Congenital Muscular Torticollis

Key Points:

  • Congenital muscular torticollis is the most common cause of torticollis in the infant and young child
  • The physical examination findings are caused by pathologic contracture and fibrosis of the sternocleidomastoid muscle
  • Physicians should rule out other causes of torticollis and be aware that DDH is present in up to 20% of patients with congenital muscular torticollis
  • Manual stretching, caregiver education, and environmental modifications represent the primary initial treatments. For resistant cases and children who present after 12 months of age a bipolar release of the sternocleidomastoid muscle is indicated

Description:

Congenital muscular torticollis (CMT) is the most common cause of torticollis in the infant and young child. The median age at presentation is 2 months. The characteristic head tilt seen in this pathology is caused by abnormal contracture of the sternocleidomastoid muscle. With contracture of the muscle, the head tilts toward the involved side with the chin rotated towards the opposite shoulder. Most often, this diagnosis is associated with breech or difficult deliveries, but it may occur in uncomplicated spontaneous vaginal delivery  or Cesarean delivery. The deformity is most often present on the right side and up to 20% of patients will also have developmental dysplasia of the hip (DDH).   Consideration should be given to screening patients with CMT for DDH with physical examination and imaging.

Epidemiology:

Overall, the estimated incidence of congenital muscular torticollis is <1% of all live births. The incidence may as be low as 0.3% in uncomplicated deliveries with vertex position or as high as 1.8% in patients with breech presentation.

Clinical Findings:

The typical head posture results from the contracted SCM: the ear on the side of contracture is tilted towards the ipsilateral shoulder while the chin is rotated toward the contralateral shoulder.   Occasionally a firm mass or knot may be palpated in the SCM, typically in a child less than 3 months of age.  Plagiocephally is commonly present on the side of the contracted SCM and is related to the contracture and the recommendation of supine sleep positioning for prevention of sudden infant death syndrome.  When viewed from above, a typical plagiocephaly deformity will demonstrate anterior displacement of the ear on the same side as the occipital flattening.  

Imaging Studies:

The diagnosis of congenital muscular torticollis is primarily clinical.  However, ultrasound may be a useful modality in the presence of a palpable mass. Radiographs of the cervical spine may be obtained to rule out any associated congenital abnormalities.   Every child with CMT does not need imaging, however they should be considered if the physical exam findings are not consistent with congenital muscular torticollis or if the deformity does not respond to conservative treatment.  MRI has been performed on some patients for research purposes. Findings include increased thickness of the SCM muscle (2-4x larger than the contralateral side) in younger patients whereas atrophy and fibrosis was seen in older patients.  MRI of the central nervous system can be considered in atypical torticollis or prior to surgical intervention if the deformity does not improve with conservative measures. 

Etiology:

There are currently many theories regarding the etiology of congenital muscular 
torticollis. These are outlined below
  • Birth Trauma
    •  Compression on the neck leads to venous compression of the sternocleidomastoid musculature. Ultimately, this result in compartment syndrome and resultant fibrosis of the sternocleidomastoid musculature.
  • In utero crowding
    • Supported by the fact that children with uncomplicated deliverieshave congenital muscular torticollis.
    • Also supported by the association with other “packaging” problems such DDH and metatarsus adductus.
  • Neurogenic Myopathy
    • A combination of trauma and ischemia lead to progressive fibrosis, which entraps the SCM branch of the spinal accessory nerve. This nerve compression leads to contracture and deformity.
  • Mesenchymal Precursor Cells
    • Cell lines from embryogenesis remain in the SCM musculature with ability to differentiate into myoblasts and fibroblasts. If fibroblast differentiation predominates then excessive collagen is formed and results in a scar-like contracture.

Treatment:

The mainstay of treatment is nonoperative with manual stretching exercises, caregiver education, and environmental modifications. The primary technique of stretching the SCM involves rotating the chin towards the ipsilateral shoulder while tilting the head toward the contralateral shoulder.  The range of motion and cosmetic appearance is adequate in greater than 90% of patients following this treatment.  In patients with resistant pathology or who present after 1 year of age then a lengthening of the SCM muscle may be considered.  The ideal age to consider surgery is controversial with some authors advocating delaying until closer to school age.  

Complications:

  • Plagiocephaly
  • Facial asymmetry
  • Limited range of motion
  • Missed diagnosis of congenital cervical dislocation, cervical instability, or a neurogenic cause of torticollis
  • Surgical complications related to SCM lengthening
    • Poor cosmesis with abnormal contour of the SCM column related to early surgery
    • Injury to the SCM branch of the spinal accessory nerve
    • Recurrence

Differential Diagnosis:

  • Vertebral anomalies (congenital vertebral anomalies or atlantoaxial rotatory subluxation
  • Ocular torticollis
  • Trauma / fracture
  • Inflammatory (Grisel’s syndrome)
  • Tumors of cervical spine / CNS
  • Paroxysmal torticollis of infancy
  • Sandifer syndrome (reflux associated with torticollic posture)

References:

  1. Canale ST, Griffin DW, Hubbard CN. Congenital muscular torticollis. A long-termfollow-up. J Bone Joint Surg Am. Jul 1982;64(6):810-816.
  2. Cheng JC, Au AW. Infantile torticollis: a review of 624 cases. J Pediatr Orthop.1994 Nov-Dec 1994;14(6):802-808.
  3. Herman MJ. Torticollis in infants and children: common and unusual causes.Instr Course Lect. 2006;55:647-653.
  4. Kaplan SL1, Coulter C, Fetters L.  Physical therapy management of congenital muscular torticollis: an evidence-based clinical practice guideline: from the Section on Pediatrics of the American Physical Therapy Association. Pediatr Phys Ther. 2013 Winter;25(4):348-94.
  5. Lee YT, Yoon K, Kim YB, et al. Clinical features and outcome of physiotherapy inearly presenting congenital muscular torticollis with severe fibrosis onultrasonography: a prospective study. J Pediatr Surg. Aug 2011;46(8):15261531.
  6. Lee YT, Cho SK, Yoon K, et al. Risk factors for intrauterine constraint areassociated with ultrasonographically detected severe fibrosis in early congenitalmuscular torticollis. J Pediatr Surg. Mar 2011;46(3):514-519.
  7. Loder RT. The Cervical Spine. Lovell and Winter's Pediatric Orthopaedics.Philadelphia, PA: Lippincott Williams & Wilkins; 2014.
  8. Morrison DL, MacEwen GD. Congenital muscular torticollis: observationsregarding clinical findings, associated conditions, and results of treatment. JPediatr Orthop. 1982;2(5):500-505.
  9. Robin NH. Congenital muscular torticollis. Pediatr Rev. Oct 1996;17(10):374-375.
  10. Sudesh P, Bali K, Mootha AK, Dhillon MS. Results of bipolar release in thetreatment of congenital muscular torticolis in patients older than 10 years of age.J Child Orthop. Jun 2010;4(3):227-232.

Top Contributors:

Grant Hogue MD