Basics
Description
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A limitation of motion of the cervical spine that causes the head to be held in a tilted position
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May result from muscular, skeletal, or neurologic abnormalities (Fig. 1)
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Classification :
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Congenital abnormalities
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Acquired abnormalities
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Synonyms: Skeletal wry neck; Congenital wry neck; Cock-robin deformity; Sandifer syndrome (torticollis resulting from gastroesophageal reflux disease and hiatal hernia)
General Prevention
The condition cannot be prevented, but prompt referral and treatment may preclude the need for surgery.
Epidemiology
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The most common cause is rotatory subluxation of the atlantoaxial joint, an acquired condition.
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Congenital muscular torticollis usually is evident in the first 6-8 weeks of life.
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Other causes may appear throughout childhood or may become evident well into adulthood.
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Males and females are affected equally.
Incidence
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Because of the multiple causes, it is difficult to give a specific figure for incidence.
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It affects an estimated 1 per 100 to 1 per 1,000 patients.
Fig. 1. Torticollis typically produces lateral flexion to 1 side and rotation to the other.
Risk Factors
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Local trauma to the infant’s neck during delivery, especially during a difficult delivery
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For atlantoaxial rotatory subluxation: Upper respiratory infection, pharyngitis, or trauma
Genetics
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Multiple congenital causes of torticollis exist, of which a few have a genetic predisposition.
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Skeletal dysplasias are the most common genetic syndromes associated with torticollis.
Etiology
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Congenital muscular torticollis is caused by contracture of the sternocleidomastoid muscle.
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Congenital bony torticollis may be secondary to occipitocervical abnormalities.
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Acquired torticollis may result from neurogenic, traumatic, inflammatory, or idiopathic causes (see Differential Diagnosis).
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Bony abnormalities: Atlanto-occipital synostosis, basilar impression, odontoid abnormalities, cervical hemivertebrae, or asymmetry of occipital condyles
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Atlantoaxial rotatory subluxation, the most common bony abnormality, is characterized by rotatory displacement of C1 on C2 and may be congenital or secondary to inflammation or trauma.
DiagnosisSigns and Symptoms
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Hallmark sign: Tilting of the head to 1 side with limitation of ROM
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Usually the patient rotates the head away from the neutral (straight) position, but not toward it.
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Patients may present with a neck mass (contracted sternocleidomastoid muscle).
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Neck pain is a common complaint, but it usually occurs in adults.
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Patients also may complain of occipital pain, vertigo, or dizziness aggravated by certain movements of the head.
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If torticollis persists beyond infancy, secondary asymmetry of the cranium (plagiocephaly) may remain.
Physical Exam
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The patient’s head is tilted, with the ear toward the involved side and the chin rotated away, with limitation of ROM toward the corrected position.
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In some cases of muscular torticollis, a palpable mass may be present on the involved side (contracted sternocleidomastoid muscle).
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Remodeling of the head or face may result from pressure while sleeping.
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A short, broad neck with a low hairline may be seen in patients with bony abnormalities or Klippel-Feil syndrome.
Tests
Lab
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No specific laboratory tests, unless an inflammatory or neoplastic origin is being considered
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Ophthalmologic, audiologic, and gastroenterologic evaluations sometimes are needed if no obvious skeletal causes are seen.
Imaging
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AP and lateral radiographs of the cervical spine should be obtained for any patient with torticollis to identify bony abnormalities.
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CT is used to evaluate rotatory subluxation, dislocation, or fracture.
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MRI is used if a neurologic lesion of the brainstem or neck is suspected.
Pathological Findings
In congenital muscular torticollis, the sternocleidomastoid muscle is fibrotic, replaced by scar tissue in a nonspecific fashion.
Differential Diagnosis
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Neurogenic causes:
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Spinal cord tumors of the cervical spine
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Cerebellar tumors
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Syringomyelia
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Ocular dysfunction
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Traumatic causes:
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Subluxations
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Fractures and dislocations of the occipitocervical junction
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Inflammatory causes:
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Cervical adenitis
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Rheumatoid arthritis
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Idiopathic causes:
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Atlantoaxial rotatory subluxation or displacement
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TreatmentGeneral Measures
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Congenital muscular torticollis:
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Responds to stretching exercises in nearly 100% of patients treated before 1 year of age
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Positioning of toys in the crib will encourage the child to stretch the involved side.
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Atlantoaxial rotatory subluxation:
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Patients usually recover with physical therapy if the condition is detected within the 1st week of onset.
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Use of a soft collar and analgesics is for patients with atlantoaxial rotatory subluxation patients.
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Patients with recalcitrant cases may require muscle relaxants and a hard collar or brace.
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If treatment is delayed, traction or even surgery is required.
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P.469
Activity
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Contact sports and vigorous athletics should be restricted until the condition has been treated.
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Specifics depend on the underlying cause.
Special Therapy
Physical Therapy
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Stretching exercises may be beneficial in patients with muscular torticollis or recent-onset rotatory subluxation.
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Specific instructions should be given to the therapist.
Medication
Analgesics (acetaminophen, ibuprofen)
Surgery
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Congenital muscular torticollis that is refractory to stretching may require release of the sternocleidomastoid muscle.
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Severe atlantoaxial rotatory subluxation or other severe bony abnormality may require fusion of C1 and C2.
Follow-upPrognosis
Most cases resolve spontaneously or with treatment.
Complications
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Fixed subluxation
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Plagiocephaly (in late-treated muscular torticollis)
Patient Monitoring
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Neurologic status should be followed closely.
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Bony abnormalities, such as rotatory subluxation, may require repeated CT scans.
MiscellaneousCodes
ICD9-CM
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754.1 Congenital torticollis
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847.0 Traumatic torticollis
Patient Teaching
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Once the cause is known, anatomic models may be used to explain the cause of the torticollis to patients and families.
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Patients and families should be made aware of the usual course of the condition and the possible need for different methods of therapy.
FAQ
Q: When is congenital torticollis usually 1st evident clinically?
A: In the first 6-8 weeks of life.
Q: What does a neck mass in a patient with torticollis often represent?
A: A contracted sternocleidomastoid muscle. However, additional evaluation may be required in some cases to rule out other causes.
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