1. Congenital Muscular Torticollis and Plagiocephaly:
Evidence-based Evaluation and Intervention
Theresa Miller-Ferri, MPT, PCS
Physical Therapy Coordinator
The Children’s Institute
November 2012

2. At the conclusion of this presentation, participants will be able to:
Objectives
At the conclusion of this presentation, participants will be able to:
Define congenital muscular torticollis (CMT)
Outline differential diagnoses
Identify the anatomy generally involved with CMT
Understand possible etiologies of CMT
Demonstrate an awareness of key components of examination and assessment of a child with CMT
Identify possible implications of CMT in a variety of areas of function and development
Identify when referrals to other professionals is indicated
Follow recommended treatment guidelines and discharge criteria
Identify appropriateness of bracing, surgery, and other interventions

3. Torticollis “wry neck”
Condition occurring in which the head is held in side-bending toward one side and rotated toward the other side
Usually resulting from unilateral fibrosis and shortening of the sternocleidomastoid (SCM) muscle; may also involve scalenes, upper trapezius, and other muscles of the face and neck
Plagiocephaly (facial and skull asymmetry) often present

5. Case Study:
Male infant referred at 2 months of age
Resting position in supine: with 15 degrees left lateral flexion and 30 degrees right rotation
Passive lateral flexion 40 degrees to the right and 45 degrees to the left
In supine: active rotation to the right 80 degrees, and to the left 20 degrees once positioned in midline
Plagiocephaly Severity Score 6/15
History of GI reflux – not well managed

6. Congenital Torticollis
A neck deformity primarily involving unilateral shortening and fibrosis of the SCM that is detected at birth or within the first two months of life
Can be sub-divided into three types:
Torticollis with a palpable mass or tumor in the tight SCM (SMT)
Muscular torticollis - Without a palpable mass or tumor in a tight SCM (MT)
Postural torticollis – that w/ all the clinical features of torticollis but w/ no demonstrable tightness nor pseudotumor of the SCM (postural head tilt, late-onset ocular torticollis, SCM imbalance, “positional preference”?) (POST)
** the term “Congenital Muscular Torticollis” (CMT) often includes all of these groups
** the “involved” side is the side of tightness or in which a mass is present – therefore, a child with right-sided involvement would generally present with right cervical lateral flexion and left rotation as their preferred position

7. In one study by Cheng, et al 1, (group of orthopedists) of 1,086 CMT infants:
42.7% had a pseudotumor ( found clinically in lower 1/3 of SM (35%), mid-1/3 of SM (40.4%), upper 1/3 (11.9%), in the whole muscle (12.6%) )
Tumor ranged in size from 1cm-4cm w/ over 70% >2cm
Had higher incidence of breech presentation (19.5%), difficult labor including higher rate of forceps delivery and vacuum extraction (56%), and hip dysplasia (6.81% - compared to 1.9% in MT group and .9% in postural group)
Tend to be referred earlier
30.6% had muscular torticollis
22.1% had postural torticollis

8. Spasmodic Torticollis 9
A clinically diagnosed movement disorder in which many authors describe psychologic accompaniments
Incidence ~ 1 in 100,000 w/ either insidious or an abrupt onset
Abrupt onset is usually secondary disease rather than the subtle onset of idiopathic disease
Tx. options vary but usually include psychologic and oral pharmacologic therapies
Usually marked by involuntary hyperkinesis of neck musculature resulting in abnormal head postures or sustained movements of the head
Usually occurs in later life

9. Acute Torticollis 10 (in children, adolescents, and young adults)
Characterized by atlantoaxial rotary fixation (rotational subluxation) of sudden onset
Usually attributed to synovial fold entrapment in the C1 –C2 interspace or possibly a tear and invagination of capsular ligaments about the atlantoaxial synovial joints
Rarely may be caused by an ear or upper respiratory infection
Often a h/o trauma
Sometimes a click is heard at onset
In up to 25% of cases, no clear cause may be identified
Tx. is dictated by the cause

10. Cause of CMT (the jury is still out on this one!)
Birth trauma
Cheng, et al 1 showed an increased incidence of difficult labor and assisted delivery in the group w/ pseudotumor present in the SCM
Tang, et al 3 did a histopathological study using light and electron microscopy in which results were inconclusive and could not support the “trauma theory”

11. Cause continued……… Intra-uterine malposition
increased incidence of CMT w/ breech position, multiple births, other musculoskeletal anomalies
Endogenous Origin – being the result of an anomaly in the muscle blastoma itself
Ischemia
Heredity
Neurogenic
Infection
Compartment syndrome

