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Toe walking and TorticollisToe walking and Torticollis

Carolyn Forsman, MD

April 20, 2012

Pediatric Physical Medicine and Rehabilitation

Why do These 2 Topics go Together?

• Both toe-walking and torticollis are common pediatric presentationspresentations

• The management of each is controversial and there is much discussion about the natural course and potential long-term consequences, as well as conservative vs. aggressive treatment

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Toe Walking

Torticollis

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Objectives

• Define toe walking and formulate a differential diagnosis of toe walking

• Explain the treatment options of idiopathic toe walking

• Define torticollis and formulate a differential diagnosis of torticollis

• Explain the treatment options of torticollis

• Provide appropriate referral for evaluation of toe walking and torticollis

Normal Gait Pattern

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Normal Gait Pattern

First Rocker

Second Rocker

Normal Gait Cycle

• http://www.youtube.com/watch?v=occFkFbl3ms

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Normal Gait Development

• The normal toddler gait is wide-based with excessive knee and hip flexionknee and hip flexion

• Toddlers walk with a flat-foot or on their toes

• In typically developing children, consistent heel strike during initial stance occurs by 18 months of age OR at a mean time frame of 22.5 weeks (about 5 and a half months) after the onset of independent ambulation

Burnett, et al; Sutherland, et al.

Normal Gait Development

• Reciprocal arm swing also develops by 18 months

• Idiopathic toe-walking has been described as a normal variant of early gait by some, but this is not supported by the literature

• Gait characteristics should be mature by 5 years of age

Burnett, et al; Sutherland, et al.

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Toe walking is defined as the failure of the heel to contact the floor at the onset of stance during gait:

Video of Toe Walking

• http://www.youtube.com/watch?v=IQRkSrmcH5E

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Differential Diagnosis of Toe-Walking• Idiopathic Toe Walking

• Cerebral Palsy: secondary to an injury to the brain; spasticity causes one to walk on their toes; also has abnormal tone in their hamstrings, hip flexors, adductors; clonus is present

• Congenital triceps surae contracture: present at birth with foot in equinovarus

• Pervasive Developmental Disorder: autism spectrum, often behavioral

• Myopathy: weakness causes the patient to compensate their gait patterngait pattern

• Peripheral Neuropathy (i.e. Charcot Marie Tooth): again causes weakness

• Tethered Spinal Cord: secondary to a spinal dysraphism

Toe Walking in Cerebral Palsy

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Initial Evaluation of Toe-Walking - History

• Was the child born prematurely? - More concern for cerebral palsycerebral palsy

• Were there any heel cord contractures at birth?

-Think about a congenital contracture

• Are there any other concerns for developmental delays? - Could be PDD, CP

• Is there a family history of toe walking? - More likely to be idiopathic, could also be a myopathy or neuropathy

Initial Evaluation of Toe-Walking - History

• Is the child having any foot or leg pain? - Concern for spasticityspasticity

• Are there balance problems? - Ataxia may lead down more of a neurologic workup

• Is there any bowel or bladder dysfunction?

- Spinal cord more likely involved

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Initial Evaluation of Toe-Walking –Physical Exam

• Gait observation - toe walking is often clinically less obvious when walking in shoes vs. barefootobvious when walking in shoes vs. barefoot

• Neurologic exam –check strength, reflexes, look for clonus

• Passive and active lower extremity range of motion -normal ankle dorsiflexion is 15 to 20 degrees

Ankle Dorsiflexion

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Idiopathic Toe-Walking (ITW)

• Probably the same condition as congenital short tendo calcaneus

• Also known as habitual toe walking• Also known as habitual toe walking

• Always bilateral and symmetrical

• Incidence appears to be as high as 5/500 births

• Can be autosomal dominant with a familial incidence of to 32%

• True etiology is not known

• ITW is a diagnosis of exclusion

Sala, et al.

Idiopathic Toe-Walking

• Children with ITW have abnormal muscle firing when studied by EMG (electromyography)studied by EMG (electromyography)

• There is abnormal co-contraction of the tibialis anterior muscle (controls dorsiflexion) and the gastrocnemiusmuscle (controls plantarflexion)

• Children with ITW can temporarily control their gait to walk with a normal heel-toe pattern when cued, but it iswalk with a normal heel toe pattern when cued, but it is not lasting

Eastwood, et al.

