Torticollis Paediatric

Background

Torticillis = "twisted neck" (torus and collum). Sometimes present as "Wry neck"

• Child holds head tilted to one side, chin rotated to opposite side (unilateral muscle contraction)
• Differential Dx questions
  • Hx trauma
  • Evidence infection / inflammation?
  • Spinal cord involvement?

Diagnostic flow diagram Fig 1 Fig 2.

Diagnostic approach to Paediatric Torticollis (Wry neck)

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Neck stiffness after trauma

Neck stiffness associated with infections / inflammatory conditions

Potentially life threatening

• Bacterial meningitis (see adult meningitis page)
  • Children (especially infants) may presernt with torticollis rather than neck stiffness
• Retropharyngeal abscess
  • Fever present - infection in space between posterior pharyngeal wall and anterior body Cx spine (see diagram )
  • Commonest Gp A Strep, occasrinally Staph or anaerobes
  • Clinically toxic, drooling, stridor
  • Lateral neck x-ray may show soft tissue swelling
• Infections of the spine (osteomyelitis, abscess, discitis)
  • More commonly occur in thoracic or lumbar spine
  • Localised pain, fever, high ESR
  • Usuall under 3 yo
  • Usually bacterial (staph aureus) occasionally atypical, TB etc
  • Local bone distruction, soft tissue sqwelling, reduced disc space on plain x-rays
  • Bone scan may be positive before plain x-ray changes

Diagnostic flow diagram Fig 1 Fig 2

Generally non-life threatening

• Atlantoaxial subluxation from local inflammation
  • Rheumatoid (Still's) arthritis, SLE, tonsillitis, phartyngitis or after ENT pocedures  = Grisel's syndrome
    • Sublux is rotatory, without displacement of atlas
    • Usually neck pain and tender SCM, fever & dysphagia
    • Head tilted to one side and rotated to side opposite the facet dislocation
    • Plain x-rays may be normal, CT best to visualise  (Most commonly no ant displacement of axis )
    • If severe - early neurosurgical opinion
• Cervical lymphadenitis
  • Tender swelling over lateral aspect of neck ± fever
  • Usually local S. aureus or strep, occasionally atypical e.g. TB
• Intervertebral disc calcification (IDC)
  • Uncommon, self limiting, calcification of nucleus pulposus. Cause unknown
  • Usually present with 1 - 2 days neck pain, torticollis, ± fever
  • ESR usualy raised. WCC up in 1/3
  • May require LP to exclude meningitis

Diagnostic flow diagram Fig 1 Fig 2

Neck stiffness and CNS space occupying lesions (SOL)

Potentially life threatening

• Brain tumours - posterior fossa commonest site for childhood brain tumours
  • Check for raised ICP, cranial nerve (particularly eye signs), lateralising or long tract signs
• Spinal cord tumours
  • Astrocytoma typically caues pain at tumour site and neulological signs (incl. sphincter symptoms)
  • Patients may hold their heads in forward flexion ("hanging head sign")
  • Urgent MRI
• SOL of head / neck
  • Nasopharyngeal carcinoma may present with epistaxis, neck pain and cervical adenopathy
  • Others include acoustic neuromas, orbital tumours, mets, Arnold-Chiari malformations, AVM and syringomyelia

Generally non-life-threatening

• Benign tumours of head & neck
  • Osteoid osteoma (older children and adolescents). Symptoms worse at night. Plain x-rays diagnostic

Diagnostic flow diagram Fig 1 Fig 2

Congenital causes of Neck Stiffness

• Congenital muscular torticollis
• Aetiology unclear, probable birth trauma, SCM haematoma with resultant scarring
• Often palpable mass in inferior part of SCM. Mass not at birth but appears in neonatal period
• Head held with chin pointing away from affected, contracted SCM
• Associated craniofacial asymmetry(contralateral flattening of occiput and ipsilateral depression of malar prominence)
• X-ray to outrule other causes. Treat with passive stretching of affected muscle
• If deformity persists more than 6 months, may need surgical release (< 5%)
• Skeletal malformations
• Commonest is Kippel-Feil (congential fusion Cx vertebrae) may have atlantoaxial instability
• Scoliosis develops in 50%
• Classical triad (<50%) is limited neck ROM, low hairline and short neck
• Atlantoaxial instability
• Down synd., Kippel-Feil, other skeletal dysplasias, os odontoideum, Morquio syndrome mucopolysaccharidopsis)
• Benign paroxysmal torticollis of infancy
•  Episodes of torticollis in association with pallor, agitation and vomiting
• Onset at 2 - 3 months, remits by 2 - 3 years

Diagnostic flow diagram Fig 1 Fig 2

Miscellaneous causes torticollis

• Ophthalmologic, neurologic and /or vestibular causes (chheck visual acuity and exclude diplopia in all)
• Myaesthenia gravis (torticollis, ptosis, extra-ocular eye muscles and cranial nerve palsies
• Sandifer syndrome (torticollis, gastro-oesophageal reflux and hiatus hernia present as FTT and vomiting)
• Pneumomediastinum (usually after bout coughing or retching.)
• Spasmus nutans (I bet this one is new to you too)
  • Acquired torticollis, nystagmus and head nodding
  • Become symptomatic in first 2 years
  • Rarely underlying brain tumour (CT all), most benign self limiting condition
• Dystonic reaction (diphenhydraminbe 1 mg/kg will be diagnositc and therapeutic)

Diagnostic flow diagram Fig 1 Fig 2

Evaluation child with torticollis

• Is there cord involvement?
• Is there a history of trauma
• Any signs infection / inflammation

Diagnostic flow diagram Fig 1 Fig 2

Links

Reference - Fleisher G. Textbook of Paediatric Emergency Medicine. Lipppincott Williams & Wilkins.