Updated: 10/17/2020

Congenital Muscular Torticollis

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Introduction
  • Overview
    • Congenical muscular torticollis is a musculoskeletal deformity seen in infants and children caused by contraction of the sternocleidomastoid muscle.
    • , characterized by a persistent head tilt toward the involved side with the chin rotated towards the contralateral side
    • Treatment is typically passive stretching
    • Failure to treat may result in craniofacial deformities
  • Epidemiology
    • Incidence
      • Most common cause of infantile torticollis
      • 0.3 - 2.0%
    • Risk factors
      • Oligohydramnios
      • First pregnancy (limited intrauterine space)
      • Traumatic delivery
      • Breech delivery
  • Pathophysiology
    • Contracture of the SCM causing cervical rotational deformity away from the affected side, with chin 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
      • DDH (5 - 15% association) 
      • Metatarsus adductus 
      • Calcaneovalgus feet
    • Plagiocephaly (asymmetric flattening of the skull)
    • Congenital atlanto-occipital abnormalities
  • Natural history
    • If left untreated
      • Permanent rotational deformity 
      • Positional plagiocephaly
      • Craniofacial deformities
        • Facial asymmetry
        • Facial hemihypoplasia
      • Compensatory scoliosis
Anatomy
  • Sternocleidomastoid (SCM) muscle
    • 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
  • History
    • Parents usualy notice head tilt and rotation or palpate a mass on neck
  • Symptoms
    • Head tilt and rotation
    • Painless passive motion
  • Physical exam
    • 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  
    • In older children - restriction of rotation and lateral flexion of neck
      • Mass becomes a tight band
    • Conduct routine baby exam
      • Assess visual function, auditory assessment, and neurologic exam 
      • Examine for hip dysplasia, foot deformities, as well as spine abnormalities
Imaging
  • Physical examination is the easiest and most effective means of diagnosis
  • Ultrasound
    • Indications
      • Head tilt and rotation with decreased ROM in the presence of a palpable mass
    • Inexpensive, non-invasive, and quick
    • Findings
      • Larger and hyperechoic (due to fibrosis) SCM on involved side when compared to contralateral side
    • Can help differentiate congenital muscular torticollis from more serious underlying neurologic or osseous abnormalities 
  • Radiographs - cervical spine
    • Indications
      • Head tilt and rotation with no palpable mass present
      • Rule out other bony conditions that can cause torticollis
  • CT Scan (dynamic)
    • Indications
      • Rule out atlantoaxial rotatory subluxation
    • Technique
      • Scan at the C1-2 level with head straight, then in maximum rotation to the left and to the right
  • MRI of cervical spine and brain
    • Indications
      • Rule out non-muscular and central causes of torticollis
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
      • Technique
        • Opposite of the deformity
          • Lateral head tilt away from affected side with chin rotation toward the affected side 
      • 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
  • Bipolar release of SCM or Z-lengthening
    • Short, proximal incision behind the ear to divide SCM
      • SCM branch of CN XI (spinal accessory nerve) is at risk
    • Single or dual incision to reach sternal and clavicular attachments of SCM
Complications
  • Permanent rotational deformity
  • Positional plagiocephaly
  • Craniofacial deformities
    • Facial asymmetry
    • Facial hemihypoplasia
  • Compensatory scoliosis
 

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Questions (2)

(OBQ10.218) A 6-week-old female infant presents with the neck deformity and palpable mass shown in Figure A. She has had persistent lateral tilting of her head to the right since birth, and rotation of the neck is restricted. In this age group, what is the most common cause of this rotational abnormality? Tested Concept

QID: 3317
FIGURES:
1

Congenital muscular torticollis

88%

(3258/3691)

2

Klippel-Feil syndrome

0%

(13/3691)

3

Arnold-Chiari malformation

0%

(8/3691)

4

Atlantoaxial rotatory displacement

1%

(41/3691)

5

Paroxysmal torticollis of infancy

10%

(352/3691)

L 1 C

Select Answer to see Preferred Response

(OBQ09.2) Tightness in which of the following muscles has been implicated as an etiology for congenital muscular torticollis? Tested Concept

QID: 2815
1

platysma

0%

(12/3438)

2

omohyoid

0%

(15/3438)

3

longus colli

1%

(21/3438)

4

sternocleidomastoid

98%

(3365/3438)

5

trapezius

0%

(12/3438)

L 1 C

Select Answer to see Preferred Response

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