Viseceradiology Update Musculoskeletal Findings

C1 C2 - Rheumatoid Spondylitis Example of DC to MD Communication

Michael J Vives, MD, Associate Professor, Department of Orthopedics, Division of Spine Surgery, New Jersey Medical School, University of Medicine and Dentistry of
New Jersey
Steven R Garfin, MD, Professor, hair, Department of Orthopedics, University of alifornia at San Diego Medical enter
Updated! Aug "#, "$$%
Introduction
History of the Procedure
&he most common sites of rheumatoid arthritis '(A) are the metatarsophalangeal *oints, followed +y the metacarpophalangeal *oints and the
cervical spine 'an,ylosing spondylitis, rheumatoid spondylitis)- Much of the understanding of spinal afflictions in (A was advanced +y studies
pu+lished in the ./0$s and ./#$s-
1. 2
3n ./0., Davis and Mar,ley detailed medullary compression as a cause of death in patients with (A-
1" 2
3n ./#/,
Mathews reported that "045$6 of patients with (A who were admitted to the hospital had radiographic evidence of cervical spine involvement-
15 2
Problem
(heumatoid spondylitis 'an,ylosing spondylitis) primarily affects the cervical spine- Affliction of the thoracic or lum+ar spine is rare- &he anatomic
a+normalities occur as a conse7uence of the destruction of synovial *oints, ligaments, and +one- A+normalities of the rheumatoid cervical spine
generally can +e grouped into 5 categories- Atlantoa8ial insta+ility 'AA3) or atlantoa8ial su+lu8ation 'AAS) is the most common- AAS can +e a fi8ed
deformity or can +e partially or fully reduci+le- Superior migration of the odontoid 'SMO) is the ne8t most common a+normality and has alternately
+een referred to as cranial settling, pseudo+asilar invagination, or vertical9upward translocation of the odontoid-
1: 2
&he third and least commonly
seen deformity is su+a8ial su+lu8ation- &his may +e seen at multiple levels, producing a stepladder deformity- &he 5 deformities may +e seen in
isolation, or com+ined involvement may occur-
Frequency
(heumatoid arthritis '(A) affects $-%6 of the white population in the United States and ;urope- Nec, pain is reported in :$4%%6 of patients with
(A- &he prevalence of cervical spine involvement 'rheumatoid spondylitis, an,ylosing spondylitis) in (A ranges from "04%$6, depending on the
diagnostic criteria applied-
10 2
<owever, only =45:6 of patients with (A have a neurologic deficit- A su+stantial num+er of patients with radiographic
insta+ility or nec, pain do not develop neurologic deficits-
1#,=,% 2
3nvolvement of the cervical spine typically +egins early in the disease process and often parallels the e8tent of peripheral disease- Of the 5 types of
involvement, AA3 is the most common, occurring in up to :/6 of patients-
1/ 2
>hile most of these su+lu8ations are anterior, appro8imately "$6 are
1
lateral and appro8imately =6 are posterior- SMO is seen in up to 5%6 of patients with (A- Su+a8ial su+lu8ation is seen as a discrete pathologic
entity in .$4"$6 of patients-
Su+a8ial su+lu8ation also develops after previous upper cervical fusions-
1.$,.. 2
3n one series of =/ patients, 5#6 developed su+a8ial su+lu8ation an
average of "-# years following occipitocervical fusion, and 0-06 e8perienced su+a8ial su+lu8ation an average of / years following atlantoa8ial
fusion-
1.$ 2
Pathophysiology
(ecent theories on the pathogenesis of rheumatoid arthritis '(A) suggest that the synovial cells of these patients chronically e8press an antigen
that triggers the production of rheumatoid factor, an immunoglo+ulin molecule directed against other autologous immunoglo+ulins- An
inflammatory response is initiated, involving immune comple8 formation, activation of the complement cascade, and infiltration of
polymorphonuclear leu,ocytes- &he proliferating fi+ro+lasts and inflammatory cells produce granulation tissue, ,nown as rheumatoid pannus,
within the synovium- &he pannus produces proteolytic en?ymes capa+le of destroying ad*acent cartilage, ligaments, tendons, and +one- &he
destructive synovitis results in ligamentous la8ity and +ony erosion with resultant cervical insta+ility and su+lu8ation-
1.",.5 2
Atlantoa8ial su+lu8ation results from erosive synovitis in the atlantoa8ial, atlanto4odontoid, and atlanto4occipital *oints and the +ursa +etween the
odontoid and the transverse ligament 'see image +elow)-
2
3
Rheumatoid spondylitis. Depiction of anterior subluxation of C1 on C2, retrodental pannus, and osseous erosions; the spinal
cord is compressed between the pannus anteriorly and the posterior arch of the atlas.
