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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Diagnostic flow diagram Fig 1 Fig 2

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Diagnostic flow diagram Fig 1 Fig 2

Neck stiffness after trauma

Potentialy life threatening causes

  • Cervical fractures
    • Mainly upper vertebrae, often alert on presentation, neurological signs rare. Immobilise and image
  • Subluxation Cx spine
    • More common than #. Can result from minor trauma. 
    • Commonest is rotatory altantoaxial subluxation (cord intact as transverse lig of atlas intact).
      • Beware in children with Down syndrome - transverse lig very lax
    • Sternomastoid (SCM) and neck tendernes localised to same side as head rotation. Contrary to inflammatory muscular torticollis (tender SCM is opposite to direction of head rotation)
    • Open mouth peg view x-ray abnormal. Confirm with CT
    • Refer orthopaedics - most treated with soft collar & NSAIDs

Potentially left threatening

  • SCIWORA -(Spinal Cord Injury WithOut Radiological Abnormality)
  • Ligament laxity in children. SCIWORA make up to 50% cord injuries in children
  • Usually < 8 yo. Significant and / or progresive neulology up to 48 hours after trauma
  • Admit all, even those with transient neurology as may remit and progress later
  • Epidural haematomas of cervical spine
    • Progressive neulology. 
    • Treat ABCs. Immobilise. Immediate neurosurgical opinion ± MR

Non life threatening

  • Clavicular fracture
  • Traumatic muscular contusion of neck
    • Dx of exclusion only

Neck stiffness associated with infections / inflammatory conditions

pharyngeal space
Pharyngeal space

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

retro-pharyngeal abscess1
Retro-pharyngeal abscess

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



Content by Dr Íomhar O' Sullivan Published 29/03/2004. Reviewed by Dr ÍOS 28/09/2005, 03/08/2007. Last review Dr; ÍOS 16/06/21