space available for the spinal cord (sub-axial stenosis if the space available for the cord is less than 13mm) (Figs.3.2, 3.3, 3.4, and 3.5). It is important to remind here CT scan can have false positives (images suggestive of subluxation but normal anatomical variant); careful imaging assessment is warranted.

In case of AAD secondary to tumor, CT scan and MRI are very important to assess size, range, density and involvement of the tumor, as well as the damage of the body and the pedicle of the vertebra. MR angiography can also be performed to identify the course of the vertebral artery.

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Fig. 3.4 Odontoid (C2) disruption (lateral radiograph)

F. Canavese

Fig. 3.5 Odontoid (C2) tumor ((a, b) CT scan; (c) MRI)

3.5 Differential Diagnosis

AAD should be considered in a child with the inability or unwillingness to turn the head (evidence of torticollis) when history and physical examination are inconsistent with congenital torticollis (Chap. 28).

The most frequent and relatively benign form of AAD in children without associated fracture is rotatory dislocation; most children with rotatory dislocation have underlying pathologic conditions.

A purely traumatic AAD in the absence of another predisposing risk factor is extremely rare; traumatic osseous injuries may also result in atlanto-axial instability (e.g., type II odontoid fractures).

3 Atlanto-Axial (C1–C2) Subluxation andDislocation

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Table 3.1 Causes and relative incidence of atlanto-axial dislocation

Causes of atlanto-axial dislocation Incidence Traumatic Lesion of the transverse ligament (C2) Rare Chromosomal disorder Trisomy 21 15–20%

Sandifer syndrome

Skeletal dysplasias Goldenhar syndrome Not

known Spondyloepiphyseal dysplasia 30% Mucopolysaccharides type IV (Morquio

syndrome)

Congenital osseous abnormalities

Inammatory disease Chronic rheumatoid arthritis Adults

Tumor Benign and malignant Not

Infection Retro-pharyngeal abscess (tuberculosis)

Occipitalization of the atlas Not Congenital abnormality of the odontoid

Gastroesophageal reux (GERD) and chronic

oesophagitis

Grisel’s syndrome

40–90%

known

20–86%

Not

known

Not

known

Chromosomal disorders, skeletal dysplasia, congenital osseous abnormalities, inammatory disorders, and tumors such as aneurysmal bone cyst (Chap. 38), osteochondroma (Chap. 34), chordoma (Chap. 62), osteoblastoma (Chap. 33), brous dysplasia, eosinophilic granuloma (Chap. 35), and Ewing’s sarcoma; Chap.

39) can be frequently associated with AAD (Table3.1; Figs.3.4 and 3.5).

Grisel’s syndrome, also known as nasopharyngeal torticollis of inammatory origin, is a non-traumatic subluxation of the C1–C2 joint, caused by the contracture of the muscles in contact with an infection. Treatment includes antibiotics and immobilization of the neck; early treatment is essential to prevent long-term sequelae (xed C1–C2 dislocation). Surgical fusion may be required for residual instability of the joint or to correct a rigid deformity.

3.6 Treatment Options

The treatment of AAD varies according to the severity of symptoms and the presence/absence of neurological involvement. Children presenting acutely with AAD can be treated conservatively, in the absence of neurologic injury; orthopedic treatment consists of cervical halter traction in the supine position until AAD is reduced; traction is then followed by orthotic immobilization (rigid brace including the head, neck, and chest, or halo-vest) (Video 3.1) and subsequent active range-of-motion exercises until free-motion returns (Figs.3.1, 3.2, and 3.3).

Surgical treatment for patients with symptomatic AAD is indicated in children when one or more of the following clinical and/or radiological signs are present: (1) neurologic involvement; (2) ADI greater than 4 –5 mm with the reduction of the

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F. Canavese

space available for the cord; (3) deformity present for more than 3months; (4) recurrence of deformity following 6weeks of immobilization.