&he superior migration of the odontoid is attri+uted to erosion and +one loss in the occipitoatlantal and atlantoa8ial *oints 'see image +elow)-
4
Rheumatoid spondylitis. Depiction of superior migration of the odontoid into the foramen magnum with compression of the
spinal cord.
Su+a8ial su+lu8ation results from destruction of the facets, interverte+ral discs, and interspinous ligaments- Unli,e degenerative disease,
involvement of "45 and 54: is common, and osteophytes seldom are seen-
Presentation
(heumatoid involvement of the cervical spine 'rheumatoid spondylitis, an,ylosing spondylitis) is *ust one element in a systemic disease process-
ervical involvement often correlates with the degree of hand and wrist erosion- ervical involvement also has +een associated with the presence
of rheumatoid nodules and the use of corticosteroids- lassically, craniocervical nec, pain often is associated with occipital headaches-
ompression of the " sensory fi+ers supplying the nucleus of the spinal trigeminal tract can cause facial pain- ompression of the " sensory
fi+ers supplying the greater auricular nerve may result in ear pain- Occipital neuralgia results from compression of the " sensory fi+ers supplying
the greater occipital nerve- A history of myelopathic symptoms should +e sought carefully- Patients may e8perience wea,ness, decreased
endurance, gait difficulty, paresthesias of the hands, and loss of fine de8terity- Patients with involvement may e8perience urinary retention and,
eventually, incontinence-
@erte+ro+asilar insufficiency may +e found, particularly in patients with atlantoa8ial insta+ility 'AA3)- omplaints may include vertigo, loss of
e7uili+rium, visual distur+ances, tinnitus, and dysphagia- Similar symptomatology can also +e caused +y mechanical compression of the
+rainstem- 3n some patients, nec, motion can elicit shoc,li,e sensations through the torso or into the e8tremities 'ie, Ahermitte sign)-
&he physical inventory of these patients fre7uently is confounded +y the severity of their peripheral rheumatoid involvement- >ea,ness in these
patients can also +e due to tenosynovitis, tendon rupture, muscular atrophy, peripheral nerve entrapment, or articular involvement, ma,ing
neurologic impairment less o+vious- Signs of myelopathy should raise suspicion of cervical involvement- (arely, cranial nerve dysfunction can
occur secondary to compression of the medullary nuclei +y the odontoid- Other rare findings in patients with advanced +rainstem compression
include vertical nystagmus and heyne4Sto,es respirations-
&he (anawat classification can +e used to categori?e patients with rheumatoid myelopathy +ased on their clinical history and physical findings
'see +elow)-
1.: 2
&his classification has some utility in determining potential for neurologic recovery following surgery-
&he (anawat classification of neurologic deficit is as follows!
lass 3 4 No neural deficit
lass 33 4 Su+*ective wea,ness, dysesthesias, and hyperrefle8ia
5
lass 333A 4 O+*ective wea,ness and long4tract signsB patient remains am+ulatory
lass 333C 4 O+*ective wea,ness and long4tract signsB patient no longer am+ulatory
Indications
Numerous investigators have attempted to elucidate the natural history of rheumatoid arthritis '(A) as it affects the cervical spine 'rheumatoid
spondylitis, an,ylosing spondylitis), with wide variation in their findings-
1.0,.#,.=,.%,./ 2
Depending on the diagnostic criteria applied, the prevalence of
cervical involvement in (A ranges from "04%$6- &he li,elihood of cervical involvement appears to increase with the duration of rheumatic disease-
Cecause neurologic deficit is seen only in =45:6 of cases, many patients with pain and radiographic criteria for insta+ility do not develop
neurologic se7uelae- <owever, .$6 of patients with (A may die from +rainstem compression that is unrecogni?ed +efore their sudden death-
1"$ 2
&he identification of a su+set of patients with impending neurologic deficit has +een elusive due to the poor correlation of neurologic symptoms
with radiographic indicators of insta+ility- &herefore, universally accepted surgical indications have +een slow to develop-
Contraindications
ontraindications to surgery for rheumatoid spondylitis 'an,ylosing spondylitis) include medical conditions that suggest the patient would not
