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Orthopaedic Management of Ankylosing Spondylitis

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

Ankylosing spondylosis (AS) is a seronegative inammatory disease of


unknown etiology characterized by
inammation in the axial skeleton.
The sacroiliac joints are often involved initially, followed by enthesopathy in the paravertebral zygapophyseal joints and disk spaces.
Enthesopathy is inammation at the
site of tendon insertions that predisposes to the development of brosis
and calcication; left untreated, it
leads to fusion of the zygapophyseal
joints and intervertebral disk space.
This fusion in turn can lead to a
xed, hyperkyphotic posture and
compromised sagittal balance. Hip
and knee exion contractures occur
when patients compensate for loss
of sagittal balance by assuming a
crouched position. Degeneration
and ankylosis of peripheral joints,
such as the hip, knee, and shoulder,

Vol 13, No 4, July/August 2005

may ensue. Deformity resulting


from inammation and fusion of the
sacroiliac joints, spine, and hips
leads to severe functional impairment in approximately 30% of patients.1 Several diagnostic and treatment strategies are available for
early and late manifestations of AS.

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-

B27positive.2 Carriers of this gene


have a 16% to 50% increased risk of
developing AS, but other genetic
factors are likely involved as well.4
HLA-B27 is present among the inhabitants of Eurasia, North Africa,
and North America; it is virtually
absent among the aboriginal populations of Australia and South
America.2 Although both HLAB27negative and HLA-B27positive AS patients have similar articular manifestations, the former
usually develop the disease at an
older age and lack a positive family
history. AS tends to occur at a later
age in females than in males, and diagnosis is often delayed.2

Dr. Kubiak is Resident, Musculoskeletal Research


Center, NYUHospital for Joint Diseases Department of Orthopaedic Surgery, New York, NY. Dr.
Moskovich is Associate Chief, Spine Service, Musculoskeletal Research Center, NYUHospital for
Joint Diseases Department of Orthopaedic Surgery. Dr. Errico is Chief, Spine Service, Musculoskeletal Research Center, NYUHospital for
Joint Diseases Department of Orthopaedic Surgery. Dr. Di Cesare is Chief, Adult Reconstructive Service, Musculoskeletal Research Center,
NYUHospital for Joint Diseases Department of
Orthopaedic Surgery.
None of the following authors or the departments
with which they are affiliated has received anything
of value from or owns stock in a commercial company or institution related directly or indirectly
to the subject of this article: Dr. Kubiak, Dr. Moskovich, Dr. Errico, and Dr. Di Cesare.
Reprint requests: Dr. Di Cesare, Musculoskeletal Research Center, NYUHospital for Joint Diseases, 301 E 17th Street, New York, NY 10003.
Copyright 2005 by the American Academy of
Orthopaedic Surgeons.

267

Orthopaedic Management of Ankylosing Spondylitis

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

pulmonary brosis, aortitis, and


genitourinary problems, with prevalence up to 40%.10,11

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-

Figure 1 Lateral radiograph of the lumbar


spine in a 46-year-old man demonstrating the
owing syndesmophytes and posterior element fusion consistent with bamboo spine.
Disk spaces are often maintained, especially
in the lumbar spine. Anteriorly, osteophytes
are often oriented vertically rather than horizontally, which is common in spondylitis and
diffuse idiopathic skeletal hyperostosis.

racic kyphosis. Patients become


tachypneic when the degree of kyphosis compresses the abdominal
cavity, thereby limiting diaphragmatic excursion. Pulmonary function testing should be performed before surgery on all patients with AS. Aortic
and mitral regurgitation murmurs
can occur as a consequence of aortitis in patients with long-standing disease. Right bundle branch block and
varying degrees of atrioventricular
block occur in up to 30% of patients
as a result of brosis extending from
the aorta to the intraventricular septum.13
Early in the disease, examination
of the sacroiliac joints may reveal tenderness to palpation, which can be ex-

Journal of the American Academy of Orthopaedic Surgeons

Erik N. Kubiak, MD, et al

acerbated by hyperextension of the


hips. Patients with hip involvement
develop hip exion contractures.
Some patients exhibit loss of lordotic curvature of the lumbar spine and
loss of spine movement in all planes.
Patients with decreased mobility of
the thoracolumbar spine have an abnormal Schober signthat is, lack of
at least a 5-cm increase in distance
from a midline point 5 cm below and
10 cm above the posterior superior iliac spine, measured in both the erect
standing position and full forward
exion. Late in the disease, the cervical spine can become rigid, with
loss of exion and extension. Chinbrow angle, occiput-to-wall distance,
and gaze angle are used to evaluate
functional deformity involving the
cervical spine (Fig. 2). Most patients
are neurologically normal, with preservation of deep tendon reexes. A
slow, progressive cauda equina syndrome may appear late in the course
of the disease.

