4/22/2021

Torticollis and Cranial Deformity in Infants Interventions and Impacts on Gross Motor Development

Danielle Rupp, PT, DPT

Course Objectives

● Review typical Congenital Muscular Torticollis (CMT) presentation and common cranial deformities (Plagiocephaly, Brachycephaly, Scaphocephaly) ● Understand key points of assessment ● Review Clinical Practice Guidelines for treatment of CMT ● Discuss various treatment strategies and specific interventions ● Identify when to refer out during assessment of an infant with CMT ● Discuss roll of CMT and cranial deformity on gross motor milestones and development ● Explore long term outcomes of untreated CMT and cranial deformity ● Determine prognosis, treatment frequency and discharge criteria

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Presenter’s Bio - Danielle Rupp, PT, DPT

● Undergraduate: University of Delaware - majoring in Exercise Physiology with concentrations in both Biomedical Engineering and Disabilities Studies ● Graduate : University of Delaware Doctor of Physical Therapy ● Career: Pediatric outpatient physical therapy for 7 years, treating kids from birth to age 21 with a wide variety of diagnoses

Torticollis

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Torticollis

In Latin: Tortus = twisted & Collum = neck

● Torticollis describes the tilting of an infant's head to one side with rotation to the opposite side.

Credit: Kim Lephart, PT, DPT, MBA, PCS - Treating Torticollis: What’s in your toolbox? Evidence-based Assessments & Interventions

Description

● Head tilt to one side & chin points to opposite shoulder ● Limited range of motion makes it difficult for baby to turn head side to side ● Flattening to one side of the face and head may occur

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Differential Diagnosis

● Ocular Torticollis ○ Abnormal head position due to ophthalmic conditions ○ Driven by a conscious or unconscious effort to maintain horizontal line of orientation (head righting) ● Neurologic Torticollis ○ Benign Paroxysmal Torticollis – Periodic episodes of torticollis, due to decreased blood flow to the brain, may randomly alternate side to side ● Brachial Plexus Injury ○ Scarring and contracture of SCM causes head to be pulled down and towards affected arm ● Cervical dystonia(Spasmodic torticollis) ○ Constant contraction of muscles force movement of the neck ○ Believed to be a disease of the basal ganglia ● Orthopedic Torticollis ○ Bony and spinal deformities that can cause malalignment of the neck ● Gastro-Intestinal Torticollis: Sandifer’s Syndrome ○ Combination of gastro-esophageal reflux (GERD) disorder along with spastic torticollis and dystonic body movements

Muscular Torticollis

● Two layers of muscle ○ Superficial (long neck muscles) ■ The sternocleidomastoid is the most targeted muscle ■ Bony attachments: sternum (sternum furcula) and clavicle (two-thirds medial) occipital region (two-thirds side of the neckline) and mastoid apophysis ■ Fibers have an obliquely upward and outward direction ○ Deep (paravertebral muscle)

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Muscular Torticollis

● SCM Tumor (50%) ○ Hard, immobile, fusiform swelling in the SCM ○ Develops between 7-14 days of life ○ Increases in size for 2-4 weeks and plateaus for 1 month ○ Decreases in size until disappears by 5-8 months ○ Most severe form of muscular torticollis ● Congenital Muscular Torticollis (30%) ○ SCM tightness and passive range of motion limitations ● Postural Torticollis (20%) ○ Mildest form of muscular torticollis ○ Infant’s postural preference ○ Without muscular or passive range of motion restrictions

Congenital Muscular Torticollis (CMT)

● CMT is a congenital musculoskeletal disorder characterized by unilateral tightening and shortening of the SCM, with contralateral weakness and over- lengthening ● Present at birth or develops soon after ○ Usually discovered in the first 6 to 8 weeks of life- when newborn begins to gain head control ● Can persist for a year or longer ● Typically unilateral, but in rare cases can be bilateral ● High occurrence in traumatic childbirth ● During the baby's first few weeks, a visible, palpable swelling, or soft lump, known as a sternomastoid tumor, may be present ○ Appears in 50% of cases ○ This lump is not painful and gradually goes away, generally before 6 months of age

Credit: National Center for Biotechnology Information, U.S. National Library of Medicine: https://www.nlm.nih.gov/ The American Academy of Orthopaedic Surgeons: https://orthoinfo.aaos.org/en/diseases--conditions/congenital-muscular-torticollis-twisted-neck

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Incidence

● CMT was considered a rare congenital musculoskeletal disorder ○ 0.3% to 2% of newborn infants or young children ● Recent research studies, however, have noted an incidence as high as 16% ● Male to female predominance: 3 to 2 ratio ● Traumatic deliveries = 2% ○ 19.5% forceps, suction 56% ● More than 50% of cases are first pregnancy ● Diagnosis is generally before two months in 50% of cases; parents identify most cases and may correlate with plagiocephaly

Potential Causes

● The etiology is unknown ● Theories ○ Ischemia ○ Trauma during childbirth ○ Decreased amniotic fluid volume ○ Uterine compression syndrome. ○ Intrauterine malposition (pelvic position) or space (crowding) ■ Having tighter space in the uterus is more common for first-born children ● No known prevention of congenital muscular torticollis

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Cranial Abnormalities & Deformities

Cranial Deformities

● Plagiocephaly ● Brachycephaly ● Scaphocephaly

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Plagiocephaly & Back to Sleep Campaign

● When compared to 1996-1999, Plagiocephaly increased ○ 214% in 2000-2003 ○ 390% in 2004-2007

Head Shape

Normal Head Shape– 3 month Normal Head Shape– 6 month Normal Head Shape– 9 month old old old

Normally, the head is about 1/3 longer than it is wide and rounded at the back.

