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Torticollis

System(s) affected: musculoskeletal; nervousSynonym(s): acute wryneck; idiopathic generalized torticollis; SCM torticollis; neonatal torticollis; idiopathic cervical dystonia; focal dystonia; nuchal dystonia ### Epidemiology

Posttraumatic fracture or dislocation

Neoplastic: spinal cord tumor, acoustic neuroma, osteoblastoma, orbital tumor, fibromatosis, metastasis

Selective peripheral nerve denervation

para>Congenital muscular torticollis (CMT): seen at birth or in early infancy " accounts for 80% of torticollis cases presenting in infancy. Results from unilateral fibrosis and shortening of the sternocleidomastoid (SCM) muscle

Acquired torticollis

  • Acquired torticollis (also known as "wryneck " ) " usually self-limited.
  • Spasmodic torticollis, (cervical dystonia) caused by recurrent involuntary muscular contractions
  • Other less common forms of torticollis include oculogyric, gastroesophageal reflux " induced, arthritis-related, scoliosis-related, and hysterical torticollis.

Epidemiology

  • ’ Ό90% of cases occur in individuals aged 31 " 60 years.
  • Predominant age: CMT: newborn and infants; pediatric acquired torticollis, <10 years; adult acquired torticollis, 30 " 60 years; spasmodic torticollis, 30 " 50 years (mean age 40 " 43 years)
  • Predominant sex: spasmodic torticollis, female > male (1.6:1); CMT, male > female (3:2)

Incidence

Congenital: up to 1/250 births; spasmodic: 1/100,000; overall incidence for torticollis is 24/1 million persons.

Prevalence

All focal dystonias combined: 295/1 million persons; no reliable data for acquired torticollis.

Etiology and Pathophysiology

  • CMTIntrauterine malpositioning may lead to foreshortening and fibrosis of the SCM.Birth trauma (e.g., clavicular fracture) can lead to CMT as well.
  • Pediatric acquired torticollisCNS disorders, increased intracranial pressureOcular disorders " abnormal head/neck positioning as visual compensationBony abnormalities, soft tissue pathologiesGastrointestinal diseaseDrug inducedConversion disorder
  • Adult acquired torticollisEmotional stress, postural factors (e.g., work, sleep, lying while reading or watching TV, prolonged unusual positioning of neck), or exposure to cold. Many cases are idiopathic.Medication reactions (most commonly anticholinergics, amphetamines, and certain anesthetic agents)
  • Spasmodic torticollisMuscular spasm secondary to traumatic, infectious or inflammatory insultCervical spine injuries and spondylosis, ocular disorders, organic CNS disorders, psychogenic causes, certain tumors and vestibular dysfunction can all contribute to cervical dystonia.

Genetics

Spasmotic torticollis may also have a genetic basis.

Risk Factors

  • CMT: intrauterine crowding, breech presentation, ischemia, birth injury
  • Pediatric acquired torticollis: soft tissue inflammation or infection, neurologic disease, visual disturbances, trauma
  • Adult acquired torticollis: stress, unusual neck position (particularly when sleeping), exposure to cold, medications, trauma, infection
  • Spasmodic: family history of dystonia, soft tissue inflammation or infection, neurologic conditions, visual disturbances, trauma

Pediatric Considerations

CMT: without treatment becomes a fibrous cord and may be associated with persistent craniofacial deformities and persistent torticollis

Commonly Associated Conditions

  • >80% of infants with CMT present with craniofacial asymmetry, deformational plagiocephaly, and developmental dysplasia of the hip (DDH).
  • CMT and pediatric acquired torticollis: Consider Klippel-Feil syndrome (congenital fusion of cervical vertebrae).
  • Pediatric and adult acquired torticollis: Consider spinal abnormalities, tumors.
  • Spasmodic torticollis often accompanied by behavioral health conditions

Diagnosis

History

  • Abnormal head posture, neck pain, headache, neck muscle stiffness, restricted neck range of motion, neck mass or swelling
  • Birth history in children
  • Family history of dystonias
  • Medication history
  • Recent cervical spine trauma

Physical Exam

  • Normal ROM: flexion 60 degrees; extension 75 degrees; rotation 90 degrees; side-bending 45 degrees
  • Torticollis presents as rotational (twisting), anterocollis (flexion), laterocollis (side bending), and retrocollis (extension) with the head tilting to the affected side (80%, to right side) and the chin rotating to the opposite side.
  • Spasm of trapezius, SCM, and other neck muscles
  • Tenderness over the affected SCM
  • Neck mass, lymphadenopathy
  • Craniofacial asymmetry (plagiocephaly) consistent with congenital or chronic torticollis
  • Phasic jerking or tremor of antagonist muscles
  • Physical gesturing (geste antagoniste) such as touching face or chin to reduce dystonia (pathognomonic for spasmodic torticollis)
  • Ocular irregularities (e.g., diplopia)
  • Spinal abnormality: Short neck with low posterior hairline may indicate occipitocervical synostosis.
  • Structural abnormalities of the hips or feet
  • Common physical findings in different types of torticollisCMT: Firm, nontender, palpable enlargement of the SCM may appear from birth or develop over weeks.Pediatric and adult acquired torticollis: unilateral neck stiffness, pain, spasm, or decreased ROM without traumaSpasmodic torticollis may initially present with neck stiffness progressing to pain, head jerking, and neck spasms.

