CHAPTER 47 Medical Causes of Neck Pain
INTRODUCTION
Neck pain is a frequent complaint to doctors in Western societies.1 Valkenburg et al.2 demonstrated in a large epidemiological study in the Netherlands that the lifetime prevalence of neck pain was 30% in males and 43% in females. By definition, neck pain is pain perceived in a region bounded superiorly by the superior nuchal line, laterally by the lateral margins of the neck, and inferiorly by an imaginary transverse line through the T1 spinous process.3 Pain in the neck may arise from any of the structures in the neck, which include the ligaments, osseous structures, intervertebral discs, muscles, zygapophyseal joints, dura, and nerve roots.
TUMORS
Primary cervical spine bone tumors are rare, accounting for only 0.4% of all tumors4 and for only 4.2% of the primary bone tumors that occur in the spine above the sacrum.5 Cervical spine primary bone tumors occur disproportionately less frequently than those in the thoracic and lumbar spine. Dreghorn et al.6 found that five of 55 primary spinal bone tumors were cervical. Boriani and colleagues5 noted 63 cervical tumors out of 366 primary bone tumors of the spine. In another series, Weinstein and McLain7 reported only six of 82 primary spinal bone tumors involved the cervical spine.
Primary benign tumors of the cervical spine
Primary benign bone tumors of the cervical spine occur most commonly in the first 20 years of life, with the incidence of malignant tumors increasing significantly with age. In a series of 41 benign tumors of the cervical spine (patient ages 4–50 years old) treated at the Istituto Ortopedico Rizzoli between 1952 and 1988, Levine and colleagues8 noted that 32 patients were younger than 20 years old. Another study, by Bohlman et al.,9 noted that nine out of nine patients under 21 years old had benign cervical tumors, but 10 of 12 patients over 23 years old had a primary malignant tumor. Neurologic findings may be common on initial presentation. The most common initial symptom is neck pain that is often worse at night, with continued pain at rest that is not typically relieved with analgesics.9 The duration of symptoms may be prolonged, averaging 19 months in some series.10
Benign primary bone tumors are generally distributed to all levels of the cervical spine. The most common vertebral levels are C2, C4, and C7; C1 is the least frequent site.11 Radicular symptoms can be a common complaint but neurologic abnormalities may be absent. In a series by Levin et al.10 of 41 patients, most of the benign tumors were located in the posterior elements. Osteoid osteomas and osteoblastomas were found in the pedicles and facet joints. Giant cell tumors, eosinophilic granulomas, and aneurysmal bone cysts were most common in the vertebral body, although aneurysmal bone cysts commonly occur in the posterior elements as well.10
Hemangiomas
Hemangiomas are the most common benign spinal tumor and occur in about 10% of the population. While they are usually asymptomatic, pathological fractures or expansion of the bony architecture into neural structures may create symptoms.12 These tumors demonstrate a slight female predominance. Hemangiomas of the cervical spine are much less common than in the thoracic and lumbar spines. In a review of literature, Nguyen and colleagues found 10 of 148 reported cases of hemangiomas were located in the cervical spine. Radiographically, hemangiomas have a striated or honeycomb texture, which is the expression of hamartomatous growth of the vascular tissue within the vertebral body. The posterior spinal elements are rarely involved.10
Aneurysmal bone cyst
Aneurysmal bone cysts typically occur in the first two decades of life without male or female predilection.13 Isolated disease commonly occurs in the posterior arch, but patients may have circumferential involvement.14 Aneurysmal bone cyst is a pseudotumoral, hyperplastic, and hemorrhagic lesion whose pathogenesis is unknown. These cysts frequently occur in the spine, most commonly in the thoracic or lumbar spine.15 Twenty-five percent of aneurysmal bone cysts of the spine observed at the Rizzoli Institute presented in the cervical spine.16
Fibrous dysplasia
Fibrous dysplasia is a hamartomatous condition rarely occurring in the vertebrae. When it does occur, it is usually asymptomatic and generally found incidentally. It can, however, cause weakening of trabecular structures and vertebral collapse, thus causing neck pain, muscle spasm, and associated torticollis. Typical radiographs may show an expanded vertebral outline with faded radiolucency of the vertebral body. No treatment is necessary for latent lesions, but stability is indicated when mechanical failure occurs or is impending.17 Radiotherapy should be avoided in active lesions as it is ineffective and can induce sarcoma. Rarely, bone grafting and curettage may be advised.18
Osteoid osteoma
The most common benign primary tumor in the cervical spine is osteoid osteoma, which is frequently found in the posterior arch.8 The male to female ratio of occurrence is 2:1 with most cases presenting before the age of 25. One-tenth of all osteoid osteomas occur in the spine and 30% of those in the cervical region. One should consider this diagnosis among adolescents or young adults complaining of persistent neck pain frequently associated with nondermatomal radiation to the upper limb. The pain often interferes with sleep, sometimes causing muscle spasm or torticollis. Patients may respond favorably to aspirin. On imaging, this tumor appears as a small radiolucent area which is more commonly identified by the zone of reactive sclerosis around it. Bone scans are often helpful to focus the examinations on standard radiographs and localize the CT/MRI scan to the suspected lesion. Although there have been some cases of spontaneous regression, curettage of the nidus is usually necessary. In addition, one potential developing technology is percutaneous radiofrequency denervation, which has shown effectiveness in nonspinal lesions and may be useful for the cervical spine.