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Eastwood, et al.

Gait Deviations Seen in Toe-Walking

• Ankle plantarflexion in stance and swing phase

• Lack of first rocker

• Lack of second rocker

• Decreased push-off

• Premature heel-off

• Out-toeing (to compensate)

• Knee hyperextension

• Increased anterior pelvic tilt

Sutherland, et al.

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Gait Deviations

Other Implications of Toe Walking

• Positive correlation between language delays and toe-walkingtoe walking

• Positive correlation between learning disabilities and toe-walking

• Potential correlation with anecdotal evidence between sensory processing dysfunction and toe-walking, but not confirmed by the literatureconfirmed by the literature

Shulman, et al.

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Natural History of Toe-Walking• This is controversial

• The consensus is that true toe-walking persists into adulthood although may improve without intervention inadulthood although may improve without intervention in up to 50%

• Some studies say that toe-walking resolves on its own as the patient gets heavier with age

• “Outgrowing” of toe walking is more likely due to other compensatory changes:

F t ti– Foot pronation– Excessive external tibial torsion– Out-toeing

• http://www.youtube.com/watch?v=IQRkSrmcH5E

Shulman, et al.

Consequences of Prolonged walking on One’s Toes…• Gastrocnemius, soleus, and Achilles tendon tightness is

acquired after years of toe-walking making treatment much more difficult

• This can result in a fixed equinus contracture

• Limitation in ankle dorsiflexion passive range of motion is associated with increased frequency of ankle injuries in children

• Limitation in ankle dorsiflexion is also associated with increased forefoot, midfoot, and/or hindfoot pain in adulthoodc eased o e oot, d oot, a d/o d oot pa adu t ood

• Older children with a history of toe-walking often demonstrate excessive external tibial torsion with an increased positive thigh foot ankle and out toeing to accommodate their plantar flexion contracture.

Shulman, et al; Tabrizi, P.

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Consequences of Prolonged Walking on One’s Toes…

• Type II vs. Type I muscle fibers in the gastrocnemius(tonic, slow-contracting, fatigue resistant) thought to be(tonic, slow contracting, fatigue resistant) thought to be adaptive (there are actual histological changes!)

• Risk of increased lumbar lordosis with spondylolysis

• Risk of osteochondritis dessicans of the talus and/or femoral condyles

Shulman, et al; Tabrizi, P.

Treatment Options

• Physical Therapy

• Orthotics

• Serial Casting

• Botox

• Surgery

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What Happens When You Refer to PT?• Stretching of ankle plantarflexors

• Strengthening of anterior tibialis muscle

• Strengthening of all other trunk and lower extremity muscles

• Neuromuscular electrical stimulation

• Ankle joint mobilization

• Orthotic intervention

• Treadmill training

• Night splintingNight splinting

• Home exercise program development

• Stretching alone is often not effective likely because there is often only a minimal limitation in range of motion

Physical Therapy Outcomes

The efficacy of therapy alone is dependent upon:

1.The amount of contracture present at the time of evaluation

2.The percentage of time the child spends toe-walking

3. The age of the child at initial evaluation

Older children over the age of 5 years are not as– Older children, over the age of 5 years, are not as likely to be as successful with conservative treatment

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Physical Therapy Goals

• To improve ankle dorsiflexion passive range of motion to greater than or equal to 10 degrees with knee extendedgreater than or equal to 10 degrees with knee extended

• For the patient to achieve heel-toe ambulation at least 75% of the time in spontaneous gait

• For the patient and family to become independence in their home-exercise program

• To maximize the patient’s gross motor skills if there are other delays

Tidwell, et al.

Physical Therapy Setbacks

• Plateaus in range of motion even after successful treatment may occur withtreatment may occur with

– Growth spurt

– Anxiety

– Fatigue

– Illness

– Lack of follow through at home

• If these plateaus last longer than 4 weeks, a therapy reassessment is indicated

Tidwell, et al.

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Orthotics

• Braces are almost always indicated to help reinforce a normal gait pattern wtihout consistent verbal remindersnormal gait pattern wtihout consistent verbal reminders

• Night stretching splints can also be helpful

Articulated vs. Solid

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Serial Casting

• Application of a series of below knee walking casts with the foot in neutral

– Casts are changed ever 1-2 weeks

– 6-8 weeks total

• These can be very effective in the short term:

– Gain ankle dorsiflexion range of motion

– Improve gait EMG with a reciprocal contraction of tibialis anterior and gastrocnemius instead of co-contraction

• Children can relapse

Brouwer, et al.