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-

Vol 13, No 4, July/August 2005

tients hips and knees fully extended


(Fig. 2). Occiput-to-wall distance is
measured to grossly determine the
patients sagittal balance. The horizontal distance from the occiput to the
wall is measured with the patients
buttocks and heels against the wall,
with hips and knees extended. Clinically, occiput-to-wall distance should
be 0 to 2 cm. The degree of hip exion contracture is determined by
placing the patient supine with the
lumbar spine pressed at to the examining table, then measuring the
angle that each femur makes with
the horizontal.
Normal sagittal balance places the
center of mass just in front of the S1
vertebral body. Radiographically, a
plumb line drawn from the center of
the C7 vertebral body should just
touch the anterior edge of the body
of S1 on the lateral radiograph. Normal individuals are able to compensate for small changes in sagittal balance through changes in sacral
inclination; hip, knee, and ankle exion; or extension. In patients with AS,
these normal compensatory changes
are ameliorated by loss of motion in
these areas.15

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.

Figure 2 Measurement of occiput-to-wall


distance, gaze angle, and chin-brow angle.

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

Orthopaedic Management of Ankylosing Spondylitis

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

space, sclerosis, and fusion with loss


of sclerosis at the hips.
The ascending osseous changes of
the cervical, thoracic, and lumbar
spine lead to the characteristic bamboo spine in advanced stages of the
disease. The erosive sclerotic processes that occur in the spine are different from those that occur in diffuse
idiopathic skeletal hyperostosis
(DISH, also known as Forestiers disease). AS can be erroneously diagnosed in patients who have ankylosis of the spine for other reasons.
Spine lesions in AS are the result of
wedging of vertebral bodies and ossication of disk spaces leading to a
rigid kyphotic spine. Enthesopathy of
the zygapophyseal joints leads to fusion of the posterior elements. The
bone loss and reabsorption that lead
to these deformities occur early in
young persons with active disease.16
Erosions at the discovertebral junctions are called Romanus lesions; sclerosis in this region gives rise to socalled shining corners. In AS, the
syndesmophytes are bilateral and
have their insertions at the upper and
lower margins of adjacent vertebrae;
in DISH, the syndesmophytes are
larger and asymmetric, with nonmarginal vertebral insertions consistent
with their status as ligamentous ossications. Additionally, DISH spares
the sacroiliac joints and is not associated with HLA-B27. In patients with
AS, the zygapophyseal joints are pre-

270

dominantly involved and frequently


obliterated by fusion. Four other
spondyloarthropathiespsoriatic arthritis, reactive arthritis, arthritis associated with inammatory bowel
disease, and undifferentiated spondyloarthropathycan have radiographic changes similar to those of AS.
Both computed tomography (CT)
and MRI are more sensitive than plain
radiographs for detecting early disease. In a review of imaging techniques for sacroiliitis, Braun et al17 advocated the use of CT for evaluation
of erosions and regional ankylosis.
MRI is the most sensitive instrument
for detecting early disease, with a sensitivity of 95% and a specicity of
100%.18 Early detection may be important in determining which patients would likely benet from
immune-modulating medication regimens when radiographic evidence is
lacking. Bone scans are unreliable, although a negative bone scan should
create suspicion of an AS diagnosis
in a patient with sacroiliitis or low
back pain.

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-

Journal of the American Academy of Orthopaedic Surgeons

Erik N. Kubiak, MD, et al

al disease; and (6) no medical contraindications to the initiation of


treatment.21 Braun et al22 summarized
the results of antiTNF- medications
in more than 300 patients enrolled in
clinical trials, which indicated significant (P < 0.0001) short-term improvement in disease activity, function, and
quality of life. Long-term efficacy of
these medications is being studied in
ongoing clinical trials. Early medical
intervention may improve patient
function, lessen pain, and delay disease progression.
The physician is responsible for
educating the patient about the disease and its natural history. An activity program of physical exercise, including stretching and postural
alignment, should be consistently followed. A medium-rm mattress is
recommended, and the patient may
benet from sleeping supine with one
pillow to maintain a more normal
sagittal spinal alignment and prevent
hip exion contractures. Ultimate
ankylosis of the spine in an anatomic
position, an achievable outcome for
patients who receive early diagnosis
and counseling, is far preferable to
progressive kyphosis.

