CHAPTERS

Chapter 1: Introduction to Torticollis

The word “torticollis” comes from the Latin “tortus” for twisted and “collum” for neck. In a nutshell, that explains this condition, which you may also see referred to as wry neck or loxia. Breaking down the structure of the word, however, is the only simplistic thing about an ailment that can have many roots.

Presentation of Torticollis

Torticollis presents as abnormal positioning of the head or neck that may also be accompanied by asymmetry of the cranium and facial features. The abnormality may be fixed or dynamic, and present as a combination of tilt, rotation, and flexion. The most typical variant is for the neck to twist to one side causing a tilting of the head. There are both pediatric and adult forms of torticollis.

Pediatric and Adult Torticollis

When torticollis appears in infants, it can be congenital (present at birth) or acquired. The major causes are believed to be intrauterine malposition, breech birth, or cervical malformation.

In adults, the distortion may develop slowly over time or appear acutely after a trauma or as adverse pharmaceutical reaction.

If twisting of the neck appears in people with a family history of the disorder, the condition is called spasmodic torticollis. It appears most often between the ages of 31 and 50 and can become permanent if not treated.

Simply bending or twisting the neck beyond the normal range of motion can lead to acute or episodic torticollis. The person will experience pain and discomfort if they try to turn their head or to hold it straight. There will also be pain and palpable tenderness present in the neck muscles.

Torticollis and SIDS

Although there is no direct causal link between torticollis and Sudden Infant Death Syndrome (SIDS), preventing one can lead to an increased incidence of the other.

Groundbreaking research into the tragic death of babies to SIDS determined that children who sleep on their stomachs at night face a higher risk for SIDS.

Consequently, parents now put babies down on their backs leading to an increased need for children to have “tummy time” during the day. This position forces babies to lift their heads, building the necessary muscle strength to carry the weight of the skull and to engage in a full range of motion.

Although there is no specific way to prevent torticollis per se, ensuring adequate tummy time is certainly key to correct muscular development in a baby’s neck.

Causes of Torticollis in Adults

The causes of twisted neck in adults are many and varied. When the condition is spasmodic in nature (also called cervical dystonia) the specific causal agents are neurological and may be related to an imbalance of neurotransmitters affecting the brain’s basal ganglia.

The neurotransmitter acetylcholine is thought to play a major role in spasmodic torticollis by increasing the frequency of nerve signals to the region. Ultimately, muscle spasms begin to fire and pull the head to one side (most often to the left.)

Spasmodic torticollis appears gradually, beginning with slight pain at the base of the skull and minor stiffness of the neck. Increasingly, everyday motions cause pulling sensations and pain that becomes severe as the torticollis progresses.

The discomfort is more pronounced on one side, and may radiate into the shoulder, with tingling and numbness moving down the arm and into the hand. The individual may also suffer from head tremors. An episode can last years, sometimes resolving spontaneously only to recur in the future.

Spasmodic torticollis related to neurotransmitter imbalances is, however, only one way in which adults may suffer from twisted neck.

Muscular Fibrosis

An injury to the neck may lead to a case of torticollis due to the formation of scar tissue. Muscular fibrosis refers to the excessive formation of such tissue between the muscle fibers themselves. This results in weakness and fatigue in the muscles with abnormal contractions and shortening.

Muscular fibrosis is also the leading cause of torticollis in infants. If the child does not have sufficient room in the womb the neck may be pushed to one side thus damaging the muscle, which then heals after birth. The damage may also be due to a breech birth.

As the tears in the muscle grow back together, the fibrous scar tissue fills open spaces in the muscle’s structure. This growth hampers the muscle’s ability to both contract and relax. Therefore, any kind of neck injury, in infants or adults, can lead to an episode of torticollis.

Congenital Spinal Abnormalities

Abnormalities of the spine at birth are not uncommon, and may include scoliosis, kyphosis, torticollis, and vertebral defects.

Scoliosis is an abnormal curving of the spine, which normally forms an S shape when viewed from the side, but is straight when seen head on. In individuals with scoliosis, however, the spine also curves side to side.

Kyphosis is an abnormal rounding or curving of the vertebrae that leads to the condition commonly called “hunchback.” In less pronounced instances, the affected person merely seems to be slouching.

With torticollis, there is an abnormality of the top seven vertebrae of the neck known as the cervical spine. This is the region of the backbone that not only supports the weight of the head, but is also responsible for the head’s wide range of motion from side to side, up and down, and to all rotational points in between.

If one or more of these vertebrae do not function properly, twisting of the neck may result. When spinal abnormalities are involved, torticollis can be more difficult to correct and may require surgery.

