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Erik N. Kubiak, MD, Ronald Moskovich, MD, Thomas J. Errico, MD, and Paul E. Di Cesare, MD
Abstract
Ankylosing spondylitis is an inammatory disease of unknown etiology that affects
an estimated 350,000 persons in the United States and 600,000 in Europe, primarily Caucasian males in the second through fourth decades of life. Worldwide, the
prevalence is 0.9%. Genetic linkage to HLA-B27 has been established. Ankylosing
spondylitis primarily affects the axial skeleton and is characterized by inammation
and fusion of the sacroiliac joints, spine, and hips. The resultant deformity leads to
severe functional impairment in approximately 30% of patients. Orthopaedic management primarily involves correction of hip deformity through total hip arthroplasty and, less frequently, correction of spinal deformity with spine osteotomy.
Closing wedge osteotomies have the lowest incidence of complications. Whether patients with ankylosing spondylitis are at increased risk for heterotopic ossication
remains controversial, but comparison with age- and sex-matched counterparts
suggests no dramatically higher risk. Because of the high rate of missed fractures
and complications after minor trauma in patients with ankylosing spondylitis,
plain radiographs are usually not sufficient for evaluation. Thorough patient assessment should include a comprehensive history, physical examination, and laboratory studies.
J Am Acad Orthop Surg 2005;13:267-278
Epidemiology
AS typically affects young adults,
most commonly males (M:F = 3:1) in
their second through fourth decades. The incidence of AS in North
America is reported to be 1 to 2 per
1,000.2 Worldwide prevalence is up
to 0.9%.3 Fifteen percent to 20% of
patients with AS have a positive
family history of the disease. AS
is linked to HLA-B27; 80% to 95%
of patients with AS are HLA-
267
Etiology
Not everyone who is HLA-B27
positive develops AS; it is likely that
other genetic and/or environmental
factors play a role in the etiology.5
One recent theory is that AS is an
autoimmune disorder that occurs
subsequent to a Klebsiella pneumoniae
infection in HLA-B27positive individuals, with molecular mimicry between HLA-B27 and K pneumoniae
bacterial antigens.6 Elevated levels
of antibodies to K pneumoniae have
been noted in AS patients. Elevated
IgA levels in AS patients may reect
mucosal immunity to a persistent
bacterial infection and may be the
autoimmune trigger for AS, idiopathic bowel disease, and other reactive arthritides. No causal relationship has been established
between AS and other autoimmune
diseases, but the incidence of AS in
patients with idiopathic bowel disease is approximately 3.7%.7
Pathophysiology
The pathognomonic feature of AS is
a combination of inammation and
bony destruction at the site of tendon insertion (ie, enthesopathy).
Pannus or broblastoid tissue and
inammatory cell inltrates invade
the bone adjacent to entheseal attachments; new bone formation in
response to inammation leads to
ankylosis of affected joints. Preferential involvement of the zygapophyseal joints has been noted on
magnetic resonance imaging (MRI).
Ankylosis and subsequent loss of
motion of the zygapophyseal joints
leads to syndesmophyte formation,
resulting in the characteristic bamboo spine of individuals with AS
(Fig. 1). Enthesopathy also can occur
in peripheral joints, leading to synovitis-like symptoms and joint degeneration.8 Other manifestations of
AS include inammatory bowel disease, psoriasis, uveitis/iritis (25%),9
268
Clinical History
Patients usually present with symptoms and physical ndings consistent
with the nonmechanical and inammatory nature of AS. Onset is often
insidious; patients typically cannot
give the precise time of onset or even
pinpoint the initially affected site.
These individuals frequently present
in the second through fourth decades
of life with low back pain and stiffness, particularly in the morning, at
night, or after prolonged periods of
sitting and/or recumbency. Chest
wall pain is a frequent complaint, particularly with deep inhalation. Back
pain not relieved by recumbency and
the persistent discomfort may compel the individual to leave the bed at
night.12 Patients also may have buttock pain that radiates down the posterior thigh. Enthesopathy involving
the Achilles and plantar tendon insertions with associated symptoms also
is common. Some patients experience
no pain but present with increasing
stiffness in the hips and spine. As with
other inammatory diseases, symptoms often improve with exercise.