Credit: Cranial Technologies: https://www.cranialtech.com/plagiocephaly/

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Normal Head Shape– 3 month old

Normal Head Shape– 6 month old

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Normal Head Shape– 9 month old

Plagiocephaly Brachycephaly Scaphocephaly – Head is flat on one side – Head is wider than normal – Head is longer, taller and – Head appears skewed to one – Back of head is flat rather than narrower than normal side from above curved – Unilateral facial asymmetries – Head may be widest above the generally involving ear, eye and ears cheek

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Plagiocephaly

Plagiocephaly means “oblique head” (Greek “plagio” = oblique & “cephale” = head)

● Head is flat on one side ● One ear is more forward than the other ● One eye is smaller than the other ● One cheek is fuller than the other ● Top of the head is not level ● Head shape resembles a parallelogram from above

Plagiocephaly head shapes, ranging from normal to severe.

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Brachycephaly

Brachycephaly means “short head” (Latin “brachy” = short & “cephaly” = head)

● Head is wider than normal ● Head is abnormally tall ● Back of head is flat rather than curved ● Face appears small relative to the size of the head ● Widest part of the head is just above the ears ● Tips of ears protrude ● Head shape resembles a trapezoid from above

Brachycephaly head shapes, ranging from normal to severe.

Scaphocephaly

Scaphocephaly, aka Sagittal synostosis or Dolichocephaly meaning “long head” (Greek) ● Premature closure of the sagittal suture of the skull ● The sagittal suture is located on the top of the head running between the parietal bones from the anterior fontanelle (soft spot) and coronal sutures to the lambdoid sutures ● Causes abnormal growth of the skull resulting in a long and narrow head shape ● Fullness (bossing) of the forehead ● Most common form of isolated (non-syndromic) craniosynostosis (about 50% of all cases) ● Male to female ratio 4:1

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Craniosynostosis

Craniosynostosis is premature fusion of one or more of the sutures in the skull. True synostosis may limit the size of the cranial vault (skull) and therefore impair brain growth.

● Untreated, build-up of pressure in the baby’s skull can lead to blindness, seizures, or brain damage ● 1 in every 2,500 babies in the US ● Many types require surgery Many of the problems a baby can have depend on:

● Which sutures closed early ● When the sutures closed ○ before or after birth and at what age ● Whether or not the brain has room to grow

National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention

How are CMT and Cranial Deformity related?

● Estimated 87% of babies with CMT also had incidence of plagiocephaly ● CMT impacts the way the baby holds his or her head and ultimately assists in developing bad sleeping and positioning habits ● Once the baby gets into a negative pattern, it almost certain that the soft head of a newborn will receive more pressure in one area and ultimately result in a slight misshapen head

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Clinical Assessment of CMT

Key Points of Assessment

● Birth and Medical History ● Body Systems Review ○ Commonly Impacted Systems: Vision, Hearing, Neurologic, GI, Respiratory, Muscolo-skeletal (shoulder, spine, hips and feet), Integumentary ● Palpation ● Muscle Tone ● Positional Preferences and Posture ● Range of Motion ○ Active vs Passive ■ Cervical Lateral Flexion and Rotation ● Strength Assessment ○ Muscle Function Scale ● Physical Assessment ● Gross Motor ○ TIMP or AIMS

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Birth and Medical History

● Age of initial visit ● Age of onset of symptoms ● Pregnancy history ● Delivery history ○ Cephalic vs. breech ○ Vaginal vs. Cesarean ● Use of assistance during pregnancy ○ Forceps ○ Suction ● Family history of torticollis or other congenital/developmental conditions ● Any other known or suspected medical conditions ● Developmental milestones and age reached

Body Systems Review

● Vision ○ Symmetrical eye tracking with emphasis on visual field defects ○ Nystagmus ○ Strabismus ○ Resolution of tilt in supine ● Hearing ● Neurological ● Gastrointestinal ○ History of reflux or constipation ● Cardiorespiratory ○ Symmetrical coloration, rib cage expansion, and clavicle movement ● Musculoskeletal ○ Symmetrical alignment of the shoulder and hip girdles with particular attention to cervical vertebral anomalies, rib cage symmetry and hip dysplasia ○ Symmetrical passive ROM of the neck ● Integumentary ○ Skin fold symmetry of hips and cervical region

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Common Comorbidities

● Craniofacial asymmetry (90.1%) ● Plagiocephaly (87%) Bercik et al (2019) ● Hip dysplasia (up to 20%) ● History of Reflux ○ Peak at 10-12 months ○ 27% vs 5.4% of normal population

