Torticollis

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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
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