Osteoblastoma
Osteoblastomas are similar to osteoid osteomas but are larger (>2 cm) and less common. Forty percent of reported osteoblastoma cases were found in the spine19 with 25% of those occurring in the cervical region. There is a male predominance and they generally occur before the age of 25. They most commonly arise from the posterior elements, sometimes invading the vertebral body. Symptoms range from nocturnal pain to severe neck pain with muscle spasm.20 Plain radiographs can usually identify osteoblastomas. Patterns vary from that of a giant osteoid osteoma, a rounded radiolucent mass larger than 2 cm with variable amount of scanty ossifications, to that of a highly aggressive purely lytic lesion, which is distinguished from low-grade osteosarcoma.19 The ‘active’ lesions are positive on bone scan and present on CT scan with well-marginated sclerotic borders and a thin reactive shell that thickens the cortical outline.21 These lesions can be treated with curettage with low recurrence rates (5–10%). More aggressive lesions can also be treated with curettage but have higher recurrence rates (20%). If feasible, selective arterial embolization is mandatory to reduce bleeding before curettage and may serve as a treatment option or adjuvant. Cryotherapy or postoperative radiotherapy may also be indicated.
Giant cell tumors
Giant cell tumors are ironically considered ‘benign’ by some despite the fact that they have a high recurrence rate and sometimes metastasize into the pulmonary region. Their prevalence in the spine is low (2–10%). Giant cell tumors are more common in females, with a peak incidence in the third decade. On plain radiographs, they often manifest as purely lytic lesions. Staging utilizes a combination of plain films, bone scan, CT scan, MRI, and biopsy. Surgical treatment depends on the staging of the tumor and the degree of involvement of critical vascular and neural structures. Stage 2 lesions may be treated with curettage plus adjuvant therapy (phenol, liquid nitrogen, or methylmethacrylate), whereas stage 3 lesions may require marginal en bloc resection. If the tumor demonstrates malignant transformation, then radiation therapy may be initiated. Preoperative embolization should be considered to reduce blood loss and morbidity. Giant cell tumors have a high recurrence rate and re-resection is often not an option. Therefore, repeated embolization may be necessary to control or perhaps even cure a very large lesion.22 In addition, cryosurgery,23 bisphophonates,24 and interferon and radiotherapy may hold some potential for control of the lesion.
Osteochondroma
Osteochondromas are the second most common tumor of the bone but occur infrequently in the spine (1–3% of all osteochondromas). They are often asymptomatic but may become painful if an exostosis grows into the spinal canal or if it develops a large subcutaneous protuberance. In most cases, a palpable painless mass arising from the anterior or anterolateral surface of the vertebral body is found by the patient during skeletal maturation. The tumor forms dense conglomerate masses containing radiolucent areas with clusters of calcifications; typically, the cortex of the host is evaginated, with the cancellous bone continuing directly into the vertebral cancellous bone.25
Chondrosarcoma
Chondrosarcoma is a malignant bone tumor whose cells tend to differentiate into cartilage. Its prevalence in the spine is greater than 6%26 but shows no specific prevalence in the cervical, thoracic, or lumbar segments. Patients may present with a slow-growing mass or neurological symptoms if the tumor compresses nearby neural or vascular structures. Plain radiographs may show an extraosseous mass found arising from the posterior elements of the involved vertebra. This may appear irregularly ossified, lobulated, or cauliflower-like, with irregular limits extending out toward the soft tissues. On CT scan, a cartilaginous cap may be appreciated. Treatment is surgical excision, as radiation and chemotherapy are not effective.
Chordoma
Chordomas are malignant tumors that transform from ectopic notochordal remnants and are typically seen in the spheno-occipital region (including the first cervical vertebra) and sacrum. They are slow-growing tumors which generally manifest clinically after the fifth decade of life. Fewer than 5% occur before age 20. Chordomas of the cervical spine occur almost exclusively in the vertebral body. The most common complaint is a long history of mild neck pain together with symptoms related to an anterior slow-growing mass: dysphagia, upper respiratory obstruction, and Claude-Bernard-Horner syndrome, or slow cord and nerve root compression from expansile chordomas within the spinal canal.9,22 In rare cases, a palpable mass may be appreciated. Cranial nerve compression can be provoked by craniocervical chordomas.28 On plain radiographs, chordomas generally appear completely radiolucent, although calcifications may occasionally be present within the tumor mass.29 Treatment options include surgery and radiation therapy. Metastases are rare and late; however, local progression of the disease is the most frequent cause of death.
