Eric Harris
CHAPTER
119
Cervical Instability and Kyphosis
(Rheumatoid, Dwarfism,
Degenerative, Others)
INTRODUCTION
The function of the cervical spine depends greatly upon proper
alignment and stability. An unstable, unbalanced cervical spine
will not facilitate the range of motion (ROM) or alignment
needed to correctly position the head to allow for even the
most basic activities of daily living. Minor abnormalities of sagit-
tal balance are amplified when repetitive movements or pro-
longed forward gazes are required. This chapter will provide a
brief overview of the anatomy and pathophysiology surround-
ing both instability and kyphotic deformity in the cervical spine.
We will then discuss some of the more common etiologies and
the accepted treatments of these processes. Finally, we will
review several cases that serve to illustrate the complexities of
diagnosis and treatment of cervical instability and kyphosis.
The osseous cervical spine consists of seven vertebrae. These
can be subdivided into the atlantoaxial complex, consisting of
the C1 and C2 vertebrae, and the subaxial spine, made up of
vertebrae C3 through C7. The atlantoaxial complex forms a base
upon which the skull rests. The unique anatomy of C1, the atlas,
and C2, the axis, allows for approximately 50% of the total rota-
tional and sagittal plane motion of the cervical spine. The ring-
like atlas is distinguished from the other cervical vertebrae by the
absence of a true vertebral body and spinous process and the
presence of bilateral posterior and superior grooves within which
the paired vertebral arteries run prior to ascending through the
foramen magnum. In addition to the articulations with the skull
base, a true synovial joint lies on the posterior aspect of the ante-
rior portion of the C1 ring where it articulates with the peg-like
portion of the C2 vertebrae known as the odontoid process, or
dens. This articulation allows the majority of rotation of the head
while preventing excessive anteriorposterior translation of the
skull. A ligamentous complex consisting of oblique, transverse,
and alar ligaments maintains the stability at this articulation,
which is essential to ensure adequate space for the spinal cord.
The vertebrae of the subaxial cervical spine are similar in
morphology to the thoracic and lumbar vertebrae. A generally
cylindrical anterior vertebral body with upsweeping lateral
uncovertebral joints is attached by bilateral pedicles to the pos-
terior elements. The anterior joint complex, consisting of two
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James Lawrence
Jeffrey Rihn
Joseph Gondusky
Alan Hilibrand
bodies and the intervening intervertebral disc, allows for con-
trolled motion at each motion segment while providing resis-
tance to compressive and shear forces. The facet joints, lateral
masses, and spinous processes and their respective intercon-
necting ligamentous structures act as a posterior tension band
that resists hyperflexion while accommodating motion. The lat-
eral masses at C3 through C6 contain the foramina transver-
saria through which the vertebral arteries course en route to
their junction to form the basilar artery intradurally.
All vertebrae in the cervical spine enclose the spinal cord.
Proper alignment and kinematics are paramount in protecting
this vital structure. Instability or sagittal malalignment can ulti-
mately lead to spinal cord deficits ranging from subtle fine
motor skill deficits to gait abnormalities to death.
OVERVIEW OF CERVICAL SPINE
BIOMECHANICS
Cervical instability is classically defined as the inability of the
cervical spine under physiological loads to maintain its normal
pattern of displacement so that there is no neurological dam-
age or irritation, no development of deformity, and no inca-
pacitating pain.13 By necessity, this region of the spine must
accommodate a high degree of mobility while maintaining
appropriate stability. The ROM of the cervical spine can be
divided into a neutral zone, characterized by minimal passive
resistance or restraint, and an elastic zone, within which
increased motion in a given plane requires increased passive
resistance to maintain spinal integrity.
Panjabi et al described three subsystems within the cervical
spine necessary for stability.12 The passive subsystem is composed
of the bony and ligamentous structures responsible for physically
stabilizing the spine and limiting the size of the neutral zone.
The active subsystem, composed of the cervical musculature,
controls the motion of the spine within the neutral zone and
maintains the size of the neutral zone. Both the active and pas-
sive subsystems also provide feedback to the third subsystem. The
third subsystem, the neural control subsystem, composed of
peripheral sensory and motor neurons and integrating centers
1281
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 1282 Section XI Kyphosis and Postlaminectomy Deformities
in the central nervous system (CNS), receives information from
the other two subsystems and then directs the actions of the
active subsystem to maintain appropriate position and stability.
PATHOPHYSIOLOGY OF CERVICAL
INSTABILITY
Cervical instability typically occurs secondary to traumatic or
degenerative processes. Traumatic causes are beyond the scope
of this discussion, which will focus on degenerative and iatrogenic
etiologies including poor posture, degenerative disc disease, facet
arthropathy, spondylosis, muscular insufficiency, and postsurgical
instability. These processes lead to a relative increase in the size