12. Conditions that may present as torticollis, but w/o involvement of the SCM or other muscle:
Cervical vertebrae subluxation (refer to orthopedics)
Posterior fossa tumor (refer to neurology)
Spinal abnormalities (refer to orthopedics)
Ocular problems (refer to opthalmology)

13. CMT Related Incidences:
Incidence of CMT reported as .3% - 1.9%
Ratio of boys : girls (3 : 2)
Ratio of right vs. left involvement (varies per study, but appears to be left > right)
Presence of a mass in the SCM (25% 4 – 43% 1)
complicated labor and delivery (29% 4 – 62% 5)
Breech presentation (16% 4 – 28%) ….. 34% incidence of torticollis in single breech (head and knees extended) presentation 11
Hip dysplasia (2.5% % % 4 ) – most consistent in literature, but up to 29% in some studies, and generally ipsilateral to SCM involved
Developmental asymmetry (~25%)

14. Incidences, continued…..
Cranial facial asymmetry (80% 5 – 90% 1 )
Spinal anomalies (2%)
Other musculoskeletal anomalies (6% 1,5 ) including varus toes, metatarsus adductus, postural and structural talipes equino varus, calcaneal valgus foot
Obstetrical paralysis 11 (Erb’s Palsy, Klumpke’s Palsy, whole arm palsy)
In study by Suzuki, et al ...
torticollis developed in 51% of babies who had O.P. (26% in cephalic presentations, 80% in breech presentations)
Torticollis on same side as paralysis in 95% of those w/ O.P.

15. Physical Therapy Diagnosis
Physical Therapists’ diagnoses “identify the impact of a condition on function at the level of the system (especially the movement system) and at the level of the whole person.” The diagnostic label indicates the primary dysfunction addressed by physical therapy interventions.
The PT Diagnostic Classification pattern typically most consistent with torticollis is Impaired Posture (4B)
Guide to Physical Therapy Practice, 2003

16. Evaluation and Assessment Guidelines for a Child Referred to PT for Torticollis:
History
Pregnancy, labor, delivery (vaginal vs. c-section, vertex vs. breech) including any complications
Presence of skull and/or facial asymmetry at birth
Birth weight, length, and gestational age
Any medical complications since birth
Breast or bottle fed (any feeding difficulties)
Any known familial history of torticollis or other musculoskeletal problems

17. Sleep position
Amount of time spent on stomach and in positioning devices
h/o the specific problem of head position and neck motion
Identify the primary caregivers
Has child been seen by an orthopedist or neurologist? Any x-rays taken?
Any changes in symptoms over time

18. Screening Neurological
ATNR, abnormal muscle tone, presence of sustained clonus
Hip
Asymmetry, hip clunk, leg length discrepancy
Vision
Midline visual focus, tracking, ocular alignment
Integumentary
Clinical appearance of skin
Pain
Appropriate pain scale

19. Physical Examination Begin with general observation of the infant
Resting position of head (record degree of rotation and lateral flexion) in supine, prone, and supported sitting (if possible)
If child is old enough to sit or stand unsupported, measure from anterior and posterior views as any cranial or facial asymmetry may effect your measures
Resting position of trunk and limbs
assess for plagiocephaly and cranial facial asymmetry, including location, by examining the head from the front, back, side, and looking down on the top of the head
Check spine and limbs for other musculoskeletal deviations including hips for Ortalani sign or limitations in abduction that may indicate dysplasia (would warrant an orthopedic referral)

20. AROM
Observe and elicit limb movements to assess ROM as well as functional strength
Measure cervical rotation (if infant is unable to rotate out of preferred position, may need to bring infant’s head closer to midline before asking them to turn away)
Assess visual tracking
If infant is > than 4 months of age, can assess active cervical lateral flexion through elicitation of vertical head righting response
Muscle Function Scale (MFS) for Infants

21. PROM
Trunk (may find tightness on side of tight SCM) and extremities (remember, typical infants are more flexible in some areas and tighter in others compared to children and adults, so be sure of what you are measuring!)
Cervical rotation ( degrees is normal for an infant, 90 degrees = chin in line with shoulder)
an arthrodial goniometer is helpful
Cervical lateral flexion (infants can have up to 90 degrees = ear to same shoulder)
– be aware of any facial asymmetry that may affect your alignment (an arthrodial goniometer is helpful)
** stabilize shoulders when taking measurements!

22. Palpation
Find both portions of SCM on each side and compare (may need to laterally flex away and rotate head toward same side to find the muscle)
Does one side feel tighter than the other?
Is one SCM more pronounced than the other?
Is there a mass present? If so, measure it and define it’s location.
Is the upper trapezius or other cervical musculature tight as well?