Malignant fibrous histiocytoma
Malignant fibrous histiocytoma is a sarcoma of histiocytic origin that consists generally of histiocytes and fibroblasts and is quite rare in the cervical spine.18 In the cervical region, pathologic fracture and neurological symptoms may occur at onset as a result of the rapid collapse of bone. Cases of malignant fibrous histiocytoma can be confused with eosinophilic granulomas. However, because of their rapid progression, one should suspect malignancy in these rapidly growing, large, soft tissue masses that occurs during the second and third decade of life. Treatment of fibrous histiocytomas in the cervical region consists of palliative curettage combined with radiation and chemotherapy. Prognosis is poor.
Osteosarcoma
Osteosarcoma is the most common malignant bone tumor but is extremely rare in the spine. Some reported cases of osteosarcoma of the spine are secondary, arising from Paget’s disease, or after radiation therapy.30 If osteosarcoma does occur in the spine, it arises from the vertebral body31 or from the posterior elements, but frequently involves the whole vertebra at the time of diagnosis.32 Varied patterns may appear on plain radiographs, ranging from total radiolucent vertebra with collapse and kyphosis to so-called ‘ivory’ vertebra with variable amounts of surrounding neoplastic tissue. The course of the disease is rapid, with early lung metastases. Treatment considerations include intralesional excision combined with chemotherapy and radiation therapy.
METABOLIC CAUSES OF NECK PAIN
Paget’s disease
Paget’s disease of the bone (osteitis deformans) is a chronic skeletal disorder, which may result in enlarged or deformed bones in one or more regions of the skeleton. Paget’s disease has a predilection for the axial skeleton and may be widespread at the time of diagnosis. It can present as chronic neck pain and stiffness when the cervical spine is involved. The condition commonly affects the pelvis and spine, particularly the lumbar spine, with a frequency of 30–75%. The sacrum is involved in 30–60% of cases and the skull in 25–65% of cases.33 The proximal long bones, especially the femur, also are affected in 25–35% of cases.34
In many cases, Paget’s disease is asymptomatic. Often, the disease is so mild that it is not diagnosed or symptoms may be mistaken with arthritis or other disorders. In other cases, the diagnosis is made only after complications have developed. The excessive bony remodeling associated with Paget’s disease may result in pain, fractures, limb bowing, and hearing loss if the auditory ossicles are affected. Complications associated with fractures, such as articular and neurologic problems, increase mortality in these patients. Sarcomatous degeneration also may occur but is less prevalent. The prognosis is extremely unfavorable if the patient has any type of sarcomatous degeneration, especially those with multicentricity. The 5-year survival rate for a patient with Paget’s disease and sarcoma is 5–7.5% but increases to 50% for those who undergo operative tumor ablation and chemotherapy before metastases. The 5-year survival rate for elderly patients with primary nonpagetic sarcoma is 37%. A more axial lesion carries a less favorable prognosis since higher doses of radiation can be delivered to the appendicular skeleton.35
Laboratory and radiographic studies are beneficial in arriving at the diagnosis. Biochemical indices reveal elevated alkaline phosphatase levels of bone origin, due to increased osteoblastic activity and bone formation. Analysis of alkaline phosphatase isoenzymes helps to identify the hepatic contribution to total levels of alkaline phosphatase. Radiographs reveal a typical expanding lytic lesion, transverse lucent areas or osteoporosis circumscripta, thickened cortices, sclerotic changes, and bone expansion with coarse disorganized trabecular patterns. Bone scanning is the most sensitive test for evaluating the extent of lesions in Paget’s disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget’s disease.36
The causes of Paget’s disease are still not clearly defined. Research suggests that Paget’s disease may be caused by a ‘slow virus’ infection of bone, a condition that is present for many years before symptoms appear. There is also a hereditary factor, which may be the reason that family members with Paget’s disease are susceptible to the suspected virus. Paget’s disease is the second most common bone disease in the United States, after osteoporosis. Paget’s disease is rarely diagnosed in people under 40. Men and women are affected almost equally. Prevalence in the population ranges 1.5–8% in older adults, depending on the patient’s age and where he or she lives. Several effective therapies for Paget’s disease have recently been approved in the United States.37
INFECTIOUS CAUSES OF NECK PAIN
Neck pain is present in over 90% of patients with a cervical spinal infection.38 While cervical spine infections are less frequent than infections in the lumbar or thoracic spine, they deserve important consideration because they have the highest risk for neurologic compromise and disability.39 In this section, infections are classified by anatomic location for easier diagnosis.