of the neutral zone relative to the total ROM of the cervical spine.
Once this occurs, the stabilizing systems are overwhelmed and
unable to compensate for this increased motion. Motion then
becomes uncontrolled and patients become symptomatic.
Patients with cervical instability present with a variety of com-
plaints including axial pain, headaches, and muscle spasm. In
addition, patients may present with neurological complaints such
as radicular pain, loss of fine motor skills, or gait abnormalities
that may be secondary to myelopathy caused by the instability
itself. Pain is the most common symptom of instability and, espe-
cially in the setting of neurological signs or symptoms, patients
with neck pain should be appropriately evaluated for instability.
DIAGNOSIS
Of paramount importance in evaluating these patients is a com-
plete and careful neurological examination. This portion of
the physical examination should include a motor examination
of all muscle groups in both the upper and lower extremities; a
complete sensory evaluation, including light touch, pinprick,
and joint position sense; and evaluation of reflexes, including
the presence of any upper motor neuron signs such as
Babinskis or Hoffmanns sign, hyperreflexia, or clonus. In addi-
tion, a gait assessment and the results of a Romberg test should
be documented. ROM should be assessed but it is important to
remember that ROM measurements are only end-range values
and provide no real evaluation of neutral zone motion.
Plain radiographs are the foundation of a comprehensive
radiographic evaluation of patients with cervical instability with
computed tomography (CT) reserved as a secondary modality.6
Several specific measurements, which may suggest instability may
be made on plain radiographs. On a lateral radiograph, measure-
ment of sagittal plane translation is performed from the poste-
rior superior aspect of the inferior vertebral body to the posterior
inferior aspect of the superior vertebral body. The sagittal dis-
placement should be less than 3.5 mm.14 Measurement of sagittal
plane rotation is made through a comparison of the angle of the
interspace in question (inferior end plates of bodies above and
below) to the angle of adjacent interspaces. A difference of
greater than 11 suggests instability.14 Finally, the distance between
the basion and the tip of the odontoid process should be between
4 and 5 mm. An increase of more than 1 mm in this measure-
ment on flexion/extension radiographs suggests instability.
Numerous lines may be applied to a lateral plain radiograph
(Fig. 119.1) including McRaes, Chamberlains, or McGregors
from which a measurement is then made to the tip of the odon-
toid to evaluate the relationship of the skull base to the dens.4,10,11
The tip of the dens should not project more than 0, 3, or 4.5 mm,
LWBK836_Ch119_p1281-1294.indd 1282
McRaes Line
Clivus
Inion
Hard Palate
Chamberlains Line
Ranawats
Line
Figure 119.1. Some of the numerous lines used to evaluate the
occipitocervical junction for basilar invagination.
respectively, above these lines. The Ranawat method for assess-
ing this abnormality involves measuring from the center of the
C2 pedicle to a line bisecting the ring of C1 with a value less
than 13 mm considered abnormal.15 Redlund-Johnell and
Pettersson described measuring the distance from the inferior
end plate of C2 to McGregors palato-occipital line with values
greater than 34 mm in men or 29 mm in women being abnor-
mal.16 Finally, Clark described three levels of settling based on
the relationship of the C1 ring to C2. Level I is present when the
ring is at the top of the odontoid and is referred to as the nor-
mal station. In level II disease, the ring would be lower than
normal station but above the lower third of the axis. Level III is
present when the ring is seen at the lower third of the axis and
indicates severe migration.5 In 2001, Riew et al evaluated all of
the aforementioned radiographic criteria for reliability at detect-
ing instability in a rheumatoid arthritis (RA) population. Their
results showed that a combination of the Clark station, the
Redlund-Johnell criterion, and the Ranawat criterion, scored as
positive for basilar invagination if any of the three were positive,
proved to be better than any single criterion with a sensitivity
and specificity of 94% and 91%, respectively.18 Riew further con-
cluded that none of the previously widely used criteria were
adequate when used alone and that even when the combined
criteria are negative, further imaging studies such as CT or mag-
netic resonance imaging (MRI) should be utilized to further
evaluate potential instability in the patient with RA.
MRI should be used to further evaluate patients who dem-
onstrate signs and symptoms of instability, especially when plain
radiographs indicate the presence of instability. MRI provides
the most detailed information about the soft tissues of the neck,
including the presence of RA pannus, the space available for
the spinal cord (SAC), and the presence or absence of spinal
cord compression or myelomalacia. For further information
using this imaging modality, especially in RA patients, flexion/
extension MRI protocols have been described. Possible indica-
tions for performing dynamic MRI include radiographic insta-
bility of the upper cervical spine, myelopathy, superior migration
of the odontoid process, and obliteration of bony landmarks
on plain radiographs. In patients with RA, it may also aid in
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 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others) 1283