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Botox

• Botox is a neurotoxin derived from the bacteria Clostridium botulinumClostridium botulinum

• It produces a protein that inhibits the release of acetylocholine and results in temporary localized reduction in muscle activity

• Effects last for up to 3 months

Botox

• It appears that Botox A treatment can normalize the ankle EMG pattern during gait and a more normal foot-strike pattern is obtained

• Botox is often used in association with therapy, casting and/or orthotics

Brunt, et al; Engstrom, P; Jacks, et al.

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Botox Video

• http://www.youtube.com/watch?v=l7l0csoCQkM

Surgery

• Toe walking may persist after all conservative treatment methods, even in the absence of significant Achillesmethods, even in the absence of significant Achilles contracture

• Surgical options include gastroc-soleus lengthening, tendo Achilles lengthening

• Toe-walking can STILL recur

• There is a risk of over-lengthening and functionally weakening the gastrocnemius

• Surgeons usually wait until children are 8-10 years of age

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Other Attempted Treatments

• Supportive shoes

• Auditory biofeedback

• Electrical stimulation

Auditory Biofeedback

• Pressure-sensitive heel switch

• Heel strike makes a sound

• Feedback for at least an hour a day for at least 3 months

• Not good evidence to support

Conrad, et al.

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When Should You Refer?

• If a child is over the age of 2 and continues to walk on their toes, they should be treated

• If you are uncomfortable ordering physical therapy refer• If you are uncomfortable ordering physical therapy, refer to pediatric physical medicine and rehabilitation or pediatric orthopedics

• If you have tried physical therapy and bracing and the child is still toe walking after 6 months to a year, refer for further treatment options

• If the child is older than the age of 5 years old, refer

• If there is any concern that there is an underlying diagnosis other than just idiopathic toe walking, refer appropriately (ie neurology, PM&R, ortho)

Questions???

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Definition and History of Torticollis

“Torticollis” literally means “twisted neck”

Latin “torquere” for twisted and “collum” for neck

It is not a diagnosis, but a word used to describe the twisted posture of the neck

Torticollis be a sign of an underlying pathology or may be benignbenign

Cheng, et al. 2000

Underlying pathologies can include:

• Muscular

• Skeletal

• Neurologic

• Inflammatory

• Neoplastic

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Differential Diagnosis of Torticollis

• Congenital muscular torticollis

• Sternocleidomastoid tumor

• Postural torticollis

more of a preference than a muscle problem

• Posterior fossa tumor

• Hemiplegia –causing weakness and spasticity on one side

• Abnormal vertebral structure

– Klippel-Feil Anomaly (congenital fusion of any 2 cervical vertebrae)

– Hemivertebrae

Cheng, et al. 2000; Cooperman.

Differential Diagnosis of Torticollis (continued)

• Fracture or dislocation of vertebrae – acute onset

• Occular abnormalities – head tilt to try to prevent diplopia

• C1-C2 rotary subluxation – acute onset

• Clavicle fracture – typically a birth injury

• Brachial plexus palsy again typically a birth injury• Brachial plexus palsy – again, typically a birth injury. Causes weakness on one side.

Cheng, et al. 2000; Cooperman.

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Cervical Hemivertebra

Klippel-Feil Anomaly

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Sternocleidomastoid Tumor

Congenital Muscular Torticollis

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Can you tell the difference between each of those pictures?

NOPE!

They all look the same!!!

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Congenital Muscular Torticollis (CMT)• Over 80% of all infants presenting with a torticollis

posture have congenital muscular torticollis

• That means about 20% have one of the other underlying abnormalities!!!

• CMT is observed at birth or early infancy

• CMT results from unilateral fibrosis and shortening of the sternocleidomastoid muscle and/or upper trapezius muscle

• This is the 3rd most common musculoskeletal condition in infants

• The incidence is 0.3%-2%

• The etiology of congenital muscular torticollis appears to be multifactorial

Cheng, et al. 2000

Initial Evaluation of Torticollis - History

• Was the child born prematurely?

More concern for hemiplegia

Wh t th bi th i ht? W th hild l f• What was the birthweight? Was the child large for gestational age?

