Torticollis Treatment & Management

Updated: Oct 22, 2018
  • Author: Michael C Kruer, MD; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Approach Considerations

A comprehensive approach to the medical care of torticollis includes several treatment goals. All underlying reversible causes of torticollis should be explored and treated appropriately.

Medications include nonsteroidal anti-inflammatory drugs (NSAIDs), benzodiazepines and other muscle relaxants, anticholinergics, and local intramuscular injections of botulinum toxin, [17, 34, 35, 36] or phenol. Physical therapy includes stretching exercises, massage, local heat, analgesics, sensory biofeedback, and transcutaneous electrical nerve stimulation (TENS). [37]

When conservative treatment measures fail, patients may undergo brain stimulation procedures, a sternocleidomastoid release, selective denervation, or dorsal cord stimulation. Surgical therapy may consist of the following:

  • Unipolar sternocleidomastoid release

  • Bipolar sternocleidomastoid release

  • Selective denervation

  • Dorsal cord stimulation

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Conservative Management

In torticollis, the conventional dopamine agonists and antagonists are not effective (dopamine receptors D1=D2), as distinguished from the L-dopa–responsive dystonias (a set of inherited generalized dystonias). Anticholinergics (eg, trihexyphenidyl, benztropine) may be somewhat effective but are typically less so than in generalized or torsion dystonias.

Try unconventional dopamine agonists (dopamine receptors D2 >D1, such as pramipexole or ropinirole) or antagonists with D2, D3, D4 blocking activity, such as olanzapine or risperidone, or try glutamate release inhibitors (eg, riluzole) or glutamate receptor blockers (eg, high-dose amantadine, lamotrigine, memantine).

Botulinum toxin injection is the current popular treatment of choice. Selective medication choices may include clonazepam, especially if blepharospasm is part of the syndrome, or baclofen, especially if oromandibular dystonia is part of the syndrome.

Nonspecific medication choices include propranolol or primidone if prominent oscillatory components, or nonsteroidal anti-inflammatory drugs (NSAIDs) for arthritic components.

Patients may respond well to sensory feedback training. Muscle relaxation techniques may be tried. Cervical braces occasionally are made that reproduce the tactile advantage of a sensory trick, which controls movement and/or reduces dystonia.

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Surgical Intervention

Typically, surgical care has been tried as a last resort for patients whose symptoms are refractory to botulinum injections; however, advances in brain stimulation technology and popular use has encouraged US Food and Drug Administration (FDA) approval for stimulator use, especially in the subthalamic nucleus (STN), as follows:

  • Selective ramisectomy for cervical musculature (late delayed recurrence can be a problem)

  • Deep brain stimulator electrode implants in the globus pallidus (GP) or the STN to control contralateral dystonia

Other procedures such as sternocleidomastoid release (unipolar/bipolar), selective denervation, or dorsal cord stimulation may be indicated. Preoperative electromyography (EMG) may be helpful in defining the exact muscles and nerves involved.

Surgery is contraindicated in patients in whom underlying reversible causes have not been excluded and in those in whom conservative therapy has not been attempted. In congenital muscular torticollis, a trial of nonoperative treatment for 12-24 months is allowed before surgical intervention is pursued, because 90% of these patients respond to passive stretching within the first year of life. [38]

Stereotactic neurosurgery

Stereotactic neurosurgery using brain stimulator technology is now a subspecialty that was originally confined to conditions of Parkinson disease (STN or GP placement) and non-Parkinsonian tremor (ventrointermediated thalamic nucleus [VIM] or STN placement) but has been expanded to include FDA-approved brain stimulator applications for dystonia (STN or GP placement) and experimental adaptations to Tourette syndrome (STN or GP placement), obsessive-convulsive disorder (OCD) and major affective disorders (cingulate placement), and phantom pain.

Torticollis involves cervical dystonia and, as such, qualifies as a candidate for consideration of treatment with deep brain stimulation. Now FDA approved, deep brain stimulation (DBS) [39] should be considered a competitive option to botulinum injections in consultation with a stereotactic neurosurgeon familiar with this application.

Stereotactic procedures targeting the pallidofugal fibers or Forel field have not had results encouraging enough for them to become widely accepted.

Sternocleidomastoid release

Sternocleidomastoid muscle release is often used in congenital muscular torticollis. For mild deformity, unipolar release of the muscle is performed distally. For moderate and severe torticollis, bipolar technique is used to release the muscle proximally and distally.

Although sternocleidomastoid release is described mainly for congenital torticollis, it may also be used in the other forms as well. Some patients may ultimately require a combination of several different surgical procedures for correction of torticollis.

Unipolar sternocleidomastoid release for congenital muscular torticollis

Make an incision 5 cm long just superior and parallel to the medial end of the clavicle and to the depth of the tendons of the sternal and clavicular attachments of the sternocleidomastoid muscle. Incise the tendon sheath longitudinally and pass a hemostat or other blunt instrument posterior to the tendons. Next, using traction on the hemostat, draw the tendons outside the wound and then superior and inferior to the hemostat; clamp them and resect 2.5 cm of their inferior ends. If contracted, divide the platysma muscle and adjacent fascia. Next, with the patient's head turned toward the affected side and the chin depressed, explore the wound digitally for any remaining bands of contracted muscle or fascia and, if any are found, divide them under direct vision until the deformity can, if possible, be overcorrected.

