Congenital Muscular Torticollis
Synonyms and related keywords: idiopathic spasmodic
torticollis, IST, involuntary contractions of neck muscles, focal
dystonia, congenital torticollis, birth trauma, intrauterine
malpositioning, acquired torticollis, acute wryneck, painful neck
spasms, cervical muscle spasm, tonic head deviation, clonic head
movements, head torsion, spasmodic torticollis, head shaking,
cervical osteomyelitis, retropharyngeal space infection, occipital
condyle fracture, cervical muscle spasm following motor vehicle
accident, odontoid fractures, cervical disk disease, L-dopa,
neuroleptics, ocular condition, palsy of the inferior oblique muscle,
essential head tremor.
Torticollis Overview
Torticollis is one of a broader category of disorders that
exhibit flexion, extension, or twisting of muscles of the
neck beyond their normal position. In torticollis your neck
tends to twist to one side. The condition can either
develop slowly if you have a family history of the
disorder, acutely from trauma, or as an adverse reaction
to medications.
Child with right SCM
When the disorder occurs in people with a family history,
it is referred to as spasmodic torticollis. The characteristic
twisting of the neck is initially spasmodic and begins
between ages 31-50 years. If you leave the condition
untreated, it likely will become permanent.
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Bending or twisting your neck too far can lead to acute
torticollis. This condition appears with few symptoms,
although often you will appear uncomfortable and will hold
your head straight or rotated to one side. It will hurt to
move your head to the opposite side. Your neck muscles
on the side that hurts often are tender to the touch. The
doctor will check your nerve and motor function to rule
out spinal cord injury.
Certain drugs of abuse such as ketamine, amphetamines,
and cocaine as well as commonly prescribed neuroleptic
drugs such as prochlorperazine (Compazine), haloperidol
(Haldol), and chlorpromazine (Thorazine) can cause acute
dystonia (a lack of normal muscle control). This is a
condition that involves the sudden onset of involuntary
contractions of the muscles of the face, neck, or back. In
addition to bending of the head to one side (acute
torticollis), you may experience deviation of the eyes
(oculogyric crisis) and protrusion of the tongue
(buccolingual crisis).
Background:
Torticollis (from the Latin torti, meaning twisted and collis,
meaning neck) refers to presentation of the neck in a
twisted or bent position. Torticollis manifests in
involuntary contractions of the neck muscles, leading to
abnormal postures and movements of the head. Idiopathic
spasmodic torticollis (IST) is considered a focal dystonia.
What is torticollis?
Torticollis (also called congenital muscular torticollis) is a
condition that causes a baby's head and neck to tilt to one
side. It affects about 2 percent of newborns. The cause is
unknown, but doctors suspect that when a baby is
positioned in the uterus so that her head is tilted to the
side and her neck is down, the blood supply to the neck is
cut off. This results in some tightness in one of the two
strap (or sternocleidomastoid) muscles that connect the
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breastbone, head, and neck, and allow a baby to turn her
neck. Though the baby may look uncomfortable, this
condition causes no pain. About 8 percent of babies born
with torticollis will also have other problems such as hip
dislocation.
Pathophysiology:
Torticollis is a symptom, as well as a disease, and it has a
host of underlying pathologies. Torticollis can be divided
into 2 types: congenital and acquired.
Congenital torticollis is usually not encountered in the ED,
but it is worthy of mention because of its presentation.
Infants born with torticollis appear healthy at delivery, but
over days to weeks, they develop soft-tissue swelling over
an injured sternocleidomastoid. Injury may be due to birth
trauma or intrauterine malpositioning. This mass, which
may be confused with a cystic hygroma or branchial cleft
cyst, regresses and leaves a fibrous band in place of the
sternocleidomastoid muscle, causing contracture of the
neck.
Acquired torticollis has an identical presentation, but it
has a host of underlying pathologies that must be
excluded before diagnosis of IST can be made.
An acute form of torticollis, known as acute wryneck, is
the type most frequently encountered in the ED. Acute
torticollis develops overnight in young and middle-aged
adults. Patients present with painful neck spasms. On
examination, cervical muscle spasm is visible and
palpable. Symptoms usually resolve spontaneously within
2 weeks. Treatment is symptomatic and consists of the
use of heat, massage, supportive cervical collar, muscle
relaxants, and analgesics.