tolerate the stress of surgery, such as unsta+le angina or a recent myocardial infarction or stro,e- Active infection with li,ely +acteremia would also
+e a relative contraindication to surgery, especially in the setting of planned instrumentation- &he patientDs medical condition should +e optimi?ed
prior to proceeding with any planned surgical intervention-
Workup
Laboratory Studies
(heumatoid factor seropositivity has +een correlated with more e8tensive cervical involvement 'rheumatoid spondylitis, an,ylosing spondylitis)-
&he use of the rheumatoid factor as a predictor of neurologic involvement has not +een esta+lishedB therefore, it does not have a role in the
surveillance of patients with rheumatoid arthritis '(A) with cervical involvement-
Imaging Studies
All patients with rheumatoid arthritis '(A) should have radiographic e8amination of the cervical spine +ecause cervical involvement 'rheumatoid
spondylitis, an,ylosing spondylitis) can remain asymptomatic- >hile prediction of the onset of myelopathy in any particular patient is difficult,
studies of large populations of patients have sought to esta+lish parameters for predicting neurologic involvement-
1#,".,"" 2
6
Plain radiography
&he initial imaging assessment should consist of plain radiographs of the cervical spine, including lateral fle8ion and e8tension views-
Several measurements that can help direct management can +e made on plain radiographs-
&raditionally, the anterior atlantodental interval 'AAD3) has +een used to monitor patients with (A over time- &his measures the interval
from the posterior margin of the anterior ring of . to the anterior surface of the odontoid- An interval of more than 5 mm in an adult or :
mm in a child is considered a+normal- @arious authors have recommended surgery for values of more than % mm, / mm, or .$ mm-
Anterior atlantoa8ial su+lu8ation may also +e assessed +y measurement of the posterior atlantodental interval 'PAD3), as measured from
the posterior aspect of the odontoid to the anterior margin of the lamina of .- Cecause the synovial pannus may occupy .45 mm of the
retro4odontoid space, this interval does not represent the true space availa+le for the cord-
>hile the AAD3 was used commonly to monitor patients with cervical involvement, a num+er of investigations have shown that the AAD3
does not relia+ly discriminate patients who are neurologically intact from those with neural deficit-
1"5 2
&his is due in part to the 54dimensional
changes that ta,e place with progressive su+lu8ation- As the deformity progresses, the anterior arch of the atlas displaces in an
anteroinferior direction as superior migration of the odontoid 'SMO) com+ines with atlantoa8ial insta+ility 'AA3)- >ith continued SMO, the
AAD3 decreases, although this vertical translocation is associated with a more unfavora+le prognosis-
Coden and associates demonstrated this pitfall +y e8amining the sensitivity, specificity, accuracy, and positive and negative predictive
values of the AAD3 in predicting paralysis +ased on varying critical intervals 'see &a+le at the end of the imaging section)-
1": 2
Eor e8ample,
raising the cut4off value for the AAD3 from % mm to .$ mm increases the specificity from 0%6 to /$6- <owever, the sensitivity with such a
change in threshold value decreases from 0/6 to 506- More recently, the PAD3 has +een recommended as a more relia+le predictor of
whether neurologic compromise will develop-
&he PAD3 was compared with the traditional anterior interval in a long4term series involving =5 patients-
1": 2
Using a critical PAD3 of less than
or e7ual to .: mm resulted in a sensitivity 'a+ility to detect those with paralysis) of /=6, a level superior to that using the AAD3- More
important, the negative predictive value, using a critical PAD3 of .: mm, rises to /:6- &herefore, if the PAD3 is more than .: mm, there is
a /:6 chance that the patient will not have paralysis- Such a high negative predictive value ma,es the PAD3 e8tremely relia+le as a
screening test-
A variety of measurements have +een used to assess SMO- All of these measurements attempt to identify and grade the degree of
odontoid encroachment on space normally occupied +y the spinal cord and +rainstem- Unfortunately, many of these are difficult to
reproduce- &he image +elow depicts pertinent measurements-

7
8
Rheumatoid spondylitis. Pertinent measurements of superior migration of the odontoid; cranial migration distance
C!D".
