Updated: 9/26/2020

Congenital Muscular Torticollis

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Questions
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Introduction
  • Overview
    • a congenital "packaging deformity" typically caused by contracture of the sternocleidomastoid (SCM) muscle
  • Epidemiology
    • incidence
      • the most common cause of infantile torticollis
    • risk factors
      • traumatic delivery
  • 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 
    • plagiocephaly (asymmetric flattening of the skull)
    • congenital atlanto-occipital abnormalities
  • Natural history
    • untreated may lead to
      • permanent rotational deformity 
      • positional plagiocephaly
      • facial asymmetry
      • dysplasia of
        • skull base
        • atlas (articular facets, laminae, body)
        • axis
Physical Exam
  • Symptoms
    • head tilt and rotation
      • usually noticed by parents
    • typically painless
      • if painful then suggestive of an alternative diagnosis
  • Physical exam
    • head tilt & rotation
      • tilt towards the affected SCM
      • chin rotation away from the affected SCM  
    • palpable neck mass (contracted SCM) 
      • is noted within the first four weeks of life 
        • gradually subsides, becoming a tight band as the patient ages
Imaging
  • Radiographs
    • indications
      •  if no palpable mass present to rule out other conditions that cause torticollis including
        • rotatory atlanto-axial instability
        • Klippel-Feil syndrome
  • Ultrasound
    • indications
      •  in the presence of a palpable mass
    • can help differentiate congenital muscular torticollis from more serious underlying neurologic or osseous abnormalities 
  • CT Scan (dynamic)
    • indications
      • to rules out atlantoaxial rotatory subluxation
    • technique
      • 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 cosmetic deformity
      • outcomes
        • good results reported even in older children (4-8 years)
Techniques
  • Bipolar release of SCM or Z plastic lengthening
    • postoperative
      • consider immobilization in over corrected position
 

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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%

(3235/3664)

2

Klippel-Feil syndrome

0%

(13/3664)

3

Arnold-Chiari malformation

0%

(8/3664)

4

Atlantoaxial rotatory displacement

1%

(41/3664)

5

Paroxysmal torticollis of infancy

9%

(348/3664)

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%

(12/3417)

2

omohyoid

0%

(15/3417)

3

longus colli

1%

(21/3417)

4

sternocleidomastoid

98%

(3345/3417)

5

trapezius

0%

(12/3417)

L 1 C

Select Answer to see Preferred Response

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Topic COMMENTS (8)
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