Introduction
Background
Torticollis (from the Latin torti, meaning twisted and collis, meaning neck) manifests as involuntary contractions of the neck muscles, leading to abnormal postures and movements of the head.1 It is not a diagnosis but a sign of an underlying disease process. Cervical dystonia, spasmodic torticollis, torsion dystonia, and acute wryneck all refer to the same process. Most of the cases presenting to the emergency department present as acute, acquired torticollis, although other congenital and infectious causes as well as trauma must be considered.
A 69-year-old woman presents with torticollis and a fever.
Pathophysiology
Torticollis is a symptom with a host of underlying pathologies and can be divided into congenital and acquired causes.
Congenital torticollis
Congenital torticollis is rare and usually not encountered in the ED. The most common form of congenital torticollis is congenital muscular torticollis. The cause is unknown, but theorized that the sternocleidomastoid (SCM) muscle is injured during birth trauma, resulting in fibrosis or that intrauterine malpositioning leads to unilateral shortening of the SCM.2 The incidence is less than 2% and is more common in breech presentations. Other causes of congenital torticollis include postural torticollis, pterygium colli (webbed neck), SCM cysts, vertebral anomalies, odontoid hyperplasia, spina bifida, hypertrophy or absence of cervical musculature, and Arnold-Chiari syndrome. It can also be seen with clavicular fractures, especially in neonates secondary to birth trauma.
Acquired torticollis
The pathophysiology of acquired torticollis depends on the underlying disease process. Cervical muscle spasm causing torticollis can result from any injury or inflammation of the cervical muscles or cranial nerves from different disease processes.
Patients most frequently present to the ED with torticollis unprovoked or after sleeping in an awkward position. Acute torticollis develops overnight and results in painful, palpable neck spasms the following morning. Symptoms usually resolve spontaneously within a few days lasting no more than 1-2 weeks. It is treated with conservative, symptomatic management like analgesics, massage, exercise, and stretching.
Any abnormality or trauma of the cervical spine can present with torticollis. Trauma, including minor trauma (sprains/strains), fractures, dislocations, and subluxations, often result in spasms of cervical musculature. Spinal epidural hematomas are a potential life-threatening cause to consider. Torticollis rarely also presents secondary to intervertebral disk calcifications, cervical spine tumors, spondylitis, arteriovenous malformations, and other bony abnormalities.
Upper respiratory and soft tissue infections of the neck can cause an inflammatory torticollis secondary to muscle contracture or adenitis. Torticollis has been associated with retropharyngeal abscesses and is important to consider because it is potentially life-threatening.3,4 It is most commonly seen in children aged 2-4, but the incidence in adults is increasing. Patients typically present with neck discomfort, fever, stridor, dysphagia, drooling, odynophagia, and respiratory distress.
Other causes can be secondary to any infection involving surrounding tissue or structures of the neck including pharyngitis, tonsillitis, epiglottitis, sinusitis, otitis media, mastoiditis, myositis, tuberculosis, nasopharyngeal abscess, upper respiratory tract infections, and upper lobe pneumonias.
Soft-tissue neck radiograph demonstrates retropharyngeal abscess appearing as torticollis.
Torticollis often presents as a dystonic reaction secondary to medications including phenothiazines, metoclopramide, haloperidol, carbamazepine, and phenytoin. Dystonic reactions cause acute muscle spasms of certain muscle groups often resulting in torticollis, trismus, fixed upper gaze, grimace, clenched jaw, and difficulty with speech. It is treated with diphenhydramine or benzodiazepines.
Torticollis is also often seen as compensatory mechanism for another disease or symptoms. Patients present with a head tilt to compensate for an essential head tremor or for diplopia secondary to an ocular muscle or nerve palsy. Pediatric patients need a thorough eye examination to rule out a cranial nerve palsy or congenital nystagmus.
Benign paroxysmal torticollis is a self-limited condition common in infants characterized by repetitive episodes of head tilting with vomiting, pallor, irritability, ataxia, or drowsiness and usually presents in the first few months of life. Episodes can alternate sides. It is thought to be a migraine equivalent disorder.
Sandifer syndrome is a term used to describe gastroesophageal reflux with abnormal posturing including torticollis in infants. Torticollis occurs intermittently and can alternate sides. Other symptoms of reflux may be present including regurgitation, anorexia, irritability, anemia, failure to thrive, coughing, asthma, and hoarseness. Treatment is antireflux therapy.
Atlantoaxial rotary subluxation (AARS) of C1 on C2 is important to consider and leads to a presentation similar to torticollis. It is predominant in children and generally occurs after minor trauma, pharyngeal surgery, an inflammatory process, or upper respiratory tract infection. It is thought to be precipitated by retropharyngeal edema leading to laxity of ligaments and structures at the atlantoaxial level, permitting the rotational deformity. In contrast to congenital muscular torticollis, the head tilts away from the affected SCM muscle. Known as the "cock robin" position, the head is rotated to the side opposite the facet dislocation and laterally flexed in the opposite direction.5 Patients may also complain of unilateral occipital pain. AARS can also be a result of torticollis, though extremely rare.Idiopathic spasmodic torticollis (IST) is a chronic, progressive form of torticollis classified as a focal dystonia. The etiology is unclear, although a thalamic lesion has been suspected. It is characterized by having an acquired, nontraumatic origin consisting of episodic tonic and/or clonic involuntary contractions of neck muscles. Symptoms last more than 6 months and result in considerable somatic and psychological disability.
Clinical
History
- Patients with congential muscular torticollis often have a palpable soft tissue mass in the sternocleidomastoid (SCM) muscle shortly after birth. The mass later subsides, leaving a contracted SCM muscle. The head characteristically tilts toward the side of the mass with the chin rotated in the opposite direction.
- Patients with acquired torticollis present similarly with the head twisted to the affected side and the chin pointed toward the opposite shoulder. Patients complain of pain, neck stiffness, and decreased range of motion. Onset is generally acute. A thorough history and physical examination is necessary to identify treatable causes.
- The presence of craniofacial asymmetry indicates congenital or long-standing torticollis.
- Patients with traumatic torticollis should be immobilized. Midline cervical tenderness suggests cervical spine trauma or osteomyelitis. In other cases, active and passive range of motion should be evaluated.
- The posterior pharynx should be examined for signs of inflammation and infection. The neck should be palpated for masses, adenopathy, or focal tenderness. A complete neurological examination should be performed, including strength testing, sensory deficits, and gait.
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Further Reading
Keywords
torticollis, idiopathic spasmodic torticollis, IST, involuntary contractions of neck muscles, focal dystonia, congenital torticollis, birth trauma, acquired torticollis, acute wryneck, painful neck spasms, cervical muscle spasm, tonic head deviation, clonic head movements, head torsion, spasmodic torticollis, cervical osteomyelitis