&he (anawat inde8 targets disease in the .4" segment +y utili?ing a lateral radiograph- A line is drawn from the pedicles of "
superiorly along the vertical a8is of the odontoid until it intersects a line connecting the anterior and posterior arches of .- A value less
than .5 mm is diagnostic of vertical settling-
&he Mc(ae line connects the front of the foramen magnum to the +ac,- &he upper tip of the odontoid process should not pro*ect a+ove
this line and should normally +e . cm +elow the anterior margin of the foramen magnum-
&he ham+erlain line is drawn from the posterior margin of the hard palate to the posterior margin of the foramen magnum- Pro*ection of
the tip of the odontoid # mm a+ove this line is considered pathologic- <owever, the margins of the foramen magnum fre7uently are difficult
to delineate without a tomogram-
&he McFregor line has +ecome the most consistent reference +ecause it connects the posterior margin of the hard palate to the most
caudal point of the occiput- @ertical settling is defined here +y migration of the odontoid more than :-0 mm a+ove this line-
&he (edlund4Johnell value assesses the occiput4to4" comple8- &he value measures the distance +etween the midpoint of the inferior
margin of the +ody of the a8is to the McFregor line- @alues less than 5: mm in males and "/ mm in females are considered a+normal and
correlate with increased ris, of neurologic in*ury-
1"0 2
Plain radiographs also are useful in detecting su+a8ial verte+ral su+lu8ations- &hese su+lu8ations may +e 7uantitated on lateral cervical
radiographs as translation forward in millimeters or as a percentage slip of the total anteroposterior diameter of the inferior verte+ral +ody-
<istorically, focus was directed on the num+er of millimeters of listhesis or the percentage of verte+ral slip- <owever, recent
demonstrations show that the sagittal diameter of the su+a8ial canal correlates with the presence and degree of paralysis more often than
does the percentage of verte+ral slip- Patients with canal diameters of .5 mm or less are at higher ris, for neurologic involvement-
Magnetic resonance imaging
Magnetic resonance imaging 'M(3) has provided an increased a+ility to visuali?e the e8tent of spinal cord compression, particularly when
due to pannus-
1"# 2
Dvora, and colleagues showed that two thirds of patients with AAS have a pannus of more than 5 mm in diameter-
1"= 2
&herefore, the +ony
canal diameter measured on plain radiographs may not represent the true space availa+le for the cord-
9
Gawaida et al demonstrated spinal cord compression in all patients with (A when the space availa+le for the cord 'as measured on M(3)
was less than or e7ual to .5 mm-
1"% 2
Using M(3, the cervicomedullary angle is an effective indicator of cord distortion from SMO- &his angle incorporates lines drawn along the
anterior aspects of the cervical cord and along the medulla- &he normal range is .504.=0H- Angles less than .50H indicate +asilar
invagination and have +een associated with myelopathy- See image +elow for a patient e8ample-

10
Rheumatoid spondylitis. !R# of a patient with superior migration of the odontoid and subaxial subluxation. Courtesy of
$te%en R. &arfin.
11
Dynamic cord compression can +e detected with functional M(3 scans o+tained in fle8ion and e8tension-
1"/ 2
Polytomography and computed tomography
<istorically, tomograms were useful for 7uantitating the degree of +asilar invagination and to measure AAD3 and PAD3 more accurately in
patients with a+normal radiographs-
omputed tomography '&) scan with sagittal and coronal reformatting largely has supplanted +iplanar tomography- A & scan com+ined
with intrathecal contrast provides e8cellent +ony detail and the a+ility to detect spinal cord compression from synovial pannus-
>hile the noninvasive nature of M(3 has made it the preferred modality for this type of evaluation, & myelography is useful for patients
with contraindications to M(3-
(elia+ility of the Anterior 'AAD3) and Posterior 'PAD3) Atlantodental 3ntervals in Predicting Paralysis in Patients with (heumatoid Arthritis
Test SES! SPE"# $""% PPV& PV''
AAD3 % 0/ 0% 0% #. 0#
AAD3 / :. == 0% #= 00
AAD3 .$ 50 /$ #" %$ 0#
AAD3 .. .% /= 00 %# 0"
PAD3 ." =# /$ %5 /$ =%
PAD3 .5 /. =. %" =% %%
PAD3 .: /= 0" =0 #/ /:
12
Note that all values are e8pressed as percents- &hese num+ers were calculated from a series of =5 patients-
IS;NS J Sensitivity
KSP; J Specificity
L A J Accuracy
MPP@ J Positive predictive value
NNNP@ J Negative predictive value
(eprinted with permission from Coden SD, Dodge AD, Cohlman <<, and (echtine F(-
1": 2
Treatment
edical Therapy