23. Development Pull to sit - Protective reactions
Age-appropriate gross and fine motor skills
If infant is rolling, make sure they do so over both sides, as they tend to prefer to roll over the side ipsilateral to the SCM involved
Look for symmetry of active use, particularly UE use
May see persistent ATNR on involved side
In many cases, a decrease in use of the UE ipsilateral to the involved SCM is present
Pull to sit - Protective reactions
Head righting - Equilibrium reactions
Trunk righting

25. Appropriate Referrals
Opthalmology – visual dysfunction is observed
Orthopedics – fails hip screen; patient presents with a bony end feel in cervical ROM; child is 18 months or older at presentation
Neurology – fails neurological screen
Craniofacial Clinic – child demonstrates Plagiocephaly Type I – V (has any degree of skull or facial asymmetry = immediate referral)

26. Treatment Recommendations
Initial Visit23
Treatment should begin at time of initial evaluation
Caregiver instruction in home program
Education on basic pathology of CMT
Education on environmental adaptations and positioning to reduce deformational forces to face and skull, and to encourage active movement out of preferred cervical position; stretching tight muscles out of preferred position

27. Second and Subsequent Visits23
2nd visit should occur within two weeks of initial evaluation
Review/parent demonstration of current HEP
Update HEP as indicated with additional exercises, developmental activities and environmental adaptations as appropriate
Reassessment of cervical ROM, skull/facial shape, posture/positioning
Developmental activities as appropriate
Screen of trunk, UE, and LE ROM

28. Possible complications of stretching:
SCM spasm
Rupture or “snapping” of the muscle (akin to a manual myeotomy) – signs include a sudden “giving way” of the muscle, sudden increase in ROM, bruising, a “snapping” sound (chance increases w/ severity of torticollis and in infants <2 months old)
Fracture of clavicle
Respiratory and circulatory changes

29. Frequency and Progression of Intervention
Refer to Cincinnati Children’s Hospital Medical Center: Evidence-based clinical care guideline for Therapy Management of Congenital Muscular Torticollis

30. Of note:
- Plateaus and/or regression in active ROM
and/or midline control may occur during periods of:
* fatigue
* illness
* growth spurt
* gross motor progression
- Even after the time of discharge from therapy,
caregivers should resume their HEP during these times.
- If the above persists for more than days, a
new therapy assessment may be indicated

31. Surgery may be indicated if…
After 6 months of treatment:
Child has less than 75 degrees of cervical rotation
Persistence of palpable tumor
Child is 18 months or older at time of initial presentation to therapy
** bracing and intensive therapy are required after surgery

32. Plagiocephaly Asymmetry of the cranium 13
Synostotic plagiocephaly – primary to abnormal sutural development; occipitoparietal flattening caused by lambdoid synostosis (premature closure of the lambdoid suture in the skull), rare ~3/100,000 births
deformational plagiocephaly – secondary to abnormal forces acting on an intrinsically normal, developing cranium

34. Deformational Posterior Plagiocephaly
There has been a rather dramatic increase in the incidence of deformational plagiocephaly from before 1992, from 1/300 births to 1/60 births or as high as 48%, depending on the sensitivity of the criteria used to make the dx. 15
Attributed to the initiation in 1992 of the “Back to Sleep” (supine sleeping) campaign to reduce the incidence of Sudden Infant Death Syndrome (SIDS)
It is estimated that a newborn spends some 700 hours on the back of his soft skull in the first 2 months of life22

35. Insurance required 2 months of aggressive positioning before would consider paying for a helmet
Insurance also required sequential computer scans of the skull to determine if positioning was effective
Mother also took considerable convincing

36. Benik Contralateral Torticollis Bracing System23
Consists of a neoprene cap, trunk orthotic, and two straps (Y = facilitate active contraction of contralateral SCM; I = facilitate active contraction of contralateral upper trapezius and/or decrease rotational element of the Y strap)
Trial during several sessions before ordering for child

37. Benik23 Indications Contraindications
Child must be at least 4 months of age and not responded to standard conservative treatment
Child must be receiving standard treatment
Child has a consistent 5-degree lateral head tilt
Child must have at least 10-degrees lateral flexion toward the contralateral side
Child must have active head righting reactions to lift away from the involved side
Caregivers must demonstrate adequate ability to supervise child with the device on and to properly don/doff the system
C-spine not cleared by physician based on previous x-ray results (cervical x-rays may not be indicated for all CMT patients)
Family non-compliance or decreased safety awareness; inability to properly supervise child when using system, and/or to properly don/doff the system

