Torticollis

Author(s): Nancy Yeh, MD and Melissa Trovato, MD

Originally published:09/20/2013

Last updated:08/01/2017

1. DISEASE/DISORDER:

Definition

Torticollis is a postural abnormality of the neck leading to head tilt and rotation.  It can occur in people of all ages, from the newborn to adults.  Classically, torticollis is categorized as congenital or acquired.  Congenital muscular torticollis (CMT) is the most common form.

A newer classification proposed in 2012 uses nonparoxysmal (nondynamic) and paroxysmal (dynamic) torticollis as the two main categories.1

Etiology

  • Nonparoxysmal or nondynamic torticollis1
    • CMT
    • Osseous torticollis
    • Central and peripheral nervous system torticollis
    • Ocular torticollis
    • Non-muscular, soft tissue torticollis
  • Paroxysmal or dynamic torticollis1
    • Benign paroxysmal torticollis
    • Spasmodic (cervical dystonia)
    • Sandifer syndrome induced torticollis secondary to gastroesophageal reflux
    • Drug induced torticollis
    • Increased intracranial pressure induced torticollis
    • Torticollis secondary to conversion disorder

Epidemiology including risk factors and primary prevention

  • CMT
    • Incidence: 0.3% to 2%.1
    • Predilections: Right, male (3:2), first-born, breech and singleton delivery2
    • Risk factors include intrauterine malposition and complicated labor and delivery.

Patho-anatomy/physiology

The patho-anatomy and pathophysiology behind torticollis is dependent on the primary etiology.  In CMT, in the inferior one-third of the affected SCM, a well-circumscribed “tumor” can form, due to a hematoma or a fibroma.  As this shrinks the sternocleidomastoid (SCM) shortens, tilting the head toward and rotating the chin away from the affected SCM.

In general, disruption of arterial or neural supply to the SCM or trapezius can result in torticollis.1  For example, during an acute infection, muscle spasms or referred pain can lead to abnormal head positioning.

Another common cause of torticollis is from atlantoaxial rotary subluxation (AARS).  Retropharyngeal edema is thought to cause laxity of the atlantoaxial ligaments.  Laxity of these ligaments allows for subluxation of the facets of C1 and C2 causing abnormal tilt and rotation of the head.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

  • Age of presentation1
    • CMT: By 6 weeks of age.
    • Older age of onset suggests other acquired conditions.
  • 50-70% of SCM tumors resolve spontaneously by one year old with minimal deficits.2
  • Resolution rates by 12 months old with conservative management (manual stretching, home exercise and positioning program) range from 70-97%.4
  • 9 to 21% with CMT require surgery, especially if they persist past 1 year of age.4
  • Osseous abnormalities may need traction or surgery.3
  • Long-term development of craniofacial and postural deformities in children with untreated or inadequately treated CMT have been described.5,6,7

Specific secondary or associated conditions and complications

  • Craniofacial defects: 80% of cases, improves if treated early.8
  • Scoliosis: Commonly associated with genetic syndromes with bony abnormalities (Klippel-Feil and Larsen). Late-onset, asymptomatic thoracic scoliosis (8-13 degrees) in CMT also described.7
  • Hip dysplasia: 2 to 20% incidence versus 2% in general population.1
  • Motor developmental delay and disorders of attention, coordination, language and affect: In a cohort of 38 children ages 7 and 8 years with history of CMT, 14 (37.8%) were diagnosed with ADHD, 1 with autism spectrum disorder, 5 (15.5%) with definite motor problems (defined as scores < 5th percentile on Movement Assessment Battery for Children or the Bruininks-Oseretsky Test).9  Alberta Infant Motor Scores were found to be lower in infants with CMT (n=82) versus healthy controls (n=40) at 2 and 6 months. Reason remains unclear, but authors speculate impaired midline posture results in asymmetrical development of sensory frame of reference for perception and action.10