Palpation

● Sternocleidomastoid ○ Perform contralateral rotation, ipsilateral lateral flexion, and flexion of the head. ● Splenius ● Trapezius ● Scalenes ● Quadratus Lumborum ● Rib Cage ● Scapulae

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Muscle Tone

● High tone vs Low tone ● Neck and throughout body

Positional Preferences and Posture

● Positioning ○ What is positional preference? ○ What positions does the baby avoid, if any? ○ Recommended: Prone positioning up to 1 hr per day ■ No minimum requirement ■ Shown to offset supine sleeping on motor skill acquisition ● Check Posture ○ Sitting ○ Supine ○ Prone

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Range of Motion - Active vs Passive: Cervical Lateral Flexion and Rotation

● Rotation ○ Passive: ■ Measured in supine, head in neutral, nose aligned with the 90 degree vertical reference ○ Active: ■ Younger than 3 months: tested in supine ■ Older than 3 months: tested in sitting on the clinician’s lap on a rotating stool with the parent enticing the infant to maintain eye contact while the PT rotates the baby away from the parent ● Lateral Flexion ○ Passive: ■ Measured in supine with the shoulders stabilized using an oversized or arthrodial protractor ○ Active: ■ Older than 2 months: the Muscle Function Scale provides an objective strength assessment of active lateral flexion

Credit: Kim Lephart, PT, DPT, MBA, PCS - Treating Torticollis: What’s in your toolbox? Evidence-based Assessments & Interventions

Range of Motion - Reference Values for Range of Motion

● Cervical Rotation: ○ 100◦ - 120◦ (mean 110◦) ■ < 1 year of age: ≥ 100◦

● Lateral Flexion: ○ 65◦ - 75◦ (mean 70◦) ■ <1 year of age: ≥ 65◦

Credit: Kim Lephart, PT, DPT, MBA, PCS - Treating Torticollis: What’s in your toolbox? Evidence-based Assessments & Interventions

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Strength Assessment - Muscle Function Scale

● Objective measurement of active lateral flexion ● Used for infants greater than 2 months ● 6-point scale ● Hold infant in front of a mirror and tip horizontally

Muscle Function Scale for Infants

0 = Head below horizontal

1 = Head on horizontal

2 = Head slightly above horizontal

3 = Head high above horizontal < 45◦

4 = Head high above horizontal > 45◦

5 = Head almost vertical > 75◦

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Physical Assessment

● Feet check ○ Association between hip dysplasia and clubfoot (one in 54 neonates = 1.9%) ● Pectus excavatum ● Ribs and Pelvis alignment ● Face symmetry ○ Eye and ear size and position, pupils, checks, forehead bossing, jaw symmetry ● Hands to midline/cross body ● Spine – curvature/flattening ● Scapulae & shoulder elevation and symmetry ● Quad/Creep/Crawl ● Transitions: pivot, sit to stand, stand to squat, rolling ● Prone suspension ● Pull to sit – head lag or tilt ● Pull to stand ● Weight Shift & Weight Bearing side preference ● Balance, Righting, Protective responsive

Gross Motor Standardized Assessment Tools

● Test of Infant Motor Performance (TIMP)

○ 34 weeks post-conceptional age to 5 months post-term

● Alberta Infant Motor Scale (AIMS) ○ Infants 0-18 months

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TIMP

● Motor outcome measure used to assess posture and selective control of movement needed by infants under four months of age for functional performance in daily life ● The TIMP was developed to ○ 1) identify infants with delayed motor development ○ 2) discriminate among infants with varying degrees of risk for poor motor outcome ○ 3) measure change resulting from intervention ● Age Range ○ Between 34 weeks post-conceptional age to 5 months post-term ● Validity with Alberta Infant Motor scale (AIMS) ○ Correlation between scores on the TIMP and the AIMS was highest ■ TIMP tests at 90 days ■ AIMS testing at 6 months

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AIMS

Purpose: ● To measure the motor development for infants at risk for motor delay, focusing on attaining motor milestones and components necessary to attain the milestones. ● The AIMS takes into consideration three criteria related to quality of movement: weight distribution, posture and movement against the force of gravity. Type of Test: ● Performance based, norm-referenced observational measure Target Population and Ages: ● Infants 0-18 months or until child is able to independently walk

Test Administration

Scoring:

The AIMS consists of 58 items, including 4 positions

● Prone (21 items) ● Supine (9 items) ● Sitting (12 items) ● Standing(16 standing)

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Does symmetry of the movements matter?

“In scoring bidirectional motor items, such as rolling, pivoting, and cruising, the examiner should use clinical judgment. If after observing the infant move, the examiner has no concerns about symmetry, the infant may be credited for a bidirectional item, even though it was observed in only one direction. In most instances an infant will move spontaneously in both directions… If, however, the examiner has doubts about the infant’s ability to move symmetrically, the infant should be encouraged to perform the item in both directions. If, in this instance, the examiner is unable to motivate that infant to move in both directions and there is still a concern about symmetry, the item should be scored as ‘not observed’.”