The twisting of the neck becomes apparent shortly after birth in most cases, but can present much later in life as the spinal column begins to deteriorate with age.

Toxic Brain Injury – Encephalopathy

A person who has any type of encephalopathy is presenting with impaired brain function. Generally if there is no injury to the brain and no tumor present; the abnormality is due to the presence of a toxin.

For instance, in heavy drinkers encephalopathy may progress to Wernicke-Korsakoff Syndrome, which is a type of early onset dementia.

There are, however, many toxins in our environment to which we are exposed not by choice or conscious action, like organic solvents or even chemicals that have leached into the groundwater and thus into our drinking supply.

The classic symptoms of toxic brain injury include dizziness, disorientation, and diminished intellectual skills, but loss of motor control is a definite possibility. This may manifest as an unsteady or shambling gait, trembling in the hands, and an inability to control the movement and positioning of the head.

Traumatic Brain Injury

Traumatic brain injuries are a consequence of external forces causing dysfunction and impairment that is often permanent.

This may come from an actual physical blow to the head or may be the result of being in close proximity to an explosion, a common cause of brain injury to soldiers fighting in Iraq and Afghanistan.

Mild traumatic brain injury or concussion may lead to only temporary functional issues, while more severe brain trauma where bruising and bleeding are present can cause a life time of impairment.

Just as is the case with toxic brain injury, an inability to control the movement and position of the head is a possible outcome.

Scarred or Diseased Cervical Vertebrae

Any injury or illness that affects the cervical vertebrae can alter a person’s ability to move their head. These seven vertebrae begin just above the shoulder blades and continue to the base of the skull. Their function is aided by the adjacent muscles and ligaments, with nerve endings interweaving the entire structure.

When problems occur, the issues tend to cascade through the head, neck, and shoulders, with corresponding disruptions in motion and flexibility. A good example of an injury to this region that causes the neck to twist is whiplash in the aftermath of an automobile accident.

Arthritis

Since the cervical vertebrae are one of the areas of the body prone to the development of arthritis, this gradual degeneration of the cushioning discs between the vertebrae can also lead to a twisting of the neck.

This causes not only an abnormal positioning of the head, but also constant pain and stiffness. The good news in regard to this kind of degenerative disc disease is that it actually tends to improve over time.

Tonsillitis

The tonsils are two tissue masses at the rear of the throat that trap germs before they can enter the airway. Additionally, the tonsils produce infection-fighting antibodies. Often, however, the tonsils themselves become infected if they are overwhelmed by bacteria and viruses.

The consequent inflammation and swelling called tonsillitis can cause the nerves at the base of the head to also become inflamed causing short-term torticollis. The twisting of the neck resolves when the infection is treated with antibiotics or the tonsils are removed.

Retropharyngeal Abscess

Although an abscess at the back of the throat is rare, retropharyngeal abscesses are seen in infants and children, but are difficult to diagnose since they occur in an area of deep tissue.

Any time a child displays symptoms including a stiff or twisted neck, difficulty in swallowing, and an overall sense of being unwell, an abscess should be one of the conditions ruled out rather than glossed over.

If treatment is delayed, such a deep-seated infection can be life threatening. Retropharyngeal abscesses are not commonly diagnosed in adults, but they are certainly possible at any stage of life.

Swollen Lymph Nodes

Any swelling or enlargement of the lymph nodes due to the presence of infection or disease can have the same effect on the neck muscles as an infection in the back of the throat.

In some cases, if the swelling is significant, the lymph nodes themselves may impair the movement of the neck. This type of swelling can be caused by seasonal malady like a cold or the flu, or it can be an indication of more serious illnesses including cancer.

Brain Tumors

There are many types of tumors that affect the brain and spinal cord. These abnormal cell masses can be benign or malignant. If a tumor is present, and is growing into a portion of the brain that controls movement or is adjacent to the cervical vertebrae, torticollis can result.

Typically tumors will appear on various imaging scans, which should be performed for an accurate diagnosis of the cause of torticollis. Once detected, management of the twisting of the neck is no longer the primary medical concern, with focus shifting to treating the tumor.

Tuberculosis of the Spine

Tuberculosis of the spine is also called Pott’s Disease. It is most often present in the lower thoracic and lumbar vertebrae, but can affect the cervical vertebrae.

The disease causes the cushioning discs between the bony vertebrae to collapse, with subsequent compression of the spine. When this occurs in the cervical vertebrae, it can lead to torticollis.