Figure 119.2. (A) Lateral flexion

and (B) extension radiographs of a
patient with Downs syndrome. Note A B
the significant atlantoaxial transl ation.

determination of the contribution of RA pannus to any cord
compression. Sagittal T1 MRIs in the flexed, neutral, and
extended positions can be evaluated for dynamic changes lead-
ing to cord compression and help guide the surgeon toward
appropriate operative or nonoperative treatment.2
CAUSES OF CERVICAL INSTABILITY
The most common etiologies of cervical instability include rheu-
matologic disorders, neoplasms, postsurgical instability, and
traumatic instability. Other, more rare causes include
warfarin-related chondrodysplasia and congenital abnormalities
including Downs syndrome, Morquios syndrome, and Golden-
hars syndrome, which may lead to instability secondary to associ-
ated odontoid hypoplasia. Figures 119.2 to 119.4 are images of a
patient with Downs syndrome, which illustrate the radiographic
findings associated with the etiology of cervical instability.
One of the most common causes of cervical instability is RA.
Patients with RA may develop three specific types of instability
depending upon the nature of their disease. Basilar invagination
is caused when the inflammatory processes associated with the
disease destroy the occipitocervical and atlantoaxial joints.
Instability of these joints leads to the skull gradually sinking rela-
tive to the odontoid process with subsequent compression of neu-
ral elements. Sometimes referred to as cranial settling, this pro-
cess leads to a predictably higher rate of neurological compromise
than the other types of instability typically associated with RA.
Atlantoaxial subluxation is the most common type of cervi-
cal instability seen in RA. This type of instability results from

A B

Figure 119.3. (A and B) Computed tomography images show a significant increase in the atlantodental
interval and corresponding decrease in the atlantodens interval of the same Downs patient.

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 1284 Section XI Kyphosis and Postlaminectomy Deformities

A B

Figure 119.4. (A and B) T2-weighted magnetic resonance images performed with the neck flexed and
extended. The degree of cord compression varies greatly with position change as the C1 ring shifts relative to
the hypoplastic odontoid.