2. ESSENTIALS OF ASSESSMENT

History

To help identify the etiology of the torticollis, obtain information in the following areas

  • Age of onset.
  • History of onset: Acute or insidious, trauma, fever, vomiting, neurologic deficits, exposure to medications.
  • Laterality of involvement
    • CMT – Right more common, head tilted toward and chin rotated away from affected SCM.
    • AARS- Head tilted away from and chin rotated toward affected SCM.
  • Aggravating activities
    • Supine positioning: Sandifer syndrome.
    • Neck extension: Juvenile idiopathic arthritis and AARS.
  • Associated Symptoms
    • Posterior fossa tumor: Increased intracranial pressure signs (vomiting, progressive headache, diplopia), balance/coordination problems.
    • Brachial plexus palsy: Unilateral arm weakness, pain and/or numbness, +/- unilateral ptosis.
    • Infection: Neck tenderness, fever, irritability, dysphagia, drooling, odynophagia and respiratory distress.
    • Sandifer syndrome: Regurgitation, vomiting, hematemesis, anorexia, failure to thrive, asthma, chronic cough and/or hoarseness.
  • Time spent in prone while awake.
  • Prenatal and birth history.
  • Feeding history.
  • Developmental history.
  • Family history on past interventions and response will aid treatment planning.

Physical examination

  • Inspection: Head/neck posture, pharynx, plagiocephaly, facial dysmorphism, scoliosis, metatarsus adductus, and clubfoot can be associated with CMT.
  • Palpation: Tenderness or spasms of paracervical and trapezius muscles or spinous processes, thickening or mass of the SCM or trapezius, vertebral deformity, lymph nodes.
  • Cervical range of motion (ROM): Average passive range of motion in infants is 110° (100° to 120°) rotation and 70° lateral flexion (65° to 75°).11Hip exam: Asymmetry in gluteal folds, ROM, Barlow, Ortolani, Galeazzi maneuvers.
  • Postural assessment: Compensatory scoliosis with attempts at head righting
  • Neurologic: Visual and auditory fixation and tracking, fundi for papilledema, cranial nerves, sensory testing, tone, and reflexes.
  • Functional strength testing: Consider symmetry and compensatory movements.

Functional assessment

  • Age-appropriate motor skills (rolling, hand dexterity and dominance).
  • Muscle Function Scale (MFS): scale of infant’s head righting response. Infant initially held in a vertical position, then lowered to horizontal position with head position (both sides) sustained for > 5 seconds. Scored: 12
    • Head held below horizontal line.
    • Head held on horizontal line.
    • Head held slightly over horizontal line.
    • Head held high over horizontal line but <45°.
    • Head held high over horizontal line and >45°.
    • Head held very high Mean MFS for <1 year old:11
Age MFS mean MFS range
2 months 1.0 0-2
4 months 2.6 1-4
6 months 3.0 2-4
10 months 3.4 3-4

Laboratory studies

  • Differential blood count +/- rapid streptococcal test if infection suspected.
  • Karyotype testing for trisomy 21, if indicated based on history of dysmorphism.
  • Genetic testing (sequence, deletion/duplication analysis) to check for GDF 6 or 3 mutations in Klippel-Feil syndrome, as needed based on history and exam.

Imaging

Newborn and Infants

  • In CMT, ultrasound (US) of SCM is study of choice. Graded13
    • Grade 1: Increased SCM thickness
    • Grade 2: Well-demarcated heteroechoic region
    • Grade 3: Diffuse hyperechoic fibrous mass
  • Cervical spine radiographs (anteroposterior and lateral views): C1-C2 subluxation or congenital bony abnormalities but low yield (< 1%).1
  • Routine MRI in CMT is not recommended.
  • If hip exam normal, routine hip US in CMT is not recommended.14
  • No clear diagnostic guidelines are available for Sandifer syndrome.  Barium swallow test may be considered for suspected cases.

