Torticollis

Introduction

• Torticollis is the term used to describe the clinical findings of tilting (lateral bending) of the head/neck to the right/left side, in combination with rotation of the head/neck to the opposite side
• Torticollis is not a diagnosis, but rather is adescription of a manifestation of a variety of underlying conditions

Anatomy

• Torticollis is caused by tightness of the sternocleidomastoid
• The sternocleidomastoid is so named because it originates from the sternum and the clavicle and inserts on the mastoid process of the temporal bone
• Its function is to rotate the head to the opposite side or obliquely rotate the head. It also flexes the neck

Pathogenesis

Congenital

• Most cases discovered at or near the time of birth represent congenital muscular torticollis
• The exact aetiology is unknown
• Proposed mechanism is abnormal positioning in utero and 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

Hereditary

• Familial basis for torticollis, as well as hereditary muscle aplasia, has been reported

Clinical Presentation

• Contracture of the right sternocleidomastoid muscle (75% of cases) results in tilt of the head to the right and rotation to the left and vice versa
• Cervical mass
  • A palpable mass (fibrous tissue) within the substance of the sternocleidomastoid muscle is present in approximately half of the patients
  • The mass disappears during infancy and is replaced by a fibrous band
• Chronic muscle contraction may cause plagiocephaly and facial asymmetry, both of which usually resolve with restoration of cervical motion
• Associated positional musculoskeletal deformities
  • Metatarsus adductus
  • Calcaneovalgus feet
  • Hip dysplasia
    • 20% co-incidence in older literature
    • 5-8% co-incidence in recent studies
• The evaluation of torticollis becomes more complex when
  • The typical findings associated with CMT are absent (mass and/or contracture)
  • The usual clinical response is not observed
  • The deformity presents at a later age
• In atypical cases, obtain
  • A careful history and physical examination
  • Consultation with an opthalmologist and/or neurologist
  • Plain radiographs
  • MRI of the brain and cervical spine in a subset of cases

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
  • Ultrasound of hip (1 month of age)
  • Plain radiograph of the hip (4-5 months of age)
• AP and lateral X-Rays of the cervical spine to rule out congenital anomalies are indicated when
  • The typical clinical features associated with congenital muscular torticollis are absent
  • The deformity does not respond to treatment

Differential Diagnosis

Congenital

• Congential muscular torticollis
• Positional deformation
• Klippel-Feil syndrome
• Unilateral absence of sternocleidomastoid
• Hemivertebra of 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
• Cervical discitis
• Rheumatoid arthritis
• Spontaneous subluxation with adjacent head and neck infection (rotary subluxation syndrome)
• Upper lobe pneumonia

Neurogenic

• Visual disturbances
  • Nystagmus
  • Superior oblique paresis
  • Strabismus
• Dystonic drug reactions
  • Phenothiazines
  • Haloperidol
  • Metoclopramide
• Cerebral palsy
• 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

Atlanto-axial rotatory displacement

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

Traumatic injuries

• Usually minor
• May lead to the development of rotatory displacement
• Will occasionally complicate surgical procedures in the oropharynx, ear or nose

Neurogenic torticollis

• Uncommon
• Aetiology
  • Tumors of the posterior fossa / brainstem
  • Syringomyelia
  • 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

• Quite rare
• Results from vestibular dysfunction
• Episodes may last from minutes to days
• The side of the deformity may alternate
• Self limited
• No specific treatment is required other than ruling out other treatable diagnoses

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
• With early diagnosis and treatment, surgery should be required in a minority of cases
• 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
  • Several surgical techniques have been described 

Atlanto-axial rotatory displacement

• If the patient is seen within a few days of the onset of symptoms, 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, 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

Outcome

• Surgical management results in adequate function and acceptable cosmesis in 90% of patients