Wryneck





Definition

Wryneck, also called twisted neck or torticollis, is a deformity in which the neck is twisted and held at an angle to one side. A congenital (present at birth) form called congenital torticollis is the most common type of wryneck seen in children.

Description

The sternocleidomastoid (SCM) muscle runs down either side of the neck. One end is attached to the occipital bone of the skull. The other end splits, with one end attaching to the clavicle (collarbone) and the other to the top of the sternum (breastbone). This muscle is involved in the complex movements of flexing the neck bones (cervical spine) and rotating the head up and down and sideways. Wryneck affects the SCM muscle, usually on only one side of the neck, causing the neck to spasm painfully and twist.

There are several different types of wryneck. Acute wryneck is the most common type. It develops suddenly, often for no apparent reason, and causes painful spasms that make the individual tilt the neck at an angle. The condition lasts one to two weeks, then symptoms disappear on their own without medical intervention. This type of wryneck is seen most often in older children and adults.

Adults can also develop spasmodic torticollis with head tilt and jerky head movements. This condition can develop from injury to the bones of the neck or because of infection, inflammation, or tumors of the soft tissue of the head and neck. Most often adult torticollis develops between the ages of 30 and 60. Adult onset torticollis is not be discussed here.

Congenital muscular torticollis is a neck deformity that affects newborns. It limits the range of neck motion and causes infants to tilt their head. It is the most common type of wryneck seen in young children and is different from acute wryneck, because it does not cause pain and does not resolve on its own. It arises from different causes than adult-onset torticollis.

Infants who have congenital muscular torticollis appear normal when they are born. However, within about a month, they often develop a non-tender lump on the side of the neck. Although this lump disappears by itself after about three months, the SCM muscle becomes tight, contracted, and fibrous. It does not stretch. The child then begins to tilt his head toward the affected side and point his chin toward the opposite shoulder. About three-quarters of the time, the right side is affected, causing the head to tilt to the right.

Demographics

Congenital torticollis is a rare disorder. It affects fewer than 0.4 percent of newborns. It is more common in first children than in later children and appears to be more common in babies born with a breech presentation (feet first). For reasons that are not understood, about 20 percent of children with congenital muscular torticollis also have congenital hip dysplasia . Hip dysplasia is a deformity in which the ball and socket of the hip joint do not mate properly.

Causes and symptoms

Congenital torticollis is thought to be caused by trauma around the time of birth. There are two theories about how this trauma occurs. One theory suggests that damage occurs during the birth causing a blood clot to form in the SCM muscle. This blood clot eventually leads to scarring in the muscle. The scar tissue does not stretch and causes the muscle to shorten and pull the neck out of position. Support for this theory comes from the observation that children with congenital torticollis are often breech or difficult forceps deliveries.

A second theory is that the trauma occurs before birth. It is believed that either pressure on the SCM muscle due to position of the head in the uterus causes the muscle to become fibrous and shorten or that the blood supply to the muscle is disturbed and the muscle becomes scarred. In either case, the result is scarring and shortening of the SCM muscle.

In rare cases, congenital torticollis can also be a symptom of other congenital disorders including abnormalities of the neck vertebra such as spina bifida or Arnold-Chiari syndrome. Torticollis can also be caused at an older age by fracture or dislocation of the neck vertebra or juvenile rheumatoid arthritis.

The causes of acute wryneck in older children and adults are not usually clear but seem to be related to wrenching the neck muscles, sleeping with the neck in an odd position or similar causes. Acute wryneck is briefly uncomfortable but not serious.

Symptoms of congenital torticollis are a painless mass on the neck appearing during the first two months of life and a persistent tilt of the head to one side for no other apparent reason. The child has limited ability to turn his head or move his neck (limited range of motion).

Symptoms of acute wryneck are sudden development (often overnight) of pain and stiffness in the neck sometimes accompanied by muscle spasms that cause an individual to hold the neck at an angle to try to relieve the pain.

When to call the doctor

Parents of newborns should call the doctor if they notice a lump on their child's neck or any time that their child persists in holding the head at an angle.

Diagnosis

Normally x rays of the neck are done to check for fractures . A computed tomography (CT) scan and/or a magnetic resonance imaging (MRI) scan is done to check for abnormalities in the soft tissue, such as tumors. Electromyography (EMG) is a technique that records the electrical activity of skeletal muscles. This exam can be useful in determining the extent of muscle and nerve involvement. Electromyography is usually done before surgery.

Treatment

Treatment should begin immediately for infants with torticollis. Delayed treatment increases the chance that the head tilt will not be reversed by non-surgical means. In addition, as the child grows, the face on the tilted side may become flattened. This flattening can be reversed while the bones are young and soft but after one year of age is likely to be permanent. Another reason to begin treatment early is that children with head tilt have more difficulty learning to walk and fall more often because their balance is affected by the way their head is twisted to one side of the body.

Conservative treatment

Conservative treatment for congenital torticollis should begin as soon as the condition is diagnosed. Physical therapy is begun with turning and bending the child's head four to six times per day for 15 to 20 minutes at a time. The goal of the physical therapy is to stretch and loosen the muscle and improve the range of motion. Physical therapy is continued until the child is at least one year old before surgery is considered.