Physical Examination
A systematic examination of the entire patient is imperative. Ocular pain,
scleral redness, and photophobia are
often indicative of acute anterior uveitis and may be clinically apparent before other symptoms or signs of AS
appear. Chest expansion measured at
the fourth intercostal space typically
is limited to <2.5 cm after fusion of
the costovertebral joints; the patient
then becomes an obligate diaphragmatic breather. Total lung volume and
vital capacity usually are preserved;
the diaphragm excursion is affected
only in the patient with severe tho-
Assessment of Deformity
Correction of deformity most often
is performed to improve the patients visual eld, respiratory function,14 balance, sitting position,
swallowing function, or ambulation.
Determining the site of deformity is
critical before surgically treating the
deformity. Flexion contracture of the
hips, loss of lumbar lordosis, and
progressive thoracic and cervical kyphosis all may contribute to the
functionally disabling stooped posture of patients with AS. Deformity
must be assessed both clinically and
radiographically as part of preoperative planning. The level of deformity will aid in determining the
benet of total hip arthroplasty
(THA) and/or the level of corrective
spine osteotomy and degree of correction necessary.
The chin-brow angle is the angle
formed by the vertical line drawn
from the chin to the brow with the pa-
Laboratory Evaluation
Baseline laboratory investigation in patients suspected of having AS should
include a basic metabolic panel, complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and rheumatoid factor.
Although routine HLA-B27 status is
not required to make a diagnosis of
AS, it is helpful in the differential diagnosis. The clinician should be suspicious of a diagnosis of AS in patients
who are HLA-B27negative. Patients
in the active phase of the disease have
mildly elevated ESR, CRP level, and
white blood cell count. In most cases,
rheumatoid factor is negative and
HLA-B27 is positive. Patients with
chronic AS may have a normocytic,
normochromic anemia.
Imaging Studies
The sacroiliac joints usually are the
rst joints affected in patients with
AS. Sacroiliitis initially presents as a
widening of the sacroiliac joints
with progressive sclerosis of the
joint margins, leading to eventual
bony fusion across the joints with
subsequent loss of sclerosis. Care
must be taken to disregard congenital deformities and degenerative
changes as well as osteitis condensans ilii (symmetric sclerosis on the
iliac sides of both sacroiliac joints
without erosions, seen in women
who have borne children). Standard
anteroposterior radiographs of the
pelvis are inadequate to fully evaluate the sacroiliac joints. For accurate diagnosis, a Ferguson view of
the pelvis (anteroposterior with the
x-ray tube tilted 30 cephalad) and
oblique radiographs are required.
Hip involvement presents as ossication of the ligamentous origins
and insertions about the trochanters,
iliac crests, and ischial tuberosities.
Late ndings include loss of joint
269
Table 1
The Modied New York Criteria for Diagnosing Ankylosing Spondylitis19
Clinical Criteria
1. Low back pain of at least 3 months duration improved by exercise and
not relieved by rest
2. Limitation of lumbar spine motion in sagittal and frontal planes
3. Chest expansion decreased relative to normal values for age and sex
Radiographic Criteria
4. Bilateral sacroiliitis, grade 2-4
5. Unilateral sacroiliitis, grade 3-4
270
Diagnostic Criteria
The Rome criteria for AS, established
in 1961, were modied by the American Academy of Rheumatology in
1966 and renamed the New York classication. The New York criteria were
modied in 1984 by van der Linden
et al19 to improve sensitivity and retain specicity (Table 1). The modied New York criteria combine clinical and radiographic ndings for the
diagnosis of AS. Radiographic evaluation (eg, Fergusons view, oblique
views) of the sacroiliac joints is used
to determine the degree of the sacroiliitis, which is graded 0 (normal), 1
(suspicious), 2 (minimal sacroiliitis),
3 (moderate sacroiliitis), and 4 (ankylosis). Limitations of this system are
an inability to detect all sacroiliac
and/or spinal involvement on plain
radiographs, failure to include patients with other forms of spondyloarthropathy, and an assumption that the
duration of inammatory back pain
has exceeded 3 months. The presence
of HLA-B27 is not necessary to make
the diagnosis.
