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Stiff Neck and Torticollis

Stiff neck and torticollis (head turned to1 side) can occur separately or together.

Principal Causes of Neck Stiffness and Torticollis

1. Congenital
   1. Congenitalmuscular torticollis (fibromatosis colli)
   2. Skeletal anomalies
   3. Atlantoaxial instability
2. Infection/inflammation
   1. Upperrespiratory tract infection
   2. Pharyngitis/tonsillitis
   3. Cervical adenitis
   4. Myositis
   5. Meningitis
   6. Cervical spine osteomyelitis
   7. Spinal epidural abscess
   8. Discitis
   9. Juvenile rheumatoid arthritis
3. Trauma
   1. Muscle strain or contusion
   2. Cervical spine fracture, subluxation,and dislocation
4. Drugs
5. Neoplasm
   1. Brain tumor
   2. Cervical spine and spinal cord tumors
   3. Leukemia
6. Subarachnoid hemorrhage
7. Benign paroxysmal torticollis
8. Torticollis with gastroesophageal refluxand hiatal hernia (Sandifer syndrome)
9. Spasmus nutans
10. Ocular or vestibular disturbances
11. Psychogenic

Clinical Features and Diagnosis

Congenital

Congenital Muscular Torticollis (Fibromatosis Colli)

Usuallypresents at 1–2 wks of age with firm, nontender mass inlower aspect of sternocleidomastoid muscle.

It is thought that fibrosis of musclein fetal life produces the mass.

Diagnosis is usually clinical.

Skeletal Anomalies

Short neckwith limited motion and low occipital hairline characterize Klippel-Feil syndrome.Variable number of cervical vertebrae are fused in this syndrome.

Failure of normal descent of scapulais known as Sprengel deformity, which may cause limited neck movement.

Malformation of first cervical vertebracalled hemiatlas also may cause torticollis.

Cervical spine radiography is oftendiagnostic for these anomalies. CT or MRI also may be necessaryin some cases.

Atlantoaxial Instability

Some conditions (e.g., trisomy 21, Morquiosyndrome, Klippel-Feil syndrome, and odontoid aplasia/hypoplasia)may be associated with atlantoaxial instability, which predisposesindividual to cervical subluxation. Cervical spine radiography isdiagnostic.

Infection/Inflammation

Fever, neckpain, and stiffness with or without torticollis may occur with manyinfections, including viral upper respiratory infection, pharyngitis,tonsillitis, cervical adenitis, viral myositis, meningitis, cervicalspine osteomyelitis, and spinal epidural abscess. These disordersare discussed in other chapters.

Discitis may produce neck pain andstiffness but is more common in lumbar and thoracic areas (see Chap. 5, Back Pain).

Juvenile rheumatoid arthritis may involvecervical spine and lead to neck pain and stiffness. Seldom occursas isolated event, and other joints are usually involved. Radiographyof cervical spine may show ankylosis of apophyseal joints, narrowingof disk spaces, and atlantoaxial dislocation (see Chap. 37, Limp).

Trauma

Muscle Strain or Contusion

Upon awakeningin morning, individuals may have stiff neck and torticollis, caused bylying in particular position.

Muscle strain is common with any typeof trauma, especially whiplash injury from automobile accident.

Head is often fixed in 1 position,with spasm and tenderness of affected neck muscles.

History and physical exam are usuallydiagnostic. Cervical spine radiography should be performed withhistory of neck trauma.

Cervical Spine Fracture, Subluxation, and Dislocation

Almost alwayscaused by traumatic injuries (e.g., automobile accidents, falls,or athletic injuries).

Fracture of cervical vertebra usuallyproduces pain and tenderness over involved vertebra. Cervical subluxationsor dislocations in children usually occur between C1 and C2 andbetween C2 and C3. Tenderness over involved joint, limited rangeof motion of neck, and torticollis are frequent manifestations.

Cervical spine radiography usuallyconfirms diagnosis; however, CT may be necessary if uncertaintyexists about location and extent of injury. MRI is invaluable inevaluation of spinal cord and epidural space.

Drugs

Drugs (e.g., haloperidol and metoclopramide)may produce acute dystonic reactions resulting in torticollis. Thesemanifestations resolve with use of diphenhydramine.

Neoplasm

Brain Tumor

Tumors ofposterior fossa may cause neck stiffness and torticollis as wellas headache, vomiting, ataxia, and visual disturbances. Head tiltmay occur to compensate for diplopia.

Brainstem tumors also may produce neckstiffness as well as cranial nerve palsies and pyramidal tract signs.

CT and MRI locate and define extentof these tumors. Histologic diagnosis is definitive.

Cervical Spine and Spinal Cord Tumors

Tumors ofcervical spine may produce neck pain and torticollis. Most commontumor is osteoid osteoma.

Spinal cord tumors also may produceneck pain and torticollis as well as arm and leg weakness, impairedsensation below the level of lesion, and bowel and bladder dysfunction.Most common spinal cord tumor is astrocytoma.

Combination of cervical spine and skullradiography, CT, and MRI locate and define extent of mass. Histologicdiagnosis is definitive.

