Stiff Neck

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Chapter 25 Stiff Neck

Nicholas Tsarouhas, MD, Marla J. Friedman, DO

1 What is the pathophysiology of meningismus?

Flexion of the neck stretches the inflamed nerve roots and meninges of the cervical region. The patient’s protective muscle spasm manifests as neck stiffness, or meningismus.

2 Is meningismus usually seen in neonates with meningitis?

Neonates rarely manifest meningismus or nuchal rigidity. The most common symptoms of meningitis in young infants are fever, lethargy, irritability, and poor feeding.

Pong A, Bradley JS: Bacterial meningitis and the newborn infant. Infect Dis Clin North Am 13:711–733, 1999.

3 At what age is meningismus or nuchal rigidity reliable in evaluation of children for meningitis?

While this is controversial, most experts feel that after 18–24 months this finding becomes more useful. It may be a late finding in meningitis in younger infants.

4 What are Kernig’s and Brudzinski’s signs?

These are physical examination maneuvers to evaluate for the presence of meningeal inflammation. Kernig’s sign refers to extension of the patient’s leg causes neck pain and flexion. Brudzinski’s sign involves flexion of the patient’s neck and produces flexion at the knees and hips.

5 What are the indications for lumbar puncture (LP) in a child with stiff neck?

Consider performing an LP to rule out meningitis in any febrile child with a stiff neck and no other obvious source of infection. This is especially important in children who are not well appearing.

Ostenbrink R, Moons KGM, Theunissen, CCW, et al: Signs of meningeal irritation at the emergency department: How often bacterial meningitis? Pediatr Emerg Care 17:161–164, 2001.

6 What are the contraindications to doing an LP?

LP may be dangerous in an unstable, critically ill child. “Curling” the young patient into position for the LP may limit ventilation and lead to respiratory arrest in an already compromised infant. If the child is unstable, draw a blood culture and administer antibiotics. Defer the LP until the child is more stable. Other contraindications to LP include increased intracranial pressure and lumbosacral cutaneous infection.

Cronan K, Wiley J: Lumbar puncture. In Henretig FM, King C (eds): Textbook of Pediatric Emergency Procedures. Baltimore, Williams & Wilkins, 1997, p 542.

7 Describe some possible complications of LP

While complications are rare, they do occur. Examples include hematomas, persistent cerebrospinal fluid (CSF) leaks, radiculopathies, nerve injuries, epidermoid cysts, post-LP headache, and infection. Headaches after LP in children are uncommon because a small needle is used for the procedure and minimal CSF leak occurs. Life-threatening and extremely rare complications include herniation, respiratory arrest, and cardiac arrest.

Cronan K, Wiley J: Lumbar puncture. In Henretig FM, King C (eds): Textbook of Pediatric Emergency Procedures. Baltimore, Williams & Wilkins, 1997, p 542.

8 What other spinal infections may present with a stiff neck?

Osteomyelitis, epidural abscess, and discitis may occur in the cervical region. Focal spine tenderness in the presence of fever should raise suspicion for these infections. Conventional radiography is sometimes helpful, but radionuclide bone scanning or magnetic resonance imaging are more diagnostic.

9 Which serious deep neck infection can present with stiff neck?

Retropharyngeal abscess. Retropharyngeal infections (cellulitis, adenitis, abscess) can develop in the potential space between the anterior border of the cervical vertebrae and the posterior wall of the esophagus. These infections generally occur in infants and toddlers and are rarely seen in children older than 5 years. They are usually caused by group A streptococcus, Staphylococcus aureus, or anaerobes. These ill-appearing children may present with fever, respiratory distress, drooling, difficulty swallowing, stridor, or meningismus. Obtain a lateral neck radiograph to investigate for widening of the upper cervical prevertebral tissues. Computed tomography is confirmatory. Management includes IV antibiotics and, sometimes, surgical drainage.

Harries PG: Retropharyngeal abscess and acute torticollis. J Laryngol Otol 111:1183–1185, 1997.

10 What are the most common causes of torticollis in well-appearing, afebrile children?

Minor irritation, muscle spasm, and awkward sleep malposition are quite common in young children. The onset is usually sudden, often occurring after waking from sleep. These children have no history of fever or trauma, and should have a completely normal neurologic examination. Supportive care and analgesics/anti-inflammatories are usually the only therapies indicated.

11 What are some common causes of stiff neck in well-appearing, febrile children?

Cervical adenitis. A large, tender node can often be visualized and palpated in the examination of a child with a stiff neck. Most are febrile. Group A streptococcus or S. aureus are the most likely organisms responsible, but Bartonella henselae (cat scratch disease), and mycobacterial disease should also be considered. Pharyngitis/tonsillitis and upper respiratory tract infections may also be associated with stiff neck.