Nonsurgical Management
Nonsteroidal
anti-inammatory
drugs (NSAIDs)specically, indomethacinremain the mainstay of
medical therapy. Cyclooxygenase-2
inhibitors offer the potential benets
of decreased gastrointestinal morbidity, but their efficacy in the treatment
of AS remains to be proved. Sulfasalazine, methotrexate, thalidomide, and
antitumor necrosis factor (TNF)-
agents (iniximab, etanercept, adalimumab) are frequently used in longterm AS management.20 Indomethacin and radiation therapy have been
employed to manage ankylosis and
myositis ossicans. In 2003, the Assessment in Ankylosing Spondylitis
Working Group recommended that
antiTNF- be used to treat patients
with AS when any of the following
exists: (1) a denitive diagnosis of AS;
(2) presence of the disease for at least
4 weeks; (3) presence of refractory disease (dened by the failure of two
types of NSAIDs during a single
3-month period); (4) failure of local
corticosteroid injection into inamed
sacroiliac joints; (5) failure of sulfasalazine in patients with peripher-
Surgical Management
Indications for surgical treatment of
patients with AS are to lessen pain
and to improve function. Additionally, the psychosocial effects of severe
deformity are devastating, and correction may help the patient recover
vital social skills and possibly accept
having the disease. THA is the most
common surgical intervention in AS
patients, followed by spinal osteotomies for correction of cervical deformity and thoracolumbar sagittal balance. Few patients require surgical
intervention for correction of peripheral joint abnormalities.
Hip Deformity
Thirty percent to 50% of patients
with AS exhibit involvement of the
271
Table 2
Review of Total Hip Arthroplasty in Patients With Ankylosing Spondylitis
Study
Finsterbush et
al32
Shih
et al29
Brinker
et al25
Sochart
and
Porter30
Tang
and
Chiu27
Lehtimki
et al31
Joshi
et al23
Patients/
Hips (N)
Cemented/
Uncemented
Preoperative
Ankylosis
(N)
Mean
Mean
HO:
Age at Follow-up Brooker
Surgery
(yrs
Class
(yrs)
[range]) 1-2/3-4
Mean
Survival at
10/15/20
yr (%)
Patients
Without
ReviPain
sions
(%)
(N)
Ambulation
Status
23/35
13
0/4
46/74
25
36
10 (3-15)
48/6
78//
23
97
12/20
35
6 (2-10)
6/0
60
24/43
29
6/0
91//73
12
88
58/95
46 C;
49 U
39
23
(1 mo30 yr)
11.3
(2-28)
70/20
96.8/66.3/
19
79
54/76
40
80/66/62
83
103/181
42
47
10 (2-27)
40/0
87.3/81.4/
25
95
272
morbidity justied total knee arthroplasty as a reliable means of providing durable pain relief and modest
improvement in function, regardless
of the high incidence of postoperative
stiffness.
In their review of 158 patients with
AS, Emery et al44 identied 52 with
shoulder involvement, 6 of whom
had acute inammatory arthropathy
of the sternoclavicular or acromioclavicular joints. Most symptoms responded to NSAIDs. Forty-one of the
52 patients with shoulder involvement experienced restriction of
scapulothoracic motion, which was
always bilateral; of these, 34 patients
had involvement of the scapulothoracic joint with loss of ROM (forward
exion, abduction, and external rotation), and 7 patients had asymmetric
scapulothoracic and glenohumeral
involvement. Most shoulder involvement was minimally disabling and responded to nonsurgical management.
Destructive glenohumeral involvement, although rare, was the most
disabling. The presence of limited
scapulothoracic ROM may complicate treatment of AS patients, similar
to the difficulties encountered during
treatment of other patients with destructive glenohumeral arthropathy.
