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Torticollis (Wry Neck)

  • A congenital or acquired deformity the leads to head and neck dystonia
  • Results in a posturing of the head and neck in tilt, rotation and flexion

1. CONGENITAL TORTICOLLIS

Epidemiology

  • More common in females
  • Presents at 1-4 weeks of life

Etiology

  • Deformity caused by the contraction of the sternocleidomastoid (SCM) muscle
  • Risk factors – oligohydraminos, first pregnancy, traumatic delivery, breech delivery
  • Associated conditions
    • Pterygium colli (webbed neck)
    • Spina bifida
    • Arnold- Chiari syndrome – downward displacement of one or both cerebellar tonsils through the foramen magnum
    • Developmental dysplasia of the hip

Anatomy – sternocleidomastoid

  • Function – ipsilateral neck flexion, contralateral head rotation
  • Originates at sternum and clavicle; inserts on mastoid process
  • Innervation – spinal accessory nerve (CN XI)
  • Arterial – occipital artery and superior thyroid artery

Pathophysiology

  • Birth trauma to the SCM can result in fibrosis
    • Caused by venous occlusion and pressure on the neck in the birth canal
    • Compression can cause decreased blood supply and subsequent Compartment syndrome
  • Intrauterine malpositioning (e.g. in oligohydraminos/first pregnancy) can lead to unilateral shortening of the SCM
    • Can result in formation of a hematoma, followed by muscular contracture

Clinical features

  • Head tilt towards the affected side with chin rotation away from affected side
  • Painless passive motion
  • Palpable neck pass – from contracted SCM

Diagnosis

  • Obstetric history and physical exam
  • Ultrasound – large, hyperechoic SCM (due to fibrosis) compared to contralateral side
  • XR – to rule out other bony conditions that could cause torticollis
  • CT – to rule out atlantoaxial subluxation

Treatment

Non-operative

  • Physiotherapy – excellent outcomes when compliance with physiotherapy is good, especially in newborns
    • Tilt head away from affected side and rotate chin towards affected side

Operative

  • Release of SCM (unipolar or bipolar) – failed response to at least 1 year of stretching

Differential diagnosis

  • Atlantoaxial subluxation – painful compared to congenital torticollis
  • Klippel-Feil syndrome – classic triad: short webbed neck, low posterior hairline, limited cervical range of motion

2. ACQUIRED TORITCOLLIS (AKA Cervical Dystonia)

Epidemiology

  • More common in females
  • 30-50 year olds
  • Family history of acquired torticollis

Etiology

  • Mostly idiopathic
  • Family history – genetic predisposition
    • DYT-6 and DYT-7 genes implicated
  • Neck trauma
  • Drugs – metoclopramide, prochlorperazine
  • Can occur as a symptom of other diseases – Huntington’s, Wilson’s, cerebral palsy, perinatal asphyxia
  • Infections – tonsillitis, adenitis

Pathophysiology

  • Centrally activated contraction in skeletal function, resulting in abnormal posture
  • Abnormalities in basal ganglia and the sensory and motor cortices

Clinical features

  • Involuntary twisting/deviation of neck
  • Pain
  • Abnormal head posture
  • Head tremor
  • Limited range of motion
  • Asymmetrical hypertrophy of neck muscles

Diagnosis

  • Mostly based on history and physical exam
  • Cervical XR
  • CT/MRI of brain and neck
  • Genetics – DYT-1 gene

Treatment

Non-operative

  • Physiotherapy – 1st line
  • Medications – anticholinergics, baclofen, clonazepam (limited efficacy)
  • Botulinum toxin – intramuscular

Operative

  • Deep brain stimulation of globus pallidus pars interna
  • Radiofrequency ablation
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