Q. What is torticollis?

 

Torticollis is an abnormal, involuntary positioning of the head and neck caused by unilateral contraction or dysfunction of the cervical muscles, most commonly the sternocleidomastoid. It results in the head tilting to one side and rotating to the opposite side. It may be congenital, acquired, or secondary to neuromuscular, ocular, skeletal, or dystonic causes.

 

Q. What are the types of torticollis?

 

Torticollis is broadly divided into congenital muscular torticollis and acquired torticollis. 

Congenital muscular torticollis usually results from fibrosis or shortening of the sternocleidomastoid, often associated with birth trauma, intrauterine malposition, or associated musculoskeletal anomalies. 

Acquired torticollis may arise from atlantoaxial subluxation, posterior fossa pathology, Chiari malformation, syringomyelia, spinal tumors, cervical infections, dystonia, ocular imbalance, drug-induced dystonia, or inflammatory conditions of the cervical musculature.

 

Q. What is the pathophysiology of congenital muscular torticollis?

 

Congenital muscular torticollis results from fibrosis or shortening of the sternocleidomastoid because the injured muscle undergoes scarring after perinatal trauma or intrauterine compression, and the resulting contracture pulls the head into ipsilateral tilt with contralateral rotation. It may also be associated with intrauterine malposition because constant asymmetric pressure on the fetal neck leads to muscle ischemia and subsequent fibrosis. Musculoskeletal anomalies may contribute by altering neck alignment and creating compensatory muscle imbalance that fixes the head posture.

 

Q. How is congenital muscular torticollis classified, and why is this important?

 

Congenital muscular torticollis is classified into three subtypes: the sternocleidomastoid mass type, the muscular torticollis without mass type, and the postural torticollis type. 

The sternocleidomastoid mass type presents with a firm, fusiform enlargement of the muscle in infancy and represents the most fibrotic subtype, often requiring longer physiotherapy and sometimes earlier surgical intervention. 

The muscular torticollis without mass type has no discrete swelling but still demonstrates true muscle tightness, usually responding well to therapy. 

Postural torticollis is the mildest subtype, characterized by positional preference without true fibrosis, and resolves quickly with repositioning and stretching.

 

Q. What causes acquired torticollis?

 

Acquired torticollis arises from multiple structural or functional disturbances. 

Atlantoaxial subluxation causes torticollis because rotational or lateral displacement of C1 on C2 forces the head into a fixed tilted posture to maintain comfort and avoid pain from ligamentous strain. 

Posterior fossa pathology, including Chiari malformation, causes abnormal head posture because tonsillar descent and cervicomedullary crowding preferentially increase tone or spasm in one group of cervical muscles, leading to asymmetric neck positioning. 

Syringomyelia produces torticollis when the expanding syrinx distorts spinal cord pathways regulating neck muscle tone, causing imbalance in motor output. 

 

Cervical or spinal tumors cause unilateral muscle spasm or nerve root irritation, producing protective head tilt to reduce discomfort.

Cervical infections, such as in the paraspinal soft tissues or the retropharyngeal space, lead to torticollis because inflammation and pain trigger reflex muscle spasm that locks the neck in the least painful position. 

Dystonia causes torticollis due to basal ganglia dysfunction that generates involuntary, asymmetric contraction of cervical muscles. Drug-induced dystonia, particularly from dopamine-blocking agents, causes sustained involuntary contraction of neck muscles due to acute extrapyramidal dysfunction, leading to abnormal head positioning. 

 

Ocular imbalance results in torticollis because the patient positions the head to optimize visual alignment and reduce diplopia, and the habitual compensatory posture becomes fixed over time. 

Finally, inflammatory conditions of the cervical musculature cause focal pain and reflex spasm, and the resulting contracture forces the neck into a position that minimizes pain, producing torticollis.

 

Q. What is ocular torticollis, and why is it significant?

 

Ocular torticollis refers to abnormal head posture adopted to compensate for impaired ocular alignment, most commonly superior oblique palsy, horizontal muscle imbalance, or vertical strabismus. The child tilts or rotates the head to maintain binocular vision and reduce diplopia, and because this compensatory posture becomes habitual, it mimics cervical torticollis. The sternocleidomastoid is often normal on palpation and full passive range of motion is preserved. 

 

Recognizing ocular torticollis is critical because neck surgery or aggressive physiotherapy would not correct the underlying issue, and the head tilt resolves only when the ocular misalignment is managed by an ophthalmologist. Missing this diagnosis may expose the child to unnecessary orthopaedic or neurosurgical intervention.