Intrauterine positioning plays a large role

• Was the infant a multiple?

Again, less room means malpositioning

• Was there any shoulder dystocia?

Question clavicle fracture or brachial plexus injury

• Is the child using both hands equally?

Think about brachial plexus injury

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Initial Evaluation of Torticollis – Physical Exam

• Observation of the infant at rest - look at head position/preferenceposition/preference

• Observe from the front, from above, and from behind- look for head shape and soft tissue deformity

• Palpate the neck musculature - for presence of a mass

• Passive and active range of motion of the neck –should be symmetric, should not have a “hard end feel”

Initial Evaluation of Torticollis – Physical Exam

• Look for symmetry in arm and leg use

• Hip Exam – increased risk for hip dysplasia

• Look at the feet! – check for metatarsus adductus

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Presentation of Torticollis

• Limitation of active and passive neck motion

• Posturing of the neck in lateral flexion to the ipsilateral side

• Rotation to the contralateral side causing chin to point toward the contralateral shoulder

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Three Classifications of CMT1. Sternomastoid tumor group

– A hard mass within the substance of a tight SCM

– Recognized at 1-4 weeks of age

Size of lesion from 1 3 cm– Size of lesion from 1-3 cm

– Firm and smooth and movable beneath the skin

– Reaches maximize size and then recedes within the first year

– Muscle can become fibrotic

2. Muscular torticollis without palpable tumor

3. Postural torticollis– All the clinical features of torticollis

– No tumor or muscle tightness

• Has prognostic significance

Cheng, et al. (2000, 2001)

Work-Up - Imaging

• X-ray of cervical spine (AP and lateral) to look for bony abnormalitiesabnormalities

• Ultrasound to look for fibrosis or tumor

– Confirms diagnosis of congenital muscular torticollis

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Ultrasound

Other Implications of Torticollis

• Can be seen in association with metatarsus adductus

• Can be seen in association with developmental dysplasia of the hip

• Torticollis, metatarsus, and DDH are all associated with fetal intrauterine malposition

– There is a high correlation with breech positioning

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Positional Plagiocephaly

• Over 80% of infants with CMT also present with craniofacial asymmetry and deformational plagiocephaly

Natural History of Torticollis

• 54-70% of sternocleidomastoid tumors resolve within the first year of lifefirst year of life

• Some patients have persistent residual fibrosis without clinical problem

• 9-21% have progression to frank muscular torticollis and clinical deformity

– Similar outcomes in both SCM tumor and muscular torticollis groups

Do, TT.

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Treatment Options

• Physical therapy

• Orthoses

• Botox

• Surgery

What happens when you refer to PT?

• Physical Therapy

P iti i– Positioning

– Environmental adaptations

– Passive stretching of tight SCM

– Active stretching of tight SCM

– Strengthening of weak neck and trunk musclesg g

– Movement therapy

– Home program

• Stretching by caregivers

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Range of Motion

Physical Therapy Outcomes

• Over 90% of children achieve a good to excellent outcome with conservative treatment

• Outcomes are best when therapy is initiated during theOutcomes are best when therapy is initiated during the first 12 months

• Required length of treatment range from 1 to 36 months (average is 4.7 months)

– Longer treatment if:

• SCM tumor

• Right sided involvement

• Associated with birth “difficulties”

• Initial visit at greater than one month old

• Rotation deficit of >15 degreesBinder, et al; Cheng, et al. (2001); Taylor, et al.

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Physical Therapy Goals

• Cervical range of motion within 5 degrees of normal in lateral flexion and rotationlateral flexion and rotation

– Active

– Passive

• Symmetric posture in all functional positions

• Head in midline majority of the time when active• Head in midline majority of the time when active

• Symmetric gross motor skills

Binder, et al; Cheng, et al. (2001); Taylor, et al.

Physical Therapy setbacks

• Plateaus in range of motion or temporary decrease in midline control may occurmidline control may occur

– During gross motor progression (i.e. starts walking, etc)

– During a growth spurt

– With fatigue

– With illness, especially an ear infection

• If these plateaus last more than 10-14 days, a therapy reassessment should occur

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Complications of manual stretching

• Sudden give-way or snapping of the SCM

– Up to 8% of the time

– Increased risk if:

• Hip dysplasia

• Left sided involvement

• Rotational deficit of >15 degrees

• Less than one month old at presentation

Followed by bruising and increased range of motion– Followed by bruising and increased range of motion

– Signifies potential tear or rupture of muscle

– Does not seem to result in increased need for operative treatment

Cheng, et al. (2001); Taylor, et al.