38. Benik23 Benefits Limitations
Constant support to maintain cervical midline during gross motor activities
Supports the weak SCM in its proper resting length to promote more effective contraction
Longer lever arm with straps vs. tape
Limited evidence, no formal research
May be challenging to don/doff
Straps/vest may need further adjustment during wear
Very hot to wear, excessive sweating
Difficult to keep clean with child toilet training
not flame resistant, use during only active, waking hours
Monitor closely for increased balance issues
Monitor skin sensitivity
Do not use over open sores

39. TOT Collar:
Consists of: a loop of flexible tubing with adjustable height plastic posts that are fitted on the tight side to prevent lateral flexion to that side, yet allow movement in other directions, and a velcro closure
The TOT collar is NOT a passive device – the child needs to have sufficient active lateral flexion to be able to move away from the device – their head should not be resting on the collar
Should be worn ONLY while the child is AWAKE AND SUPERVISED

40. TOT Collar Indications Contraindications
Child at least 4 months of age and not responded adequately to conservative treatment
Child must be receiving standard conservative treatment
Child must have a consistent 5-degree or greater lateral head tilt
Child must have adequate cervical ROM (i.e. at least 10-degrees lateral flexion towards the non-involved side)
Child must have positive head righting reactions to lift head away from side of collar on involved side
Karmel-Ross, 1997
C-spine not cleared by physician based on previous x-ray results
NOTE: cervical x-rays may not be indicated for all CMT patients
Family non-compliance or decreased safety awareness of TOT collar

41. Intervention Tools cont.
Kinesio Taping Techniques
“Kinesio Tex tape can be used to assist in facilitating over lengthened muscles and promoting optimal alignment in infants and children with torticollis.” Kase, Martin, Yasukawa, 2006

42. Kinesio Tape23 Benefits Limitations
Supports muscles
Removes congestion
Corrects joint problems
Activates endogenous analgesic system
Skin sensitivity/poor integrity
Application difficulty
Caregiver willingness to have child wear tape for multiple days and in public

43. Kinesio Tape23
Always test for hypersensitivity using a test patch for at least 24-hours
Inhibit muscle function by taping from insertion to origin
Facilitate muscle function by taping from origin to insertion
For a small baby, Hypafix underwrap may be enough (no tape needed)

44. Manual Therapy Techniques23
In conjunction with stretching
Evidence does not support use of manual therapy techniques alone for the treatment of CMT, but myofascial release, craniosacral therapy, massage, and heat may be beneficial to address joint and fascial system issues
Should be used with caution and ONLY with proper training

45. Factors leading to longer durations of treatment and overall outcomes include:
Older age at presentation
Being in SMT group
Significant initial rotation limitation
Breech presentation at birth
Complications/difficulties at birth
Side of torticollis (Right worse than Left)

46. Prognosis23 Fair: Poor: Excellent:
Positional preference as opposed to muscle tightness; under age 4 months
Good:
Cervical rotation limitation of 10 degrees or less; age 0-8 months
Fair:
Cervical rotation limitation of degrees; over age 8 months
Poor:
Cervical rotation limitation over 30 degrees; over age 12 months

47. Discharge Criteria
Cervical PROM and AROM is within 5 degrees of normal limits for lateral flexion and rotation
Demonstrate symmetrical posture in all functional positions
Head is held in midline most of the time during activity
Demonstrate symmetrical and age-appropriate gross motor skills

48. References
Cheng, Tang, Chen, Wong, Wong: The Clinical Presentation and Outcome of Treatment of Congenital muscular Torticollis in Infants – A study of 1,086 Cases. Journal of Pediatric Surgery, Vol 35, No 7 (July), 2000: pp
Celayir, Aysenur Cerrah: Congenital muscular torticollis: Early and intensive treatment is critical. A prospective study. Pediatrics International (2000) 42,
Tang, Liu, Quan, Qin, Zhang: Sternocleidomastoid Pseudotumor of Infants and Congenital Muscular Torticollis: Fine-Structure Research. Journal of Pediatric Orthopaedics, Vol 18(2), March/April 1998, pp
Emery, C.: The Determinants of Treatment Duration for Congenital Muscular Torticollis. Physical Therapy/Vol 74, No. 10/Oct. 1994, pp
Cheng JC, Au AW: Infantile torticollis: a review of 624 cases. Journal of Pediatric Orthopaedics, 1994 Nov-Dec; 14(6): 802-8
Binder H, Eng G, Gaiser J, Koch B: Congenital Muscular Torticollis: Results of Conservative Management with Long-Term Follow-up in 85 cases. Arch Phys Med Rehabil Vol 68, April 1987 pp
Leung, Leung: The Efficacy of Manipulative Treatment for Sternomastoid Tumours. The Journal of bone and joint surgery. British volume 1987 May;69(3):473-8

