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Introduction
  • A congenital "packaging deformity" typically caused by contracture of the sternocleidomastoid (SCM) muscle
  • Epidemiology
    • found in infants
  • Pathophysiology
    • muscular (sternocleidomastoid)
      • true etiology is unclear
      • some studies suggest association with intrauterine compartment syndrome of SCM muscle
    • non-muscular causes include
      • Klippel-Feil
      • ophthalmologic
      • brachial plexus palsies
      • lesions of central nervous system
  • Associated conditions
    • often associated with other packaging disorders
      • DDH (5 - 20% association) 
      • metatarsus adductus 
    • traumatic delivery
    • plagiocephaly (head asymmetry)
    • congenital atlanto-occipital abnormalities
Physical Exam
  • Symptoms
    • head tilt
      • usually noticed by parents
  • Physical exam
    • head tilt towards the affected side with chin rotation away from the affected side
    • palpable neck mass and fibrosis is noted within the first four weeks of life 
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 
Differential
  • Rotatory atlanto-axial instability / Grisel's disease 
    • Grisel's disease similar presentation but presents in older children
  • Klippel-Feil syndrome 
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
  • Operative
    • Z plasty lengthening or distal bipolar release of SCM
      • indications
        • condition present for greater than 1 year
        • limitation greater than 30°
 

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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? Review Topic

QID:3317
FIGURES:
1

Congenital muscular torticollis

89%

(1826/2060)

2

Klippel-Feil syndrome

0%

(6/2060)

3

Arnold-Chiari malformation

0%

(5/2060)

4

Atlantoaxial rotatory displacement

1%

(22/2060)

5

Paroxysmal torticollis of infancy

9%

(193/2060)

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

Congenital muscular torticollis (CMT) is the most common cause of torticollis in infancy. Neurologic disorders and osseous abnormalities, such as Klippel-Feil, are less common. Ultrasonography is considered the modality of choice for differentiating (CMT) from other more serious pathologies in the neck when a palpable mass is present. The initial treatment of CMT is conservative, and good outcomes can be expected in the majority of these cases. Tang et al used US to examine affected sternocleidomastoid muscles in patients with CMT classified as having one of four types of fibrosis. They determined the change in fibrosis type over time and concluded that CMT is a dynamic disease which can be accurately assessed and followed by ultrasonography. Benign paroxysmal torticollis is a self-limiting condition occurring during infancy. It resolves by the age of two to three years. Paroxysmal torticollis of infancy is a rare disorder characterized by periodic episodes of torticollis which are associated with pallor, agitation and ataxia. The etiology is unknown and no treatment is effective, however the condition is usually benign and self-limiting.


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

QID:2815
1

platysma

0%

(5/2063)

2

omohyoid

0%

(8/2063)

3

longus colli

1%

(12/2063)

4

sternocleidomastoid

98%

(2027/2063)

5

trapezius

0%

(9/2063)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

Tightness of the sternocleidomastoid muscle leading to a stiff and tilted neck is associated with the muscular variety of congenital torticollis. The differential diagnosis of the wry neck however includes sequelae to inflammatory, ocular, neurologic or orthopedic diseases so therefore a thorough and systematic work-up is warranted, including a complete physical and neurologic examination and cervical spine radiographs. The treatment for the muscular variety is observation and physical therapy (stretching: lateral head tilt away from the affected side and chin rotation toward the affected side), then botox or sternocleidomastoid muscle lengthening for refractory cases. Overall, it is important to differentiate muscular from nonmuscular torticollis because the muscular type is benign while the nonmuscular type could be potentially life threatening. The illustration below shows an example of muscular torticollis.

ILLUSTRATIONS:

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