 

Q. What is Grisel’s syndrome, and why is it an important cause of torticollis?

 

Grisel’s syndrome is a non-traumatic atlantoaxial rotatory subluxation resulting from inflammatory laxity of the transverse and alar ligaments following upper airway infection or ENT surgery, most commonly adenotonsillectomy. The inflammatory process spreads to the atlantoaxial joint through the pharyngovertebral venous plexus, leading to ligamentous weakening and subsequent subluxation. Children present with acute painful torticollis, limited rotation, and resistance to passive correction. It is an important cause because early recognition allows treatment with anti-inflammatory therapy, immobilization, and sometimes traction, while delayed diagnosis may lead to fixed deformity requiring surgical stabilization.

 

Q. What clinical features do you expect in torticollis?

 

Patients typically present with a fixed head tilt toward the affected side with rotation away from it. Congenital cases present early in infancy with palpable tightness or mass in the sternocleidomastoid. Long-standing cases may show facial asymmetry, plagiocephaly, and restricted neck range of motion. Acquired cases may present acutely or insidiously with neck pain, neurological deficits if due to Chiari or syrinx, restricted ocular movements in ocular torticollis, and dystonic twisting movements in spasmodic forms.

 

Q. What associated anomalies may accompany congenital torticollis?

 

Common associations include developmental dysplasia of the hip, metatarsus adductus, clavicular fractures, brachial plexus birth injuries, and craniofacial asymmetry. Cervical spine anomalies may coexist, though less frequently.

 

Q. What investigations will you perform for a case of torticollis?

 

Investigation of torticollis depends on determining what the suspected cause is, and therefore a structured multilevel approach is required. 

 

MRI of the brain and craniovertebral junction is the most important investigation. CT scan of the cervical spine and craniovertebral junction is essential for evaluating bony abnormalities. EMG of the neck muscles helps differentiate dystonic torticollis from extrapyramidal or neuromuscular causes because abnormal grouped discharges support dystonia, whereas normal EMG favors structural or ocular etiologies.

 

In congenital muscular torticollis, ultrasound of the sternocleidomastoid is commonly added because it identifies fibrosis, muscle thickness, and mass-type lesions, helping differentiate the sternocleidomastoid mass subtype from postural preference. 

 

In cases suspicious for atlantoaxial instability, dynamic cervical X-rays in flexion and extension help evaluate instability, although CT remains more definitive for C1–C2 alignment. 

 

When ocular torticollis is suspected, a full ophthalmologic evaluation including ocular motility testing and cover–uncover tests is necessary to identify superior oblique palsy or strabismus. In suspected infections, inflammatory markers such as CBC, ESR, and CRP can also be done. 

 

Q. How will you distinguish spasmodic torticollis from tardive dystonia?

 

Spasmodic torticollis is a primary cervical dystonia arising from abnormal basal ganglia output, producing involuntary twisting, pulling, or jerking of the neck, often with a sensory trick that temporarily improves posture. In contrast, tardive dystonia results from chronic exposure to dopamine-blocking medications such as antipsychotics or antiemetics, usually developing in adolescents or young adults. Tardive dystonia tends to be more generalized, more painful, and more persistent, and may coexist with other tardive syndromes. 

 

The distinction is important because spasmodic torticollis often responds well to botulinum toxin and deep brain stimulation of the globus pallidus internus, while tardive dystonia requires withdrawal of the offending drug, anticholinergic therapy, benzodiazepines, and, in severe cases, deep brain stimulation tailored specifically for medication-induced dystonia. Misidentification of tardive dystonia leads to incomplete treatment and continuation of the causative agent.

 

Q. What is the natural history of untreated congenital muscular torticollis?

 

Untreated congenital muscular torticollis leads to progressive craniofacial asymmetry because persistent tilt alters skull molding, producing plagiocephaly, orbital asymmetry, mandibular deviation, and ear displacement. Over time, the cervical spine compensates by developing cervicothoracic scoliosis and lateral curvature. 

 

The sternocleidomastoid fibrosis becomes more rigid, reducing the chance of recovery with physiotherapy and often necessitating surgical release. The visual system may also adapt abnormally, producing ocular tracking asymmetry. Long-standing deformity can persist into adulthood, causing aesthetic concerns, neck pain, restricted rotation, and compensatory malalignment of the shoulders and upper spine. Early intervention is essential to prevent these long-term musculoskeletal and facial consequences.