An approach to surgical and nonsurgical management for rheumatoid arthritis '(A) and for rheumatoid spondylitis 'an,ylosing spondylitis) can +e
developed +ased on the natural history of rheumatoid involvement of the cervical spine and radiographic predictors of paralysis- As suggested +y
Coden, 5 goals should +e ,ept in mind-
15$ 2
&he first goal is to avoid the development of an irreversi+le neurologic deficit, as patients with more
severe deficits have less recovery and higher mor+idity- A second goal is to prevent sudden death from unrecogni?ed neural compression, as has
+een reported in up to .$6 of deaths in (A- Einally, as half of patients with (A with radiographic evidence of insta+ility remain asymptomatic, it is
+est to avoid surgery if the patient can +e identified as +eing one who will most li,ely not develop neurologic pro+lems-
onsurgical treatment
Nonoperative treatment of rheumatoid involvement of the cervical spine 'rheumatoid spondylitis, an,ylosing spondylitis) is supportive- ;arly
aggressive medical management is important in the glo+al sense, as cervical involvement has +een correlated with disease activity-
15. 2
ollars can
+e used for comfort purposes- (igid cervical collars most li,ely do not prevent su+lu8ationB however, they may prevent reduction of a deformity +y
limiting e8tension-
15",55 2
S,in sensitivity in this population also causes pro+lems with rigid orthoses- Patients +eing monitored need careful
surveillance for long4tract signs or for radiographic findings suggesting impending neurologic compromise-
Surgical Therapy
Patients with rheumatoid arthritis '(A) or, particularly, rheumatoid spondylitis 'an,ylosing spondylitis) who have refractory pain, clearly evident
neurologic compromise, or intrinsic spinal cord signal changes on M(3 generally are candidates for surgical intervention- ontroversy surrounds
treatment for patients with little or no pain, no neural deficit, and radiographs suggestive of insta+ility- &o facilitate understanding of the operative
indications and perioperative details, categori?ation of these patients +y their pathologic lesion is helpful-
15:,50,5#,5= 2
13
$tlantoa(ial su)lu(ation * Preoperative details
Patients with AAS and no symptoms or signs of myelopathy can +e o+served when the posterior atlantodental interval 'PAD3) on the lateral
cervical radiograph is more than .: mm- &hose who have a PAD3 that measures less than .: mm should have an M(3 scan to determine the true
space availa+le for the cord- M(3 findings of less than .5 mm of space availa+le for the cord and a cervicomedullary angle of less than .50H
generally are indications for surgical sta+ili?ation-
$tlantoa(ial su)lu(ation * +ntraoperative details
&he type of procedure performed is determined +y whether or not the su+lu8ation is reduci+le, the individual surgeonDs preference and e8perience,
and the patientDs condition-
3f the deformity is reduci+le, posterior atlantoa8ial fusion can +e accomplished +y a variety of techni7ues-
15% 2
Fallie reported a techni7ue for posterior
atlantoa8ial arthrodesis in ./5/, a techni7ue that has +een used with different modifications since that time-
15/ 2
3n essence, the procedure consists
of a +loc, of autologous +one graft fi8ed +y wire loop to the posterior arch of the atlas and the spinous process of the a8is- >hile technically
straightforward to perform, rotational sta+ility and translational sta+ility are inferior to other techni7ues- &he image +elow depicts modified Fallie
fusion-
14
Rheumatoid spondylitis. !odified &allie fusion. 'ote the ()shaped bone bloc* wired o%er the spinous process of C2.
&he Croo,s fusion uses " posterior paramedian autologous structural grafts, usually attached with su+laminar wires- &he +ilateral fi8ation improves
rotational sta+ility- Multistrand titanium ca+les are increasingly favored over monofilament stainless steel wires +ecause of improved strength and
15
ease of contouring, as well as postoperative M(3 and & scan imaging 7ualities- All techni7ues using su+laminar wire or ca+le fi8ation have the
potential ris, of spinal cord in*ury during passage or from late failure of the implants- Additionally, the posterior arch of the atlas may +e
osteoporotic or partially deficient, there+y limiting its use- &he image +elow depicts Croo,s4type fusion-
16
Rheumatoid spondylitis. +roo*s)type fusion. Rectangular structural grafts are be%eled to fit between the arches of C1 and C2;
then they are secured by bilateral doubled)twisted wires.
3mmediate multidirectional sta+ility can +e achieved +y .4" transarticular screw fi8ation 'see image +elow)-
1:$ 2
17
Rheumatoid spondylitis. C1)C2 transarticular screw fixation. Courtesy of $te%en R. &arfin.
