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Introduction
  • A congenital "packaging deformity" typically caused by contracture of the sternocleidomastoid (SCM) muscle
  • Epidemiology
    • the most common cause of infantile torticollis
  • Pathophysiology
    • contracture of the sternocleidomastoid (SCM) causing cervical rotational deformity away from the affected side, tilt toward the affected side
    • suspected result of intrauterine compartment syndrome of SCM muscle
      • thought to be caused by venous outflow obstruction
  • Associated conditions
    • often associated with other packaging disorders
      • DDH (5 - 20% association) 
      • metatarsus adductus 
    • traumatic delivery
    • plagiocephaly (asymmetric flattening of the skull)
    • congenital atlanto-occipital abnormalities
Physical Exam
  • Symptoms
    • head tilt and rotation usually noticed by parents
  • Physical exam
    • head tilt towards the affected side with chin rotation away from the affected side  
    • palpable neck mass (the contracted SCM) is noted within the first four weeks of life 
      • gradually subsides, becoming a tight band as the patient ages
    • not typically painful, if painful then suggestive of an alternative diagnosis
Imaging
  • Radiographs
    • indicated if no palpable mass present to rule out other conditions that cause torticollis including
      • rotatory atlanto-axial instability
      • Klippel-Feil syndrome
  • Ultrasound
    • indicated in the presence of a palpable mass
    • can help differentiate congenital muscular torticollis from more serious underlying neurologic or osseous abnormalities 
  • CT Scan
    • rules out atlantoaxial rotatory subluxation
    • Scan at the C1-2 level with head straight, then in maximum rotation to the right, and then in maximum rotation to the left
Differential
  • Atlantoaxial Rotatory Subluxation 
    • Post-traumatic
    • Post-infectious (Grisel's disease)
    • painful, patient will resist correction of deformity
  • Klippel-Feil syndrome 
    • has classic triad of:
      • short neck
      • low hairline
      • restricted neck motion
  • Ophthalmologic conditions
  • Vestibular conditions
  • Lesions of central and peripheral nervous system
Treatment
  • Nonoperative
    • passive stretching
      • indications
        • condition present for less than 1 year
        • limitation less than 30°
      • stretching technique
        • should include lateral head tilt away from the affected side and chin rotation toward the affected side (opposite of the deformity) 
      • outcomes
        • 90% respond to passive stretching of the sternocleidomastoid in the first year of life
        • associated plagiocephaly does remodel and improve, but this process is delayed in older children
  • Operative
    • bipolar release of SCM or Z plastic lengthening
      • indications
        • failed response to at least 1 year of stretching
        • Significant deformity
      • good results reported even in older children (4-8 years)
      • Post-op:Consider immobilization in over corrected position
Complications
  • Untreated may lead to
    • permanent rotational deformity 
    • positional plagiocephaly
    • facial asymmetry
    • dysplasia of
      • skull base
      • atlas (articular facets, laminae, body)
      • axis
 

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