Torticollis

Torticollis (wryneck) is a postural deformity characterized by head tilt and/or neck rotation. Torticollis may be congenital, acquired, or idiopathic. The diagnostic workup and treatment of torticollis vary based on the presence of red flags for torticollis and the suspected underlying etiology. Congenital muscular torticollis (CMT), the most common type of congenital torticollis, may present with palpable abnormalities of the sternocleidomastoid (SCM) muscle, such as thickening, tightening, and/or mass. CMT generally does not require diagnostic studies and is managed conservatively, e.g., with physical therapy; refractory cases may require diagnostic studies, botulinum toxin injections, or surgery. For nonmuscular congenital torticollis (e.g., due to bony abnormalities) and acquired torticollis (e.g., due to muscular, neurologic, bony, and ocular abnormalities), clinical features of the underlying etiology may be present, and management is aimed at addressing the underlying cause.

• Congenital torticollis ^[1]
  • Develops prenatally
  • ∼ 80% of cases are due to CMT, a contraction of the SCM muscle. ^[1][2]
  • Causes of nonmuscular congenital torticollis include bony abnormalities and neck masses.
  • See “Congenital torticollis” for additional information.
• Acquired torticollis ^[3]
  • Develops after birth and has an identifiable cause
  • See “Acquired torticollis” for additional information.
• Idiopathic torticollis ^[4][5]
  • Develops after birth and does not have an identifiable cause
  • Age of onset: ∼ 50 years ^[4]
  • Sex: ♀ > ♂ (2:1) ^[4]
  • See “Dystonia” for additional information.

• Signs of infection ^[3][6]
  • Fever
  • Localized signs of infection
    • Odynophagia, dysphagia
    • Tender neck mass ^[7]
    • Vertebral point tenderness
• Signs of neurological emergencies ^[3][8]
  • Acute or progressing focal neurologic deficits
  • Ataxia
  • Signs of increased ICP
  • Diplopia
  • Preceding trauma
• Signs of osseous emergencies ^[3][8]
  • Severe neck pain
  • Vertebral point tenderness
  • Preceding trauma
  • Conditions associated with atlantoaxial ligamentous laxity ^[6]

Etiology ^[1][3][9]

• Prenatal intrauterine malpositioning or constraint, e.g., from
  • Breech presentation
  • Multiple gestation
  • Oligohydramnios
  • Macrosomia
• SCM injury during birth: hematoma → fibrosis and/or contracture → unilateral shortening of the SCM
• Abnormal development, hypertrophy, or unilateral absence of an SCM muscle

Clinical features ^[3][8]

• Head tilt or postural preference
  • May not appear until 2–8 weeks of age ^[3]
  • The head is tilted toward the contracted SCM with contralateral chin rotation. ^[8]
• Limited passive ROM and, in more severe cases, limited active ROM ^[9]
• SCM thickening, tightening, and/or palpable mass (findings differ by severity) ^[3]
• Associated abnormalities are common, e.g., ^[2][8]
  • Craniofacial asymmetry
  • Foot deformities (e.g., clubfoot, metatarsus adductus)
  • Developmental hip dysplasia
  • Developmental delay

Congenital muscular torticollis most commonly affects the right SCM muscle. ^[8]

Diagnostics ^[3][6][8]

Diagnostic studies are not indicated for most infants with suspected CMT. ^[9]

• Indications for imaging
  • Red flags for torticollis
  • Suspected nonmuscular congenital torticollis
  • Neonates
  • Persistent symptoms despite physical therapy
• Modalities
  • Ultrasound cervical spine: initial evaluation in neonates and for persistent CMT ^[1]
  • X-ray cervical spine (AP and lateral) or CT neck: for suspected bony abnormalities ^[7]
  • MRI brain and neck: for suspected CNS causes

Differential diagnosis

• Nonmuscular congenital torticollis ^[9]
  • Etiology
    • Bony abnormalities (e.g., cervical spine scoliosis, hemivertebra, Sprengel deformity, Klippel-Feil syndrome)
    • Congenital neck masses (e.g., branchial cleft cyst, cystic hygroma, SCM cyst)
    • Pterygium colli (webbed neck)
  • Clinical features: in addition to head tilt, clinical features of the underlying etiology may be present.
  • Management: Refer to the appropriate specialist for treatment of the underlying cause.
• Acquired torticollis: e.g., Sandifer syndrome, benign paroxysmal torticollis of infancy, ocular torticollis

Management ^[3][9]

The following recommendations are for patients with CMT.

• Promptly refer to physical therapy. ^[1][7]
• Educate caregivers on: ^[9]
  • Home passive stretching exercises
  • Supervised tummy time
  • Passive positioning of the infant
• Refractory symptoms: Consider specialist referral for procedural therapy, e.g.: ^[1][7]
  • Botulinum toxin injection
  • Surgery (e.g., myotomy, bipolar tenotomy): may be indicated if symptoms persist past 12 months of age ^[3]

Do not delay referral to physical therapy. Earlier intervention increases success rates and decreases the need for invasive interventions. ^[9]

Complications ^[10]

• Craniofacial asymmetry (e.g., positional plagiocephaly)
• Scoliosis of the cervical spine

Etiology

Clinical features ^[6]

• Acquired muscular torticollis
  • Spontaneous onset; may occur overnight without provocation ^[3]
  • Head tilt ipsilateral to the muscle spasm with contralateral chin rotation ^[8]
  • Limited cervical ROM
  • SCM pain and tightness
  • Acute dystonic reaction: other extrapyramidal symptoms
• Acquired nonmuscular torticollis
  • Features of the underlying cause
  • See “Red flags for torticollis.”

Diagnostics ^[3][6][8]

• No red flags for torticollis: diagnostic studies not routinely required
• Red flags for torticollis present: Obtain diagnostic studies based on suspected underlying cause.
  • Infection: CBC with differential, cultures, inflammatory markers
  • Osseous emergencies: X-ray cervical spine, CT neck
  • Neurological emergencies: MRI brain and neck, CT neck

Treatment ^[3][8]

Treatment is based on the underlying condition; involve appropriate specialists as needed.

Muscular torticollis

Acquired muscular torticollis is typically self-limiting with resolution in 7–10 days. Treatment is mainly conservative.

• Soft cervical collar
• Passive and active stretching exercises
• Localized heat, massage
• NSAIDs or muscle relaxants ^[13]
• Refractory symptoms: See “Cervical dystonia.”

Drug-induced acute dystonic reaction ^[14]

• Stop offending agent.
• Initiate IV anticholinergics (e.g., benztropine, diphenhydramine).
• See “Acute dystonia.”

Ocular torticollis ^[7]

• Treatment depends on the underlying cause.
• Options include eye patches, glasses, and/or extraocular muscle surgery.