Discitis and vertebral osteomyelitis
Spondylodiscitis can develop from open spinal trauma, infections in adjacent structures, hematogenous spread of bacteria to a vertebra, or it can occur postoperatively. The cervical spine is only involved in 6% of cases of spondylodiscitis.40,41 Older and immunocompromised patients, including those with diabetes, organ transplantation, malnutrition, cancer, and HIV, are particularly susceptible to developing spondylodiscitis.39,42 Of note, the incidence of spondylodiscitis among intravenous drug users is in the cervical spine.38
Patients may present acutely, subacutely, or with chronic symptoms. Intravenous drug users generally present most acutely.38 Fifty percent of patients have symptoms for over 3 months before presentation.41
Pain over the infected intervertebral segment is the first and most common clinical symptom, with over 90% of patients with cervical spondylodiscitis presenting with pain.38 The pain commonly radiates to the upper trapezius region or the shoulder but may present with a true radicular component if it is complicated by contiguous spread to an epidural abscess. Approximately only 50% of patients are febrile at the time of diagnosis.41 Neurologic compromise occurs in 17% of all patients with spondylodiscitis, but 50–82% of cervical patients have neurological involvement.43 The most common physical examination findings are bony tenderness, paravertebral muscle spasm, and range of motion limitations.44,45
White blood cell count may be elevated in patients with acute cervical infection, but is often normal in patients with chronic infections. However, the erythrocyte sedimentation rate (ESR), though non-specific, is elevated in over 90% of patients and may be a useful indicator of treatment response.41 C-reactive protein (CRP), which has a shorter half-life, is also valuable in monitoring disease response. Blood cultures are positive in 24% of patients.41
Plain radiographic abnormalities are usually not evident until 2–4 weeks after infection. Early findings include disc-space narrowing and abnormal prevertebral soft tissue contours. This is followed by destructive changes in the vertebral endplates and the anterior aspect of the vertebral body which may lead to fracture, collapse, and kyphosis.40,41
Computed tomography may be helpful, as may gallium radionuclide studies and single-photon emission CT (SPECT). However, MRI with gadolinium is considered the diagnostic test of choice, with 96% sensitivity, 93% specificity, and 94% accuracy in detecting spondylodiscitis.46 Decreased signal intensity on T1-weighted imaging, increased signal intensity on T2-weighted imaging, and enhancement on T1-weighted fat suppression imaging are noted47,48 Paravertebral infection, collections under the posterior longitudinal ligament, and epidural abscesses can also be best delineated on T1-weighted fat suppression images.48
However, absolute diagnosis must be based on bacteriologic or histologic examination.39 Percutaneous needle biopsies and open biopsies can safely be performed, with open biopsies yielding a greater percentage of definitive histologic findings.
The choice of an antibiotic agent should be based on culture and sensitivity results. If no neurologic compromise is noted, antibiotic treatment should be withheld until culture results become available. In septic or neurologically compromised patients, however, immediate broad-spectrum antibiotics should be started as soon as cultures are obtained. Once cultures become available, these should be changed to the most specific and least toxic agent. In consultation with an infectious disease specialist, patients usually receive 6 weeks of intravenous antibiotics, which may be followed by an oral regimen.39 The clinical picture and ESR should be used as a guide to successful treatment.49 In addition, the patient should be afebrile, have painless cervical spine range of motion, and have a nonfocal neurologic examination.
Immobilization may limit pain and prevent deformity. In general, upper cervical spinal infections may require a halo vest and lesions in the lower cervical spine can be managed in a hard collar or cervicothoracic orthosis.39
Surgical treatment is indicated in cases requiring decompression for neural compromise, persistent pain, or elevated ESR despite treatment or to obtain bacteriologic diagnosis if needle biopsy has been equivocal.50 After debridement of the focus of infection, bone grafting or posterior instrumentation are performed to maintain stability.
The mortality is less than 5%, but is much higher in elderly patients and those with diabetes or rheumatoid arthritis.47 Fewer than 7% of all patients have residual neurologic deficits.47
Epidural abscess
The incidence of pyogenic infections in the epidural space is seen in approximately 2/10 000 hospital admissions but appears to be increasing.51 Most studies place the incidence of cervical epidural abscess at 6–18% of all epidural abscesses.52,53 The lower incidence is likely due to the epidural space in the cervical region being smaller than the thoracic or lumbar spine. Most cervical abscesses are located anteriorly in the epidural space where thoracic and lumbar epidural abscesses are commonly seen posteriorly.54,63
Clinical presentation is quite variable, resulting in a 50% incidence of initial misdiagnosis. Patients generally appear sicker than they do with spondylodiscitis and may present with fever, axial pain, spinal tenderness, and nuchal rigidity in acute cases. All the above symptoms/signs may be absent in chronic cases.53,54 In general, the disease can progress from local spinal pain to radicular pain, followed by weakness and potential paralysis.39
The ESR is generally increased in acute cases, as is the white blood cell count. Definitive diagnosis is, however, based on culture results, which are positive in 90% of cases if taken from the abscess directly, 60% if blood cultures are taken, and as low as 11% if taken from the cerebrospinal fluid (CSF).52,53
An epidural abscess, especially one in the cervical region, is a true emergency and surgical decompression and intravenous antibiotic treatment need to be considered immediately.52,54 Broad-spectrum antibiotics should be started after cultures are obtained and should be changed based on culture results. Most authors recommend 3–4 weeks of intravenous therapy if an epidural abscess is the only lesion, and 6 weeks if spondylodiscitis is also present.52 Since most cervical epidural abscesses occur anteriorly, an anterior approach is often utilized for decompression and debridement. Instrumentation and fusion may be necessary if the decompression jeopardizes the stability of the spine.