Spine
In AS, chronic inammation of the
zygapophyseal joints leads to progressive fusion of the spine, typically in a caudal to cranial fashion. As
spinal segments become progressively fused, pain often disappears. Osteopenia is common in patients with
AS. Rigid fused sections of the spine
concentrate and convert compressive
stresses to shear stresses at the
atlanto-occipital, cervicothoracic, and
thoracolumbar junctions. This can
lead to fracture, pseudarthrosis, and
spondylodiscitis. Additionally, progressive increases in cervical and thoracic kyphosis, combined with loss of
lumbar lordosis and of motion at the
hip joints, can result in loss of sagittal balance. The resultant stooped
posture can lead to signicant functional decits and a deformed appearance. Incapacitating symptoms
include the inability to lie in bed or
to look straight ahead. Patients with
cervical ankylosis may have difficulty with horizontal gaze even after
correction of lumbar alignment. Surgical intervention also may be indicated for management of deformity,
pseudarthrosis, fracture, and spondylodiscitis.
Cervical Deformity
Correction of deformity at the level of the cervical spine is indicated for
patients in whom sagittal balance has
been maintained or regained surgically, but whose kyphotic chin-brow angle has signicantly impaired the visual eld or interfered with either
hygiene or swallowing. These patients may exhibit chin-on-chest deformity. Patients with evidence of
fracture, hypermobility of the cervical spine, and subluxation of C1-C2
leading to kyphotic deformity should
be treated with gradual halo traction
to restore accurate alignment of the
spinal canal and chin-brow angle. Patients with C1-C2 instability should
be treated initially with cervical traction in a halo, followed by posterior
arthrodesis. Osteotomy of the cervical spine should be limited to patients
with rigid deformities. Evidence of recent fracture, characterized by an
overt fracture or by new onset of pain
in the cervical spine of a person previously without pain, is a contraindication to osteotomy.
Urist45 rst described extension osteotomy of the cervical spine for correction of cervical deformity. This
operation is performed under local
anesthesia with the patient awake to
facilitate neurologic monitoring during the reduction. The level of correction is centered at C7-T1 because the
canal is relatively wide at this level
and the cervical cord and nerve roots
are relatively mobile. In addition, the
vertebral vessels do not enter the lateral masses at this level. In 1998, Liew
Thoracolumbar Deformity
Corrective osteotomy of the lumbar spine is indicated to correct sagittal balance when correction of hip
exion contractures does not restore
sagittal balance and the patient is
markedly disabled. For patients with
pseudarthrosis, Simmons48 advocated correction of kyphotic deformity
by spine osteotomy to convert translational forces to compressive forces
at the site of pseudarthrosis.
Van Royen et al15 devised an equation correlating sagittal balance and
physiological end plate angle. Nomograms constructed from this information allow the surgeon to correlate the
correction angle, horizontal position
of the C7 plumb line, and the level
of the spinal osteotomy, predicting the
optimum level and degree of correction.
Most authors advocate general anesthesia and beroptic intubation for
thoracic and lumbar deformity correction. In a review of 66 spinal osteotomies, Bridwell et al49 stressed the
importance of the wake-up test for
intraoperative patient evaluation.
Five neurological sequelae were
missed by somatosensory evoked po-
273
Figure 3
Bone removed for opening wedge osteotomy (A), polysegmental closing wedge osteotomy (B), and closing wedge osteotomy (C).
274
can be performed even when the anterior longitudinal ligament is completely ossied. The anterior column
is not forcibly extended in this technique, thereby minimizing the risk of
abdominal aortic injury. In their series of 45 patients undergoing pedicle subtraction closing wedge osteotomy, Kim et al55 demonstrated an
ability to effect sagittal and coronal
balance correction with one- and twolevel closing wedge osteotomies with
no long-term neurologic sequelae.
Berven et al56 also reported correction
of sagittal balance with closing wedge
osteotomy. Pedicle subtraction osteotomy avoids intraoperative nerve root
compression resulting from closing
down the neural foramina as the
spine is extended.