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Torticollis Severity Grading

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Grading Severity

Grade 1: Early Mild

Grade 2: Early Moderate

Grade 3: Early Severe

Grade 4: Late Mild

Grade 5: Late Moderate

Grade 6: Late Severe

Grade 7: Late Extreme

Grade 1: Early Mild ● Infant presents between 0 and 6 months ● Postural preference or muscle tightness less than 15 degrees of cervical rotation Grade 2: Early Moderate ● Infant presents between 0 and 6 months ● Muscle tightness of 15-30 degrees of cervical rotation Grade 3: Early Severe ● Infant presents between 0 and 6 months ● Muscle tightness of more than 30 degrees of cervical rotation or an SCM mass

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Grade 4: Late Mild ● Infant presents between 7 and 9 months ● Postural preference or muscle tightness of less than 15 degrees of cervical rotation Grade 5: Late Moderate ● Infant presents between 10 and 12 months ● Postural preference or muscle tightness of less than 15 degrees cervical rotation Grade 6: Late Severe: ● Infant presents between 7 and 12 months ● Muscle tightness of more than 15 degrees cervical rotation Grade 7: Late Extreme: ● Infant presents after 7 months with an SCM mass OR ● Infant presents after 12 months with muscle tightness of more than 30 degrees cervical rotation

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Clinical Assessment of Cranial Deformity

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Assessment Tools for Head Shape

● Argenta Classification ○ A validated and reliable tool for classifying progressive head shape deformities ○ Five ratings for plagiocephaly and three for brachycephaly and scaphocephaly ● Cranial Anthropometric Measures ○ Manual cranial measurements using tape measurer or calipers ● Laser Guided Scans ○ 3-D laser guided imaging

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Cranial Anthropometrics

l. Measurements

● Head Circumference ○ glabella & opisthocranion (g. & op.) ● Head Length (aka A/P) ○ glabella to opisthocranion (g. & op.) ● Head Width (aka M/L) ○ eurion to eurion (eu & eu) ● Head Oblique ○ ipsilateral fronto-temporal point to contralateral lambodial point (ft & l.)

Cephalic Ratio (CR) and Cranial Vault Asymmetry Index (CVAI)

CR CVAI

● Measures head length vs head ● Measures longest and shortest width diagonals from forehead to ● CR should be < 90 posterior skull

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Cephalic Ratio (CR) Cranial Vault Asymmetry Index (CVAI)

(AKA Cranial Index (CI) )

CVAI = (A-B) x 100

CR = (M/L) A or B

(A/P) x (whichever is larger) 100

Example CR = (M/L) CR = 270 (A/P) x 100 310 x 100

CR = 87 Measurements CVAI = (A-B) x 100 ● Head Circumference = 490mm

● Head Length (A/P) = 310mm A or B (whichever is larger) ● Head Width (M/L) = 270mm ● Right to Left Head Oblique = 315mm ● Left to Right Head Oblique = 290mm CVAI = (315 - 290) x 100

315

CVAI = 7.9

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Laser Guided Scans

Ex: STARscanner

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Clinical Practice Guidelines for CMT

Clinical Practice Guidelines for treatment of CMT

The CPG for PT treatment of infants with CMT is intended as a reference to guide physical therapy practice and to inform the need for continued research

● 16 action statements organized under 4 major headings

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Action Statements

Action Statement 1: Identify newborns at risk for torticollis

Action Statement 2: Refer Infants with asymmetries to physician and physical therapist

Action Statement 3: Document Infant History

Action Statement 4: Screen Infants

Action Statement 5: Refer infants from physical therapist to physician if red flags are identified.

Action Statement 6: Request Images and Reports

Action Statement 7: Examine body structures

Action Statement 8: Classify the Level of Severity

Action Statements

Action Statement 9: Examine Activity and Developmental Status Action Statement 10: Examine Participation Status Action Statement 11: Determine Prognosis Action Statement 12: Provide First Choice Interventions Action Statement 13: Provide Supplemental Interventions Action Statement 14: Refer for Consult When Outcomes Are Not Fully Achieved Action Statement 15: Document Outcomes and Discharge Infants From Physical Therapy When Criteria Are Met Action Statement 16: Provide a Follow-up Screening of the Infant 3-12 Months Post Discharge

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Treatment for CMT

Treatment Strategies

First Choice Interventions

● Neck PROM ● Neck and trunk active ROM ● Development of symmetrical movement ● Environmental adaptations ● Parent/caregiver education

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Supplemental Interventions

Interventions with Evidence of Efficacy Interventions without Evidence of Efficacy

● Microcurrent ● Cervical Manipulation ● Myokinetic stretching ● Soft tissue massage ● Kinesiotaping ● Craniosacral therapy ● TAMO approach ● Total Motion Release ● TOT collar ● Feldenkrais Method ● Soft foam collars ● Surgery ● Custom-fabricated cervical ○ Approximately 10% of children with orthosis CMT require surgery ● Surgery/Botox ○ Typically in preschool years. ○ Lengthen the short SCM ● Helmets

Treatment for Cranial Deformity

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Treatment - Positioning vs Cranial Remodeling Orthosis

● Sutures generally close by 18 months ○ Need orthosis before this age ● Also need head control generally for helmet, but can use before head control emerges ○ 12 weeks ● One study found toddlers with plagiocephaly continue to exhibit developmental delays compared to toddlers without plagiocephaly, especially in cognition & language ○ IMPORTANT: These findings do not necessarily imply a causal relationship between plagiocephaly and development because children with delays may be more likely to develop plagiocephaly! ● Red Flag: skull shift is opposite of what you’d expect based on torticollis side