KEY POINTS: COMMON INFECTIOUS CAUSES OF STIFF NECK

1 Meningitis: Ill appearance, irritability, lethargy; fever; neck held stiffly or pain with flexion

2 Tonsillitis/pharyngitis: Red, inflamed tonsils, but not always exudative; consider peritonsillar abscess

3 Cervical adenitis: Single, enlarged, tender cervical node; fever common, but not universal

4 Viral myositis/myalgia: Cervical muscles diffusely tender to palpation; other viral symptoms present

5 Upper respiratory tract infection: Similar to viral myositis, with prominent upper respiratory tract symptoms

12 Define torticollis

Torticollis, which is derived from the Latin tortus, meaning “twisted,” and collum, meaning “neck,” is a characteristic tilting of the head to one side secondary to some underlying disorder. The child with torticollis may or may not have pain, and usually holds the head tilted to one side with the chin rotated in the opposite direction. There is unilateral neck muscle contraction. This condition can be due to a variety of causes, including minor trauma.

Kahn ML, Davidson R, Drummond DS: Acquired torticollis in children. Orthop Rev 20:667–674, 1991.

13 What common pulmonary condition may be associated with torticollis?

Upper lobe pneumonia, which causes referred pain to the neck. It is sometimes difficult to distinguish this from meningitis as both conditions can cause fever, meningismus, and ill appearance.

14 Which gastroenterologic condition may be associated with torticollis?

Sandifer syndrome, which is characterized by intermittent torticollis, opisthotonus, and irritability. This is caused by severe gastroesophageal reflux with esophagitis in infants. Many infants with this condition exhibit failure to thrive.

15 What is the most common oncologic cause of torticollis in children?

Posterior fossa tumors. In addition to head tilt, torticollis, or stiff neck, children with these tumors may present with headache, early-morning vomiting, clumsiness, ataxia, strabismus, visual changes, or papilledema.

Gupta AK, Roy DR, Conlan ES, et al: Torticollis secondary to posterior fossa tumors. J Pediatr Orthop 16:505–507, 1996.

16 What is the most common form of torticollis in infancy?

Congenital muscular torticollis (also called sternocleidomastoid [SCM] tumor of infancy or pseudotumor of infancy). This usually presents in the first 2 weeks of life as a unilateral, hard, immobile, and fusiform swelling in the inferior aspect of the SCM muscle. The infant’s head is tilted toward the affected side. The etiology is controversial. The most common explanation implicates birth trauma with resultant hematoma formation followed by muscle contracture. Another theory postulates that intrauterine abnormal fetal position causes unilateral shortening of the SCM muscle. Passive stretching of the involved muscle is usually curative; recalcitrant cases may require surgical release (< 5% of all cases). Intramuscular injections with botulinum toxin have also been employed.

Cheng JCY, Tang SP, Chen TMK: Sternocleidomastoid pseudotumor and congenital muscular torticollis in infants: A prospective study of 510 cases. J Pediatr 134:712–716, 1999.

Robin NH: Congenital muscular torticollis. Pediatr Rev 17:374–375, 1996.

17 Define paroxysmal infantile torticollis

This is a benign, self-limited condition characterized by intermittent episodes of torticollis that last for minutes to hours and may recur for weeks to months. Onset is usually in the first few months of life. It usually remits by 2–3 years of age. These episodes are associated with pallor, vomiting, agitation, or lethargy. The etiology is unknown.

18 Which congenital syndrome is identified by the triad of short neck (brevicollis), limited neck motion, and low occipital hair line?

Klippel-Feil syndrome. This is a skeletal malformation characterized by the fusion of a variable number of cervical vertebrae. It may also be associated with other bony anomalies and significant scoliosis. These children may have anomalies of multiple organ systems as well. The cause is unknown.

Jones KL: Klippel-Feil syndrome. In Smith’s Recognizable Patterns of Human Malformation, 5th ed. Philadelphia, WB Saunders, 1997.

19 What is Sprengel’s deformity?

This is a congenital failure of the scapula to descend to its correct position. In its most severe form, the scapula is connected by bone to the C-spine and limits neck motion.

Ballock RT, Song KM: The prevalence of nonmuscular causes of torticollis in children. J Pediatr Orthop 16:500–504, 1996.

20 What are the first priorities in managing a trauma victim with a stiff neck?

While maintaining in-line stabilization of the C-spine, ensure adequacy of the airway. The physical examination should then focus on the presence of neurologic deficits (weakness, paresthesias, bowel or bladder dysfunction). If neurologic deficits are present, obtain emergent neurosurgical consultation.

Kupperman N: Neck stiffness. In Fleisher GR, Ludwig S, Henretig FM (eds): Textbook of Pediatirc Emergency Medicine, 5th ed. Philadelphia, Lippincott Williams & Wilkins, 2005, pp 437–447.

21 Which initial radiographs should be obtained in the trauma victim with neck pain?

The routine initial views include an anterior-posterior cervical spine film, lateral cervical spine, and an open-mouth odontoid view.

Swischuk LE: Injured neck with mild pain. Pediatr Emerg Care 12:241–242, 1996.