Ideopathic Torticollis

Some cases of torticollis in adults seem to defy all explanations even when rare factors have been explored. In idiopathic cases, the torticollis often resolves just as it appeared — mysteriously.

So long as life-threatening potential culprits have been ruled out, idiopathic torticollis is more a nuisance than a serious medical condition.

Although the sufferer may be uncomfortable, and may experience limitations in their day-to-day activities, idiopathic torticollis can be managed and is often temporary.

Causes of Pediatric Torticollis

Pediatric torticollis, which typically occurs in the first two months of life, is almost always related to some injury to the muscles of the neck before or during birth.

During the first two months of life, an infant should be gaining more muscle control. The limited range of motion present with torticollis as compared to the increasing mobility of other children of the same age makes the tilting or twisting of the head visibly apparent to caregivers.

The contraction of the muscles is a consequence of the healing process. Scar tissue forms that causes the tissue to contract, twisting the neck and rotating the head.

It is also not unusual for a baby with torticollis to have developmental hip dysplasia, a condition in which the thighbone does not sit securely in the socket of the hip.  Hip dysplasia can also be the consequence of a difficult delivery.

In the vast majority of cases, pediatric torticollis responds to physical therapy with no need for surgical intervention.

Muscular Damage at Birth

Whether the damage to the muscle occurs in the womb due to a lack of adequate room, or is the result of a breech birth, the real culprit is scar tissue present in the sternocleidomastoid muscle of the neck.

With first pregnancies, babies typically have less space to move in the uterus, a fact that correlates with a higher incidence of both torticollis and hip dysplasia in first-born children.

The damage to the healing neck muscle in infants with torticollis may be visible to the naked eye as a small lump of tissue at the spot where the injury occurred. Torticollis happens in about 0.3-0.2 percent of births.

Physical therapy stretches the fibers to release the contraction while strengthening the muscles to enable normal function. A TOT (Tubular Orthosis for Torticollis) collar may also be used to encourage the correct position of the head for longer periods of time.

Sternocleidomastoid Tumor

In a situation similar to that which can be created by enlarged lymph nodes, a tumor on the long muscles on either side of the neck, the sternocleidomastoid, can likewise impair movement and distort the position of the head.

Such tumors are the most common masses found on the necks of infants and are actually nothing more than a severe instance of muscle fibrosis. The mass is shaped like a spindle and is not malignant and rarely will a biopsy be required.

Typically the mass will be on the right side of the neck and is more common in male babies. In only 5-10% of cases is surgery necessary to release the tissue. Typically the “tumor,” no matter how severe, will respond to physical therapy.

Klippel-Feil Syndrome

Klippel-Feil Syndrome is a rare congenital condition in which the bones of an infant’s neck are not properly formed, abnormalities. This is different from other cervical malformations, however, in that the bones of the ear will also be malformed resulting in hearing deficits.

The syndrome is characterized by fusion at birth of any of vertebrae 2-7, leading to a pronounced shortening of the neck with limited movement immediately evident.

Acquired Torticollis in Infants

Acquired torticollis, like spasmodic and acute torticollis in adults, presents in the aftermath of some accompanying trauma or illness. Resolution of the twisted neck typically means addressing the concurrent condition.

Self-Limited Incidents

Self-limiting episodes of torticollis are often caused by exposure to cold drafts or the repeated assumption of unusual postures.

Since infants bodies are still developing, their muscles are prone to experiencing abnormal contractions, especially the sternocleidomastoid and trapezius muscles.

In such cases, a program of parent-administered physical therapy is used to stretch and strengthen the muscles, with the twisted neck typically resolving in 1-4 weeks.

Posterior Fossa Tumors

Posterior fossa tumors are located at the base of the skull and are often responsible for compressing nerves in the region. In instances of acquired torticollis in infants and young children, such tumors should be ruled out as part of a differential diagnosis so that appropriate treatment can be administered without delay. Typically, surgery will be needed to remove the mass.

Throat, Ear, and Adenoid Infections

Infections of the posterior pharynx (including strep throat), ear infections, and inflamed adenoids can all cause secondary inflammation of the nerves at the base of the skull.

Since these nerves supply the neck muscles, a temporary case of torticollis may accompany any such infection. Typically, the twisting of the neck resolves when the infection is treated with antibiotics.

Drug Reactions

Although more rare, drug interactions can also cause transient torticollis. This effect is seen with the use of both antiemetic (nausea) medications and antipsychotics.

The use of antipsychotics in young children is rarely if ever an issue, but drugs for nausea and vomiting are used in this age group. The torticollis side effect can normally be treated with doses of diphenhydramine (Benadryl).

Continue Reading…