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Updated: Jan 15 2023

Congenital Muscular Torticollis

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  • SUMMARY
    • Congenital Muscular Torticollis is a musculoskeletal deformity caused by the abnormal contraction of the sternocleidomastoid muscle.
    • The condition typically presents in infants and children with a persistent head tilt toward the involved side.
    • Diagnosis is made clinically with the presence of a palpable neck mass from a contracted sternocleidomastoid muscle with the chin rotated towards the contralateral side.
    • Treatment is typically passive stretching with the condition resolving within a year. Surgical lengthening of the SCM muscle is indicated with failed response to at least 1 year of stretching.
  • Epidemiology
    • Incidence
      • most common cause of infantile torticollis
      • 0.3 - 2.0%
    • Demographics
      • 3:2 male to female ratio
    • Anatomic location
      • neck
    • Risk factors
      • oligohydramnios
      • first pregnancy (limited intrauterine space)
      • traumatic delivery
      • breech delivery
  • Etiology
    • Pathophysiology
      • contracture of the sternocleidomastoid (SCM)
        • cervical rotational deformity with chin rotation away from the affected side and head tilt towards the affected side
      • suspected muscle injury from compression and stretching of SCM
      • venous outflow obstruction
        • compression leading to decreased blood supply and subsequent compartment syndrome
    • Associated conditions
      • associated with other packaging disorders
        • developmental dysplasia of the hip (5 - 15% association)
        • metatarsus adductus
        • calcaneovalgus feet
      • plagiocephaly (asymmetric flattening of the skull)
        • occurs on contralateral side
      • congenital atlanto-occipital abnormalities
  • ANATOMY
    • Muscles
      • sternocleidomastoid muscle (SCM)
        • origins
          • sternal head - anterior surface of manubrium sterni
          • clavicular head - superior surface of medial third of clavicle
        • insertions
          • lateral mastoid process on temporal bone
          • lateral occipital bone
        • innervation
          • cranial nerve XI - spinal accessory nerve
            • at risk when operatively releasing SCM
        • function
          • ipsilateral neck flexion, contralateral head rotation
  • PRESENTATION
    • Symptoms
      • head tilt and rotation
      • painless passive motion
    • Physical exam
      • inspection
        • palpable neck mass from contracted SCM
          • usually noted within the first four weeks of life or during newborn exam
        • head tilt & rotation
          • neck tilt towards the affected SCM
          • chin rotation away from the affected SCM
        • conduct routine baby exam
          • assess visual function, auditory assessment, and neurologic exam
          • examine for hip dysplasia, foot deformities, as well as spine abnormalities
      • motion
        • in older children - restriction of rotation and lateral flexion of neck
          • mass becomes a tight band
  • IMAGING
    • Radiographs
      • recommended views
        • AP and lateral cervical spine
      • indications
        • head tilt and rotation with no palpable mass present
        • rule out other bony conditions that can cause torticollis
    • CT
      • recommended views
        • dynamic CT scan
        • scan at C1-C2 level with head straight, then in maximum rotation to left and right
      • indications
        • rule out atlantoaxial rotatory subluxation
    • MRI
      • recommended views
        • MRI brain and cervical spine
      • indications
        • rule out non-muscular and central causes of torticollis
    • Ultrasound
      • indications
        • head tilt and rotation with decreased ROM in the presence of a palpable mass
      • findings
        • larger and hyperechoic (due to fibrosis) SCM on involved side when compared to contralateral side
        • differentiate congenital muscular torticollis from more serious underlying neurologic or osseous abnormalities
  • DIFFERENTIAL
    • Atlantoaxial rotatory subluxation
      • painful (compared to painless for congenital muscular torticollis)
      • post-traumatic or post-infectious (Grisel's disease)
    • Klippel-Feil syndrome
      • classic triad:
        • short webbed neck
        • low posterior hairline
        • limited cervical range of motion
    • Ophthalmologic and vestibular conditions
    • Lesions of central and peripheral nervous system
  • TREATMENT
    • Nonoperative
      • passive stretching
        • indications
          • condition present for less than 1 year
          • less than 30° limitation in ROM
        • outcomes
          • 90-95% respond to passive stretching in the first year of life
    • Operative
      • bipolar release of SCM or Z-lengthening
        • indications
          • failed response to at least 1 year of stretching
        • outcomes
          • good outcomes (92% success), even in older children
          • facial asymmetry can improve as long as release done prior to 10 years of age
  • TECHNIQUES
    • Passive stretching
      • technique
        • opposite of the deformity
          • lateral head tilt away from affected side
          • chin rotation toward the affected side
    • Bipolar release of SCM or Z-lengthening
      • technique
        • short, proximal incision behind the ear to divide SCM
        • single or dual incision to reach sternal and clavicular attachments of SCM
      • complications
        • SCM branch of CN XI (spinal accessory nerve) is at risk
  • COMPLICATIONS
    • Permanent rotational deformity
      • risk factors
        • left untreated or unnoticed
    • Positional plagiocephaly
      • risk factors
        • left untreated or unnoticed
    • Craniofacial deformities
      • facial asymmetry
      • facial hemihypoplasia
    • Compensatory scoliosis
  • Prognosis
    • Typically resolves with stretching within the first year
    • If left untreated
      • permanent rotational deformity
      • positional plagiocephaly
      • craniofacial deformities
        • facial asymmetry
        • facial hemihypoplasia
      • compensatory scoliosis
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