Torticollis (Congenital and acquired) Children, Emergency Department, Paediatrics (365)

Torticollis is the tilting of the head to one side caused by contraction of the neck muscles e.g. sternocleidomastoid. It is not a diagnosis in itself, but is a sign of underlying pathology. Torticollis can be divided into two broad categories: Congenital and acquired.

Congenital muscular torticollis (CMT) – previously called Sternocleidomastoid tumour - is the most common cause of abnormal head posture in infants. It is usually noticed within the first month of life. It is seen as a result of birth trauma, oligohydramnios or foetal position within the uterus. CMT causes shortening and fibrosis of the sternocleidomastoid muscle which can be palpated as a mass within the muscle. CMT can occur in the absence of a palpable mass. It can be associated with gross motor delay until about 1 year of age.

There are other rarer conditions which result in congenital torticollis e.g. malformed cervical spine, Arnold-chiari malformation, spina bifida. These also include conditions involving CNS, eye, skin and bone abnormalities.

Acquired torticollis has a broad spectrum of aetiologies:

Musculoskeletal

• Muscle spasm ("wry neck")

Infection

• Head and neck (URTI, otitis media, mastoiditis, cervical adenitis, retropharyngeal abscess, dental infection, pharyngeal infection)
• Spine (osteomyelitis, discitis, epidural abscess)
• CNS (meningitis)

Atlantoaxial rotatory fixation

• Trauma and ligamentous laxity (e.g. as part of underlying disorders)
• Post head/neck surgery
• Grisel syndrome

Inflammation

• Juvenile idiopathic arthritis

Neoplasm

• CNS tumours
• Bone tumours

Dystonic syndromes (idiopathic spasmodic torticollis, drug reactions)

Conditions that mimic torticollis

• Ocular dysfunction e.g. Extra-ocular muscle palsy secondary to intracranial tumour

If torticollis occurs with a history of trauma manage as a cervical spine injury

• Is there a history of an awkward head/neck posture for a prolonged period of time which could cause the symptoms? E.g. playing X-box.
• Fever, increased drooling, sore throat and dysphagia suggest an infective cause.
• Duration of symptoms – acute muscular torticollis should resolve within 7-10 days.
• Antenatal/birth history – oligohydramnios, birth trauma
• Any neurological symptoms e.g. headache, strabismus, diplopia, photophobia, ataxia, seizures?
• Underlying conditions e.g. Down Syndrome
• Recent head and neck surgery
• Any medications that might cause dystonia e.g. metoclopramide?

• Record observations including temperature and pulse
• ENT examination
• Examine the neck - assess for mid line tenderness, active range of movement, soft tissue neck tenderness. Is there a palpable neck mass? E.g. lymphadenopathy, abscess, sternomastoid mass (congenital muscular torticollis).
• Assess for plagiocephaly and clicky/dislocated hips if congenital muscular torticollis suspected.
• Neurological examination
• Examination of spine for signs of spina bifida e.g. hairy tufts/dimples
• Ophthalmology examination – pay particular attention to normal eye movements (abnormal in extra ocular nerve palsy)
• Location of tenderness might assist in diagnosis but some deep seated pathology will show no external signs.

If torticollis occurs with a history of trauma manage as a cervical spine injury

• Discuss with Senior ED doctor prior to requesting investigations
• In general imaging is not useful, however consider:
• Cervical Spine Plain X-ray - If cervical spine tenderness following analgesia, persistent symptoms >1 week, severe pain, limited range of movement, patient has an underlying condition associated with increased ligamentous laxity.
• Ultrasound
• CT +/- MRI neck +/- brain

Specialty referral should be guided by history and clinical findings. Discuss with ED senior on the floor prior to referral. Discussion with ENT, neurology, ophthalmology, orthopaedics or general paediatrics can help in decisions about appropriate imaging modality and timing.

If torticollis occurs with a history of trauma manage as a cervical spine injury

• If the patient is unwell/ septic adopt an “ABC” approach
• Analgesia 

Further management depends upon diagnosis:

• For muscle spasm reassure patient/carer that it should resolve in about 1 week.
• Appropriate antibiotics in the case of infection – may require IV
• Referral to ENT if parapharyngeal or retropharyngeal abscess suspected.
• Refer to orthopaedics if bony cause
• Routine referral to physiotherapy is not indicated for acute muscular torticollis
• Use of other guidelines as appropriate e.g. Neck Lumps (diagnosis and management of)

Congenital Muscular Torticollis (CMT)

• Acute referral to physiotherapy (they will refer onto orthopaedics for outpatient follow up and to arrange hip scans).
• Do not use the “opt in” physiotherapy service for patients with CMT

All patients/ carers discharged with torticollis from the ED should be given advice on appropriate doses and frequency of analgesia. They should be advised to seek review by GP if the torticollis persists for longer than 1 week or return to the ED if any additional symptoms develop.