&he screws are inserted posteriorly +y entering the inferior aspect of the facet of ", crossing the .4" facet *oint, and then entering into the
lateral mass of .- Safe insertion re7uires thorough understanding of the upper cervical anatomy and e8posure of the medial +order of the "
pedicle-
1:. 2
A preoperative & scan should +e reviewed carefully +ecause some " pedicles may have a small diameter, the lateral mass may +e
partially resor+ed, or the verte+ral artery may course superomedially- &hese conditions preclude safe transarticular screw placement- 3f there is
good +one in the lateral masses, the patient may re7uire only a cervical collar- <owever, patients with poor fi8ation may re7uire a halo device
postoperatively-
Patients with irreduci+le deformity and posterior compression can +e treated with a . laminectomy and transarticular sta+ili?ation- Patients who
have irreduci+le deformity and +ony anterior compression may +e decompressed +y an anterior transoral approach, particularly in end4stage
conditions-
1:",:5 2
&his route has several difficulties, such as limited opening of the mouth in patients with concomitant temporomandi+ular disease,
postoperative infection, and pharyngeal mucosal edema- <owever, several authors have reported good results using this procedure- &his
techni7ue generally is followed +y posterior sta+ili?ation as a "4stage same4day procedure-
Patients who are healthier '(anawat class 3 and class 33) may +e treated ade7uately with atlantoa8ial sta+ili?ation and fusion alone, even in the
presence of irreduci+le deformity- Some authors recommend that patients with anterior cord compression from proliferative retrodental pannus +e
treated with ventral transoral decompression- <owever, recent data have documented resorption of retrodental pannus if a sta+le posterior fusion
is achieved-
1:: 2
Of these options, initial treatment with a posterior fusion followed +y M(3 and clinical follow4up is the most common- 3f the pannus
does not resor+ and neurologic deficit persists, late transoral decompression can +e performed-
Superior migration of the odontoid * Preoperative details
;ven in the a+sence of neurologic deficit, patients with any degree of +asilar invagination should have an M(3 in fle8ion to evaluate spinal cord
compression- Surgical treatment should +e considered in any patient with cord compression or neurologic deficit- Preoperatively, cervical traction
can +e used to attempt a gradual reduction-
Superior migration of the odontoid * +ntraoperative details
Occipitocervical fusion is the procedure of choice in patients with SMO- Several devices have +een descri+ed, ranging from wire loops securing
tricortical +one graft supplemented with cement or metal mesh, contoured rods, and more recently, plates and screws 'see image +elow)-
1:0,:# 2
18
Rheumatoid spondylitis. ,ccipitocer%ical fusion combined with lateral mass plating for a patient with combined superior
migration of the odontoid and subaxial subluxation. Courtesy of $te%en R. &arfin.
&he more rigid fi8ation afforded +y plating has +een associated with a lower pseudarthrosis rate when compared with wiring techni7ues-
1:= 2
Occipitocervical fusion with plating generally involves screw placement into the " pedicles under fluoroscopic guidance through a precontoured
plate- &his allows easier su+se7uent placement of su+a8ial screws in the lateral masses and in the occiput- Screws usually are not placed a+ove
the inion to avoid the intracranial venous sinuses-
19
>hile the inner ta+le can +e thin in places, holes drilled "45 cm from the midline, halfway +etween the foramen magnum and the transverse sinus,
generally are safe- No matter which implant techni7ue is used, autologous +one grafting should always +e performed- 3f the deformity is irreduci+le
in traction, decompression either posteriorly or anteriorly '+ased on the location of the compression) should +e considered as an ad*unct to the
fusion, as discussed a+ove- Anterior compression in these patients is predominantly osseous, not from synovial pannus- &herefore, a ventral route
provides more relia+le decompression-
Su)a(ial su)lu(ation * Preoperative details
Patients with su+a8ial su+lu8ation and no evidence of neurologic deficit can +e o+served- Plain radiographs are sufficient for surveillance- Patients
with a +ony canal diameter less than .: mm should have an M(3 to evaluate the true space availa+le for the cord- 3f the space availa+le for the
cord is less than .5 mm or if significant segmental hypermo+ility is present, surgical consideration is warranted-
Su)a(ial su)lu(ation * +ntraoperative details
Most patients with su+a8ial su+lu8ation can +e treated with posterior cervical fusion using autograft +one- 3nternal fi8ation with wires, plate4screw,
or rod4screw constructs allows for earlier mo+ili?ation and increased rates of fusion- Patients with irreduci+le su+lu8ations or significant neurologic
deficit may +est +e treated with anterior decompression and fusion alone or in concert with posterior fusion-
1:% 2
"om)ined su)lu(ations * Preoperative details
Some patients may have upper cervical involvement as well as early su+a8ial su+lu8ation- 3n these instances, the fusion should +e e8tended to
include the involved su+a8ial segments to avoid early deterioration of +orderline su+lu8ations +elow a rigid upper cervical fusion 'see image
+elow)-
20
Rheumatoid spondylitis. ,ccipitocer%ical fusion combined with lateral mass plating for a patient with combined superior
migration of the odontoid and subaxial subluxation. Courtesy of $te%en R. &arfin.