Orthotics – the TOT Collar

• The TOT Collar™ is designed to provide a noxious stimulus to the lateral aspect of the skull. The userstimulus to the lateral aspect of the skull. The user moves away from this stimulus towards a new, central corrected position. Adoption of a new, normal head position provides the ability to reset perception of horizontal and so maintain the corrected head position.

• The TOT Collar use is added to the conservative treatment of infants with congenital muscular torticollis iftreatment of infants with congenital muscular torticollis if they are 4 months of age or older and show a consistent head tilt of 5 degrees or more despite 2-3 months of treatment.

Cottrill-Mosterman, et al.

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The TOT Collar

Botox• Used in combination with therapy

• Goal is to temporarily weaken the affected SCM or upper trapezius muscle to allow for easier and more successful stretching and an improved ability to strengthen opposing neck musculature

• Has been used for years in adults with cervical dystonia

• Low doses used

• Transient adverse affects uncommon (self-limited)– Dysphagia

Neck weakness– Neck weakness

• Used in hopes of eliminating need for surgery

• One study showed 75% of patients with significant improvement– 11% needed repeat injections

– The remainder may still need surgical release

Joyce, et al; Oleszek, et al.

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Surgery• Before the mid 1960’s, surgical release of the SCM

during the first few months of life followed by immobilization was the standard of careimmobilization was the standard of care

• Is more likely needed in. . .

– Children in the SCM tumor group (8%)

– Children with initial deficit in cervical rotation of >30 degrees

– Children who initiate therapy after 12 months

Cheng, et al. (2001); Do, TT.

Surgery (continued)• Goal to is achieve a functional and cosmetically

acceptable outcome

• Never indicated in postural torticollis

• Indications:

– Deficits of passive rotation and lateral flexion greater than 15 degrees

– Presence of a tight band or SCM tumor

f– Inadequate response to therapy after 6 months

• Best results within 6 months to 2 years age range

• Technically difficult, typically involves an orthopedic surgeon and ENT

Cheng, et al. (2001); Do, TT.

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When to think about something other than congenital muscular torticollis…

• Sudden onset – think about a subluxation or a fracture

• Other associated anomalies – think about a vertebral anomaly

• Hard-end feel with passive range of motion – more likely a bony abnormality

• Weakness associated with neck position – consider brachial plexus injury, hemiplegia

When should you refer? • If a child is over the age of 1 and continues to have a

head tilt, you should refer.

• If you are uncomfortable ordering physical therapy, refer to pediatric physical medicine and rehabilitation or pediatric orthopedics.

• If you have tried physical therapy and the child still has a neck preference after 6 months, refer for further treatment options.treatment options.

• If there is any concern that there is an underlying diagnosis other than congenital muscular torticollis, refer appropriately (ie neurology, ortho, neurosurgery).

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When should you refer?

• Ophthalmology

– Referral if visual dysfunction is observed or suspected

• Check midline visual focus

• Look at ocular alignment, check light reflex

– Referral if residual head tilt with adequate range of motion and strength

• Orthopedics

R f l if f l i i– Referral if concern for non-muscular origin

• Bony end feel on cervical range of motion

• Abnormal hip exam

• Abnormal foot exam

Positional Plagiocephaly• A little bit about posterior plagiocephaly and torticollis

– 1992, the AAP introduced the “Back to Sleep” campaign

• The following decade showed a dramatic increase• The following decade showed a dramatic increase in the diagnosis of CMT and plagiocephaly

• It is now considered an “epidemic”!

– Purely a cosmetic problem, but may effect the child psychologically later on

Argenta, et al.

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Argenta, et al.

PT and Positional Plagiocephaly

• Physical therapy intervention for CMT may also improve positional preference and plagiocephalypositional preference and plagiocephaly

• Craniosacral therapy can be helpful

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Cranial remolding orthoses

• Ideal period for initiating is 4-6 months

• Treatment lasts 3 months on average

• Needs to be worn 23 ½ hours every day

• Its well tolerated

• Should always be tried before surgery is considered in patients less than 1 year of agepatients less than 1 year of age

• Only risks are of pressure spots if not well fitted

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Questions???

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