Approach

Infants with suspected CMT should be referred to physiotherapy as soon as possible.[29] It is much easier to perform the necessary massage and stretches on an infant than an active baby. Education and a home programme can be provided by the physiotherapist. Earlier referral can help prevent progression of torticollis and craniofacial deformities.

Referral to a paediatric physiatrist (consultant in physical medicine) or a physical medicine and rehabilitation consultant is warranted when progress is limited and more aggressive treatment is necessary. Referral to a neurosurgeon or plastic surgeon would be necessary if craniosynostosis were suspected.

Physiotherapy and home programme

Physiotherapy is a first-line treatment and is warranted in all infants with CMT with or without positional plagiocephaly. One evidence-based clinical practice guideline on physical therapy management in CMT was published in 2018, as an update to the 2013 guideline.[9][30]

The majority of published studies use a combination of physiotherapy, environmental adaptations, and a home programme completed by the carers.[2][10][16][31][32] Physiotherapy intervention is most effective when started early.[30] One study found that 98% of infants with CMT achieve normal cervical range within 1.5 months if physiotherapy is started before 1.0 months of age, but progressively fewer infants achieve normal range if therapy is started after 6 months, with the therapy duration being prolonged up to 9-10 months.[30] Regardless of when physiotherapy is initiated, education of carers to use their daily routines of carrying, positioning, and feeding to accomplish the desired postures is important.

In addition to massage and myofascial release performed by physiotherapists, physiotherapy and a home programme can include the following:

  • Gentle stretching using carrying and play techniques to promote active neck rotation towards the affected side and discourage tilting of the head towards the affected side.

  • Turning the head of the infant while sleeping supine to encourage rotation to the non-favoured side.

  • Encouraging head rotation to the side of the affected muscle by arranging the environment with visually stimulating items on that side or changing the crib orientation if necessary.

  • Strengthening the contralateral neck muscles by carrying the infant with his/her body tilted to the affected side, practising assisted rolling to the contralateral side, or side-lying on the affected side (these activities use the head righting response to strengthen the weak contralateral side).

  • Alternating carrying and feeding positions.

  • Encouraging mid-line head position in infant carriers with the use of rolled towels.

  • Prone time to play several times a day (newborns often tolerate a more inclined position). This position also facilitates bilateral sternocleidomastoid (SCM) elongation.

A tubular orthosis for torticollis (TOT) is occasionally prescribed, but it is not universal. It is a cervical collar made of soft tubing that is taller on the affected side to promote a passive stretch of the affected SCM and provide a noxious stimulus to promote lateral flexion to the opposite side. TOTs tend to be used in children with a mild torticollis who tend to tilt more habitually but have good passive range of movement (ROM) and good head control. One author prescribes the collar if the child has a head tilt of ≥6° at 4.5 months of age, while another uses it for constant tilting or if passive rotation is <45°.[10][16]

Cranial moulding orthosis

This is used for moderate to severe plagiocephaly, which is often associated with CMT. As the skull grows the fastest and is most malleable in the first year of life, the optimal response is obtained if used between 4 and 12 months of age. The orthosis is worn 23 hours a day, and treatment duration is usually 3 to 4 months. It is then adjusted upon receipt every 2 to 3 weeks. Use of a cranial moulding orthosis can indirectly improve head position in CMT as it provides a symmetrical surface when lying supine, thus removing the gravitational forces that promote turning of the head towards the flattened occiput. Evidence shows a statistically significant improvement in infants treated with a moulding orthosis.[33] One systematic review showed considerable evidence that moulding therapy may reduce skull asymmetry more effectively than repositioning therapy.[34]

Botulinum toxin type A (BTX-A)

BTX-A injections are performed in children with CMT that is refractory to physiotherapy and a home programme. They may also help avoid the need for surgical release and can be repeated in 3 months if deficits persist. BTX-A, a neurotoxin derived from the bacteria Clostridium botulinum, produces a protein that inhibits release of acetylcholine and results in localised reduction in muscle activity. The goal of BTX-A is to temporarily weaken the affected SCM or upper trapezius muscle, resulting in an easier and more successful stretching programme as well as an improved ability to strengthen the contralateral neck musculature. BTX-A has been shown to be safe and effective in the treatment of cervical dystonia in adults and has been used safely in children with limb spasticity for many years.[35][36] Retrospective studies have shown BTX-A to be safe and effective in children (mean age 10.1 months and 7.6 months) with refractory CMT.[37][38] The injections can be done safely without anaesthesia, but some physicians choose to anaesthetise the children. Transient adverse events, including mild dysphagia and neck weakness requiring no medical treatment, have been reported in a minority of patients.[37]

One meta-analysis of 10 studies found the effectiveness botulinum toxin for CMT was 84%, the adverse reaction rate was 1%, and conversion to surgery was 9%.[39]

Sternocleidomastoid muscle release

SCM release is considered when the child is refractory to physiotherapy and botulinum toxin type A treatments. Surgery is often considered when there is no improvement after 6 months of physiotherapy and there is a tight fibrotic band in the muscle, or when children aged >1 year.[2][10] A study of patients (age range 2 to 13 years) with CMT treated surgically showed excellent results in 88%.[40] The most important factor affecting the overall result and outcome was age at the time of operation: excellent results were obtained in all children aged <3 years. Endoscopic release has been shown to have a favourable long-term outcome.[41] Craniofacial deformity in children with CMT, as measured by cephalometry, has been shown to be significantly improved after surgical release of the SCM muscle, especially if the surgery was performed on children <5 years.[42]

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