IST is classified in a broad category of dystonic states, as
a type of focal dystonia (ie, dystonic movements in a
single body part). Torticollis may be associated with other
forms of focal dystonia, such as blepharospasm, writer's
cramp, spasmodic dysphonia, or orobuccal dystonia.
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Patients may present with tonic head deviation, clonic
head movements, or both. Head deviation can be
subdivided into lateral tilt or torsion. Patterns are not
fixed and may change over time. The operational
definition of IST contains the following elements:
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Acquired, nontraumatic origin
Adult or childhood onset
Clonic and/or tonic involuntary contractions of
multiple neck muscles
Sustained head torsion and/or tilt
Duration of 6 months or longer
Often associated with postural limb tremor
No history of chronic neuroleptic treatment
No associated ataxia, weakness, spasticity, or reflex
changes
Normal brain CT scan
Frequency:
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In the US: The exact incidence of IST is unknown,
but it is thought to be about 3 per 10,000
individuals.
Mortality/Morbidity: Stress and emotional events may
exacerbate symptoms of torticollis.
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Persistent neck deviation occasionally elicits
avoidance behaviors.
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Considerable somatic and psychological disability
may accompany chronic torticollis.
Sex: IST affects women more often than men, with a
4.5:1 ratio.
Age: IST may occur in children or adults. In 90% of cases,
however, symptom onset occurs in patients aged 31-60
years.
Causes:
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The most common cause of torticollis in the first year of
life is Congenital Torticollis which is more commonly called
Congenital Muscular Torticollis or CMT. Research studies
have put the incident rate of CMT to be 0.04% to as high
as 1.9% (1,2).
There are three different ways that CMT can present
itself:(3)
(1) A sternomastoid tumor that can be felt upon
examination (fibromatosis colli)
(2) Tightness or thickening of the sternomastoid muscle
(3) Torticollis caused by posture preference with no
tightness or tumor.
This exact cause of CMT has not been identified but some
theories have been made that link CMT to bad inutero
positioning or low amniotic fluid levels that restrict the
environment of the growing fetus and prohibit the ability
of the SCM muscle to stretch and grow which results in a
contracted SCM muscle (also called perinatal
compartment syndrome)(4). It is thought that the
shortened muscle may actually be a contributing factor to
a difficult birth and not its consequence. CMT is the third
most common musculoskeletal anomaly(5) after hip
dysplasia and clubfoot.
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IST has no clear etiology, although a lesion of the
thalamus has been suspected.
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In some patients, IST is seen in several generations
of their families.
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Many have relatives with other extrapyramidal
disorders, usually tremor.
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Consider other causes before establishing a diagnosis
of IST.
o
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Infection: Cervical osteomyelitis and
retropharyngeal space infection may be
confused with IST.
Tumors: Tumors may cause IST-like symptoms
by producing a mass effect on the muscle.
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Trauma: Occipital condyle fractures, cervical
muscle spasm following motor vehicle accident,
and odontoid fractures may simulate torticollis.
Cervical disk disease: Subluxation and/or
herniation can cause deviation to one side.
Drug-induced condition: L-dopa and
neuroleptics may induce focal dystonias. Alcohol
may worsen underlying torticollis.
Ocular condition: Patients with palsy of the
inferior oblique muscle hold their heads at an
angle to correct visual disturbances.
Essential head tremor: Tremor may precede or
coexist with IST and can be stopped with
intentional turning of the head. As with IST,
medication lists should be reviewed.
Imaging Studies:
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Plain cervical radiographs may be useful to exclude
bony trauma or osteomyelitis.
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MRIs of the head may help rule out brain tumor.
MRIs also may help if a herniated intervertebral
cervical disk is suspected.
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CT scans are useful to identify and exclude
retropharyngeal abscess or other neck mass. Brain
CT scans may not equal brain MRIs, but they are
more likely to be available to ED physicians.
CMT is most often diagnosed when an infant is 2-3 weeks
old due to the presence of a mass or tumor (fibromatosis
colli) in the belly of the SCM. In infants that do not have a
mass, torticollis is unusually noticed around 2 months old
when the neck muscles begin to develop and the head tilt
becomes clear. Diagnosing this condition involves a
physician taking a medical history (or reading the child's
medical records), performing a physical exam, and if
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needed, scheduling of additional testing. Since there may
be other causes for the torticollis posture, many
physicians will have additional testing performed as a
precaution. This testing includes x-rays of the cervical
spine, ultrasound, MRI, or a CT scan. If the child has a
lump/mass an ultrasound is usually performed with
additional testing of a biopsy or fine needle aspiration to
further study the cause of the lump/mass.
