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