inflammation and pannus formation occurring around the
transverse atlantal ligament. The atlantodental interval (ADI),
defined as the distance between the posterior aspect of the
anterior C1 ring and the anterior aspect of the odontoid pro-
cess, is assessed on the lateral radiograph. An ADI of greater
than 3 mm is diagnostic for a subluxation, and an interval
greater than 7 mm suggests complete disruption of the trans-
verse ligament. Although measurement of the ADI correlates
with injury to certain ligaments in the atlantoaxial complex, it
does not correlate with SAC, which is defined as the cross-
sectional area, at any given level in the spine, free of bone, soft
tissue, or other material and, therefore, available for the spinal
cord. Only decreased posterior atlantodens interval (PADI),
which is measured from the craniocaudal midpoint of the pos-
terior ring of C1 to the posterior aspect of the odontoid pro-
cess, correlates with decreased SAC, which may be decreased
even more by pannus formation, which is not seen on conven-
tional radiography. Figure 119.5 demonstrates RA pannus
surrounding the dens and effectively further decreasing the
SAC. A PADI of less than 14 mm is highly predictive of the
development of neurological compromise secondary to insta-
bility in patients with RA.3

Figure 119.5. (A and B)

T2-weighted magnetic resonance imag-
ing (MRI) images of a patient with
rheumatoid arthritis. The rheumatoid
pannus significantly reduces the space
available for the cord (SAC). SAC may
be overestimated in these patients, if
MRI is not performed as the pannus is
A B
not visible on plain radiographs.

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 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others)
Subaxial subluxation is the third type of instability seen in
the RA cervical spine. This process develops secondary to
inflammatory destruction of the apophyseal and neurocentral
joints of the spine. The incidence of preoperative neurological
deficits in patients with subaxial subluxation is higher when it is
seen in combination with atlantoaxial instability. A lateral flex-
ion/extension radiograph demonstrating greater than 3.5 mm
sagittal displacement provides a radiographic diagnosis of this
variety of instability but only measurement of the SAC provides
correlation with potential neurological deficits.3
Another of the inflammatory arthritides that can lead to
development of cervical instability is ankylosing spondylitis
(AS). The long lever created by an ankylosed section of the
spine can lead to excessive stresses at the atlanto-occipital or
the atlantoaxial joints. Instability from this etiology may be sig-
nificant and painful secondary to the extreme stresses involved.
Care must be taken when evaluating patients with AS to rule
out an occult fracture, especially in the setting of any minor
trauma or an acute increase in neck pain.
Other rheumatologic diseases that may lead to cervical insta-
bility include Reiters syndrome, psoriatic arthritis, and juvenile
rheumatoid arthritis (JRA). As many as 75% of patients with
psoriatic arthritis may have cervical spine involvement. In addi-
tion, 15% to 20% of patients with JRA have been found to have
low-grade atlantoaxial instability without neurological symp-
toms. The high incidence of cervical involvement in these con-
ditions necessitates a thorough cervical spine evaluation when
assessing this population. This workup should include careful
evaluation of PADI and SAC on flexion and extension lateral
radiograph and MRI as these measurements are associated with
signs and symptoms of neurological damage.
TREATMENT
Treatment of cervical instability is primarily operative. Patients
who have no neurological involvement or a minimal neurolog-
ical deficit and who are deemed not to be at increased risk of
catastrophic neurological injury may be suitable for a trial of
nonoperative treatment. In this population, the aim of treat-
CASE 119.1 Degenerative Cervical Instability
A 55-year-old woman presented with a complaint of 2-year his-
tory of increasing neck and bilateral shoulder and arm pain.
The pain radiated down the lateral aspect of the arms and was
accompanied by numbness, tingling, and a subjective weakness
and heaviness. Over the past year, she noted increasing diffi-
culty with upper extremity dexterity and coordination but
denied gait disturbance, loss of balance, or bowel and bladder
dysfunction. Nonoperative measures including chiropractic
manipulation, physical therapy, activity modifications, and
nonsteroidal anti-inflammatory drugs (NSAIDs) had failed to
alleviate her symptoms. Her past medical history was signifi-
cant only for mitral valve prolapse. A review of systems was neg-
ative, and the patient had no history of previous spine surgery.
Her physical examination was significant for a positive
Spurlings sign on the left, bilateral arm tingling with Lher-
mittes maneuver, positive Hoffmanns sign bilaterally, and
hyperreflexia in bilateral upper and lower extremities. Her
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Considerations in Treating
TABLE 119.1
Cervical Instability
Important Considerations in Treating Cervical Instability
Pain is the most common presentation in cervical instability.
Lateral flexionextension radiographs should be obtained to
fully evaluate potential instability.
Greater than 3.5 mm of translation indicates instability.
Patients with instability should be evaluated for rheumatologic
disease as well as myelopathy.
In treating atlantoaxial instability, careful evaluation of the
occipitocervical junction is necessary to avoid missing
concomitant instability at this location.
Anterior or posterior stabilization may be used but care must be
taken to adequately decompress the spinal cord in patients with
myelopathy.
ment is the enhancement of the function of the stabilizing sub-
systems to decrease stresses on individual spinal segments.
Specific treatments include postural education, with a goal of
reducing segmental loads at end ranges of motion through
maintenance of optimal biomechanical position, and strength-
ening exercises for targeted improvement of function of the
active subsystem. These combined modalities can improve the
quality and quantity of movement in the neutral zone in a select
group of patients.
Patients who have failed, or are not candidates for, nonopera-
tive treatment can be treated surgically with excellent results. A
variety of instrumentation options are available and are chosen
depending upon the location of the instability. Occipitocervical
instability is treated with gentle traction for reduction of the
occipitocervical joint if necessary and occiput to C2 instrumen-
tation and fusion. Isolated atlantoaxial instability may be treated
with a variety of instrumentation options but care must be taken
to rule out concomitant occipitocervical instability in which case
the construct and arthrodesis must extend from the occiput to
C2. Subaxial instability may be treated with anterior, posterior,
or combined procedures depending upon the location and
extent of involvement. Table 119.1 provides an overview of
important considerations in treating cervical instability.
straight line gait was slightly unsteady, and her sensation
was decreased to pinprick over the radial aspect of bilateral
forearms and thumbs as well as along the left lateral bicep.
Plain radiographs (Fig. 119.6) revealed severe degenera-
tive changes with 2 to 3 mm of spondylolisthesis at C3-C4,
C4-C5, and C5-C6. C6-C7 and C7-T1 were noted to have
decreased disc height but no significant deformity. MRI
(Fig. 119.7) demonstrated spinal cord compression, great-
est from C3-C6 accompanied by mild stenosis without cord
compression in the remainder of the cervical spine.
The patient desired operative intervention for her condi-
tion and the decision was made to perform anterior cervical
discectomy and fusion from C-3 to C-6 combined with pos-
terior laminectomy and instrumented fusion at the same
levels. Postoperative radiographs are seen in Figure 119.8.
Six months postoperatively, the patient is doing well with
near complete resolution of her symptoms.
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 1286 Section XI Kyphosis and Postlaminectomy Deformities