Halm et al57 collected functional
outcome data from 148 of 175 patients
who had spinal deformity correction
surgery between 1979 and 1988. Improvement was noted in 47 of the 60
items of the modied Arthritis Impact
Measurement Scales at a mean
follow-up of 4.8 years. Eighty-eight
percent of patients were very satised
with the results of surgery, and 60.9%
were able to return to work. In a series of 83 patients (5 with AS) undergoing spinal osteotomy to correct
xed sagittal and/or coronal plane
deformity, Ahn et al58 demonstrated
a statistically signicant correlation
between patient functional outcome
and satisfaction with sagittal lordosis >25 (P = 0.034) and coronal plumb
alignment <2.5 cm (P = 0.041). Suk et
al59 prospectively evaluated 34 patients with AS after they underwent
pedicle subtraction extension osteotomy for correction of sagittal plane
deformity. Correction of chin-brow
angle from 10 to 10 resulted in signicantly (P = 0.000) better horizontal gaze. Additionally, the functional
scores of items related to the horizontal gaze were higher than the overall
mean score. Because of the neck stiffness of AS patients, correcting the
chin-brow angle is better for them
than absolute correction of coronal
and sagittal balance would be, because correcting the chin-brow angle
improves these patients visual eld.
The importance of planning deformity correction so as to correct visual
elds is essential in AS patients.
It is unclear which type of spinal
osteotomy is most appropriate for the
treatment of severe kyphotic deformity, although closing wedge osteotomies appear to have a lower incidence
of complications. Deformity correction surgery in AS patients is associated with dural tears secondary to dural ectasia, transient nerve root
compression, loss of correction, implant failure, postoperative spinal instability, and aortic injury. Fortunately, the number of patients with severe
kyphotic deformity is decreasing because of improved medical management and earlier detection.60
Fracture
Generalized spinal osteopenia is
common in patients with AS. Spinal
fracture often is caused by minor
trauma and can be missed on spine
radiographs because of distortion of
the normal spinal anatomy by ectopic bone formation, erosions, sclerosis,
disk ossication, vertebral wedging,
or difficulty with patient positioning.
Onset of pain after even minor trauma should suggest a fracture, which
may not be visible on plain radiographs.61 Fractures frequently occur
at the C7-T1 junction and are difficult
to visualize radiographically. Bone
scan, MRI, or ne-cut CT can be used
to evaluate AS patients who present
with cervical pain (Fig. 5).
The rigid, unsupported, osteoporotic cervical spine is most susceptible to hyperextension injuries, and
the cervical spine is the most common
site of fracture in patients with AS. The
drastic increase in stiffness at the cervicothoracic junction, combined with
the lever arm of the fused cervical
spine and weight of the head, makes
fractures at the C6-C7 and C7-T1 levels most common. The lumbar and
thoracic spines are more resistant to
fracture because the anterior and posterior longitudinal ligaments are more
thoroughly ossied than in the cervical spine. Spinal fractures typically occur through the ossied disk and
vertebral body; vertebral body fractures are more common and have a
higher risk of neural compromise,
partly as a consequence of a higher
incidence of epidural hematoma and
the possibility of the newly mobile
fracture severely translating, resulting in neural injury.61
AS patients who sustain spinal fractures typically have high rates of neurologic injury.62 Missed fractures are
frequent and often present as rapidly
progressing cervical kyphosis. Surgical treatment is more commonly used,
especially in patients with neurologic
compromise, obscured visual elds,
pseudarthrosis, or recurrent fracture.
When traction or internal xation are
275
Summary
Figure 6 Preoperative (A) and postoperative (B) lateral radiographs of a 31-year-old man
who sustained a C5-C6 fracture through the disk space. Posterolateral fusion was done with
lateral mass plates and posterior element wiring.
276
Spondylodiscitis
Spondylodiscitis has been reported to occur in 5% to 23% of patients
with AS; 50% of these patients are
asymptomatic.63,64 Spondylodiscitis,
which presents as an erosive sclerotic process, characteristically involves
the intervertebral disk and adjacent
bone. It can be confused radiographically with diskitis and pseudarthrosis. Unlike diskitis, biopsies of these
lesions demonstrate chronic inammatory changes with no infective organisms.63,64 Spondylodiscitis lesions
occur at the level of the intervertebral
disk rather than through the vertebral
body, as in pseudarthrosis. Pseudarthrosis often presents as new back
pain in a previously asymptomatic individual and often arises after minor
trauma. Most cases of spondylodiscitis and pseudarthrosis resolve with
nonsurgical management. Surgical
stabilization may be required in patients with intractable pain, associated posterior element disruption, and
fracture. It is important to assess ky-
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