49. Morrison DL: MacEwen GD: Congenital Muscular Torticollis: Observations Regarding Clinical Findings, Associated Conditions, and Results of Treatment. Journal of Pediatric Orthopedics 1982;2(5):500-5
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Maigne J; Mutschler C; Doursounian L: Acute Torticollis in an Adolescent. SPINE Vol 28, No 1 E13-E15
Suzuki S; Yamamuro T; Fujita A: The Aetiological Relationship Between Congenital Torticollis and Obstetrical Paralysis. International Orthopaedics (SICOT) (1984) 8:
Boere-Boonekamp M; van der Linden-Kuiper L: Positional Preference: Prevalence in Infants and Follow-Up After Two Years. Pediatrics 2001; 107;
Mulliken J; Woude D; Vander; Hansen M; LaBrie R; Scott M: Analysis of Posterior Plagiocephaly: Deformational versus Synostotic. Plastic and Reconstructive Surgery Vol 103(2) Feb 1999,
Pollack I; Losken W; Fasick P: Diagnosis and Management of Posterior Plagiocephaly. Pediatrics 1997;99;
Persing J; James H; Swanson J; Kattwinkel J: Prevention and Management of Positional Skull Deformities in Infants. Pediatrics Vol 112 No 1 July 2003, pp
O’Broin E; Allcutt D; Earley M: Posterior Plagiocephaly: proactive conservative management. British Journal of Plastic Surgery (1999), 52, 18-23

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Hunt CE; Puczynski MS: Does Supine Sleeping Cause Asymmetric Heads? Pediatrics 1996 Jul;98(1):127-9
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51. Burch, C, Hudson, P, Reder, R, Ritchey, M, Strenk, M, Woosley, M;
Cincinatti Children’s Hospital Medical Center: Evidence-based clinical
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Torticollis,
ev-based/otpt.htm, Guideline 33, pages 1-13,
24. Karmel-Ross, Karen “Torticollis: Differential Diagnosis, Assessment, and Treatment,
Surgical Management and Bracing”

52. Other sources
Clarren SK; Smith DW; Hanson JW: Helmet Treatment for plagiocephaly and congenital muscular torticollis. The Journal of Pediatrics 1979 Jan;94(1):43-6
Fulford FE; Brown JK: Position as a cause of deformity in children with cerebral palsy. Dev Med Child Nerol 1976 Jun;18(3):305-14
Rekate HL: Occipital plagiocephaly: a critical review of the literature. J Neurosurg 1998 Jul;89(1):24-30
Jacques C; Karmel-Ross K: The Use of Splinting in Conservative and post-Operative Treatment of Congenital Muscular Torticollis. Torticollis: Differential Diagnosis, Assessment and Treatment, Surgical Management and Bracing, 1997 by The Haworth Press, Inc
Burstein F: Long-term Experience with Endoscopic Surgical Treatment for Congenital Muscular Torticollis in Infants and Children: A Review of 85 cases. Plast Reconstr. Surg 101:20, 1998
Canale ST; Griffin DW; Hubbard CN: Congenital Muscular Torticollis. A long-term follow-up. J Bone Jount Surg Am 1982 Jul;64(6):810-6
Cheng, et al.: Clinical Determinants of the Outcome of Manual Stretching in the Treatment of Congenital Muscular Torticollis in Infants: A Prospective Study of Eight Hundred and Twenty-one cases. The Journal of Bone and Joint Surgery, Incorporated Vol 83-A(5) May 2001,

53. Cheng, et al.: Snapping During Manual Stretching in Congenital Muscular Torticollis. Clinical Orthopaedics and Related Research No 384,
Lehtinen P; Ruuskanen O; Sonninen P: An Unusual life threatening cause of torticollis in a child. Archives of Disease in Childhood Vol 88(4) April 2003, 349-
Ngu B; Khanna J; Pak S; McCarthy E; Sponseller P: SPINE Vol 29, No 5, E98-E100
Walsh JJ; Morrissy RT: Torticollis and hip dislocation. Journal of Pediatric Orthopedics 1998 Mar-Apr;18(2):219-21
Hummer CD; MacEwen GD: The Coexistence of Torticollis and Hip Dysplasia Sep;54(6):1255-6
Ohman, A; Beckung, E: Reference Values for Range of Motion and Muscle Function of the Neck in Infants. Pediatric Physical Therapy; 53-58

54.
(Symmetric Designs LTD – TOT collar)
(DOC band)
(STAR band)
Lots of terrific kids and their families – many thanks!!!