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

Vol 13, No 4, July/August 2005

hips; of those, 90% present bilaterally.23 Hip involvement ranges from


exion contractures to complete
ankylosis, often in a disabling exed
position. THA consistently improves
both range of motion (ROM) and
function; it also decreases pain in patients with AS.
Patients with AS onset at an early
age are more likely to require THA.
Sweeney et al24 reported the mean age
of AS onset in patients undergoing
THA to be 19.5 years, an average of
3.5 years younger than matched controls with AS who had not undergone
THA. In addition, 16% of AS patients
with onset before age 16 years required THA within 20 years.
Indications for THA are correction
of hip exion contractures and reduction of pain caused by degeneration,
with the goal of improved function.
Patients with AS typically undergo
THA at a younger age than do patients having THA for osteoarthritis.
THA implant survival in AS patients
is similar to that in other young patients with THA. It was previously
thought that patients with AS were
at a signicantly greater risk of developing disabling heterotopic ossication (HO) than other patients undergoing THA; however, careful
review of the literature reveals that
there may not be dramatically higher rates of HO for age- and sexmatched counterparts.25,26
Correction of hip exion contracture with THAcan restore sagittal balance in patients with AS. Tang and
Chiu27 noted that, because of the presence of relative hyperextension of the
hips after THA, AS patients are more
prone to anterior dislocation when acetabular components are placed in
their normal position relative to the
pelvis. When positioning the acetabular component in a patient with AS,
one must account for the relationship
of the pelvis to the lumbar spine in
the sagittal plane in order to avoid an
excessively hyperextended hip once
the patient resumes an upright position. In patients with severe spinal de-

formity, corrective spinal osteotomies


should be performed before THA to
prevent future acetabular component
malposition. However, many surgeons still concur with Lee,28 who as
early as 1963 stated that THA should
be performed before considering spinal osteotomy because improvement
in hip ROM and pain relief may obviate the need for spinal osteotomy
in patients with severe hip exion deformity. Determining surgical sequence should be based on patient
circumstances.
Several studies indicate that THA
is benecial in AS patients (Table 2).
The studies listed in Table 2 evaluated a total of 524 THAs performed in
320 patients with AS. Ambulatory status improved in all patients. Sixty percent to 97% of patients reported no
pain after THA.23,25,27,29-31 Fifteen-year
survival (ie, time to THA failure)
ranged from 66% to 81.4%.23,27,31 Average age at time of surgery was 38
years.23,25,27,29-31 Thirty patients had
Brooker33 class 3 or 4 HO.27,29,32
Eighty-six revision THAs were
performed.23,27,29-32 Sweeney et al24 reviewed charts and questionnaires of
340 patients with AS who were treated with THA. Survival rates of primary THA were 90%, 78%, and 64%
at 10, 15, and 20 years, respectively.
Survival rates of revisions were 73%,
55%, and 55% at 10, 15, and 20 years,
respectively. These THA survival
rates and those listed in Table 2 are
not dissimilar to survival rates in
other young patients requiring
THA.34-40
The majority of revisions in the
studies listed in Table 2 were performed for aseptic loosening of both
the femoral and acetabular components.27,30 Tang and Chiu27 used both
uncemented and cemented implants
in their series of THA in patients with
AS; they reported loosening rates of
5% and 28% in uncemented and cemented implants, respectively. The
short duration of follow-up in patients receiving uncemented implants
prevented Tang and Chiu from fully

271

Orthopaedic Management of Ankylosing Spondylitis

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

= no data, C = cemented, HO = heterotopic ossication, I = improved, U = uncemented

advocating their use. However, more


recent preliminary clinical data39,40
support the use of uncemented acetabular and femoral components in
young, active patients, including
those with AS.
The level of risk for heterotopic
bone formation after THA in patients with AS remains controversial. Previous reports have resulted
in the impression that the rates of
postoperative HO are unacceptably
high after THA in AS patients. However, careful review of the previously reported high rates of HO after THA in patients with AS shows
rates not unlike those seen in other
young patients undergoing THA.
Additionally, patients with preoperative ankylosis often experience signicant improvement in function after THA.23,30,41 The proportion of AS
patients with functional ankylosis
secondary to HO following THA is
likely substantially lower than previously thought. Patients with class