The prognosis for patients with cervical disease is worse than for those with thoracic or lumbar infections. With aggressive treatment, a majority of patients recover with either minimal or no weakness,51 though prognosis for neurologic recovery depends on the duration and severity of the neurologic deficit. If paralysis persists for longer than 36–48 hours, the likelihood of recovering adequate neurologic function is very poor.54 Prognosis is also worse in diabetics, patients with HIV infection, and those who have concomitant spondylodiscitis. The mortality rate is as high as 38%, even with aggressive treatment.52
Subdural and intramedullary abscesses
Though subdural and intramedullary abscesses are rare, they present with spinal pain in one-third of all cases.55 They can also present with weakness, fever, and radicular pain. There are no stages of progression for either, as opposed to the four stages noted for epidural disease. As in all infections discussed thus far, the cervical spine is the least often affected region of the spine and infection is most commonly spread by the same mechanisms as epidural abscesses. Most infections are caused by S. aureus, though Streptococcus, E. coli, and Pseudomonas species have also been cultured.55
Deep neck infections
Peritonsillar infections are the most common deep neck infections (49%), followed by retropharyngeal infections (22%), submandibular infections (14%), and buccal infections (11%).56 Deep neck infections are usually secondary to contiguous spread from local sites due to pharyngitis, tonsillitis, otitis media, or after dental procedures.57 The microbiology of deep neck infections usually reveals mixed aerobic and anaerobic organisms in approximately 90% of patients, often with a predominance of oral flora. Both Gram-positive and Gram-negative organisms may be cultured.58
Most patients present with generalized symptoms, including fever, chills, and malaise. Localizing symptoms include odynophagia, dysphagia, sore throat, neck stiffness, neck pain, trismus, and voice changes. Physical examination findings include neck swelling, elevation of the floor of mouth, drooling, diaphoresis, fever, and bulging or asymmetry of the pharyngeal wall.58 Tachypnea or shortness of breath may indicate emergent airway collapse.59
The work-up should include white blood cell count with differential, blood cultures, and plain radiographs of the cervical spine. On lateral radiographic view, prevertebral soft tissue thickening greater than 7 mm anterior to the C2 vertebral body or 22 mm anterior to the C6 vertebral body indicates a space-occupying lesion which would most likely be a retropharyngeal abscess.60
In clinical studies, CT with contrast combined with physical examination findings resulted in a sensitivity of 95% and a specificity of 80% for diagnosing deep neck infections. Abscesses are seen as low-density lesions with rim enhancement, occasional air fluid levels, and loculations.61 CT scanning of the chest may be helpful if extension into the mediastinum is suggested. The use of MRI for diagnosis of deep neck infections has not been studied in detail, though MRIs can give excellent soft tissue resolution to help localize the region of involvement.
Depending on respiratory symptoms, the airway must be secured prior to any further treatment. Addressing the airway may involve observation, endotracheal or nasotracheal intubation, tracheostomy, or cricothyroidotomy for emergent situations. Subsequently, broad-spectrum antibiotics should be initiated. In patients with small fluid collections and no respiratory compromise, 50% of deep neck infections can be managed nonsurgically.62 In patients who do not improve within 48–72 hours of antibiotic therapy, or if respiratory compromise is imminent, surgical or CT-guided drainage should be undertaken. Patients treated for deep neck infections can be expected to recover fully as long as the infection is treated properly and in a timely manner.
Meningitis
Bacterial meningitis is historically considered a pediatric illness. However, since the 1990 licensure of conjugate Haemophilus influenzae type b vaccines for use in infancy, the majority of cases of bacterial meningitis now occur in adults.63 In order of frequency, the five most common organisms implicated in bacterial meningitis are Streptococcus pneumoniae, Neisseria meningitidis, Streptococcus agalactiae, Listeria monocytogenes, and Haemophilus influenzae.64
The classic presentation of meningitis includes fever, headache, neck stiffness, photophobia, nausea, vomiting, and signs of cerebral dysfunction (e.g. lethargy, seizure, confusion, or coma). However, the classic triad of fever, nuchal rigidity, and mental status change is found in only two-thirds of patients.65,66 On examination, cranial nerve palsies or other focal neurologic signs may be noted.