Positioning Devices

1. Tortle Beanie 2. Boppy Noggin Nest Head Support 3. Britax Head and Body Support 4. Diono 2 in 1 Head Support 5. Original Baby Elephant Ears Head Support Pillow 6. Babymoov Lovenest Newborn Head Support Pillow 7. Baby Side Sleep Pillow

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1. Tortle Beanie 2. Boppy Noggin Nest Head Support

3. Britax Head and Body Support 4. Diono 2 in 1 Head Support

5. Original Baby Elephant Ears 6. Babymoov Lovenest Head Support Pillow Newborn Head Support Pillow

7. Baby Side Sleep Pillow

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Cranial Orthoses, Helmets and Bands

Types:

● Active ○ Occasionally referred to as “dynamic” orthotics ○ Most common variety available ○ More snug and form fit to individual’s head ● Passive ○ Not as snug as active orthotics

*Primary difference in the two categories is the snugness of the helmet or band’s fit, which affects the overall amount of pressure applied to the baby’s skull at any point

https://www.babyflathead.org/helmet-and-cranial-band-options-for-plagiocephaly-and-brachycephaly/

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Active Helmets and Bands STARband ● Comprised of ½ inch of foam below a hard copolymer coating ● Adjustments as needed, checked every two weeks, depending on head growth ● Appropriate for moderate to severe cranial deformities STARlight Band ● Lightweight, ¼ inch clear plastic ● Can be molded using heat ● Ideal for young infants who are still learning to rotate and lift their heads ● For moderate and mild brachycephaly and plagiocephaly, not best design for use with scaphocephaly STARband Bi-valve ● Ideal for use with scaphocephaly STARlight Bi-valve ● Ideal for post-operative scaphocephaly patients STARlight Cap ● One-piece, clear plastic orthosis ● Lightweight and ideal for younger infants ● Used to treat mild brachycephaly, plagiocephaly and scaphocephaly

Active Helmets and Bands

DOC Band

● 6-ounce orthotic that is customized ● Hard plastic shell with a foam liner ● Weekly to bi-weekly follow up visits are required to adjust the band’s fit ● First FDA approved post-operative cranial remolding orthotic and the first marketed and approved cranial band Hanger Cranial Band

● Similar to DOC Band and STARband ● Available only through Hanger’s 670 clinics.

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Passive Helmets and Bands

Clarren Helmet

● Appropriate for infants ranging from 6-18 months of age with mild to severe cranial deformations ● Does not have any side or top openings apart from air holes ● Fastened with a chin strap ● Comprised of a 3/8” polypropylene cover with a 1/8”aliplast liner ● Does not require frequent follow-up visits, and only minimal adjustments can be made to the liner ● Can be used in combination with cranial shunts and following surgery due to the loose fit it provides. Boston Band

● Lightweight, comprised of a flexible plastic outer shell and a foam liner ● Fit is based on a mold or 3D scan ● Acceptable for the treatment of scaphocephaly, plagiocephaly and brachycephaly ● Has both side and top holes, and is a side opening band ● Available only through Boston Brace and their partner programs (NOPCO Clinical Network and other partners ● Claims to require less time for successful treatment than other available options

Treatment Time

● Ideal onset time for cranial remodeling treatments is 5-6 months of age ○ Treatment can begin at any point before one year of age and still produce decent results ● Orthotics manufacturers provide guidelines for the use of their individual products based on studies that they have conducted and reports from parents and care providers ● Results will vary from child to child and depend on the severity of the condition. ● Most products are designed for 3-4 months of therapy on average ● Average daily wear recommendations are for 23 hours/day

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Clarren Helmet Boston Band

Gross Motor

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Typical Gross Motor Development Outline

2 Months 9 Months ● Can hold head up and begins to push up when lying on ● Stands, holding on tummy ● Can get into sitting position ● Makes smoother movements with arms and legs ● Sits without support 4 Months ● Pulls to stand ● Holds head steady, unsupported ● Crawls ● Pushes down on legs when feet are on a hard surface ● May be able to roll over from tummy to back 12 Months ● Can hold a toy and shake it and swing at dangling toys ● Brings hands to mouth ● Gets to a sitting position without help ● When lying on stomach, pushes up to elbows ● Pulls up to stand, walks holding on to furniture (“cruising”) 6 Months ● May take a few steps without holding on ● Rolls over in both directions (front to back, back to front) ● May stand alone ● Begins to sit without support ● When standing, supports weight on legs and might bounce 18 Months ● Rocks back and forth, sometimes crawling backward ● Walks alone before moving forward ● May walk up steps and run

CDC Guidelines - U.S. Department of Health & Human Services

Roll of CMT and Cranial Deformity on Gross Motor Milestones and Development

● Infants with CMT are shown to have a higher incidence of persisting developmental delay ● If developmental delay is observed remediation of delay should be addressed in plan of care

*Guideline Development Group (GDG) recommends TIMP for infants through 4 months corrected age and AIMS for infants from 4-18 months corrected age

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Tummy Time!