 

Q. How will you manage congenital muscular torticollis?

 

Initial management is conservative and consists of physiotherapy aimed at gentle passive stretching of the sternocleidomastoid, positioning therapy, and caregiver-guided exercises. Most cases resolve with structured therapy over months. If the child presents late, has severe deformity, or fails conservative treatment beyond one year of age, surgical lengthening or release of the sternocleidomastoid is indicated. After surgery, physiotherapy and splinting ensure symmetrical neck posture and prevent recurrence.

 

Q. When is surgery indicated in congenital muscular torticollis?

 

Surgery is indicated when limitation of passive rotation or lateral bending persists beyond twelve months of age despite consistent physiotherapy, when a firm fibrotic band remains on ultrasound, or when the deformity is severe enough to cause craniofacial asymmetry that is unlikely to reverse spontaneously. Failure of conservative management, late presentation after infancy, or a residual tilt persisting after an adequate trial of therapy all justify operative release. The goal is to correct the contracture before the secondary changes in the spine and facial skeleton become fixed. After surgical release, early physiotherapy and proper postoperative positioning are essential to maintain correction.

 

Q. How will you manage acquired torticollis?

 

Management is guided by the underlying cause. Inflammatory or infectious causes respond to anti-inflammatory treatment, antibiotics, and rest. Ocular torticollis requires ophthalmologic correction. Drug-induced dystonia resolves with withdrawal of the offending agent and administration of anticholinergics. Chiari malformation–related torticollis improves after posterior fossa decompression when symptomatic or associated with syringomyelia. Cervical spine anomalies or atlantoaxial rotatory fixation require immobilization, traction, or surgical stabilization depending on severity. Dystonic torticollis may be managed with botulinum toxin injection or neuromodulatory procedures in refractory cases.

 

Q. Describe the role of deep brain stimulation in the management of torticollis.

 

Deep brain stimulation is used in the management of refractory cervical dystonia when medical therapy and repeated botulinum toxin injections fail to provide durable symptom control. DBS of the bilateral globus pallidus internus is effective because high-frequency stimulation suppresses the pathological oscillatory firing patterns responsible for dystonic motor output. 

 

By modulating this abnormal pallidal activity, deep brain stimulation reduces the excessive drive to the sternocleidomastoid, splenius, levator scapulae and other cervical muscles, allowing more balanced activation of antagonists and improved head alignment.

 

The selection of patients for deep brain stimulation requires confirmation of true dystonic torticollis rather than spastic, ocular, inflammatory, or structural causes, because deep brain stimulation targets the central motor network and will not correct peripheral or mechanical etiologies. 

 

Once implanted, deep brain stimulation gradually improves posture over weeks to months as the central motor circuits remodel in response to continuous stimulation. Patients often experience substantial reduction in pain, improved range of motion, better functional positioning, and a reduction in the severity and frequency of involuntary neck spasms. 

 

The benefit is long-lasting, reversible, and adjustable, which makes deep brain stimulation an important option in severe cervical dystonia, particularly in patients with progressive disability or fixed abnormal posturing from years of untreated dystonic drive.

 

Q. What complications may occur following deep brain stimulation for torticollis?

 

Complications may arise from the hardware, the operation, or the stimulation itself. Hardware problems include lead migration, insulation failure, or connector malfunction due to mechanical stress along the cervical–thoracic course. Surgical risks include intracranial hemorrhage along the electrode tract and device-related infection, which may require hardware removal. Stimulation can cause dysarthria, bradykinesia, imbalance, or mood changes when current spreads beyond the globus pallidus internus.

 

Q. What are the denervation procedures used for torticollis?

 

Denervation procedures are reserved for patients with focal dystonic torticollis in whom selective muscle overactivity is well localized and when botulinum toxin or medical therapy fails. The principle is to interrupt the motor innervation of the dystonic muscles so that the involuntary contractions cease, allowing the head to return toward a neutral position under the control of the non-dystonic antagonists.

 

These procedures target the spinal accessory nerve branches supplying the sternocleidomastoid or the posterior primary rami innervating the splenius capitis, semispinalis capitis, or other hyperactive posterior cervical muscles. By sectioning or coagulating only the branches supplying the dystonic portion of the muscle, selective denervation preserves the function of the remaining cervical musculature and prevents global weakness. This is based on the observation that cervical dystonia frequently involves asymmetric overactivation of one or two dominant muscle groups rather than a generalized pattern.