In general, whenever the diagnosis of meningitis is strongly considered, a lumbar puncture should be performed promptly. The cornerstone in the diagnosis of meningitis is examination of the CSF. Opening pressure is measured and fluid sent for cell count (and differential count), chemistry (i.e. CSF, glucose and protein), and microbiology (i.e. Gram stain and cultures). CSF Gram stain permits rapid identification of the bacterial cause in 60–90% of patients with bacterial meningitis, and CSF bacterial cultures yield the bacterial cause in 70–85% of cases.67 Also, white blood cell count with differential and peripheral blood cultures are required as part of the evaluation. There is minimal role for imaging of the neck in meningitis other than to rule out other etiologies.
Treatment is started with empiric intravenous antimicrobial therapy (i.e. antibacterial treatment or antivirals and antifungal therapy in selected cases) as soon as the lumbar puncture and blood cultures have been drawn.68 Once culture results are available, the antibiotic can be changed, though most cases of bacterial meningitis are treated with broad-spectrum cephalosporins for 14–21 days.65,68
Tuberculosis
Tuberculosis deserves special mention as it may lead to neck pain secondary to: tuberculous spondylitis, paraspinal or retropharyngeal abscesses, vertebral collapse, atlantoaxial subluxation and epidural, subdural, or intramedullary granulomas.39 In addition to spinal pain, patients generally present with malaise, weight loss, and intermittent fevers.
The ESR is generally elevated and the tuberculin purified protein derivative skin test is usually positive.69 Sputum culture may be helpful in patients with pulmonary disease. While radiographs may demonstrate scalloping of the anterior aspect of the vertebrae and bone rarefaction, MRI is the imaging modality of choice. Since intervertebral discs are generally resistant to tuberculosis, disc signal will likely remain preserved. Signal changes on MRI are similar to those seen in pyogenic etiologies. However, with gadolinium, granulomas can be easily differentiated from abscesses since they enhance globally, while abscesses generally enhance in the periphery.70
First-line medications currently include isoniazid, rifampin, pyrazinamide, streptomycin, and ethambutol. Multiple drugs should be used after consultation with an infectious diseases specialist to counteract resistance. Treatment may be continued on an outpatient basis, maintaining the oral regimen for 6–9 months.71 Surgery is indicated if neurologic compromise occurs, medical therapy is failing, or deformity is present. Before surgery, patients with pathologic fractures, kyphosis, or spinal instability should be immobilized in skeletal traction.39
Prognosis for patients with tuberculosis of the spine has improved since chemotherapeutic regimens have diversified. Currently, mortality rates are below 5%.69 In general, early surgery results in a better prognosis. In one study, all patients were relieved of their neck pain within a few days of surgery and kyphosis was corrected from 25.5 to 5.4 degrees.72
1 Andersson GBJ. The epidemiology of spinal disorders. In: Frymoyer JW, editor. The adult spine: principles and practice. Philadelphia: Lippincott Raven; 1997:130-141.
2 Valkenburg HA, Laar A, Van Hofman A, et al. Nek-en lage rugklachten. In: Instituut epidemiologie. Jaarverslag instituut epidemiologie en zesde voortgangsverslag van het epidemiololgisch preventief onderzoek Zoetermeer. 1980:99–109.
3 Merskey H, Bogduk N, editors. Classification of chronic pain: Descriptions of chronic pain syndromes and definition of pain terms, 2nd edn.. IASP Press, Seattle, 1994;103-111.
4 Weinstein JN. Surgical approach to spine tumors. Orthopaedics. 1989;12:897-905.
5 Boriani S, Biagini R, De Iure F, et al. Primary bone tumors of the spine: a survey of the evaluation and treatment at the Istituto Ortopedico Rizzoli. Orthopedics. 1995;18:993-1000.
6 Dreghorn CR, Newman RJ, Hardy GJ, et al. Primary tumors of the axial skeleton. Experience of the Leeds Regional Bone Tumor Registry. Spine. 1990;15:137-140.
7 Weinstein JN, McLain RF. Primary tumors of the spine. Spine. 1987;12:843-851.
8 Levine AM, Boriani S, Donati D, et al. Benign tumors of the cervical spine. Spine. 1992;17:399-406.
9 Bohlman HH, Sachs BL, Carter JR, et al. Primary neoplasms of the cervical spine: diagnosis and treatment of twenty-three patients. J Bone Joint Surg. 1986;68:483-493.
10 Levin AM, Boriani S. Benign tumors of the cervical spine. In: Editorial Committee, editor. The cervical spine. Philadelphia: Lipppincott-Raven; 1998:621-640.
11 Liu H, Liu Z. Surgical treatment of cervical spine tumors. J Chinese Med. 1992;105:564-566.
12 McAllister VL, Kendall BE, Bull JWD. Symptomatic vertebral haemangiomas. Brain. 1975;98:71-79.
13 Papagelopoulous PJ, Currier BL, Shaughnessy WJ, et al. Aneurysmal bone cysts of the spine: management and outcome. Spine. 1998;23:621-628.