Older Children and Adolescents

  • With trauma, cervical radiographs (anteroposterior and lateral views) are the first line. Add open-mouth odontoid view if AARS is suspected. Dynamic CT is useful in assessing C1-C2 rotation in different positions of neck rotation.  If cervical CT is positive, cervical MRI/MRA is obtained to check for spinal cord, ligamentous, and vascular injuries.15
  • Without trauma, CT of the neck is obtained.  If CT negative, brain and cervical spine MRI is obtained to evaluate for central nervous system (CNS) tumor, lesion, or infection such as diskitis.15

Supplemental assessment tools

  • Arthrodial protractor: Cervical lateral flexion and rotation.11
  • Plagiocephaly anthropometrics
    • Cranial vault asymmetry: Difference between diagonal (frontozygomaticus to opposite eurion) caliper measures; normal < 3 mm, mild/moderate ≤ 12 mm, moderate/severe >12 mm.16
    • Cranial vault asymmetry index: Difference in cranial diagonal diameter/longer cranial diagonal x 100; significant if >3.5%.

Early predictions of outcomes

CMT

  • Early treatment: if < 4 months of age; the average length of treatment was only 3.2 months and none required surgery.17
  • Initial deficit in rotation >15°, birth difficulties, presence of SCM tumor (especially if on the right) were associated with longer treatment duration.8
  • Severity of SCM fibrosis on US correlate with treatment duration.16
  • MFS scores and age of initiation of treatment had significant influence on treatment time in 33 infants. ROM deficits, plagiocephaly and gender had no effect.18

Sandifer Syndrome

  • Responds to antireflux medications, upper body elevation when sleeping.

Benign Paroxysmal Torticollis

  • Usually self-limited (resolves by 3 years).

Environmental

Ask parents about

  • Sleeping position
  • Feeding set-up
  • Crib positioning
  • Car seat location

Social role and social support system

Physicians should inquire about home and social situations that could impact counseling and treatment outcomes. The number of members in the household and their level of understanding, typical daily routine of the caregiver/child and the type of care setting (home versus day care) that is available should be considered.

3. REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

Currently, no standard guidelines exist for the treatment of torticollis. Treatment is largely based on best practice recommendations. Christensen and van Vlimmeren proposed conservative treatment algorithms for CMT.18, 19

At different disease stages

CMT in < 1 year of age

1. Physical therapy and parental education

  • Early (< 4 months old) initiation of physical therapy is optimal. Passive stretching exercise protocols varied: hold times ranging from 10 to 30 seconds and repetitions of 3 to 40. 8,13,17,20  One study from showed that stretching at 10 stretches per session for 10 to 15 seconds for 10 sessions was more effective at improving head tilt and cervical passive range of motion than at 5 sessions daily over an 8 week period.21
  • By 6 to 8 months of age, infants may not tolerate passive stretching. Facilitation of active movements of the unaffected SCM, utilizing righting reactions, may be a good alternative.4
  • Massage and myofascial release.4
  • Kinesiology taping.*12
  • Therapy frequencies ranges from 5 times/week to monthly.8,13,17,20
  • Handling, positioning, and exercises at home.1,18, 19, 20

2. Orthoses

  • Tubular orthosis for torticollis (TOT) collar: Adjunct for >4.5 months old, >6° head tilt; worn during waking hours. *19,22 TOT collar is approved for use in torticollis by the Food and Drug Administration (FDA).23
  • Custom cranial orthosis for plagiocephaly.

3. Botulinum toxin chemodenervation

  • Botulinum toxin Type A injections to SCM or upper trapezius may be considered for those who failed with exercises and TOT collar use.
    • Oleszek et al used 100 units in 0.5mL dilution, weight and age-based dosing.24

CMT in >/=1 year old

1. Surgical indications

  • ≥ 6 months of conservative management.
  • Residual deficits in cervical rotation and lateral flexion >15°.
  • Tight band/tumor.7

2. Surgical procedures

  • Distal unipolar lengthening works well for most. 4
  • Bipolar lengthening (distal and proximal).
  • Z lengthening.
  • Radical resection.