Leukemia

Meningeal leukemia is another cause of neckstiffness. Diagnosis of leukemia is discussed in Chap. 38, Lymphadenopathy.Analysis of spinal fluid reveals presence of leukemic cells.

Subarachnoid Hemorrhage

Onset is acute, with intense headache andstiff neck. Most common causes in pediatric population are headtrauma and rupture of cerebral aneurysm or arteriovenous malformation(see Chap. 25, Headache).

Benign Paroxysmal Torticollis

Migrainevariant that usually occurs in infants and toddlers.

Characterized by recurrent episodesof torticollis associated with vomiting, irritability, and sometimesheadache that may last hours or days.

Episodes usually resolve by 2–3yrs of age.

Torticollis with Gastroesophageal Reflux and Hiatal Hernia(Sandifer Syndrome)

Sandifersyndrome is association of torticollis with hiatal hernia and gastroesophagealreflux.

Persistent vomiting and poor weightgain are features of this syndrome.

Upper GI series can diagnose hiatalhernia.

Spasmus Nutans

Constellationof nystagmus, head nodding, and torticollis.

Typically occurs in children <2yrs and is generally self-limited.

CT or MRI should be considered to excludea lesion (e.g., glioma) that affects anterior visual pathway.

Ocular or Vestibular Disturbances

Torticollismay occur in children with visual disturbances (e.g., strabismusor refractive errors). Head may be turned to achieve binocular vision.

Disturbances of vestibular system,which are discussed in Chap.73, Vertigo, also may cause torticollis.

Careful ophthalmologic and neurologicexam is necessary.

Psychogenic

Emotional stress may cause hysterical reactionwith inability to turn or hold head normally. History and physicalexam are diagnostic.

Diagnostic Approach

Useful distinctionis whether neck stiffness or torticollis is congenital or acquired. Congenitalmuscular torticollis can usually be diagnosed by physical exam.Cervical spine radiography can diagnose skeletal spine anomaliesand atlantoaxial instability.

Most common acquired causes of neckstiffness or torticollis are muscle strain, other musculoskeletaltrauma, pharyngitis, tonsillitis, cervical adenitis, viral myositis,and meningitis.

With history of trauma, cervical spineradiography should be performed.

Presence of fever usually signifiesinfection.

Lumbar puncture should be performedwith suspected meningitis.

Fever and localized tenderness of cervicalspine suggests cervical osteomyelitis, and cervical spine radiographyand bone scintigraphy are often helpful.

History and physical exam should suggestpresence of other causes of neck stiffness and torticollis.

CT is initial procedure of choice forsuspected subarachnoid hemorrhage or primary brain tumor. This proceduremay be followed by MRI.

References

1. Beasley SW. Torticollis. In: O'NeillJA Jr, et al., eds. Pediatric surgery, 5th ed. St. Louis: Mosby-YearBook, 1998:773–778.
2. Cheng JCY, et al. Sternocleidomastoid pseudotumor andcongenital muscular torticollis in infants: A prospective studyof 510 cases. J Pediatr 1999;134:712–716.
3. Dubousset J. Torticollis in children caused by congenitalanomalies of the atlas. J Bone Joint Surg [Am] 1986;68:178–188.
4. Epps HR, Salter RB. Orthopedic conditions of the cervicalspine and shoulder. Pediatr Clin North Am 1996;43:919–931.
5. Gupta AK, et al. Torticollis secondary to posteriorfossa tumors. J Pediatr Orthop 1996;16:505–507.
6. Hensinger RN, et al. Changes in the cervical spinein juvenile rheumatoid arthritis. J Bone Joint Surg [Am] 1986;68:189–198.
7. Kuppermann N. Neck stiffness. In: Fleisher GR, LudwigS, eds. Textbook of pediatric emergency medicine, 4th ed. Philadelphia:Lippincott Williams & Wilkins, 2000:391–400.
8. McIntosh D, et al. Torticollis and bacterial meningitis.Pediatr Infect Dis 1993;12:160–161.
9. Parker W. Migraine and the vestibular system in childhoodand adolescence. Am J Otol 1989;10:364–371.
10. Smith MD. Orthopedic aspects of cervical spine disordersin children and adolescents. Curr Opin Pediatr 1993;5:374–378.
11. Stein MT, Trauner D. The child with a stiff neck. ClinPediatr 1982;21:559–563.
12. Sutcliffe J. Torsion spasms and abnormal postures inchildren with hiatus hernia—Sandifer's syndrome.Prog Pediatr Radiol 1969;2:190–197.
13. Williams CRP, et al. Torticollis secondary to ocularpathology. J Bone Joint Surg [Br] 1996;78:620–624.

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Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

Other Book Chapters Related to Stiff neck

Read excerpts from these other book chapters related to Stiff neck:

Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2008 Williams & Wilkins.

More About Causes of Stiff neck

• Back to symptom: Stiff neck: Introduction (review 127 causes)
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More About This Book: Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics Authors: Paul S. Bellet Publisher: Lippincott Williams & Wilkins Copyright: 2006 ISBN: 0-78172-899-1

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