14 Hay MC, Paterson D, Taylor TKF. Aneurysmal bone cysts of the spine. J Bone Joint Surg [Br]. 1975;60:506. 411
15 DeKluver M, Van der Heul RO, Veraart BEEMJ. Aneurysmal bone cysts of the spine: 31 cases and the importance of the surgical approach. J Pediatr Orthop. 1998;7:286-292.
16 Cappana R, Albisinni U, Picci P, et al. Aneurysmal bone cysts of the spine. J Bone Joint Surg [Am]. 1985;67:527-531.
17 Hu SS, Healely JH, Huvos AG. Fibrous dysplasia of the second vertebra: a case report. J Bone Joint Surg [Am]. 1990;72:781-783.
18 Campanacci M. Tumors of bone and soft tissues. Bologna: Aulo Gaggi. Berlin: Springer Verlag, 1990.
19 Dahlin DC, Unni KK. Bone tumors: general aspects and data on 8,542 cases, 4th edn. Springfield IL: Charles C Thomas, 1986.
20 Parrish FF, Pevey JK. Surgical management of aneurysmal bone cyst of the vertebral column. J Bone Joint Surg [Am]. 1967;49:1587-1604.
21 Enneking WF, Spainer SS, Goodman MA. A system for the surgical staging of the musculoskeletal sarcomas. Clin Orthop. 1980;153:106-120.
22 Lackman RD, Khoury LD, Esmail A, et al. The treatment of sacral giant-cell tumors by serial arterial embolisation. J Bone Joint Surg [Br]. 2002;84:873-877.
23 Kollender Y, Meller I, Bickels J, et al. Role of adjuvant cryosurgery in intralesional treatment of sacral tumors: results of a 3–11 year follow-up. Cancer. 2003;97:2830-2838.
24 Fujimoto N, Nakagawa K, Seichi A, et al. A new bisphophonate treatment option for giant cell tumors. Oncol Reports. 2001;8:643-647.
25 Wilner D. Radiology of bone tumors and allied disorders. Philadelphia: WB Saunders, 1990.
26 Camins MB, Duncan AW, Smith J, et al. Chondrosarcoma of the spine. Spine. 1978;3:202-209.
27 Shallot RF, Taekman MS, Nagle RC. Unusual presentation of cervical chordoma with long-term survival. J Neurosurg. 1982;57:716-718.
28 Giunti A, Laus M. Radicolopatie spinali. In: Gaggi A, ed. Bologna: 1992:216–220.
29 Winants D, Bertal A, Hennequin L, et al. Imagerie des Chordomes cervicaux et thoraciques. J Radiol. 1992;73:169-174.
30 Barwick KW, Huvos AH, Smith J. Primary osteogenic sarcoma of the vertebral column: clinicopathologic correlation of ten patients. Cancer. 1980;46:595-604.
31 Vail TP, Urbaniak JR. Donor-site morbidity with the use of vascularized autogenous fibular grafts. J Bone Joint Surg [Am]. 1996;78:204-211.
32 Mnaymneh W, Brown M, Tejada F, et al. Primary osteogenic sarcoma of the second cervical vertebra: case report. J Bone Joint Surg [Am]. 1979;61:460. 442
33 Hadjipavlou A, Lander P. Paget disease of the spine. J Bone Joint Surg [Am]. 1991;73(9):1376-1381.
34 Altman RD, Brown M, Gargano F. Low back pain in Paget’s disease of bone. Clin Orthop. 1987;217:152-161.
35 Posen S. Paget’s disease: current concepts. Aust NZ J Surg. 1992;62(1):17-23.
36 Potter HG, Schneider R, Ghelman B. Multiple giant cell tumors and Paget disease of bone: radiographic and clinical correlations. Radiology. 1991;180(1):261-264.
37 Meunier PJ, Vignot E. Therapeutic strategy in Paget’s disease of bone. Bone. 1995;17(5 Suppl):489S-491S.
38 Sapico FL, Montgomerie JZ. Vertebral osteomyelitis. Infect Dis Clin N Am. 1990;4(3):539-550.
39 Currier BL, Kim CW, Heller JG, et al. Cervical spinal infections. In: Clark CR, editor. The cervical spine. 4th edn. New York: Lipincott, Williams and Wilkins; 2004:858-889.
40 Malawski SK, Lukawski S. Pyogenic infection of the spine. Clin Orthopaed Rel Res. 1991;272:58-66.
41 Sapico FL, Montgomerie JZ. Pyogenic vertebral osteomyelitis: report of nine cases and review of the literature. Rev Infect Dis. 1979;1(5):754-776.
42 Sapico FL, Montgomerie JZ. Vertebral osteomyelitis in intravenous drug abusers: report of three cases and review of the literature. Rev Infect Dis. 1980;2(2):196-206.
43 Stone JL, Cybulski GR, Rodriguez J, et al. Anterior cervical debridement and strut-grafting for osteomyelitis of the cervical spine. J Neurosurg. 1989;70(6):879-883.
44 Visudhiphan P, Chiemchanya S, Somburanasin R, et al. Torticollis as the presenting sign in cervical spine infection and tumor. Clin Pediatr. 1982;21(2):71-76.