Differential Diagnosis

  • OsseousAtlantoaxial rotatory subluxationAtlanto-occipital subluxationPosttraumatic fracture or dislocationCervical disc diseaseCongenital scoliosisKlippel-Feil syndromeOccipitocervical synostosisGrisel syndromeSyringomyeliaArnold-Chiari malformation
  • NonosseousMyositis involving cervical musclesSoft tissue traumaNeoplastic: spinal cord tumor, acoustic neuroma, osteoblastoma, orbital tumor, fibromatosis, metastasisInfection: upper respiratory infection, cervical lymph node abscess, epidural abscess, retropharyngeal abscess, vertebral osteomyelitisVestibular disordersEssential head tremorBasal ganglion diseasesCranial nerve palsyPsychiatric disordersDrugs or toxinsDown syndromeSandifer syndromeMyasthenia gravis

Diagnostic Tests & Interpretation

Lab studies are only needed to exclude underlying disease.

  • Radiographs should be taken to rule out spinal pathology in traumatic and congenital cases.
  • Consider MRI or CT scan of cervical spine for patients with neurologic deficits.
  • CMT can be confirmed with US of involved SCM.

Follow-Up Tests & Special Considerations

Infants with CMT should be screened for DDH by physical exam and subsequent US if not clearly normal (1)[C]. All pediatric patients should have an eye exam.

Treatment

General Measures

  • CMT>90% of children achieve good outcome with conservative treatment if initiated by age 1 year.Conservative treatment includes positioning, environmental adaptations, passive and active stretching of the tight SCM muscle, strengthening of weak neck and trunk muscles, and movement therapy.Physical therapy (PT) and stretching should be started before ages 3 " 6 months (2)[B].Place toys on opposite side of bed from rotational deformity to encourage use of affected muscles.Surgery is indicated for refractory cases, a tight muscular band, or SCM mass.
  • Pediatric acquired torticollis
  • Adult and pediatric acquired torticollisConservative management includes soft cervical collar, intermittent heat or ice, bed rest, and analgesics for pain relief.
  • SpasmodicConservative: soft cervical collar, heat or ice, bed restSelective peripheral nerve denervationPallidal deep brain stimulation can achieve lasting improvements in medication-resistant cases (3)[B].

Medication

First Line

  • Treatment for spasmsBotulinum toxin injections may be more effective than anticholinergic drugs for cervical dystonia (4)[B],(5)[A]. Major predictors of treatment success are correct dosing and identification of affected muscles. Note that the recommended doses are general guidelines and must be individualized to the patient. EMG (6)[A] or PET-CT (7)[B] may help guide the injection site. Immunoresistance can develop but is rare (8)[A]:Botox: botulinum toxin type AAdult: initial 1.25 " 2.5 units (0.05 " 0.1 mL) IM into most active neck muscles; repeat every 3 " 4 months; not to exceed 200 units cumulative dose in 1-month periodPediatric: <12 years of age, not established; >12 years, administer as for adultsXeomin (botulinum toxin type A free from complexing proteins) has shown comparable safety and efficacy to Botox (10)[B].Myobloc: botulinum toxin type BAdult: 2,500 " 5,000 units IM divided among affected muscles in patients treated previously with any type of botulinum toxin; use lower dose in untreated patients.Pediatric: not establishedDiazepamDiphenhydramine or diazepam can be used for torticollis caused by medications (4)[C]:DiphenhydramineAdult: 25 " 50 mg PO q6 " 8h PRN, not to exceed 400 mg/day; 10 " 50 mg IV/IM q6 " 8h PRN, not to exceed 400 mg/dayPediatric: 12.5 " 25 mg PO TID/QID or 5 mg/kg/day or 150 mg/m2/day divided TID/QID. PRN, not to exceed 300 mg/day; 5 mg/kg/day IV/IM or 150 mg/m2/day, divided QID. PRN, not to exceed 300 mg/dayAnticholinergics may relieve acute muscle spasms (4)[C], but high doses are usually necessary and benefit is often delayed.Carbidopa-levodopa (4)[C] for focal dystonia of unknown cause: Trial might be warranted although some non " dopa-responsive dystonias improve, equal numbers have symptoms worsen.
  • Treatment for pain

Issues for Referral

  • Refer prolonged symptoms of idiopathic spasmodic torticollis to a neurologist.
  • Fixed deformities in children may require surgical consultation.
  • CMT: Refer if visual dysfunction (ophthalmology), failed hip screen (orthopedics), abnormal neurologic exam (neurology), plagiocephaly (plastic surgery), or patient presents with bony prominence (orthopedics).