Parents can supplement physical therapy by placing toys in positions such that the child must turn his head to see the object. This encourages use of the affected muscle. If there is improvement in the angle of head tilt and range of motion, therapy is continued. If there is no improvement after at least one year, surgery is considered.

Another conservative treatment to supplement stretching exercises is a tubular orthosis for torticollis (TOT) collar. The TOT collar is fitted by a physical therapist on infants who are at least four months old. It is made of soft plastic tubing with hard wedges of plastic inserted on the tilt side. When the head tilts into the hard plastic, it is uncomfortable, so the child tries to straighten his neck, thus exercising and stretching the affected muscles. Children wear the collar while awake and directly supervised from the age of about four months until they begin to walk.

When congenital torticollis is caused by deformities of the neck bones (vertebrae), conservative treatment involves the use of neck braces or body jackets.

Acute wryneck is treated with heat and over-the-counter non-aspirin pain medication ( acetaminophen , ibuprofen).

Surgical treatment

The goal of surgery in congenital muscular torticollis is to cut and then reattach the SCM muscle in a way that will remove the constricting bands of fibrous tissue, improve range of motion, and allow the head to be held vertically. Several different surgical techniques can be used. A uniploar SCM release, sometimes called an inferior open tenotomy of the SCM, cuts and then reattaches the SCM muscle where it meets both the breastbone and collarbone. This operation requires only one incision. A bipolar SCM release, also called a bipolar z-plasty, releases muscle where it is attached to the skull and at the collarbone. It requires two incisions. The SCM muscle is cut apart in a Z and then reattached. An endoscopic technique has been pioneered by plastic surgeons. This surgery involves making a small incision behind the ear and with the help of an endoscope clipping the muscle. Other surgeries are done when the cause of torticollis is a bone deformity.

After surgery children are fitted with a soft cervical collar that is worn continuously except during physical therapy and bathing or a stiff orthopedic collar that is worn during waking hours except for physical therapy. These collars are worn for about 10 weeks while the muscles heal and strengthen. Physical therapy begins about one week after surgery. The therapy involves stretching and strengthening exercises for the neck. Surgery does not instantly allow the head to be held vertically, so physical therapy and home exercises continue at least until the head tilt disappears.

Alternative treatment

Massage is said to be helpful both in stretching and releasing the muscles as a supplement, but not a replacement for, physical therapy.

Prognosis

When discovered during the first few months of life and treated promptly and consistently with stretching exercises, about 80 to 90 percent of children recover from uncomplicated congenital muscular torticollis with conservative treatment alone. Surgery is highly successful on children who do not respond to conservative treatment, so long as their torticollis is caused by restriction of the SCM muscle. In cases where torticollis is caused by or complicated by bone deformities or other congenital defects, the outcome is less likely to be successful. Torticollis is unlikely to recur if stretching and flexibility exercises for the neck are continued.

Prevention

There is no sure way to prevent wryneck and congenital torticollis; however, care should be taken to avoid as much trauma to the child as possible during delivery.

Parental concerns

Parents' concerns often are focused on the psychological impact of torticollis in children who do to respond completely to treatment. Holding the head at an angle is an obvious deformity that can cause a child to retreat from social situations. The parents of a young child who has head tilt and is just learning to walk may also be concerned about the frequency with which their child loses his balance, increasing the risk of injury.

KEY TERMS

Arnold-Chiari syndrome —A congenital malformation of the base of the brain.

Cervical spine —The seven bones of the neck that form the uppermost part of the spinal column.

Endoscope —A medical instrument that can be passed into an area of the body (the bladder or intestine, for example) to allow visual examination of that area. The endoscope usually has a fiberoptic camera that allows a greatly magnified image to be shown on a television screen viewed by the operator. Many endoscopes also allow the operator to retrieve a small sample (biopsy) of the area being examined, to more closely view the tissue under a microscope.

Spina bifida —A birth defect (a congenital malformation) in which part of the vertebrae fail to develop completely so that a portion of the spinal cord, which is normally protected within the vertebral column, is exposed. People with spina bifida can suffer from bladder and bowel incontinence, cognitive (learning) problems, and limited mobility.

Resources

BOOKS

Rakel, Robert E. "Torticollis." In Textbook of Family Practice , 6th ed. Philadelphia: W. B. Saunders Company, 2002, pp. 902–03.

ORGANIZATIONS

American Academy of Orthopaedic Surgeons. 6300 North River Road Rosemont, Illinois 60018–4262. Web site: http://www.aaos.org.

WEB SITES

"Online Service Fact Sheet Congenital Torticollis (Twisted Neck)." American Academy of Orthopaedic Surgeons , June 2004. Available online at http://www.orthoinfo.aaos.org/fact/thr_report.cfm?survey=survey (accessed October 27, 2004).

Othee, Gurdeep S., and Carl R. Menckhoff. "Torticollis." eMedicine Medical Library. Available online at http://www.emedicine.com/orthoped/topic452.htm (accessed October 27, 2004).

Reynolds, Norman C., and Ma Jianxin. "Torticollis." eMedicine Medical Library , July 1, 2004. Available online at http://www.emedicine.com/neuro/topic377.htm (accessed October 27, 2004).

Ross, Michael, and Susan Dufel. "Torticollis." eMedicine Medical Library , August 17, 2004. Available online at http://www.emedicine.com/emerg/topic597.htm (accessed October 27, 2004)

Tish Davidson, A.M.



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