The goals of surgery are to reduce and stabilize the C1–C2 complex and to decompress the spinal cord. Posterior C1–C2 fusion can be used alone for reducible AAD or in conjunction with anterior trans-oral decompression to treat certain types of irreducible AAD (Videos 3.2 and 3.4).

3.7 Expected Outcomes

In patients treated conservatively, the outcome is variable; resolution of symptoms should be expected in patients treated surgically, although neurological signs may not completely resolve after decompression if treatment is performed too late. Surgical stabilization aims to protect against potential respiratory failure, progressive neurologic symptoms, and death (rare).

3.8 Potential Complications

Serious sequelae include myelopathy, respiratory failure, vertebral artery dissection, neurologic compromise, and rarely quadriplegia or death if left untreated.

3.9 What Should Patient andFamily Know?

AAD in children is rarely caused by trauma; congenital abnormalities C1–C2, space-occupying lesions, genetic disorders, and syndromes are signicant risk factors for AAD in children.

Children with trisomy 21 are predisposed to it; they should be screened for AAD between 3 and 5years of age (cervical radiographs) although it is unclear if asymptomatic trisomy 21 patients with an ADI greater than 4 to 5mm are at higher risk for neurologic sequelae; similarly, patients with Morquio syndrome and Goldenhar syndrome must be advised against sports participation (in particular contact sports) although they may not require surgical treatment.

Further Readings

Jain VK.Atlantoaxial dislocation. Neurol India. 2012;60(1):9–17. Neal KM, Mohamed AS.Atlantoaxial rotatory subluxation in children. J Am Acad Orthop Surg.

2015;23(6):382–9. Song D, Maher CO.Spinal disorders associated with skeletal dysplasias and syndromes. Neurosurg

Clin N Am. 2007;18(3):499–514.

Spinal Cord Injury Without Radiographic

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Abnormality

FedericoCanavese

4.1 Definition

Spinal cord injury without radiographic abnormality (SCIWORA) is a syndrome characterized by clinical symptoms of traumatic myelopathy with no radiographic or computed tomography (CT) scan features of spinal fracture or instability; it is responsible for up to 20% of spinal cord injuries in children (mostly aged less than 10years of age). SCIWORA is typically seen in the cervical spine although it can also occur at the level of the thoracic and lumbar spine.

4.2 Natural History

The injury of the spinal cord seen in SCIWORA syndrome is caused by a contusion or ischemia of the cord due to temporary occlusion of vertebral arteries, followed by a spontaneous return of vertebrae to their original position. Specic biomechanics of the vertebral column in children allows the musculoskeletal system to move beyond the normal physiological range of motion without the risk of fracture.

Children under the age of 8years have the most unfavorable prognosis, which is associated with a large head-to-body ratio, increased mobility of the cervical spine, inherent ligamentous laxity, immaturity of neck musculature, incomplete ossication of the vertebrae, and shallow angulation of facet joints during childhood.

Supplementary Information The online version contains supplementary material available at (https://doi.org/10.1007/978- 3- 030- 80356- 8_4).

F. Canavese (*) Department of Pediatric Orthopedic Surgery, Lille University Center, Jeanne de Flandre Hospital, Lille, France

Faculty of Medicine Henri Warembourg, Nord-de-France University, Lille, France

https://doi.org/10.1007/978-3-030-80356-8_4

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In case of permanent neurological compromise, 100% of patients develop scoliosis (paralytic scoliosis) with or without pelvic obliquity.

F. Canavese

4.3 Physical Examination

History of trauma is important as SCIWORA lesions are often caused by hyperextension forces (e.g., during a rear-end car accident) or from a direct frontal impact to the face (e.g., diving, rugby, wrestling, and baseball).

Neurological examination is of paramount importance as patients with SCIWORA can develop a broad spectrum of neurological decits, from mild to extremely severe symptom; in particular, tetra/para/hemiparesis/plegia, paresthesia, changes in tendon reexes, loss of bladder and bowel function, signs of anterior/ central/posterior cord or Brown-Séquard syndrome in addition to local pain, sensitivity, abrasions, and bruising around the vertebral column. Moreover, neurological decits can be delayed from a few minutes to 48h after injury in about 50% of cases. This latency is associated with repeated micro-insults to the spinal cord from striking against the unstable vertebrae. Typically, neurological signs/decits are more severe in the upper extremities than in the lower extremities (Video 4.4).

