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CHAPTER 49 ■ NECK STIFFNESS
LEAH TZIMENATOS, CHERYL W. VANCE, NATHAN KUPPERMANN

INTRODUCTION
Neck stiffness is an important chief complaint in children evaluated in emergency
departments. Commonly, neck stiffness is accompanied by neck pain. Certain
clinical conditions, however, may lead a child to hold the neck in an abnormal
posture (malposition) without neck pain. The underlying causes of neck stiffness
or malposition in children range from relatively benign (e.g., muscle strain,
cervical adenitis) to life threatening (e.g., meningitis, fracture or subluxation of
the cervical spine).
Torticollis (meaning “twisted neck” from the Latin roots tortus and collum ) is
a subset of neck stiffness. With torticollis, the child holds the head tilted to one
side and the chin rotated in the opposite direction, reflecting unilateral neck
muscle contraction. This may result from various pathologic processes and may
or may not be associated with neck pain. Torticollis is often congenital and/or
muscular in origin. It can also be associated with acquired processes such as
trauma, infectious or inflammatory illnesses, central nervous system neoplasms,
drug reactions, and a variety of different syndromes.
This chapter reviews the differential diagnosis of neck stiffness including
torticollis, both with and without neck pain, in children. Figure 49.1 offers an
algorithmic approach to help distinguish potentially life-threatening from benign
causes of neck stiffness, while providing a broad differential diagnosis.


DIFFERENTIAL DIAGNOSIS
Most children presenting with neck stiffness are well appearing with benign,
frequently self-limited conditions; however, the differential diagnosis of neck
stiffness is broad and includes many potentially life-threatening causes, which
must be considered and excluded when appropriate. A history of trauma, signs or
symptoms of an infectious or inflammatory process, or evidence of spinal cord
involvement may be helpful in identifying a number of specific diagnoses.
Table 49.1 lists most causes of neck stiffness in children, Table 49.2 lists the
common causes, and Table 49.3 lists the life-threatening causes. The following
descriptions categorize the causes of neck stiffness in children by underlying
mechanism and severity.

Neck Stiffness Associated With Trauma


Potentially Life-Threatening Causes
Neck trauma is a common cause of neck pain and stiffness (see Chapter 112 Neck
Trauma ). Fortunately, serious injuries to the cervical spine (fractures,
subluxations, and spinal cord injuries) are uncommon, especially in children
younger than 8 years. Because of a higher fulcrum of the cervical spine and
relative weakness of the neck muscles compared to adults, these injuries generally
occur in the upper cervical spine in younger children, as opposed to more widely
across the cervical spine in older children and adolescents. Neck injuries in
children most commonly result from high kinetic energy mechanisms, such as
motor vehicle–related collisions, sports injuries, and falls.
Fractures of the Cervical Spine. Fractures of the cervical spine in children are
very uncommon, occurring in 1% to 3% of hospitalized pediatric trauma patients.
Although some children with fractures of the cervical spine are unresponsive at
the time of evaluation, most are alert and verbal, limit their neck movement

secondary to pain, and have no demonstrable neurologic deficits. At the
minimum, the cervical spine should be immobilized and imaging of the cervical
spine should be obtained on any child with an altered level of consciousness, pain
or stiffness of the neck, midline neck tenderness on examination, any neurologic
deficits, or distracting painful injuries after blunt trauma. Imaging should also be
obtained in those who are unable to perceive pain (e.g., as a result of alcohol or
drugs) or describe their symptoms. A large prospective study, the National
Emergency X-Radiography Utilization Study (NEXUS), of blunt trauma victims
identified five criteria (posterior midline cervical tenderness, altered alertness,
distracting injury, intoxication, and focal neurologic signs) that identified all
children with cervical spine injuries; however, there were few children younger
than 9 years and none younger than 2 years with cervical spine injuries in that
study. A multicenter attempt to retrospectively validate the NEXUS criteria
among a different cohort of children (including those younger than 2 years)
demonstrated a lower sensitivity, suggesting the need for refinement of these
criteria before routine use in children. A case-control study of 540 children with
cervical spine injuries identified eight important risk factors: altered mental
status, focal neurologic findings, neck pain, torticollis, substantial torso injury,
conditions predisposing to cervical spine injury, diving, and high-risk motor
vehicle collision mechanisms. A study to prospectively refine risk factors,
particularly in younger children, is currently underway. Results of the pilot study
suggest that a prediction rule in children is feasible.


Subluxation of the Cervical Spine. Traumatic subluxations of the cervical spine
are more common than fractures in children and are more likely to present more
than 24 hours after injury. Subluxation may result from minor trauma (e.g., falls
from low heights) but typically occurs after more severe trauma (see Chapter 112
Neck Trauma ). The most common subluxation is rotary (or “rotatory”)
atlantoaxial subluxation. Clinically, rotary subluxation typically causes neck pain

and torticollis without focal neurologic symptoms because the transverse
ligament of the atlas remains intact and the spinal cord is not compromised.
Sternocleidomastoid (SCM) spasm and neck tenderness are localized to the same
side as the head rotation as the SCM attempts to “reduce” the deformity. This is in
contrast to other causes of torticollis, in which the spastic, tender SCM muscle is
opposite to the direction of head rotation. In addition, in rotary subluxation, there
is palpable deviation of the spinous process of C2 in the same direction as the
head rotation. In contrast, during normal neck rotation beyond 20 degrees, the
spinous process of C2 deviates to the contralateral side.