"om)ined su)lu(ations * Postoperative details
21
&he type of orthosis used postoperatively often is +est determined on an individuali?ed +asis- Many patients have porotic +one of +oth the
posterior elements and of the structural graft harvested from the iliac crest- Upper cervical fusions in such patients may +est +e managed
postoperatively in a halo- >hen rigid collars are used, careful surveillance of the s,in is mandatory to avoid decu+iti- Also, if temporomandi+ular
*oint '&MJ) involvement is present, eating 'chewing) may +e a pro+lem in a cervical4thoracic orthosis '&O)-
Follo!"up
;ven after achieving a successful fusion, patients with rheumatoid spondylitis 'an,ylosing spondylitis) must continue to have long4term follow4up-
Su+a8ial insta+ility can develop +elow upper cervical fusions, so radiographic follow4up should +e o+tained periodically, even in patients who are
asymptomatic-
Eor e8cellent patient education resources, visit eMedicineDs Arthritis enter- Also, see eMedicineDs patient education articles (heumatoid Arthritis
and Understanding (heumatoid Arthritis Medications-
Complications
(heumatoid arthritis '(A) is a systemic disorder, and patients may have varying degrees of generali?ed de+ilitation-
1:/ 2
&he postoperative course of
such patients can +e complicated +y fragile s,in and poor wound healing- Poor preoperative nutritional status and corticosteroid dependence may
potentiate wound4healing pro+lems and predispose toward infection- Some airways are difficult to intu+ate- ;8cessive trauma during intu+ation
may +e responsi+le for postoperative +reathing pro+lems- >attenma,er et al reported a .:6 incidence of upper airway o+struction after
e8tu+ation in patients intu+ated without fi+eroptic assistance, compared with a .6 incidence in patients intu+ated fi+eroptically-
10$ 2
&he perioperative
mortality rate has +een reported to +e as high as 04.$6-
#utcome and Prognosis
ervical fusion in patients with rheumatoid spondylitis 'an,ylosing spondylitis) has a clinical success rate of #$4/$6-
10.,0",05,0:,00,0#,0= 2
&his wide range is
partly due to the definition of clinical success and +y variation in disease severity at the time of surgery- (ates of neurologic improvement also vary
widely, ranging from "=4.$$6-
10.,0",05,0:,00,0%,0/,#$ 2
Peppelman et al reviewed the neurologic recovery in /$ patients with neurologic deficits who underwent
surgery for rheumatoid deformity of the cervical spine-
1#. 2
&he investigators reported that /06 of patients treated for atlantoa8ial su+lu8ation 'AAS)
improved at least one (anawat grade, =#6 of patients with com+ined AAS and superior migration of the odontoid 'SMO) improved, and /:6 of
those treated for isolated su+a8ial su+lu8ation improved-
Age, gender, duration of paralysis, preoperative atlantodental interval 'AD3), and percentage of slippage in su+a8ial su+lu8ations all have +een
found to have no correlation with neurologic recovery- &he degree of preoperative neurologic deficit has +een shown to correlate with neurologic
recovery- &he results appear to +e less favora+le in patients with more advanced preoperative neurologic deficits- asey and colleagues reported
22
their results after surgery in patients classified as (anawat lass 333-
1#" 2
Eifty4eight percent of am+ulatory patients 'grade 333A) attained a grade 3 or
grade 33 after surgery- onversely, only "$6 of nonam+ulatory patients 'grade 333C) improved to grade 3 or grade 33 postoperatively-
(adiographic parameters also have +een shown to predict postoperative neurologic recovery- Coden et al reported that patients with AAS whose
posterior AD3 'PAD3)was less than .$ mm +efore surgery had poor return of motor function- >ith superimposed SMO, clinically significant
neurologic recovery was seen only when the PAD3 was at least .5 mm prior to surgery- Eor patients with su+a8ial su+lu8ations, less recovery was
seen in those with a residual postoperative su+a8ial canal diameter of less than .: mm-
1": 2
Nonunion rates in this population have +een estimated at 04"$6- Many of these nonunions may +e asymptomatic, so management should +e
individuali?ed-
ultimedia
23
!edia file 1- Rheumatoid spondylitis. Depiction of anterior subluxation of C1 on C2, retrodental pannus, and osseous erosions;
the spinal cord is compressed between the pannus anteriorly and the posterior arch of the atlas.
24
!edia file 2- Rheumatoid spondylitis. Depiction of superior migration of the odontoid into the foramen magnum with
25
compression of the spinal cord.
26
27
!edia file .- Rheumatoid spondylitis. Pertinent measurements of superior migration of the odontoid; cranial migration distance
C!D".
28
!edia file /- Rheumatoid spondylitis. !R# of a patient with superior migration of the odontoid and subaxial subluxation.
Courtesy of $te%en R. &arfin.
29
!edia file 0- Rheumatoid sponylitis. !odified &allie fusion. 'ote the ()shaped bone bloc* wired o%er the spinous process of C2.