The level of spinal cord injury corresponds to the location of the SCIWORA lesions. It is advisable to use the ASIA scale during a clinical examination (Appendix G).

4.4 Imaging

Conventional radiographs are of limited help due to the presence of muscle spasms. In particular, lateral radiographs of the cervical spine alone have low sensitivity and specicity; diagnostic accuracy can be improved if anterior-posterior, lateral, oblique, and open mouth or odontoid radiographs are performed. The stability of the cervical spine can also be assessed by exion and extension dynamic radiographs. Moreover, the interpretation of cervical radiographs in children can be difcult due to incomplete ossication and the presence of normal anatomical variants (e.g., pseudo-subluxation of C2–C3).

CT is most accurate in detecting bony pathology. SCIWORA should be suspected, and magnetic resonance imaging (MRI) performed, in patients with neurologic symptoms and a positive history of blunt trauma with plain radiographs and CT scans without evidence of fracture.

MRI (sagittal plane) is helpful in identifying the location and extent of the injury as it can detect the signs of acute spinal cord injury (Fig.4.1), including edema, hematoma, loss of continuity (transection) of the spinal cord, and prolapsed nucleus pulposus. MRI can highlight important prognostic factors of SCIWORA lesions. In particular, hematomas less than 30% of the spinal cord diameter or edema have a favorable prognosis and resolve over time in most cases; on the other hand, transection of the cord or hematomas greater than 50% of the spinal cord diameter have a poor prognosis (Fig.4.2) and manifest clinically as paresis or paralysis.

4 Spinal Cord Injury Without Radiographic Abnormality

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Fig. 4.1 SCIWORA.MRI shows a lesion of the spinal cord at the cervicothoracic junction

Fig. 4.2 SCIWORA.MRI (transverse section) shows a lesion of the spinal cord at the upper cervical spine

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F. Canavese

4.5 Differential Diagnosis

Differential diagnosis should include embolism from vertebral artery occlusion associated with cardiovascular diseases such as endocarditis, cardiac arrhythmia, persistent foramen ovale, arteritis, or bleeding disorder. Acute or chronic myelitis should also be excluded (Chap. 1).

4.6 Treatment Options

External immobilization of the spine (brace, collar, or halo vest) for up to 3months is the mainstay of treatment as most cases show the absence of bony involvement and malalignment (Video 4.1); moreover, the majority of published reports suggest a signicant improvement in neurological status without operative treatment. In selected cases with MRI evidence of ligamentous injury, instability, spinal cord compression, worsening, or not-improving neurological ndings should be indications for surgical decompression with or without fusion.

For asymptomatic patients who obtained stable spine xation as assessed by exion and extension dynamic radiographs, external immobilization devices can be removed earlier. Patients must avoid increased-risk activities for 6 months after diagnosis to prevent acute exacerbations of symptoms and reduce the risk of another injury.

4.7 Expected Outcomes

The two main predictors of prognosis after SCIWORA are the initial neurological status and MRI ndings. Improvement of neurological symptoms following conservative treatment should be expected in patients with incomplete neurological injury (absence of osseous lesions and instability); patients with instability or anatomical transection of the spinal cord (complete neurological decit at initial presentation) have the poorest prognosis with permanent neurologic decits.

4.8 Potential Complications

Permanent disabilities and possible long-term deformities are among the complications encountered by SCIWORA patients.

4 Spinal Cord Injury Without Radiographic Abnormality

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4.9 What Should Patient andFamily Know?

The upper cervical spine is more commonly affected in younger children while the lower cervical spine is more commonly affected in older children and adolescents as the fulcrum of movement is between C2 and C4in younger children, and between C5 and C6in adolescents and adults.