Symptoms:
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Because spasmodic torticollis is an abnormal
contraction of the muscle in one side of the neck,
people will appear with their head turned to one side.
Neck muscles and those between the neck and
shoulder will be tense and tender.
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People with acute torticollis will be unwilling to turn
their head to one side or may have their head turned
slightly away from the side of discomfort.
The infent may hold her head to one side and have
limited neck movement, or she may have a small
bump on the side of her neck. Torticollis is usually
diagnosed within the first two months of a baby's life
because even if parents don't spot it, a pediatrician
will. Babies with torticollis may also develop
positional plagiocephaly (asymmetrical head shape)
because they'll often sleep with their head turned to
the affected side. There are also a few other less
common forms of torticollis. One, called wryneck,
which happens when one of the vertebrae doesn't
develop completely. Another, called acquired
torticollis, is a serious condition that can come on
anytime after birth and is usually an indication of a
more serious problem, such as a brain or spinal cord
tumor. Your doctor may need to take X-rays or
perform other tests to determine which form your
baby has.
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How is it treated?
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If your baby is found to have congenital muscular
torticollis, you'll want to get her started on a
stretching routine to lengthen and stretch her tight
neck muscles. The doctor may show you the proper
exercises (or refer you to a physical therapist), and
you'll be required to perform them on your child
several times a day. The moves aren't complicated,
but if you're not comfortable doing them, ask your
physical therapist to help you. You'll also want to
provide as many opportunities as possible for your
baby to turn her head to the other side. To that end,
you should approach her from the non-affected side,
encouraging her to turn her head in that direction.
You can also place your baby to sleep with her head
turned to the non-affected side. When she's awake,
it's very important to give your baby plenty of time
on her stomach. This will help to develop the muscles
in her neck.
How long does it take to see results?
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You'll probably see improvement within weeks. And
as long as your baby's condition was discovered early
enough — ideally by the age of 2 or 3 months — and
she's following a rigorous stretching program, it
should be fully corrected by age 1. (Your doctor may
refer you to a physical therapist to help stretch the
muscles.) If, however, the muscles do not return to
their normal length and your baby doesn't have a
normal range of motion by the time she's 18 months
old, your doctor may refer you to an orthopedic
surgeon, who may recommend surgery to lengthen
the muscles. Surgery is necessary in about 15
percent of cases.
When to Seek Medical Care
In general, acute torticollis is not life threatening. If
symptoms are limited to muscle stiffness and pain, see
your doctor in at least a day.
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If you injure your neck but cannot reach your doctor, go
immediately to a hospital's emergency department. Other
medical conditions may appear to be torticollis and will
need immediate medical attention. For example, anyone
who experiences spasms of the neck muscles involved
with swallowing or breathing or symptoms that might
involve the central nervous system should be evaluated.
The following symptoms suggest injury or irritation to the
central nervous system:
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Trouble breathing or swallowing
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Pins-and-needles feeling or numbness in your arms
and legs
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Urinary hesitancy, urinary or fecal incontinence
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Weakness in your arms and legs
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Impaired speech
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Difficulty walking
CLINICAL ASSESSMENT:
History:
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Signs and symptoms of torticollis change as the
disease progresses.
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During later stages of the chronic disease, patients
have more pain and difficulty performing motor
activities.
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One sixth of patients report head trauma that
occurred several days, or even months, before
symptom onset.
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Definite spasmodic torticollis develops within 6
months of the first appearance of symptoms in 42%
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of patients, within 6-12 months in 20% of patients,
and after 1 year in 35% of patients.
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Head turning is the most frequent symptom (80% of
cases), and the patient often notes this finding while
driving or attempting to perform a bimanual task
involving direct visualization.
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One fourth of patients learn that their heads are
turned only when others inform them.
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Neck pain occurs in 50% of cases.
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Head shaking occurs in 50% of cases.
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Abnormal posture occurs in 25% of cases.
Exams and Tests
The doctor will take a detailed history emphasizing specific
medications that you may be taking. A physical
examination will be performed.
When there is a history of trauma, the doctor may take xrays of your neck to exclude a fracture or dislocation of
the spinal bones in your neck.