272

3 HO, nevertheless, experienced signicant (P < 0.01) improvement in


ROM, pain relief, and independent
ambulation after THA.41 Higher
rates of HO after THA have been reported in AS patients who have undergone repeat operations, who
have experienced postoperative infection, who were treated with a
transtrochanteric approach, and/or
who had an active disease (reected
by elevated ESR or CRP level).
Brinker et al25 found similar rates of
HO in 12 patients with AS and in an
age-matched control group without
AS who underwent THA. High-risk
patients with preoperative ankylosis, with previous hip surgery, with
previous infection, and/or in whom
a transtrochanteric approach to the
hip was used should be considered
for HO prophylaxis with indomethacin or low-dose radiation. The
benets of HO prophylaxis in other
patients with AS has not been denitively determined.

Other Peripheral Joint


Involvement
The rate of shoulder and knee involvement in AS ranges from 25% to
70%.42 Although little has been reported on managing the shoulder and
knee in patients with AS, Finsterbush
et al32 noted marked improvement in
walking, function, and pain in 21 of
23 patients with severe hip, knee, and
ankle involvement who were treated
with surgery and rehabilitation. They
did not address survivorship or the
treatment of knee or ankle involvement. Parvizi et al43 recently reported on 30 total knee arthroplasties in
20 patients (mean age, 55 years) who
were followed for a mean of 11.2
years. Knee Society pain scores improved signicantly (P < 0.0001), from
a mean of 14 preoperatively to a mean
of 76.3 at nal follow-up. Knee Society function scores improved from
16.3 to 58.7. There was essentially no
improvement in ROM (84.8 to 86.7).
In both series,32,43 severe preoperative

Journal of the American Academy of Orthopaedic Surgeons

Erik N. Kubiak, MD, et al

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

Vol 13, No 4, July/August 2005

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

and Simmons46 recommended resection extension osteotomy followed by


posterior fusion and immobilization,
with either halo or collar postoperatively, depending on the xation
method. In 1997, McMaster47 reported the results of extension osteotomy
at the C7-T1 level in AS patients with
severe exion deformity of the cervical spine. All 15 patients (mean age,
48 years) had a mean correction of
54. One patient became quadriparetic, two had unilateral C8 palsies, and
four experienced subluxations. Neurologic problems were attributed to
dural traction (ectasia), posterior element impingement of C8 roots, and
subluxation. Rigid xation using
plates and rod-and-screw constructs
may decrease neurologic complications by maintaining stability during
and after osteotomy.

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

Orthopaedic Management of Ankylosing Spondylitis

Figure 3

Bone removed for opening wedge osteotomy (A), polysegmental closing wedge osteotomy (B), and closing wedge osteotomy (C).

tentials but were detected by the


wake-up test.
The osteotomies can be performed
in either the lateral decubitus or prone
position. Initially plaster thoracolumbosacral orthosis with one leg included was used for postoperative immobilization. Since the advent of
segmental pedicle screw xation,
many surgeons immobilize patients
in custom rigid thoracolumbosacral
orthoses with a hip extension or forego postoperative immobilization altogether. Recommended periods of
immobilization vary from 2 to 4
months for closing wedge osteotomies and 6 to 15 months for opening
wedge and polysegmental osteotomies.
The opening wedge, polysegmental wedge, and closing wedge osteotomies (Fig. 3) are the three surgical
techniques used for correcting lumbar kyphosis. In 1945, Smith-Petersen
et al50 rst described an opening
wedge osteotomy for correction of
lumbar kyphosis in six patients.
Hehne et al51 advocated polysegmental wedge osteotomy (Fig. 4), which
was rst described by Wilson and
Turkell52 in 1949. In 1985, Thomasen53 subsequently described a monosegmental closing wedge osteotomy
of the lumbar spine, in which the posterior elements, pedicles, and a portion of the vertebral body are resected to accomplish the correction (the
so-called pedicle subtraction osteotomy).

