Orthopedics

Torticollis

Background

Torticollis, or “wryneck,” is the clinical term for a twisted or rotated neck

  • In Latin, “torus” means “twisted” and “collum” means “neck”
  • Torticollis is a common complaint in children
  • Torticollis can be congenital or acquired

Congenital Torticollis

Congenital Muscular Torticollis:

  • The unilateral contracture of the sternocleidomastoid muscle
  • The infant’s neck range of motion is restricted
  • The head tilts towards the shortened SCM and the chin points towards the opposite side
  • The most common form of congenital torticollis
  • Estimated incidence is < 1-2%
  • More common in breech babies and difficult deliveries
  • Congenital torticollis is associated with facial asymmetry (acquired torticollis is not)

Etiology:

  • The etiology of congenital muscular torticollis is not well understood
  • The main theories attribute congenital torticollis to either 1) abnormal fetal positioning in utero or 2) birth trauma
  • Malposition of the head in utero is believed to cause an “in utero compartment syndrome” that leads to unilateral fibrosis of the SCM
  • The birth trauma theory suggests that the SCM is damaged during delivery, which causes bleeding à fibrosis à contracture of the muscle
  • A familial tendency has been described

Clinical features:

  • Typically detectable by 2-4 weeks of age, once the child has developed some head control
  • The presenting sign may be a well-circumscribed mass on the involved SCM
  • Child will hold the head tilted to one side
  • Bilateral torticollis is rare
  • The right SCM is more commonly involved than the left

Physical exam:

  • Limited range of motion of the neck
  • The head tilts towards the shortened SCM and the chin rotates to the opposite side
  • A firm mass, or “pseudotumor,” may be palpable in the lower third of the affected SCM
  • In severe cases, facial asymmetry may be present, with a unilateral epicanthal fold or anterior displacement of the ear on the affected side
  • The “tumor” usually resolves spontaneously by 2-6 months of age

Associated anomalies:

  • 6% of children will have associated musculoskeletal abnormalities: hip dysplasia, metatarsus adductus, talipes equinovarus, C1-C2 subluxation
  • Facial asymmetry
  • Skull deformities (plagiocephaly) can result because the baby will repeatedly rest his/her head on one side

Diagnosis:

  • Usually a clinical diagnosis
  • Consider A-P and lateral plain films of the cervical spine to rule out C1-C2 subluxation and other associated congenital bony anomalies
  • Ultrasound of the SCM can confirm that the pseudotumor is of neuromuscular origin

Treatment:

  • May resolve spontaneously OR lead to craniofacial asymmetry if left untreated
  • The majority of cases resolve after a few months of stretching therapy and careful positioning:
    • Positioning:
      • Hold the child so he/she must rotate the chin towards the affected side while feeding
      • Position the child so that he/she must rotate the chin toward the affected side to see his/her toys or to view the room
    • Stretching exercises: for young babies, parents can do passive stretching exercises to gently stretch the shortened SCM; older babies (> 6-8 mo) are treated with active stretching exercises
    • A soft collar can be used as an adjunctive therapy once the child is 4-5 months old
    • The age at which children are referred to therapy correlates with successful conservative treatment (children who are referred earlier do better)
    • Most children require < 6 mo of therapy
  • Infants should be re-evaluated every 2-4 weeks. If home therapy doesn’t resolve the limited ROM in 4-6 weeks, outpatient PT is recommended
  • -Surgery is recommended when children have persistently limited ROM (loss of > 30 degrees of movement) of the neck, significant plagiocephaly, or facial asymmetry after 6 to 12 mo of age
    • Surgical outcomes are excellent overall
    • The severity of initial presentation is not a predictor of whether a child will develop persistent torticollis and need surgical intervention

Some rare malformations of the vertebrae can also cause congenital torticollis

Acquired Torticollis

  • Injury or inflammation from a range of conditions can cause spasms of cervical muscles or irritation of cervical nerves, leading to torticollis
  • In contrast to congenital torticollis, acquired torticollis is rarely associated with facial asymmetry

Etiology:

  • Underlying conditions range in severity from benign to potentially life-threatening
  • Most common causes: atlantoaxial subluxation, minor trauma, URI, cervical lymphadenitis, myositis, retropharyngeal abscess, superior oblique palsy
  • Less common causes include: benign paroxysmal torticollis, dystonic reaction, spasmus nutans, and Sandifer syndrome

Clinical Presentation:

  • Similar to that of congenital torticollis—the head is tilted toward the shortened SCM and the chin is rotated to the opposite side
  • The child is usually older in cases acquired congenital torticollis
  • Onset is usually acute

Evaluation of a child with acquired torticollis:

First step: Determine if the child needs immediate intervention. Torticollis in the setting of acute trauma should be treated with extreme caution, as there is potential for c-spine damage.