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Often, x-rays are sufficient to make this
determination.
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In a small number of cases, subtle abnormalities or
preexisting conditions, for example, degenerative
arthritis of the spine of the neck, may require a CT
scan of the neck.
Physical ASSESSMENT:
The physical examination should be directed at identifying
underlying etiologies as well as documenting the degree of
muscle involvement.
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Limited cervical range of motion and muscle
contraction of sternocleidomastoid and paracervical
muscles may be present.
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Severe muscle contraction and spasm may be
present in the sternocleidomastoid, levator, splenius,
and trapezius muscles.
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Head deviation to one side, with slight neck flexion,
is the presenting posture.
TREATMENT
Treatment for torticollis is targeted to relax the contracted
neck muscles involved. These treatments include
medication, physical devices, botulinum toxin, and
surgery. In most people, torticollis resolves in several
days to a few weeks. A few people will develop continuing
neck problems for months to years. Persistent neck
muscle spasms may require referral to a neurologist or
surgeon.
Prehospital Care: Immobilize the cervical spine for any
patient with acute torticollis and a history of neck trauma.
Emergency Department Care: Patients with torticollis may
present to the ED because of adverse effects of therapy
for another disease. Identify and appropriately treat the
cause. Review the medication list of all patients with IST
or essential head tremor.
Consultations:
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Refer most patients with symptoms suggestive of IST
to a neurologist for follow-up.
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Since IST progresses slowly and is stable, immediate
consultation is unnecessary.
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Referral to a neurologist specializing in movement
disorders may hasten diagnosis and treatment.
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Drugs of choice for IST include benzodiazepines,
anticholinergics, and local intramuscular injections of
botulinum toxin (BOTOX®). Emergency physicians, as
standard practice, do not administer BOTOX® injections.
Tertiary referral centers perform most injections.
In most people, torticollis resolves in several days to a
few weeks. A few people will develop continuing neck
problems for months to years. Persistent neck muscle
spasms may require referral to a neurologist or surgeon.
Drug Category: Benzodiazepines -- By binding to specific
receptor sites, these agents appear to potentiate effects of
gamma-aminobutyric acid (GABA), facilitate inhibitory
GABA neurotransmission, and assist other inhibitory
transmitters. Benzodiazepines may act in the spinal cord
to induce muscle relaxation.
Surgery
Surgery is reserved only for a few selective cases. In this
treatment, some of the upper neck nerves are selectively
severed to prevent muscle contraction. Surgical treatment
often helps, but frequently your neck will return to its
twisting position after several months.
Correction consists of conservative [non-surgical]
treatment of physical therapy with passive stretching and
exercises. Exercises concentrate on relaxing tight
muscles, lengthening shortened muscles, and improving
the range of motion in the neck.
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Stretching right SCM
Stretching of right SCM
Stretching of right SCM
Chiropractic care, massage therapy, cranial sacral
therapy, muscle taping, use of the Tubular Orthotic for
Torticollis (TOT) collar, Botox injections, and alternative
medicine approaches may also be used in addition to the
physical therapy treatment of torticollis. You should not
begin any type of alternative or home treatment program
unless your child is under the direct supervision or
guidance from a trained healthcare professional. Always
discuss your intentions with your child's healthcare
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professional, and if needed, get a second opinion before
making your decision.
Therapy is usually followed until the child is tilt free and in
many cases, follow up visits to a therapist are
recommended until a child has learned to walk because
there may be regression as new milestones are reached.
If therapy fails to produce satisfactory results, or if
symptoms worsen, then surgery to lengthen the SCM
might be considered.
Prognosis:
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In most patients, IST gradually progresses over
months to years.
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Complete remissions sometimes occur.
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Remission percentages have been reported in 12%
or 21% of patients, particularly younger patients
during the first 5 years of IST. In these reports, most
remissions were only temporary.
Outlook
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Most people with acute torticollis are successfully
treated with medication. If a drug caused the spasm,
it should be stopped. Spasmodic torticollis is
successfully treated with local injections of botulinum
A toxin in combination with medications. If these
conservative measures are unsuccessful, surgery on
the nerves of the neck can be attempted. After
surgery, many people will often have initial relief, but
most relapse after several months.
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For the great majority of people with acute torticollis,
the condition goes away in several days to a few
weeks. A small number of people will go on to
develop continuing problems with their neck for
months to years.
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