may be conservative medical management, radiofrequency ablation, or surgery.
Eosinophilic granulomas and bone cysts are other rare, benign lesions of the spine
that may cause neck pain and stiffness.

Congenital Causes of Neck Stiffness
Neck stiffness and/or torticollis from congenital abnormalities are usually not life
threatening. These congenital causes are usually muscular or skeletal in origin.
Congenital Muscular Torticollis. Congenital muscular torticollis is the most
common cause of torticollis in infancy. The etiology is unclear but is believed to
be related to birth trauma causing an injury to the SCM muscle with hematoma
formation, followed by fibrous contracture of the muscle. Other theories suggest
intrauterine malposition, infection, neurogenic causes, and intrauterine
compartment syndrome of the SCM muscle. On examination, a palpable mass can
often be detected in the inferior aspect of the SCM. The mass is generally not
present at birth but appears in the neonatal period. The head is held in a
characteristic position, with the patient’s chin pointing away from the affected,
contracted SCM muscle. Some degree of craniofacial asymmetry is commonly
found in these patients, typically with contralateral flattening of the occiput and
ipsilateral depression of the malar prominence. Ultrasound is the imaging
modality of choice. Treatment is conservative with active positioning and manual
stretching of the involved muscle. Surgical release of the SCM is required in
approximately 5% of cases if the deformity persists for longer than 6 to 12
months.
Skeletal Malformations. Klippel–Feil syndrome is characterized by congenital
fusion of a variable number of cervical vertebrae, which may result in atlantoaxial
instability. The cause is unknown. It is often associated with other bony
abnormalities, and significant scoliosis develops in more than 50% of affected
children. Limited range of motion of the neck is the most common physical sign.
The classic triad also includes a low hairline and a short neck but is seen in fewer
than half of patients.
Sprengel deformity is characterized by congenital failure of the scapula to

descend to its correct position. The scapula rests in a high position relative to the
neck and thorax. In its most severe form, the scapula may be connected by bone
to the cervical spine and limit neck movement.
Hemiatlas is a malformation of the first cervical vertebra, which may cause
severe, progressive torticollis.


Basilar impression is a condition resulting from anomalies at the base of the
skull and vertebrae, which lead to a short neck, headache, neck pain, and cranial
nerve palsies due to compression of the cranial nerves. Many congenital
conditions,
including
Klippel–Feil
syndrome,
achondroplasia,
and
neurofibromatosis, may cause basilar impression. Commonly associated with
basilar impression is occipitocervical synostosis, a condition in which fibrous or
bony connections between the base of the skull and the atlas cause neck pain,
torticollis, high scapula, and neurologic symptoms.
Atlantoaxial Instability. Several congenital conditions may be associated with
atlantoaxial instability and may predispose the patient to cervical subluxation. In
addition to Down, Marfan, and Klippel–Feil syndromes, these include other
skeletal dysplasias and os odontoideum (aplasia or hypoplasia of the odontoid
process of the axis). Morquio syndrome is a mucopolysaccharidosis resulting in
flattening of the vertebrae and multiple skeletal dysplasias. In this syndrome, the
odontoid is underdeveloped and may lead to atlantoaxial subluxation.

Miscellaneous Causes of Neck Stiffness
Head tilt, neck stiffness, and/or torticollis have been reported in other conditions,

some of which are life threatening and others generally benign.
Ophthalmologic, Neurologic, and/or Vestibular Causes. Head tilt or neck
malposition may result from abnormalities of vision (e.g., strabismus, cranial
nerve palsies, extraocular muscle palsies, refractive errors) or the vestibular
apparatus. The child attempts to correct for the disturbance through changes in
neck position. Careful ophthalmologic and neurologic examinations of the child
with head tilt are necessary to exclude these possibilities. Torticollis has also been
reported in patients with migraine headaches.
Myasthenia Gravis. Patients with myasthenia gravis may develop torticollis,
although ptosis, impairment of extraocular muscular movement, and other cranial
nerve palsies are generally earlier signs.
Guillain–Barré Syndrome. Neck stiffness has been reported in children with
Guillain–Barré syndrome. Neck stiffness in this condition is seen in association
with generalized motor weakness and areflexia.
Idiopathic Intracranial Hypertension. Stiff neck and torticollis have also been
reported in children with idiopathic intracranial hypertension, also known as


