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Congenital Muscular Torticollis and Plagiocephaly: Evidence-based Evaluation and Intervention

Congenital Muscular Torticollis and Plagiocephaly: Evidence-based Evaluation and Intervention Theresa Miller-Ferri, MPT, PCS Physical Therapy Coordinator The Children ’ s Institute November 2012. Objectives At the conclusion of this presentation, participants will be able to:

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Congenital Muscular Torticollis and Plagiocephaly: Evidence-based Evaluation and Intervention

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  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. 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

  4. 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

  5. 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

  6. 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

  7. 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

  8. Acute Torticollis10 (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

  9. 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”

  10. 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

  11. 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)

  12. 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% 5 - 4% 1 - 9% 4 ) – most consistent in literature, but up to 29% in some studies, and generally ipsilateral to SCM involved • Developmental asymmetry (~25%)

  13. 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.

  14. 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

  15. 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

  16. 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

  17. 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

  18. 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)

  19. 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

  20. 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 (100-110 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!

  21. 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?

  22. Development 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

  23. 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)

  24. 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

  25. 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

  26. 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

  27. 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 • http://www.cincinnatichildrens.org/service/j/anderson-center/evidence-based-care/evidence-based-guidelines/

  28. 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 10-14 days, a new therapy assessment may be indicated

  29. 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

  30. 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

  31. 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

  32. 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

  33. 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

  34. Benik23Indications 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

  35. Benik23Benefits 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

  36. 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

  37. TOT CollarIndications 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

  38. 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

  39. Kinesio Tape23Benefits 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

  40. 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)

  41. 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

  42. 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)

  43. Prognosis23 • 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 10-30 degrees; over age 8 months • Poor: • Cervical rotation limitation over 30 degrees; over age 12 months

  44. 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

  45. 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 1091-1096 Celayir, Aysenur Cerrah: Congenital muscular torticollis: Early and intensive treatment is critical. A prospective study. Pediatrics International (2000) 42, 504-507 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 214-218 Emery, C.: The Determinants of Treatment Duration for Congenital Muscular Torticollis. Physical Therapy/Vol 74, No. 10/Oct. 1994, pp 921-929 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. 222-225 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

  46. 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 Smith D; DeMario M: Spasmodic Torticollis: A Case Report and Review of Therapies. The Journal of the American Board of Family Practice Vol 9(6) Nov/Dec 1996 435-441 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) 8:175-181 Boere-Boonekamp M; van der Linden-Kuiper L: Positional Preference: Prevalence in Infants and Follow-Up After Two Years. Pediatrics 2001; 107;339-343 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, 371-380 Pollack I; Losken W; Fasick P: Diagnosis and Management of Posterior Plagiocephaly. Pediatrics 1997;99;180-185 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 199-202 O’Broin E; Allcutt D; Earley M: Posterior Plagiocephaly: proactive conservative management. British Journal of Plastic Surgery (1999), 52, 18-23

  47. Clarren SK: Plagiocephaly and torticollis: Etiology, natural history, and helmet treatment. The Journal of Pediatrics 1981 Jan;98(1):92-5 Loveday B; de Chalain T: Active Counterpositioning or Orthotic device to Treat positional Plagiocephaly? The Journal of Craniofacial Surgery Vol 12(4) July 2001, pp 308-313 Hunt CE; Puczynski MS: Does Supine Sleeping Cause Asymmetric Heads? Pediatrics 1996 Jul;98(1):127-9 Jantz J; Blosser C; Fruechting L: A Motor Milestone Change Noted With a Change in Sleep Position. Archives of pediatrics and Adolescent Medicine Vol 151(6) June 1997, 565-568 Dewey C; Fleming P; Golding J; and the ALSPAC Study Team: Does the Supine Sleeping Position Have Any Adverse Effects on the Child? II. Development in the First 18 months. Pediatrics 1998;101;5 Stellwagen L; Hubbard E; Vaux K: Look for the “stuck baby” to identify congenital torticollis. Contemporary Pediatrics May 2004, 55-65

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