30
!edia file 1- Rheumatoid spondylitis. +roo*s)type fusion. Rectangular structural grafts are be%eled to fit between the arches of
C1 and C2; then they are secured by bilateral doubled)twisted wires.
31
!edia file 2- Rheumatoid spondylitis. C1)C2 transarticular screw fixation. Courtesy of $te%en R. &arfin.
!edia file 3- Rheumatoid spondylitis. ,ccipitocer%ical fusion combined with lateral mass plating for a patient with combined
superior migration of the odontoid and subaxial subluxation. Courtesy of $te%en R. &arfin.
$eferences
32
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patients- Radiology- Sep ./%"B.::':)!=:040.- 1Medline2-
38
%ey!ords
rheumatoid spondylitis, an,ylosing spondylitis, Ce,hterev arthritis, Marie4Strumpell spondylitis, rhi?omelic spondylitis, rheumatoid arthritis, (A,
atlantoa8ial su+lu8ation, AAS, su+a8ial su+lu8ation, atlantoa8ial insta+ility, AA3, superior migration of the odontoid, SMO
Contributor Information and &isclosures
4uthor
Michael J Vives, MD, Associate Professor, Department of Orthopedics, Division of Spine Surgery, New Jersey Medical School, University of
Medicine and Dentistry of New Jersey
Michael J @ives, MD is a mem+er of the following medical societies! American Academy of Orthopaedic Surgeons and North American Spine
Society
Disclosure! Nothing to disclose-
Coauthors"
Steven R Garfin, MD, Professor, hair, Department of Orthopedics, University of alifornia at San Diego Medical enter
Steven ( Farfin, MD is a mem+er of the following medical societies! American Academy of Orthopaedic Surgeons, American Orthopaedic
Association, North American Spine Society, Orthopaedic (esearch Society, and >estern Orthopaedic Association
Disclosure! Nothing to disclose-
!edical 5ditor
,ee - Riley +++, MD, hief, Division of Orthopedic Spine Surgery, Assistant Professor, Departments of Orthopedic Surgery and Neurosurgery,
Johns <op,ins University
Disclosure! Nothing to disclose-
Pharmacy 5ditor
.rancisco Talavera, PharmD, PhD, Senior Pharmacy ;ditor, eMedicine
Disclosure! eMedicine Salary ;mployment
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/illiam 0 Shaffer, MD, Professor, @ice4hairman and (esidency Program Director, Department of Orthopedic Surgery, University of Gentuc,y at
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>illiam O Shaffer, MD is a mem+er of the following medical societies! American Academy of Orthopaedic Surgeons, American Orthopaedic
39
Association, 3nternational Society for the Study of the Aum+ar Spine, Gentuc,y Medical Association, Gentuc,y Orthopaedic Society, North
American Spine Society, Southern Medical Association, and Southern Orthopaedic Association
Disclosure! DePuySpine .//=4"$$= 'not presently) (oyalty onsultingB DePuySpine "$$"4"$$= 'closed) Frant9research funds SacroPelvic
3nstrumentation Ciomechanical StudyB DePuyCiologics "$$04"$$% 'closed) Frant9research funds <ealos study *ust closedB DePuySpine
"$$/ onsulting fee Design of Offset Modification of ;8pedium
C!5 5ditor
Dinesh Patel, MD, .$"S, Associate linical Professor of Orthopedic Surgery, <arvard Medical SchoolB hief of Arthroscopic Surgery,
Department of Orthopedic Surgery, Massachusetts Feneral <ospital
Dinesh Patel, MD, EAS is a mem+er of the following medical societies! American Academy of Orthopaedic Surgeons
Disclosure! Nothing to disclose-
Chief 5ditor
Mary $nn E 1eenan, MD, Professor, @ice hair for Fraduate Medical ;ducation, Department of Orthopedic Surgery, University of Pennsylvania
School of MedicineB hief of Neuro4Orthopedics Program, Department of Orthopedic Surgery, <ospital of the University of Pennsylvania
Mary Ann ; Geenan, MD is a mem+er of the following medical societies! Alpha Omega Alpha, American Academy of Orthopaedic Surgeons,
American Orthopaedic Association, American Orthopaedic Eoot and An,le Society, American Society for Surgery of the <and, and Orthopaedic
(eha+ilitation Association
Disclosure! Nothing to disclose-
.urther Reading
(itu8ima+ for the treatment of rheumatoid arthritis-
National 3nstitute for <ealth and linical ;8cellence 'N3;) 4 National Fovernment Agency 1Non4U-S-2- "$$= Aug- "# pages- NF!$$0/$"
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Related eMedicine topics2
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An,ylosing Spondylitis and Undifferentiated Spondyloarthropathy 1(heumatology2
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