CERVICAL SPINE

ABNORMALITIES IN CHILDREN
 TORTICOLLIS AND RELATED
SYNDROMES
• Torticollis is a cervical deformity :
• head is rotated, tilted towards one side with
lateral flexion: ‘cock-robin’ position
• SCM muscle is ‘shortened’ and may feel tight
and hard
• Congenital: cervical spine anomaly,
particularly of the atlas
• Acquired: Tumour eg: eosinophilic granuloma
• Infection: discitis, lymphadenitis
• Ear or upper respiratory tract infection [rarely]
• Cervicothoracic scoliosis
• 25% of cases: Idiopathic
• Children and adolescents, acute torticollis is
characterized by atlantoaxial rotational
subluxation of sudden onset
Workup: cervical spine X-ray, CT scan
INFANTILE (CONGENITAL) TORTICOLLIS
• PATHOLOGY :SCM muscles is fibrous and fails
to elongate as the child grows
• resulting in a progressive deformity
• aetiology is unclear
• intrauterine packaging disorders
• birth injury causing localized ischaemia
• history of difficult labour or breech delivery
is common
 Clinical features
• Lump : first few weeks after birth,
disappearing within few months
• No deformity or obvious limitation of
movement until the child is 1–2 years old
• classical visible deformity of the neck, with the
head tilted towards the affected side and the
face rotated towards the contralateral
shoulder
• Asymmetry of the face (hemihypoplasia]
• Plagiocephaly: Flat head syndrome
• Features can worsen and become more
obvious as the child grows
 Treatment
• Most children have a complete spontaneous
resolution with time, but some cases may
require physiotherapy
• daily muscle stretching may prevent the
incipient deformity
• benign SCM lump can completely disappear
• clinician should be aware of other causes such
as tumours and cysts in the neck, which may
need surgical excision
• If the condition persists beyond 1 year,
operative correction is required to avoid
progressive facial deformity
 SECONDARY TORTICOLLIS
• Infection:
• lymphadenitis
• retropharyngeal abscess
• discitis
• Tuberculosis
• Tumours:
• posterior fossa tumours
• Intraspinal tumours
• inflammatory disorders:
• juvenile rheumatoid arthritis
• neurogenic causes:
• benign paroxysmal torticollis
• Trauma
• idiopathic
 Atlantoaxial rotatory subluxation
• pathological displacement of atlas[ C1 ]
• associated with minor trauma
• nasopharyngeal infection, tonsillectomy
• Retropharyngeal abscess (Grisel’s syndrome]:
• inflammation of the pharynx can lead to attenuation
of atlantoaxial ligaments or the synovium
• acute onset : pain and muscle spasm
• Delayed presentation also seen
• In fixed deformities, pain subsides but motion is
restricted and the child cannot correct the deformity
• chin is shifted laterally and the head fixed in
this position
• Early diagnosis and therapy are crucial to
prevent neurological complications caused by
compression of the medulla oblongata by the
dislocated odontoid
• Plain X-ray interpretation may be challenging
• Open-mouth views should be obtained
• CT scans in both neutral and maximum lateral
rotation are the most helpful investigation
• Most cases are mild and can be managed with
a soft collar and analgesics
• IF there is no resolution after a week, halter
traction
• Bed rest and analgesics
• physiotherapy may be contraindicated
• Attempts for manual reposition without
general anaesthesia are not indicated
• Occasionally, if the articulation remains
unstable, subluxation persists or recurs easily
or if there is neurological compromise, then a
C1–C2 fusion is recommended.
 KLIPPEL-FEIL SYNDROME
• Failure of segmentation of the cervical somites
during the 3rd to 8th week of embryogenesis,
resulting in fusion of at least two cervical
segments
• fusion can occur at any level in the cervical
spine, but approximately 75% occur in the
upper cervical spine
• often associated with other skeletal and
extraskeletal abnormalities:
• Scoliosis(60%)
• renal abnormalities (35%] : unilateral renal
agenesis
• Sprengel deformity (30%)
• Deafness (30%)
• congenital heart disease (14%}: VSD
• Other associated deformities:
• hand anomalies such as syndactyly
• thumb hypoplasia
• extra digits
Classical clinical triad of children :
• short neck with various degrees of neck webbing
• low posterior hairline
• limitation of neck mobility
• Symptoms tend to arise in the 2nd or 3rd
decades
• most consistent clinical finding is a limited
range of motion of the neck, especially lateral
bending
• pain due to joint hypermobility or neurological
symptoms from instability
 Imaging
• X-rays and CT scans reveal fusion of two or
more cervical vertebrae
• vertebrae are also often widened and
flattened (so-called ‘wasp-waist appearance’,
which is considered pathognomonic

• All patients with Klippel–Feil syndrome should

have an ultrasound evaluation of the renal
system.
 Treatment
• For asymptomatic patients, treatment is
unnecessary but parents should be warned of
the risks of contact sports
• Sudden catastrophic neurological compromise
can occur after minor trauma

• Children with symptoms may need cervical

fusion
 BASILAR IMPRESSION
• Basilar impression or invagination is a disease
of the atlantoaxial facet joints causing
progressive vertical instability ,migrating
upwards {odontoid} ,obstructing foramen
magnum and compressing upon the brainstem
• congenital or acquired
• primary invagination :
occipitoatlantal fusion
hypoplasia of the atlas
bifid posterior arch of the atlas
• Odontoid anomalies
• Klippel–Feil syndrome
• achondroplasia
• Secondary basilar impression :
• osteomalacia,
• Rickets
• osteogenesis imperfecta
• Paget’s disease
• Neurofibromatosis
• skeletal dysplasia
congenital neurological anomalies, such as
Arnold- Chiari malformation and syringomyelia
 CLINICAL FEATURES
• Short neck, facial asymmetry and torticollis : not
pathognomonic
• neck pain, headaches in the distribution of the
greater occipital nerve
• cranial-nerve involvement
• Ataxia
• vertigo
• nystagmus,
• weakness and paraesthesia of the limbs
 Imaging
• Craniometric parameters such as
• McRae’sline
• Chamberlain’s line
• McGregor’s line:
• described to quantify the relationship
between the odontoid process and the
foramen magnum
 Treatment
• depends on the degree of neural compression
and reducibility of the deformity
• Surgical decompression and stabilization with
a posterior occipitocervical arthrodesis
• IF symptoms are the result of a compressive
aberrant odontoid that cannot be reduced,
odontoidectomy may be indicated
THANK YOU