Other causes1

  • Atlantoaxial rotary subluxation
    • Acute (< 1 week) and less severe – Soft cervical collar, rest, analgesics +/- diazepam or muscle relaxants.
    • Severe – Cervical traction, surgery.
  • Retropharyngeal abscess: Antibiotics (2nd or 3rd generation cephalosporins), NSAIDs, surgery if no improvement within 24 hours.
  • Primary cervical dystonia: Oral anticholinergics (i.e, trihexyphenidyl), clonazepam and other benzodiazepines, baclofen, botulinum toxin injections, deep brain stimulation for intractable cases.
  • Sandifer syndrome: Antireflux medication (proton pump inhibitors), head elevation during sleep, surgical fundoplication.
  •  Drug induced torticollis: Treat initially with diphenhydramine.

*No randomized clinical trials (RCT) with long-term follow-up found in the literature.

Coordination of care

CMT is ideally managed on an outpatient basis with coordination of care between the treating therapist and pediatric physiatrist. If trial of conservative management fails, discussion with a plastic surgeon or otolaryngologist regarding potential surgical intervention is recommended.

Patient & family education

  • Proper stretching techniques.
  • Positioning and handling skills incorporated into daily routine:
    • Feeding: Food or bottle presented to side of affected SCM to facilitate neck rotation toward that side.
    • Positioning: should be placed in a crib in such a way that he is forced to rotate his head to affected SCM and tilt toward unaffected SCM in order to see an interesting object such as a toy mobile.
    • Encouraging play in prone and facilitating neck extension to allow bilateral SCM muscle stretching.
    • Turning head to non-favored side while sleeping in supine.
  • Proper donning of TOT collar.

Emerging/unique Interventions

  • Muscle Function Scale
  • Cervical Range of Motion
  • Developmental Milestones

4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Cutting edge concepts and practice

  • Kinesiology taping as a potential adjunct to correct muscular imbalances. Immediate improvements in MFS scores with muscle relaxation taping technique versus facilitation technique has been described, although prospective studies or long-term follow-up have yet to be published.12
  • Preliminary studies show that kinesiology taping improves pain in patients with cervical dystonia.25
  • Potential application of 3D stereophotogrammetry to track changes in facial asymmetries.26
  • Two adult cases of CMT were treated with percutaneous myotomies and intramuscular cell-assisted fat grafting with improvement in head positioning and range of motion, one of which had sustained results at 18 months postoperatively.27

5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Gaps in the evidence-based knowledge

  • Factors impacting length of treatment (gender, laterality of involvement and age).
  • Efficacy of kinesiotaping.
  • Efficacy of cervical orthosis (TOT collar).

