Torticollis, also known as “wry neck,” cervical dystonia, spasmodic torticollis, and torsion dystonia, is a symptom of an underlying problem rather than a disease in itself. The term torticollis originates from the Latin torti (twisted) and collis (neck), and describes a posture in which the head is involuntarily twisted to one side.

Torticollis can be present at birth (congenital) or occur later in life (acquired). The causes of torticollis are numerous, but the prevalence can be difficult to determine; recent estimates predict approximately 1% to 16% of newborns may have the disorder.

Looking Back

One of the better-known forms of congenital torticollis is congenital muscular torticollis. According to William A. Phillips, MD, a pediatric orthopedic surgeon at Texas Children’s Hospital in Houston, the current theory is that its cause might be related to how the child was positioned in utero or during labor: The delicate cervical muscles, particularly the sternocleidomastoid, may become fibrotic and stiff when damaged by poor positioning or birth trauma. The condition is more common with firstborn children and in breech births, Phillips says.

Congenital torticollis in infants also can be caused by Klippel-Feil syndrome, a condition in which the cervical vertebrae become fused together. Additionally, torticollis may be seen with Sandifer syndrome, a spasmodic dystonia of the head and neck that is coupled with seizure-like upper body movements and is associated with gastroesophageal reflux.

Children may develop acquired torticollis, which can result from underlying cervical spine dysfunction, a reaction to certain medications, or via compensatory mechanisms brought on by ocular muscle or nerve weakness or paralysis, Phillips says. Acquired torticollis can be a sign of a brain tumor or thalamic problem, and certain upper respiratory tract or throat infections can cause it. Torticollis can occur after a minor trauma, such as falling off a slide, or following ear, nose, and throat surgery, he adds.

Infants who don’t initially appear to have torticollis can later show signs of it. Several scientific papers have noted an increase in this form of positional, or secondary, torticollis since the onset of the Back to Sleep program in 1992, which was developed to prevent sudden infant death syndrome by urging parents to position children on their backs for sleep.

Moving Forward

One of the hallmark signs of torticollis in infants is a head position of ipsilateral side bending with contralateral rotation in relation to the tight cervical musculature. Because torticollis may mimic subluxation of C1 on C2, atlantoaxial instability should be ruled out before treatment. With C1/C2 instability, the head is more likely to tilt into a position of contralateral side bending.

Treatment for torticollis varies based on the cause. For congenital muscular torticollis or positional torticollis, treatment starts with physical therapy. At Children’s Memorial Hospital in Chicago, clinical specialist Mary Weck, PT, begins by teaching parents to encourage their babies to stretch and strengthen their muscles actively.

“We work with the parents on how to position the babies, how to hold the babies, [and] how to handle the babies, so that all through the waking day, and even as much as possible during the sleeping night, the baby’s head is positioned out of the torticollis,” she says.

How long it takes for therapy to become effective depends on many factors, such as when PT begins, how much the family is able to actively interact with the child, how young the baby is, how tight the muscles are, and the baby’s personality. “Some babies are pretty set in their ways and it can take longer,” Weck says.

In general, the earlier the PT referral, the easier the torticollis case is to resolve. “You can correct this condition better earlier, even if the case is severe,” Weck says. At Children’s Memorial Hospital, PTs follow the babies until they are walking well and symmetrically. If babies aren’t correcting quickly, the therapist might try a collar cut high on the side of the head tilt, or use taping to encourage weak muscles to strengthen and strong muscles to become less active, she says.

Some researchers have explored using botulinum toxin injections as a treatment for muscular torticollis. However, Michael L. Cunningham, MD, PhD, medical director at the Children’s Craniofacial Center at Children’s Hospital and Regional Medical Center in Seattle, says he doesn’t use the injections. If a case of torticollis hasn’t resolved with PT, Botox would lead to a paralyzed muscle, he says. “I’d do surgery on that patient before I would do long-term Botox injections,” he notes. But he does find that surgery is rarely necessary. In his 16 years working in a specialized field that sees a number of these patients, he has only referred two for surgery.

Phillips agrees that in his experience, surgical intervention is rare with muscular torticollis because physical therapy works so well. When necessary, the surgery usually consists of lengthening the shortened muscle by releasing some of the tight fibers, he says.