The effects of prone positioning on the quality and acquisition of developmental milestones in four-month-old infants Dudek-Shriber et al (2007)

“Specifically, the results of this study also appear to suggest that those infants who spent slightly more than an hour or more in the prone position while awake per day achieved greater success in acquiring certain prone, supine, and sitting milestones that begin developing incrementally at approximately four months of age.”

Comparison of time spent awake in prone position for four-month old infants who did and did not achieve milestone (n = 100)

Dudek-Shriber et al (2007)

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Motor Function in School-Aged Children With Positional Plagiocephaly or Brachycephaly

Objective: ● To determine whether children with a history of positional plagiocephaly/brachycephaly (PPB) show persistent deficits in motor development.

Methods: ● In a longitudinal cohort study, we completed follow-up assessments with 187 school-aged children with PPB and 149 participants without PPB who were originally enrolled in infancy. Primary outcomes were the Bruininks-Oseretsky Test of Motor Proficiency-Second Edition (BOT-2) composite scores.

Results: ● Children with PPB scored lower than controls on the BOT-2. Stratified analyses indicated that differences were restricted to children who had moderate-severe PPB. No consistent differences were observed in children who had mild PPB.

Conclusion: ● Children who had moderate-severe PPB in infancy show persistent differences in motor function. We suggest close developmental monitoring and early intervention to address motor deficits.

Collett et al (2020)

Do Infant Motor Skills Mediate the Association Between Positional Plagiocephaly/Brachycephaly and Cognition in School-Aged Children?

Objective ● Positional plagiocephaly/brachycephaly (PPB) is associated with lower cognitive scores in school-aged children. This study tested the hypothesis that infant motor skills mediate this association. Methods ● Children with a history of PPB (cases, n = 187) and without PPB (controls, n = 149) were followed from infancy through approximately 9 years of age. Infant motor skills were assessed using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-3), and cognition was assessed using the Differential Ability Scales, 2nd edition (DAS-2). The Bayley-3 motor composite was examined as a mediator of the association between PPB and DAS-2 general cognitive ability (GCA) scores. In secondary analyses, mediation models were examined for the DAS-2 verbal ability, nonverbal ability, and working memory scores; models using the Bayley-3 fine versus gross motor scores also were examined. Results ● Cases scored lower than controls on the DAS-GCA (β = −4.6; 95% CI = −7.2 to −2.0), with an indirect (mediated) effect of β = −1.5 (95% CI = −2.6 to −0.4) and direct effect of β = −3.1 (95% CI = −5.7 to −0.5). Infant motor skills accounted for approximately 33% of the case–control difference in DAS-2 GCA scores. Results were similar for other DAS-2 outcomes. Evidence of mediation was greater for Bayley-3 gross motor versus fine motor scores. Conclusion ● Infant motor skills partially mediate the association between PPB and cognition in school-aged children. Monitoring motor development and providing intervention as needed may help offset associated developmental concerns for children with PPB. Brent et al (2021)

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Long Term Outcomes with Gross Motor Delays

● If developmental delay is related to CMT and documented at initial evaluation treatment can continue under current plan of care ● If CMT is truly resolved and developmental delay is independent of CMT ● New prescription required to reflect new diagnosis ● Re-evaluation required ● New plan of care established

Prognosis, Referrals, Home Exercise Programs & Discharge Criteria

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Determining Prognosis

Prognosis for full resolution of CMT treated conservatively

● Prior to 3 months of age is 100% ● After 3 months of age is 75%

Estimated length of episode of care

● For infants younger than 3 months: 1.5-3 months ● Infants older than 3 months or infants who initiate treatment several months or more after diagnosis: 3-6 months ● Recommendations for surgery are typically made after conservative PT intervention after a minimum of 3 months of intervention however frequently following 6-12 months of intervention

Duration Based on CMT Severity

<3 months old

CMT severity of 1-3: <6 months duration

CMT Severity of 4-7: >6 months duration

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Determining Prognosis

● Best Predictor: ○ Severity of ROM restrictions ○ Limitations of cervical rotation >15◦ or SCM mass ○ Older age at diagnosis – Strongly correlated with surgery

● With proper treatment, 90% to 95% of children improve before the first year of life, and 97% of patients improve if treatment starts before the first six months.

Red Flags

● Atypical presentations ○ Tilt and turn to same direction ○ Plagiocephaly and tilt to the same side ● Abnormal tone ● Late-onset torticollis at 6 months or older ● Visual abnormalities ● History of acute onset

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When to Refer

● After 4-6 weeks of initial intense intervention in the absence of red flags, little or no reduction in neck asymmetry is noted ● Suspected hip dysplasia ● Asymmetries of the head, neck and trunk are not resolving after 4-6 weeks of initial intense treatment ● After 6 months of treatment with only moderate resolution ● If the infant is older than 12 months on initial examination and either facial asymmetry and/or a 10-15 degree difference persists between Left and Right sides ● The infant is older than 7 month on initial examination and a tight band or SCM mass is present ● If the side of the torticollis changes

