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Congenital Muscular Torticollis

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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