REFERENCES

  1. Tomczak K, Rosman NP. Torticollis. Journal of Child Neurology. 2012; 00(0):1-14.
  2. Do T. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006; 18: 26-29.
  3. Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine. 1998;23:2174-2179.
  4. Freed S, Coulter-O’Berry C. Identification and treatment of congenital muscular torticollis in infants. Journal of Orthotics and Prosthetics. 2004;16(4S):18-23.
  5. Rogers GF, Oh A, Mulliken J. The role of congenital muscular torticollis in the development of deformational plagiocephaly. Plastic and Reconstructive Surgery. 2009;123(2):643-652.
  6. Bredenkamp J, et al. Congenital muscular torticollis: a spectrum of disease. Arch Otolaryngol Head and Neck Surg. 1990;116:212-216.
  7. Canale ST, Griffin DW, Hubbard CN. Congenital muscular torticollis: a long-term follow-up. Journal of Bone and Joint Surgery. 1982;64-A(6):810-816.
  8. Cheng JCY, Wong MWN, Tang SP, et al. Clinical determinants of the outcome of manual stretching in the treatment of congenital muscular torticollis in infants. Journal of Bone and Joint Surgery. 2001;83-A(5): 679-687.
  9. Schertz M, Zuk L, Green D. Long-term neurodevelopmental follow-up of children with congenital muscular torticollis. Journal of Child Neurology. 2012; 00(0):1-7.
  10. Tessmer A, Mooney P, Pelland L. A developmental perspective on congenital muscular torticollis: a critical appraisal of the evidence. Pediatric Physical Therapy. 2010;22:378-383.
  11. Ohman AM, Beckung ER. Reference values for range of motion and muscle function of the neck in infants. Pediatrics Physical Therapy. 2008;20(1):53-58.
  12. Ohman A. The immediate effect of kinesiology taping on muscular imbalance for infants with congenital muscular torticollis. PM&R. 2012;4:504-508.
  13. Lee YT, et al. Clinical features and outcome of physiotherapy in early presenting congenital muscular torticollis with severe fibrosis on ultrasonography: a prospective study. Journal of Pediatric Surgery. 2011;46: 1526-1531.
  14. Kim SN, Shin YB, Kim W, et al. Screening for the coexistence of congenital muscular torticollis and developmental dysplasia of hip. Ann Rehabil Med. 2011;35:485-490.
  15. Haque S, Shafi B, Musa K. Imaging of torticollis in children. RadioGraphics. 2012;32:557-571.
  16. Mortenson P, Steinbok P, Smith D. Deformational plagiocephaly and orthotic treatment: indications and limitations. Childs Nerv Syst. 2012;28:1407-1412.
  17. Celayir A. Congenital muscular torticollis: early and intensive treatment is critical. a prospective study. Pediatr Int. 2000;42:504-507.
  18. Christensen C, et al. Conservative management of congenital muscular torticollis: an evidence-based algorithm and preliminary treatment parameter recommendations. (Physical and Occupational Therapy) in Pediatrics. 2013, Early Online 1-
  19. van Vlimmeren LA, Helders PJ, van Adrichem LN, Engelbert RH. Torticollis and plagiocephaly in infancy: therapeutic strategies. Pediatric Rehabilitation. 2006;9(1):40-46.
  20. Ohman A, Mardbrink E, Stnsby J, Beckung E. Evaluation of treatment strategies for muscle function in infants with congenital muscular torticollis. Physiotherapy Theory and Practice. 2011;27(7):463-470.
  21. He L, et al. Comparison of 2 dosages of stretching treatment in infants with congenital muscular torticollis. Americal Journal of Physical Medicine & Rehabilitation. 2016; 00: 00-00.
  22. Cottrill-Mosterman S, et al. Orthotic treatment of head tilt in congenital muscular torticollis. Journal of the Association of Children’s Orthotic-Prosthetic Clinics. 1987;22:1-3.
  23. Collins A, Jankovic J. Botulinum toxin injection for congenital muscular torticollis presenting in children and adults. Neurology. 2006;67:1083-1085.
  24. Oleszek JL, Chang N, Apkon SD, Wilson PE. Botulinum toxin Type A in the treatment of children with congenital muscular torticollis. Am J Phys Med Rehabil. 2005 84:813-816.
  25. Pelosin E, et al. Kinesiotaping reduces pain and modulates sensory function in patients with focal dystonia: a randomized crossover pilot study. Neurorehabilitation and Neural Repair. 2013; 27(8): 722-731.
  26. Hammond, P. The use of 3D face shape modelling in dysmorphology. Archives of Disease in Childhood. 2007;92(12):1120-1126.
  27. Monreal, J. Functional and aesthetic recovery of congenital muscular torticollis with intramuscular stromal vascular fraction enriched fat grafting. Cureus.  2017; 9(1)e975.

Original Version of the Topic

Paola Mendoza, MD, Lainie K. Holman, MD. Torticollis. 09/20/2013.

Author Disclosure

Nancy Yeh, MD
Nothing to Disclose

Melissa Trovato, MD
Nothing to Disclose

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