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 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 and months of age, and the condition tends to remit by the age of to 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 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