  • When trauma is suspected, immediately immobilize the c-spine
  • Emergent treatment is necessary for patients with torticollis, stridor and drooling, as these patients may have a retropharyngeal abscess or epiglottis and are in danger of losing their airway

History:

  • How old is the child?
  • Duration of the torticollis? When did it develop?
  • Acute or insidious onset?
  • History of trauma?
  • History of fever?
  • Recent head or neck infection?
  • Recent surgery to the head or neck?
  • Associated symptoms?
  • History of medication use?

Exam: Focus on the head, neck, eyes, upper respiratory tract, and nervous system

  • Assess active ROM of the neck
  • Test for signs of ocular involvement: corneal light reflex test, cover-uncover test, fundal exam
  • Examine the pharynx for signs of inflammation or abscess
  • Palpate for enlarged lymph nodes
  • Perform a full neuro exam, watching for signs of weakness, ataxia or sensory deficits

Some clues to etiology that may be present on exam include:

  • Craniofacial asymmetry: suggests congenital or long-standing torticollis
  • Short neck with low posterior hairline: suggests bony c-spine anomaly
  • Tenderness over the SCM suggests trauma or myositis
  • Point tenderness over the c-spine suggests underlying fracture, subluxation, or osteomyelitis

Imaging:

  • C-spine radiographs: required for all patients with persistent torticollis, pain or findings that suggest vertebral involvement
  • CT/MRI: helpful to determine the extent of certain lesions, to rule out atlantoaxial subluxation if plain films are equivocal, and to assess patients with neurologic deficits
  • Ultrasound and CT can be helpful in patients in whom retropharyngeal inflammation is suspected

Treatment: Varies depending on the underlying cause

  • Emergent causes (vertebral subluxation, retropharyngeal abscess) require subspecialty consult and urgent treatment
  • Torticollis caused by a musculoskeletal injury may respond to NSAIDs and a cervical collar. Children who do not respond to NSAIDs may respond to diazepam or muscle relaxants. Adults are treated with botulinum toxin injections, but there is little evidence to support the use of botox in children.
  • Antibiotic treatment is necessary for children with lymphadenitis or other infectious causes.
  • Torticollis caused by atlantoaxial subluxation depends on the severity of the abnormality (see below)
  • Ocular torticollis (see below) may respond to treatment of the underlying ocular abnormality
  • Dystonic reactions caused by drugs (see below) may respond to diazepam or diphenhydramine
  • Antireflux therapy is necessary for children with torticollis caused by GERD (see below)

In sum: most children with acquired torticollis have a minor muscle injury or muscular inflammation. Their symptoms typically resolve in a few days.

Please see below for more information about some specific causes of acquired torticollis

Atlantoaxial subluxation (AAS):

  • Rotational displacement of C1 on C2
  • An important cause of acquired torticollis in children
  • Non-traumatic atlantoaxial subluxation is called “Grisel Syndrome”

Etiology:

  • Believed to be caused by retropharyngeal edema à leads to laxity of the ligaments at the atlantoaxial level à enables rotation and deformity
  • Can follow trauma, surgery, inflammatory processes of the neck, and URI syndromes
  • The classic mechanism that leads to AAS is the head striking the windshield or dashboard during a motor vehicle accident

Clinical Features:

  • Occurs in children between 6 and 12 years old
  • Most kids present with pain and decreased ROM after minor trauma to the neck
  • A portion of the second cervical nerve runs through the atlantoaxial joint, so children may have pain in the affected dermatome (over the occiput)

Physical Exam:

  • The head tilts away from the affected SCM
  • The axis may be tender and displaced to the same side as the torticollis

Associated Anomalies:

  • Children with Down syndrome, Morquio Syndrome, Larsen syndrome, Marfan syndrome, neurofibromatosis, achondroplasia, ankylosing spondylitis, and cervical spine arthritis are prone to atlantoaxial instability

Diagnosis:

  • C-spine immobilization is appropriate, as there is often a history of trauma and risk of injury to the spine
  • Cervical spine plain films are necessary when there is a traumatic mechanism of injury; CT may be necessary to confirm the diagnosis
  • Orthopedics should be consulted when there is significant pain
  • Neurology should be consulted when there are signs of neurologic deficits

Treatment:

  • Treatment depends on the severity and duration of the abnormality
  • Acute, minor cases (< 1 week): soft cervical collar, rest and analgesics
  • Severe, prolonged cases: cervical traction, immobilization, and surgery may be necessary

Retropharyngeal Abscess

  • A potentially life-threatening cause of torticollis

Clinical features:

  • Most common between 2-4 years old
  • Refusal to move the neck because of discomfort is the key presenting feature

Physical Exam:

  • Fever
  • Irritability
  • Dysphagia, odynophagia
  • Drooling, inability to fully open the mouth
  • Respiratory distress (stridor, tachypnea)

Diagnosis:

  • Lateral neck radiographs
  • CT

Treatment:

  • Immediate ENT consult
  • Emergent surgical drainage if the airway is compromised
  • Antibiotic therapy +/- surgery if the airway is intact

Ocular Torticollis:

  • Children with disorders of eye mobility or alignment (strabismus) may tilt their heads to avoid seeing double.

Diagnosis:

  • Careful eye exam, with corneal light reflex and cover-uncover tests
  • Optho referral

Treatment:

  • Involves correcting the underlying ocular abnormality

Benign Paroxysmal Torticollis

  • Recurrent episodes of head tilt that are often accompanied by vomiting, pallor, irritability, ataxia, or drowsiness

Etiology:

  • The pathogenesis is unknown
  • Considered to be a “migraine equivalent” disorder
  • Some patients later develop migraines or benign paroxysmal vertigo

Clinical features:

  • Usually presents in the first few months of life
  • The episodes of torticollis are periodic and can occur on either side (often alternate randomly)
  • Attacks tend to last for a few hours, but can occasionally last for days

Physical Exam:

  • Torticollis is accompanied by vomiting, pallor, irritability, ataxia, or drowsiness

Diagnosis:

  • Diagnosis is made based on careful history and physical exam
  • Since the clinical findings can mimic more serious illnesses (brain tumors, seizures), an EEG or neuroimaging may be necessary to rule out such disorders
  • Attacks are frequently filmed by family members to aid in diagnosis

Treatment:

  • Benign paroxysmal torticollis is a self-limited condition
  • Episodes occur less frequently with time, and tend to disappear in most cases by 5 years of age

Dystonic Reaction

  • A dystonic reaction is an acute spasm of muscle groups characterized by fixed upper gaze, neck twisting, grimace, clenched jaw, and difficulty with speech
  • Torticollis can occur as part of a dystonic reaction to certain medications (phenothiazines, carbamazepine, phenytoin, etc.)
  • Episodes are often painful and scary for the patient
  • Drug-induced dystonic reactions can be treated with diphenhydramine or diazepam

Sandifer Syndrome

  • Sandifer syndrome consists of gastroesophageal reflux with torsion spasms of the neck and abnormal posturing
  • In these patients, torticollis occurs intermittently and alternates sides
  • Additional symptoms may include other complications of GERD: regurgitation, anorexia, hematemesis, chronic cough, failure to thrive, hoarseness, and irritability
  • Treatment is anti-reflux therapy

Spasmus Nutans

  • A triad of acquired nystagmus, head nodding and torticollis (torticollis is not always present)
  • Onset tends to occur between 6 and 12 months of age, but it can also present in children who are several years old
  • This is a rare disorder; it was more common at the turn of the century
  • Most cases are transient

Other Rare Causes of Acquired Torticollis

Intervertebral disc calcification, benign tumors of the c-spine (osteoid osteoma), bony abnormalities of the c-spine (occipitocervical synostosis), spondylitis, AV malformations, and brain tumors

References

  1. Rapson A, and Rappaport D. Index of suspicion. Pediatr Rev 2007; 28: 343.
  2. Robin N. Congenital muscular torticollis. Pediatr Rev 1996; 17: 374.
  3. Tom, LW, Rossiter, JL, Sutton LN, et al. Torticollis in children. Otolaryngol Head Neck Surg 1991; 105: 1.
  4. UptoDate articles referenced: Macias and Gan, Acquired Torticollis in Children and Congenital Muscular Torticollis.
  5. Updated by Sarah Todd, MS3 (3-22-2012)