FREQUENCY DETERMINATION

Age at onset of treatment

0 – 4 months Follow every other week

4 – 5 months Follow weekly

6 – 8 months Follow weekly

9 – 12 months Follow weekly

12 – 18 months Follow once every 1-2 weeks until child is an independent ambulator

18 – 36 months Initiate treatment, follow once every 1-2 weeks while awaiting specialty consults

Credit: Kim Lephart, PT, DPT, MBA, PCS - Treating Torticollis: What’s in your toolbox? Evidence-based Assessments & Interventions

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FREQUENCY DETERMINATION

Decrease to every other week:

● Good compliance with HEP ● Improving ROM ● Good developmental progression

Increase to 2x/week:

● Poor compliance with HEP ● Family needs more hands-on-training

Credit: Kim Lephart, PT, DPT, MBA, PCS - Treating Torticollis: What’s in your toolbox? Evidence-based Assessments & Interventions

Home Exercise Program

Seattle Children’s Hospital :

https://www.yumpu.com/en/document/read/26175285/torticollis-and-your-baby- seattle-childrens

https://www.seattlechildrens.org/globalassets/documents/for-patients-and- families/pfe/pe347.pdf

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Home Exercise Program - Tips

Tips:

● Engage the visual system ● Be patient ● Timing during the day and around other activities (eat and sleep) ● Create frequency around already performed tasks ● Modify to increase endurance

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Home Exercise Program - Caution

Caution:

● Do NOT push! ● Careful around jaw

Criteria for Discharge

● Full PROM within 5 degrees of the non-affected side ● Symmetrical active movement patterns throughout the passive range ● Age-appropriate motor development ● No visible head tilt ● The parent/caregiver knows what to monitor as the child grows

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Quick Stat Reference

● CMT prevalence was 0.3-2% (1 in 250 =0.4%), however, as recent as 2014 incidence rates were as high as 16% ● 75% of the time the right side of the neck is affected ● SCM Tumor (50%) Congenital Muscular Torticollis (30%) Postural Torticollis (20%) ● Sternomastoid tumor present in 50% of cases ● Male to female ratio 3 : 2 ● More than 50% of cases of CMT are first pregnancy ● 50% of CMT diagnosed before 2 months ● Approximately 10% of children with CMT require surgery ● 90-95% of babies with CMT improve before first year of life & 97% improve if treatment starts before first 6 months ● Common Comorbidities with CMT: Craniofacial asymmetry (90.1%), Plagiocephaly (87%), Hip dysplasia (up to 20%), Reflux (Peak at 10-12 months with 27% vs 5.4% of normal population) ● Estimated 87% of babies with CMT also had incidence of plagiocephaly ● Plagiocephaly increased 214% in 2000-2003 and 390% in 2004-2007 compared to 1996-1999 ● Scaphocephaly is the most common form of isolated (non-syndromic) craniosynostosis (about 50% of all cases), with male to female ratio 4:1 ● Ideal onset time for cranial remodeling treatments is 5-6 months of age

Questions?

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References

● Ballock RT, Song KM. The prevalence of nonmuscular causes of torticollis in children. J Pediatr Orthop 1996;16:500-504. ● Bercik, Deborah PT; Diemer, Susan PT, PCS; Westrick, Stephanie PT, DPT; Worley, Sarah MS; Suder, Ryan OTR/L, BCP, PhD Relationship Between Torticollis and Gastroesophageal Reflux Disorder in Infants, Pediatric Physical Therapy: April 2019 - Volume 31 - Issue 2 - p 142-147 doi: 10.1097/PEP.0000000000000592 ● Bohnsack BL, Bhatt R, Kahana A. Non-Ophthalmic Symptoms Secondary to Ocular Torticollis From Severe Blepharoptosis: An Underappreciated But Treatable Condition. Ophthalmic plastic and reconstructive surgery 2012;28(2):e36-e39. doi:10.1097/IOP.0b013e3182191be3. ● Boyko N, Eppinger MA, Straka-DeMarco D, Mazzola CA. Imaging of congenital torticollis in infants: a retrospective study of an institutional protocol. J Neurosurg Pediatr. 2017 Aug;20(2):191-195 ● Branch L, Kesty K, Krebs E, Wright L, Leger S, David L. Deformational Plagiocephaly and Craniosynostosis: Trends in Diagnosis and Treatment after the “Back to Sleep” Campaign. Journal of Craniofacial Surgery. 2015;26(1):147-150. ● Brent R Collett, PhD, Erin R Wallace, PhD, Cindy Ola, PhD, Deborah Kartin, PT, PhD, Michael L Cunningham, MD, PhD, Matthew L Speltz, PhD, Do Infant Motor Skills Mediate the Association Between Positional Plagiocephaly/Brachycephaly and Cognition in School-Aged Children?, Physical Therapy, Volume 101, Issue 2, February 2021, pzaa214 ● Collett B, Breiger D, King D, Cunningham M, Speltz M. Neurodevelopmental Implications of “Deformational” Plagiovephaly. J Dev Behav Pediatr. 2005 October; 26(5):379- 389. ● Collett, Brent R. PhD; Kartin, Deborah PT, PhD; Wallace, Erin R. PhD; Cunningham, Michael L. MD, PhD; Speltz, Matthew L. PhD Motor Function in School-Aged Children With Positional Plagiocephaly or Brachycephaly, Pediatric Physical Therapy: April 2020 - Volume 32 - Issue 2 - p 107-112 doi: 10.1097/PEP.0000000000000687 ● Collet B, Starr J, Kartin D, Heike C, Berg J, Cunningham M, Speltz M. Development in Toddlers with and without deformational plagiocephaly. Arch Pediatr Adolesc Med. 2011 Jul; 165(7):653-658. ● Darrah J, Bartlett D. “Infant rolling abilities – the same or different 20 years after the back to sleep campaign” Early Human Development. 2013;89(5):311-314. ● Do TT. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006 Feb;18(1):26-9 ● Dudek-Shriber L, Zelazny S. The effects of prone positioning o n the quality and acquisition of developmental milestones in four-month-old infants. Pediatr Phys Ther 2007;19(1):48-55. ● Graham R, Mancher M, Wolman DM, Greenfield S, Steinberg E. Clinical Practice Guidelines We Can Trust. 2011, Washington, DC. Retrieved from http://www.nap.edu/catalog.php?record_id=13058 ● Gray GM, Tasso KH. Differential Diagnosis of Torticollis: A Case Report. Pediatr Phys Ther. 2009;21:369-374. ● Han MH, Kang JY, Do HJ, Park HS, Noh HJ, Cho YH, Jang DH. Comparison of Clinical Findings of Congenital Muscular Torticollis Between Patients With and Without Sternocleidomastoid Lesions as Determined by Ultrasonography. J Pediatr Orthop. 2019 May/Jun;39(5):226-231 ● Ho BC, Lee EH, Singh K. Epidemiology, presentation and management of congenital muscular torticollis. Singapore Med J. 1999 Nov;40(11):675-9