 

Denervation reduces involuntary spasms because it eliminates the peripheral efferent drive, interrupting the final common pathway through which abnormal basal ganglia signals manifest. Over time, the antagonistic muscles regain better control as the abnormal pulling force disappears. 

 

Denervation is most successful when the dystonia is focal, the pattern is stable, and electromyographic mapping clearly identifies the hyperactive muscles. Although not as commonly performed, it remains an option for highly selected patients with well-defined dystonic circuitry who are poor candidates for more invasive neuromodulatory procedures.

 

Q. What complications may arise after selective denervation procedures for torticollis?

 

Selective denervation may produce excessive weakness if too many branches are cut, leading to head drop or loss of postural control, while inadequate denervation results in persistent dystonia. Sensory nerve irritation or neuroma formation can generate neuropathic pain. Scar tissue may limit neck mobility, and deep dissection may injure nearby structures such as the internal jugular vein. Long-term recurrence may occur due to collateral nerve sprouting, emphasizing the need for accurate mapping and careful patient selection.

 

Q. What neuromodulatory options exist for torticollis apart from selective sectioning?

 

Dorsal column stimulation: The spinal cord stimulation at the cervical level modulates segmental and supraspinal circuits involved in dystonic posturing; this is less commonly used but may benefit selected patients with refractory dystonia. 

 

Botulinum toxin injection represents a chemical neuromodulation method, weakening dystonic muscles by blocking neuromuscular transmission and thereby reducing involuntary contractions; although its effect is temporary, it remains a first-line neuromodulatory therapy.

 

Microvascular decompression of the spinal accessory nerve is indicated in rare torticollis caused by vascular compression at the nerve entry zone, and decompression restores normal nerve firing over time. 

 

Stereotactic lesioning procedures targeting areas such as Forel’s H1 field or thalamic nuclei can interrupt abnormal motor network activity in severe refractory cases, although these are now less common due to the preference for adjustable and reversible deep brain stimulation.

 

Q. How will you surgically release the sternocleidomastoid in congenital muscular torticollis?

 

Surgical release of the sternocleidomastoid is performed when congenital muscular torticollis fails to respond to prolonged physiotherapy or when the child presents late with fixed fibrosis and persistent tilt. I will position the patient supine with slight extension and rotate the head to expose the tight sternocleidomastoid. I will make a small transverse incision along a natural skin crease low in the neck, directly over the clavicular and sternal heads of the muscle. 

 

I will carefully dissect through the subcutaneous tissue and platysma until the fibrotic sternocleidomastoid is identified. Once exposed, I will isolate the sternal and clavicular heads and then release them from their attachments at the manubrium and clavicle. If the deformity is severe or if mid-muscle contracture is evident, I will extend the release proximally and perform bipolar release by freeing the upper attachment near the mastoid, ensuring that the great auricular nerve and accessory nerve are preserved.

 

After the release, I will gently stretch the neck through its full passive range of motion to confirm that the contracture is corrected and the head can be positioned neutrally. Hemostasis will be secured and the wound closed in layers. Postoperatively, I will begin physiotherapy early to maintain the newly gained range. 

 

The child will undergo a structured program of stretching, strengthening of the contralateral sternocleidomastoid, and positioning techniques to prevent recurrence. Nighttime bracing or orthosis may be used initially to maintain correction. Over the following months, muscle balance gradually normalizes, and the facial asymmetry improves as the child grows.

 

Q. What complications may occur after surgical release of the sternocleidomastoid in congenital muscular torticollis?

 

Complications include injury to the spinal accessory nerve resulting in trapezius weakness or shoulder droop, and injury to the great auricular nerve causing sensory loss over the auricle and jaw angle. Inadequate release leads to persistent tilt, while over-release causes cosmetic deformity or imbalance between neck muscles. Hematoma or infection may limit postoperative motion and promote fibrosis. Recurrence is the most important long-term risk if physiotherapy is not maintained, making postoperative stretching and bracing essential.

 

Q. What is the prognosis?

 

Congenital muscular torticollis treated early has an excellent prognosis with near-complete correction. Late treatment may leave residual facial asymmetry. Acquired torticollis prognosis depends on etiology: inflammatory and ocular causes usually resolve completely; dystonic and neurogenic causes may persist chronically; and Chiari-related torticollis often improves significantly following appropriate decompression.