Additional Therapies

  • Osteopathic manipulation may be useful:Direct myofascial stretching of cervical region with attention to the SCMOccipital-atlantal releaseV-spread of the occipitomastoid suture on the side of restrictionMuscle energy and/or functional positional release at the cervical region
  • PT may be beneficial for acquired childhood and adult torticollis (11)[B].
  • If detected early, 90% of CMT responds to stretching exercises.

Surgery/Other Procedures

CMT: Surgical release if PT is unsuccessful by age 1 year

Complementary & Alternative Medicine

Acupuncture may also provide benefit.

Ongoing Care

Follow-up Recommendations

  • Monitor infants with CMT every 2 " 4 weeks until resolved.
  • Depression is common in protracted cases, screen accordingly.

Prognosis

  • CMT: good for correctable pathologies
  • Pediatric acquired torticollis: good when the underlying pathology is discovered and treated
  • Adult acquired torticollis: excellent; generally resolves in a few days to weeks
  • Spasmodic: may wax and wane for years, even with treatment

Complications

  • Facial asymmetry in CMT; possibly an increased risk of developmental delay/disorders
  • Dental malocclusion
  • Degenerative osteoarthritis of the cervical spine, hypertrophy of the SCM muscle, and paresthesia due to compressed nerve roots
  • Depression

References

1.Joiner ERA, Andras LM, Skaggs DL. Screening for hip dysplasia in congenital muscular torticollis: is physical exam enough? J Child Orthop. 2014;8(2):115 " 119. [View Abstract]2.Do TT. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006;18(1):26 " 29. [View Abstract]3.Walsh RA, Sidiropoulos C, Lozano AM, et al. Bilateral pallidal stimulation in cervical dystonia: blinded evidence of benefit beyond 5 years. Brain. 2013;136(Pt 3):761 " 769. [View Abstract]4.Tarsy D, Simon DK. Dystonia. N Engl J Med. 2006;355(8):818 " 829. [View Abstract]5.Patel S, Martino D. Cervical dystonia: from pathophysiology to pharmacotherapy. Behav Neurol. 2013;26(4):275 " 282. [View Abstract]6.Nijmeijer SW, Koelman JH, Kamphuis DJ, et al. Muscle selection for treatment of cervical dystonia with botulinum toxin " a systematic review. Parkinsonism Relat Disord. 2012;18(6):731 " 736. [View Abstract]7.Lee HB, An YS, Lee HY, et al. Usefulness of (18)f-fluorodeoxyglucose positron emission tomography/computed tomography in management of cervical dystonia. Ann Rehabil Med. 2012;36(6):745 " 755. [View Abstract]8.Coleman C, Hubble J, Schwab J, et al. Immunoresistance in cervical dystonia patients after treatment with abotulinum toxin A. Int J Neurosci. 2012;122(7):358 " 362. [View Abstract]9.Simpson DM, Blitzer A, Brashear A, et al. Assessment: botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008;70(19):1699 " 1706. [View Abstract]10.Dressler D, Paus S, Seitzinger A, et al. Long-term efficacy and safety of incobotulinumtoxinA injections in patients with cervical dystonia. J Neurol Neurosurg Psychiatry. 2013;84(9):1014 " 1019. [View Abstract]11.Queiroz MA, Chien HF, Sekeff-Salem FA, et al. Physical therapy program for cervical dystonia: a study of 20 cases. Funct Neurol. 2012;27(3):187 " 192. [View Abstract]

Additional Reading

  • Consky EA. Clinical assessments of patients with cervical dystonia. In: Jancovic J, Hallett M, eds. Therapy with Botulinum Toxin. New York, NY: Marcel Dekker; 1994:211 " 237.
  • Jankovic J. Treatment of cervical dystonia with botulinum toxin. Mov Disord. 2004;19(Suppl 8):S109 " S115. [View Abstract]
  • Tomczak KK, Rosman NP. Torticollis. J Child Neurol. 2013;28(3):365 " 378. [View Abstract]

Codes

ICD10

  • M43.6 Torticollis
  • Q68.0 Congenital deformity of sternocleidomastoid muscle
  • G24.3 Spasmodic torticollis
  • S13.4XXA Sprain of ligaments of cervical spine, initial encounter

ICD09

  • 723.5 Torticollis, unspecified
  • 754.1 Congenital musculoskeletal deformities of sternocleidomastoid muscle
  • 333.83 Spasmodic torticollis
  • 847.0 Sprain of neck

SNOMED

  • 70070008 torticollis (disorder)
  • 268240006 Congenital torticollis
  • 74333002 Spasmodic torticollis (disorder)
  • 360444002 Traumatic torticollis

Clinical Pearls

  • Suspect torticollis in patients with head tilt, chin lift, and restricted movement of the neck.
  • Consider secondary causes, and rule out as necessary based on history and physical exam.
  • Most adult cases are self-limiting and resolve within days to weeks.
  • In congenital and acquired pediatric cases, placing items of interest to the child (e.g., TV, toys) on the opposite side of the bed helps to speed resolution.