pseudotumor cerebri. These neck symptoms may be the presenting signs of the
condition, but more commonly patients present with headache, vomiting, and
papilledema. Therefore, clinicians should inspect the optic discs of children with
neck stiffness and/or torticollis. Lumbar puncture and removal of cerebrospinal
fluid may quickly resolve the cervical symptoms and signs.
Benign Paroxysmal Torticollis of Infancy. Benign paroxysmal torticollis of
infancy presents as recurrent episodes of head tilt sometimes accompanied by
pallor, agitation, and vomiting. Episodes subside spontaneously within a few
hours or days. Typical onset is between 2 and 8 months of age, and the condition
tends to remit by the age of 2 to 3 years. The etiology is unknown, and there is no
effective treatment.
Sandifer Syndrome. Sandifer syndrome describes intermittent episodes of

stiffening and torticollis related to gastroesophageal reflux. Children with this
syndrome may have other symptoms associated with reflux including recurrent
vomiting and failure to thrive.
Spontaneous Pneumomediastinum. Spontaneous pneumomediastinum may
present with neck pain and torticollis. A history of severe coughing and/or
retching is usually elicited. Crepitus is generally palpated along the neck.
Spasmus Nutans. Spasmus nutans is an acquired condition of childhood,
characterized by nystagmus, head nodding, and torticollis. Children with these
findings typically become symptomatic by 2 years. The condition is generally
benign and self-limited. However, some children with the symptoms of spasmus
nutans have underlying brain tumors. Therefore, imaging of the brain is
necessary.
Dystonic Reaction. Certain drugs can cause acute dystonic reactions with
torticollis. These most commonly include antipsychotic and antiemetic agents
(e.g., haloperidol, prochlorperazine, and metoclopramide). Treatment with
diphenhydramine may be diagnostic and therapeutic.
Psychogenic Disorder. Hysterical patients may present with torticollis. This
diagnosis can be made only after excluding other causes.

EVALUATION AND DECISION


The evaluation of, and treatment plan for, neck stiffness is best organized around
several important historical/clinical questions and physical examination findings:
(i) Is there evidence of spinal cord involvement?; (ii) Is there a history of
trauma?; (iii) Is there evidence of an infectious or inflammatory process (e.g.,
history or presence of fever)?; (iv) Is a cervical mass present?; (v) Are the
symptoms acute or chronic?
The approach to the child with a stiff or malpositioned neck should focus
initially on whether there is spinal cord involvement, as detailed in Figure 49.1 .

The diagnostic studies included in the figure represent suggested modalities for
evaluation of children with those signs/symptoms. Decisions regarding specific
diagnostic modalities will depend on each patient’s individual presentation,
history, and examination.
For any child with neck stiffness or pain, a history of weakness, paresthesias of
the extremities, or abnormal bowel or bladder function should be sought. In
addition, a complete ophthalmologic and neurologic examination should be
performed, with the latter focusing on spinal cord function. Included in this
examination should be an assessment of muscle strength, sensation, deep tendon
reflexes, the Babinski reflex, and anal tone. Extra vigilance must be used if the
patient is too young or incapacitated to provide an accurate history.
If spinal cord involvement is detected, immobilization, neurosurgical
consultation, and imaging of the cervical spine are necessary. Conditions causing
cervical spinal cord compromise may rapidly lead to permanent disability or
death if not immediately addressed. If secondary to trauma, one should suspect
cervical spine fracture, subluxation, or spinal epidural hematoma. In the setting of
fever, a spinal epidural abscess should be considered. Atlantoaxial subluxation
with instability secondary to otolaryngologic diseases or procedures (i.e., Grisel
syndrome) should be considered in children with spinal cord involvement and
consistent histories. Finally, spinal cord tumors and other space-occupying lesions
should be considered if the development of symptoms is gradual and not
associated with trauma or fever.
The next consideration is whether the neck stiffness is the result of an acute
traumatic event. If acute trauma is the cause of the neck stiffness, the cervical
spine should be properly immobilized (see Chapter 112 Neck Trauma ) and
imaging of the cervical spine obtained. Fractures and subluxations/dislocations
will generally be identified on plain radiography of the cervical spine. Other
modalities (e.g., CT, MRI) may be useful to detect ligamentous injury, rotary
subluxation, or spinal epidural hematomas. In the setting of trauma, cervical
muscle strain and/or contusion are diagnoses of exclusion. If other symptoms in