22 If the initial radiographs are inconclusive, which other images may be helpful?

Flexion/extension views of the cervical spine may be useful in the patient who reports pain yet has no abnormality noted on the initial films. If plain radiographs are still inconclusive, computed tomography of the cervical spine is indicated next. If focal neurologic findings are present, spinal magnetic resonance imaging is indicated.

23 What is the most common anatomic abnormality identified in cases of traumatic torticollis?

Atlantoaxial rotary subluxation. The ligamentous laxity of the pediatric cervical spine predisposes children to this condition, which is often seen after only a minor injury such as a fall from a low height. These patients report stiff neck with pain but have no neurologic deficits. Plain films of the cervical spine may reveal asymmetry of the odontoid relative to the atlas. Computed tomography, ideally with three-dimensional reconstructions or done dynamically, is confirmatory. In most cases, soft collar, rest, and anti-inflammatory agents or analgesics are curative. Severe cases, however, may require traction or surgery.

Muñiz AE, Belfer RA: Atlantoaxial rotary subluxation in children. Pediatr Emerg Care 15:25–29, 1999.

24 Is atlantoaxial subluxation always a result of trauma?

No, atlantoaxial subluxation may result from ligamentous laxity following an infection or inflammatory process. It may be associated with rheumatoid arthritis, systemic lupus erythematosus, or tonsillitis/pharyngitis, and it may follow an otolaryngologic procedure (e.g., tonsillectomy, adenoidectomy). This is called Grisel’s syndrome. Children with Down and Marfan syndromes are particularly susceptible to this subluxation, secondary to the laxity of the transverse ligament of the atlas.

Chipparini L, Zorzi G, De Simone T, et al: Persistent fixed torticollis due to atlanto-axial rotatory fixation. Neuropediatrics 36:45–49, 2005.

25 Are there other injuries to consider in a child with a stiff neck after a minor fall?

Some children with a clavicle fracture may hold their neck to one side and limit movement because of pain and sternocleidomastoid muscle spasm. Always remember to examine/palpate the clavicles of a child who has sustained a fall.

26 What is the name given to drug-induced torticollis?

Dystonic reaction. This is characterized by muscle spasm and abnormal postures, and it most commonly affects the eyes, face, neck, and throat. Patients with dystonic reactions may present with nuchal rigidity, opisthotonus, trismus, oculogyric crisis, or cogwheel rigidity. These patients are awake and often very distraught over their condition. The torticollis is caused by increased cholinergic activity and change in dopaminergic activity in the basal ganglia, which are responsible for muscle tone.

27 Which drugs most commonly cause dystonic reactions in children?

Metoclopramide (Reglan) and phenothiazines such as prochlorperazine (Compazine). These reactions are dose-related and usually begin within 2–5 days of initiating therapy.

28 How are dystonic reactions treated?

IV diphenhydramine (Benadryl) at a dose of 1 mg/kg usually terminates the reaction. Oral diphenhydramine should be continued for several days after discharge from the emergency department. Benztropine mesylate (Cogentin) is an alternate therapy. Of course, the offending drug should be discontinued.

29 What arachnid envenomation may be associated with torticollis?

The bite of Lactrodectus mactans, the dreaded black widow spider, is a neurotoxic envenomation that causes muscle pain and sometimes nuchal rigidity. There is little to no local reaction at the site of the bite. Black widow spider bites are the leading cause of death from spider bites in the United States.

30 Summarize the extensive differential diagnosis of stiff neck in children

See Table 25-1.

Table 25-1 Causes of Stiff Neck

Infectious
Meningitis*	Brain/epidural abscess
Encephalitis	Septic arthritis/osteomyelitis
Epiglottitis	Discitis
Retropharyngeal abscess	Viral myositis/myalgia*
Tonsillitis/pharyngitis*	Upper respiratory infection*
Cervical adenitis*	Pneumonia (upper lobe)
Otitis media	Mastoiditis
Traumatic
Muscle contusion/spasm*	Subarachnoid hemorrhage
Atlantoaxial rotary subluxation	Epidural hematoma
Congenital muscular torticollis*	Spinal cord injury
C-spine fracture	Clavicle fracture*
Congenital
Benign paroxysmal torticollis	Skeletal malformation (Klippel-Feil
Congenital muscular torticollis*	syndrome, Sprengel’s deformity)
Atlantoaxial instability (Down syndrome)	Arnold-Chiari malformation
Toxic
Dystonic reaction*	Black widow spider bite
Oncologic
Posterior fossa tumors	Lymphoma
Benign tumors of the head and neck	Meningeal leukemia
Miscellaneous
Migraine	Spasmus nutans
Sandifer syndrome	Syringomyelia
Collagen vascular disease (juvenile rheuma-	Pseudotumor cerebri
toid arthritis, ankylosing spondylitis)	Psychogenic

* Denotes most common causes of stiff neck.

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