Torticollis

Introduction

Torticollis is the term used to describe the clinical findings of tilting (lateral bending) of the head/neck to the right or left side in combination with rotation of the head/neck to the opposite side. Torticollis is not a diagnosis, but rather a manifestation of a variety of underlying conditions. Some of the conditions that should be considered in the differential diagnoses are listed below.

Anatomy

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Pathogeneis

Most cases discovered at or near the time of birth represent congenital muscular torticollis. Although the etiology is unknown, this deformity is felt to result from abnormal positioning in utero, and involves contracture of the sternocleidomastoid. Recent information from both muscle biopsies and MRI scans have led to the speculation that congenital muscular torticollis may be caused by an intramuscular compartment syndrome. Intrauterine muscle injury from compression and/or stretch may create localized ischemia, which results in fibrosis and contracture. Familial basis for torticollis as well as hereditary muscle aplasia have been reported. A contracture of the left sternocleidomastoid muscle results in tilt of the head to the left and rotation to the right, and vice versa. 

Patient History and Physical Findings

Congenital muscular torticollis is defined by the presence of a palpable mass (fibrous tissue) within the substance of the sternocleidomastoid muscle. However, this finding is present in approximately half of the patients. The mass disappears during infancy, and is replaced by a fibrous band. Associated findings include plagiocephaly and facial asymmetry, both of which usually resolve with restoration of cervical motion. Patients may have associated positional musculoskeletal deformities such as metatarsus adductus and calcaneovalgus feet. While the older literature suggested that up to 20% of patients also have dysplasia of the hip, more recent studies suggest that this figure is 5-8%.

The evaluation of torticollis becomes more complex when the typical findings associated with CMT are absent (mass and/or contracture within the sternocleidomastoid), the usual clinical response is not observed, or the deformity presents at a later age. In such cases, a careful history and physical examination is required, and often a consultation with an opthalmologist and/or neurologist will be helpful. Plain radiographs should be obtained, and an MRI of the brain and cervical spine will be required in a subset of cases. The differential diagnosis is large.

Imaging and other Diagnostic Studies

While standards for screening in patients with a normal clinical examination have not been established, consideration should be given to obtaining either an ultrasound (1 month of age), or a plain radiograph of the hip (4-5 months of age). Torticollis in this age range may also result from congenital vertebral anomalies, and an AP and lateral radiograph of the cervical spine is indicated when the typical clinical features associated with congenital muscular torticollis are absent, or if the deformity does not respond to treatment.

Differential Diagnosis

Congenital

• Congential muscular torticollis
• Positional deformation
• Klippel-Feil syndrome
• Unilateral absence of sternocleidomastoid
• Hemivertebra (cervical spine)
• Unilateral atlanto-occipital fusion
• Pterygium colli

Trauma

• Muscular injury (cervical muscles)
• Atlanto-occipital subluxation
• Atlantoaxial subluxation
• C2-3 subluxation
• Rotary subluxation
• Fractures

Inflammation / Infection

• Cervical lymphadenitis
• Retropharyngeal abscess
• Cervical vertebral osteomyelitis
• Rheumatoid arthritis
• Spontaneous (hyperemia, edema) subluxation with adjacent head and neck infection (rotary subluxation syndrome)
• Upper lobe pneumonia

Neurogenic

• Visual disturbances (nystagmus, superior oblique paresis)
• Dystonic drug reactions (phenothiazines, haloperidol, metoclopramide)
• Cervical cord tumor
• Posterior fossa brain tumor
• Syringomyelia
• Wilson disease
• Dystonia musculorum deformans
• Spasmus nutans

Miscellaneous

• Acute cervical disk calcification
• Sandifer syndrome (gastroesophageal reflux, hiatal hernia)
• Hysteria
• Benign paroxysmal torticollis
• Bone tumors (eosinophilic granuloma)
• Soft tissue tumor

Treatment

A stretching program should be successful in more than 90% of patients, especially when treatment is started within the first 3 months of life. For patients diagnosed late, or those in whom the stretching program has failed to correct the deformity, surgical release of the sternocleidomastoid may be considered.  The trend has been to delay surgical intervention until the child approaches school age, and several surgical techniques have been described. Surgical management results in adequate function and acceptable cosmesis in more than 90% of patients. With early diagnosis and treatment, surgery should be required in a minority of cases.

Atlantoaxial rotatory displacement refers to rotational malalignment between the atlas and the axis, which may vary from mild subluxation to complete dislocation. Although the deformity is initially flexible, within several weeks it may become fixed (irreducible).  As such, prompt diagnosis and treatment are essential. The condition is most often secondary to infection/inflammation of the tissues of the upper airway, neck, and/or pharynx (Grisel's disease).

Traumatic injuries, usually minor, may also lead to the development of rotatory displacement. This condition will occasionally complicate surgical procedures in the oropharynx, ear, or nose. Rotational malalignment at this joint is best evaluated with a CT scan, in which axial images are obtained through the upper cervical spine in different positions (right and left rotation). This study not only establishes the diagnosis, but also determines whether the displacement can be reduced passively. A "fixed" displacement persists with the head in different positions. If the patient is seen within a few days of the onset of symptoms, then a trial of analgesics and a soft collar may be attempted. Patients with symptoms for more than a week are often admitted to the hospital for analgesia, muscle relaxants, and a period of cervical traction. If this fails to reduce the displacement, than halo traction may be attempted. If the joint can be reduced, patients are typically immobilized for at least 6 weeks in a halo vest. Patients with a fixed deformity may require a posterior atlantoaxial fusion to stabilize the articulation.

Neurogenic torticollis is uncommon rare and results from tumors of the posterior fossa or brainstem, syringomyelia, and Arnold-Chiari malformation. In addition to the neurologic examination, an MRI of the brain and cervical spine is required to establish the diagnosis.

Paroxysmal torticollis of infancy is also quite rare, and is felt to result from vestibular dysfunction. Episodes may last from minutes to days, and the side of the deformity may alternate.  The condition is self limited, and no specific treatment is required other than ruling out other treatable diagnoses.

Torticollis may also be seen in association with discitis or vertebral osteomyelitis, juvenile rheumatoid arthritis, cervical disc calcification, visual problems (strabismus due to paralysis of the extraocular muscles), tumor like conditions, and in patients with cerebral palsy and chronic gastroesophageal reflux (Sandifer's syndrome).

Pearls and Pitfalls

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Postoperative Care

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Outcome

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Complications

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Selected References

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Related topics

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Useful Internet Resources

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