45 Zigler JE, Bohlman HH, Robinson RA, et al. Pyogenic osteomyelitis of the occiput, the atlas, and the axis. A report of five cases. J Bone Joint Surg [A]. 1987;69(7):1069-1073.
46 Modic MT, Feiglin DH, Piraino DW, et al. Vertebral osteomyelitis: assessment using MR. Radiology. 1985;157(1):157-166.
47 Tali ET. Spinal infections. Eur J Radiol. 2004;50(2):120-133.
48 Longo M, Granata F, Ricciardi G, et al. Contrast-enhanced MR imaging with fat suppression in adult-onset septic spondylodiscitis. Eur J Radiol. 2003;13(3):626-637.
49 Kemp HB, Jackson JW, Jeremiah JD, et al. Pyogenic infections occurring primarily in intervertebral discs. J Bone Joint Surg [Br]. 1973;55(4):698-714.
50 Emery SE, Chan DP, Woodward HR. Treatment of hematogenous pyogenic vertebral osteomyelitis with anterior debridement and primary bone grafting. Spine. 1989;14(3):284-291.
51 Hlavin ML, Kaminski HJ, Ross JS, et al. Spinal epidural abscess: a ten-year perspective. Neurosurgery. 1990;27(2):177-184.
52 Baker AS, Ojemann RG, Swartz MN, et al. Spinal epidural abscess. N Engl J Med. 1975;293(10):463-468.
53 Hancock DO. A study of 49 patients with acute spinal extradural abscess. Paraplegia. 1973;10(4):285-288.
54 Redekop GJ, Del Maestro RF. Diagnosis and management of spinal epidural abscess. Can J Neurolog Sci. 1992;19(2):180-187.
55 Levy ML, Wieder BH, Schneider J, et al. Subdural empyema of the cervical spine: clinicopathological correlates and magnetic resonance imaging. Report of three cases. J Neurosurg. 1994;81(1):160.
56 Ungkanont K, Yellon RF, Weissman JL, et al. Head and neck space infections in infants and children. Otolaryngol Head Neck Surg. 1995;112(3):375-382.
57 Brook I. Microbiology and management of peritonsillar, retropharyngeal, and parapharyngeal abscesses. J Oral Maxillofac Surg. 2004;62(12):1545-1550.
58 Asmar BI. Bacteriology of retropharyngeal abscess in children. Pediatr Infect Dis J. 1990;9(8):595-597.
59 Silvio M, Andrew JH. Deep neck infections. Am J Otolaryngol. 1996;17(5):287.
60 Tannebaum RD. Adult retropharyngeal abscess: a case report and review of the literature. J Emerg Med. 1996;14(2):147-158.
61 Miller WD, Furst IM, Sandor GK, et al. A prospective, blinded comparison of clinical examination and computed tomography in deep neck infections. Laryngoscope. 1999;109(11):1873-1879.
62 Broughton RA. Nonsurgical management of deep neck infections in children. Pediatr Infect Dis J. 1992;11(1):14-18.
63 Peltola H. Worldwide Haemophilus influenzae type b disease at the beginning of the 21st century: global analysis of the disease burden 25 years after the use of the polysaccharide vaccine and a decade after the advent of conjugates. Clin Microbiol Rev. 2000;13(2):302-317.
64 Hussein AS, Shafran SD. Acute bacterial meningitis in adults. A 12-year review. Medicine. 2000;79(6):360-368.
65 El Bashir H, Laundy M, Booy R. Diagnosis and treatment of bacterial meningitis. Arch Dis Child. 2003;88(7):615-620.
66 Goossens H, Sprenger MJ. Community acquired infections and bacterial resistance. Br Med J. 1998;317(7159):654-657.
67 Durand ML, Calderwood SB, Weber DJ, et al. Acute bacterial meningitis in adults: a review of 493 episodes. N Engl J Med. 1993;328:21-28.
68 Quagliarello VJ, Scheld WM. Treatment of bacterial meningitis. N Engl J Med. 1997;336(10):708-716.
69 Lifeso RM, Weaver P, Harder EH. Tuberculous spondylitis in adults. J Bone Joint Surg [Am]. 1985;67(9):1405-1413.
70 Kim NH, Lee HM, Suh JS. Magnetic resonance imaging for the diagnosis of tuberculous spondylitis. Spine. 1994;19(21):2451-2455.
71 Working Party on Tuberculosis of the Spine. A 15-year assessment of controlled trials of the management of tuberculosis of the spine in Korea and Hong Kong. Thirteenth Report of the Medical Research Council Working Party on Tuberculosis of the Spine. J Bone Joint Surg [Br]. 1998;80(3):456-462.
72 Hsu LC, Leong JC. Tuberculosis of the lower cervical spine (C2 to C7). A report on 40 cases. J Bone Joint Surg [Br]. 1984;66(1):1-5.