274

In their meta-analysis of articles


published between 1945 and 1998,
Van Royen and De Gast14 reported on
856 patients with AS who underwent
correction of xed kyphotic thoracolumbar deformity. There were 451
opening wedge osteotomies, 249
polysegmental wedge osteotomies,
and 156 closing wedge osteotomies.
Mean patient age was 41 years at the
time of operation; mean correction

obtained was 40.3 for opening


wedge osteotomies, 40.3 for polysegmental wedge osteotomies, and 36.5
for closing wedge osteotomies. Of the
856 patients who underwent surgery,
34 (4%) died from intraoperative or
postoperative complications. The authors noted a tendency toward less
severe complications with closing
wedge osteotomy. Jaffray et al54 reported that closing wedge osteotomy

Figure 4 Preoperative (A) and postoperative (B) weight-bearing lateral radiographs of a


41-year-old man who underwent polysegmental closing wedge osteotomy of the lumbar spine
for correction of severe kyphotic deformity and loss of sagittal balance. Lumbar lordosis was
corrected from 37 preoperatively to 60 postoperatively, which resulted in a 16-cm correction of the patients sagittal balance to neutral.

Journal of the American Academy of Orthopaedic Surgeons

Erik N. Kubiak, MD, et al

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

Vol 13, No 4, July/August 2005

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

Figure 5 T2-weighted sagittal reconstruction


magnetic resonance image of a 46-year-old
man with a C7-T1 disk space fracture with
subluxation and cord compression. Fast spinecho sequences may show edema, thereby allowing fracture localization, even without
fracture displacement.

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

Orthopaedic Management of Ankylosing Spondylitis

photic deformity before stabilization


because deformity can create shear
stresses at the site of spondylodiscitis or pseudarthrosis. These translational forces can be converted to compressive forces at the time of
stabilization, leading to higher rates
of fusion.

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.

used to manage these injuries, the neck


should be aligned to prefracture
position, not necessarily to a normal
position (Fig. 6). Minor ndings in patients with AS may be associated with
substantial instability in the cervical
spine, secondary to the altered biomechanics of the fused spine in addition to osteopenia and the concentration of forces at the cervico-occipital
and cervicothoracic junctions.
Preoperative evaluation of the
cervical spine is essential when manipulating the neck during intubation and patient positioning. Physicians also must be aware that,
because the atlanto-occipital joint is
last to fuse, atlantoaxial instability
may occur. Instability is usually
demonstrated on lateral exion-extension views of the neck, where the
atlantodens and posterior atlantodens intervals are measured. An
atlantodens interval >3.5 mm is indicative of instability. A difference of
7 mm indicates disruption of the
alar ligaments, and a difference >9 to
10 mm or a posterior atlantodens interval >14 mm is associated with an
increased risk of neurologic injury

276

and usually requires surgical intervention.

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-

AS, which affects approximately


350,000 people in North America
and 600,000 in Europe (worldwide
prevalence, 0.9%), belongs to a
larger group of spondyloarthropathies and is characterized predominantly by involvement of the sacroiliac joints in young men who
present with inammatory low back
pain in their third or fourth decade.
The disease primarily affects the axial skeleton and is characterized by
enthesopathy, which leads to fusion
of the sacroiliac joints, hips, and
spine. Young men presenting with
inammatory back pain and evidence of sacroiliitis should be
screened for AS; most patients are
HLA-B27positive.
Management of AS remains palliative, with NSAIDs continuing to
be the mainstay of treatment. However, new antiTNF- drugs offer
the promise of arresting the progression of AS. AS patients presenting
with back or neck pain after minor
trauma should be considered to
have spinal fractures until denitively proven otherwise with CT or
MRI. THA can signicantly improve
function, pain, and deformity in AS
patients with severe hip involvement. Results are comparable to
those in other young patients with
severe hip pathology. Because the
incidence of HO in AS patients
undergoing THA may not be signicantly more than that of similar
non-AS patients undergoing THA,
the routine use of HO prophylaxis in
AS patients should be reserved for

Journal of the American Academy of Orthopaedic Surgeons

Erik N. Kubiak, MD, et al

those at high risk of developing HO.


Correction of spinal deformity
should be reserved for patients with

signicant functional disability after


they have been evaluated for THA.
Among the alternative approaches

to deformity correction, closing


wedge osteotomies appear to have
the lowest rate of complications.

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Journal of the American Academy of Orthopaedic Surgeons

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