● Jawadi AH (2016) Clubfoot and Congenital Muscular Torticollis Prevalence in Children Diagnosed with Developmental Dysplasia of Hip: Review of 594 DDH Patients. Pediat Therapeut 6: 288. doi:10.4172/2161-0665.1000288 Pediat erapeut ISSN:2161-0665 Pediatrics, an open access journal Volume 6 • Issue 2 • 1000288 ● Kaplan SL, Coulter C, Sargent B. Physical Therapy Management of Congenital Muscular Torticollis: A 2018 Evidence-Based Clinical Practice Guideline From the APTA Academy of Pediatric Physical Therapy. Pediatr Phys Ther. 2018 Oct;30(4):240-290 ● Klackenberg EP, Elfving B, Haglund-Akerlind Y, Carlberg EB. Intra-rater reliability in measuring range of motion in infants with congenital muscular torticollis. Adv Physiother. 2005;7:84-91. ● Kluba S, Kraut W, Reinert S, Krimmel M. What is the optimal time to start helmet therapy in positional plagiocephaly? Plastic & Reconstructive Surgery. 2011;(128)2:492-498. ● Kuo AA, Tritasavit S, Graham JM. Congenital muscular torticollis and positional plagiocephaly. Pediatr Rev. 2014 Feb;35(2):79-87; quiz 87 ● Majnemer A, Barr RG. Influence of supine sleep positioning on early motor milestone acquisition. Developmental Medicine & Child Neurology 2005;47: 370-376. ● Petronic I, Brdar R, Cirovic D, Nikolic D, Lukac M, Janic D, Pavicevic P, Golubovic Z, Knezevic T. Congenital muscular torticollis in children: distribution, treatment duration and outcome. Eur J Phys Rehabil Med. 2010;45(2):153-158. ● Persing J, James H, Swanson J, et al. Prevention and management of positional skull deformities in infants. American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery. Pediatrics. 2003;112(1 Pt 1):199-202. Available at: http://pediatrics.aappublications.org/content/112/1/199.full. Accessed January 18, 2015. ● Pleva, Anne DPT; Pediatric Clinical Specialist; Bruk, Aimee Parent Commentary on “Associations Between Congenital Muscular Torticollis Severity and Physical Therapy Episode”, Pediatric Physical Therapy: October 2020 - Volume 32 - Issue 4 - p 321 doi: 10.1097/PEP.0000000000000735 ● Seruya M, Oh A, Taylor J, Sauerhammer T, Rogers G. Helmet Treatment of Deformational Plagiocephaly: The Relationship between Age at Initiation and Rate of Correction. Plastic & Reconstructive Surgery. 2013;(131)1:55e-61e. ● Sestokas L, Rawlini R, Rawlini V, Connor C, Vicari F. Treatment Efficacy of Deformational Plagiocephaly and Brachiocephaly: Results of a Large, Prospective Clinical Trial. Plastic & Reconstructive Surgery. 2012:130(5S-1):17. ● van Vlimmeren LA, Helders PJ, van Adrichem LN, Engelbert RH. Torticollis and plagiocephaly in infancy: therapeutic strategies. Pediatr Rehabil. 2006 Jan-Mar;9(1):40-6. ● Wei JL, Schwartz KM, Weaver AL, Orvidas LJ. Pseudotumor of infancy and congenital muscular torticollis: 170 cases. Laryngoscope. 2001 Apr;111(4 Pt 1):688-95

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