If overcorrection is not possible after this procedure, make a small transverse incision inferior to the mastoid process and carefully divide the muscle near the bone. Take care to avoid damaging the spinal accessory nerve. Close the wound or wounds and apply a bulky dressing that holds the head in the overcorrected position.

Bipolar sternocleidomastoid release

The bipolar sternocleidomastoid release, as described by Ferkel et al, for congenital muscular torticollis involves making a short transverse proximal incision behind the ear and dividing the sternocleidomastoid muscle insertion transversely just distal to the tip of the mastoid process. [7, 40] With this limited incision, the spinal accessory nerve is avoided, although the possibility that the nerve may take an anomalous route should be considered. Next, make a distal incision 4-5 cm long in line with the cervical skin creases 1 fingerbreadth proximal to the medial end of the clavicle and the sternal notch. Divide the subcutaneous tissue and platysma muscle, exposing the clavicular and sternal attachments of the sternocleidomastoid muscle. Carefully avoid the anterior and external jugular veins and the carotid vessels and sheath during the dissection.

Next, cut the clavicular portion of the muscle transversely and perform a Z-plasty on the sternal attachment in order to preserve the normal V-contour of the sternocleidomastoid muscle in the neckline. Obtain the desired degree of correction by manipulating the head and neck during the release. Occasionally, release of additional contracted bands of fascia or muscle is necessary before closure. Close both wounds with subcuticular sutures.

Selective denervation

Selective denervation is primarily used in the treatment of torticollis, and varying success rates have been reported since its introduction in the early 1980s by Claude Bertrand, MD, and his colleagues in Montreal, Canada. [41, 42, 43] Denervation involves resecting the nerves that supply the specific muscles involved and is irreversible. Because of this, an EMG is sometimes performed to correctly identify all muscles involved before the procedure.

Selective denervation using the Bertrand method involves dissection through fascial planes to expose and section the posterior primary rami throughout all cervical levels. Preoperative EMG isolates the exact muscles involved and their nerve supply, and only the involved segments are denervated. Once the nerve supply has been cut, the associated muscles will atrophy permanently.

Dorsal cord stimulation

In dorsal cord stimulation, the electrodes are inserted into the subarachnoid space laterally at the C1-C2 level, with a monopolar electrode threaded down to the C4-C5 level for a 7-10–day trial of stimulation. About two thirds of the patients have improvement in their symptoms, and most patients respond best to higher frequencies between 1100 and 1500 Hz. Patients who have significant relief and tolerate stimulation are considered candidates for permanent dorsal column stimulator electrode implantation. The epidural electrode is placed midline at the C1-C2 level and sutured in place so that it cannot become dislodged with neck movement.

Surgical complications

As with any surgical procedure within the calvarium, the possibility of bleeding or infection exists, but in the hands of a subspecialty trained stereotactic neurosurgeon, this is less than 2-3%. Previously, electrode displacement was a risk due to translational forces, but this problem has been overcome by proper technical advance in mechanical stabilization. Other complications include injury to spinal accessory nerve or nearby vasculature (including the jugular veins and carotid artery), neck muscle atrophy, loss of muscle control, instability, variable numbness or sensory loss, pain, and neck deformity.

Certain neuropsychiatric conditions may occur in a minority of postsurgical cases but are typically corrected by recalibration of impulse parameters (pulse width, frequency, and amplitude). These behavioral side effects include visual hallucinations, obsessive gambling, hypersexuality, and depression. This same set of issues occurs with medication adjustment in anti-Parkinson medications.

Electromagnetic field precautions post surgery

Patients must be wary of any interaction with major electromagnetic fields associated with electrical generators in industrial applications, field detectors used in library screening to prevent book stealing, and metal detectors in general. Preflight check-in to airlines or other security checkpoints should avoid electromagnetic probes or wands that can turn off the deep brain stimulator (DBS). Nevertheless, the patient has a handheld magnetic trigger and can either turn on or off the pacemaker controller.

Magnetic resonance imaging (MRI) has special considerations because of massive fluctuations of magnetic fields that can cause the generator to cycle on and off. Before scanning, the pacemaker should be turned off (the patient can do this), and the amplitude setting of the controller should be set to zero (done by a physician or technician with special interrogator needs). Recycling at zero amplitude is not problematic.

Similar issues occur if electroconvulsive therapy is anticipated. When electric cardioversion is needed in a cardiopulmonary resuscitation (CPR) emergency, postsurvival adjustments can be made to maximize motor performance and the status of the DBS being on or off should not detract from needed lifesaving measures.

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Long-Term Monitoring

Regular outpatient visits are needed for routine medication checkups, repeat botulinum toxin injections, or recalibration of deep brain stimulation settings.

For unipolar sternocleidomastoid release, physical therapy that includes manual stretching of the neck to maintain the overcorrected position is begun 1 week after surgery. Manual stretching should be continued 3 times daily for 3-6 months. The use of plaster casts or braces is usually unnecessary.

For bipolar sternocleidomastoid release, physical therapy involving range of motion and muscle stretching and strengthening is started early. A cervical collar may be used for the first 6-12 weeks after surgery.

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