A B

Figure 119.6. (A to C) (Case 119.1). Radiographs

demonstrating severe degenerative changes with 2 to
C
3 mm of spondylolisthesis at C3-C4, C4-C5, and C5-C6.

CERVICAL KYPHOSIS
In utero, the human spine is entirely kyphotic in form. In the
absence of congenital defects, as infants learn to sit and hold
their heads erect, secondary lumbar and cervical lordosis
develop. Normal cervical lordosis, as measured by the C2-C7
Cobb angle is between 10 and 20 with an average of 14.47 and
a plumb line dropped from the spinous process of C2 should
pass through T1, L1, and the S1 in a balanced spine. Conditions
that lead to significant deviation from these values may affect
patients ability to maintain horizontal gaze and can lead to pro-
gressive pain, deformity, and neurologic compromise.
PATHOPHYSIOLOGY
Maintenance of sagittal balance in the human cervical spine
depends upon a number of both anterior and posterior struc-
tures functioning in their intended manner. Anteriorly, the

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 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others)
Figure 119.7. The severe degree of spinal cord compression in
Case 119.1. Significant spondylolisthesis is seen at each level from C3
to C6.
vertebral bodies and intervertebral discs primarily resist com-
pression and bear approximately one third of the axial load.
Compromise of the integrity of these structures by severe
degeneration or injury will lead to anterior height loss and sub-
sequent kyphosis. While the posterior elements bear the
remaining two thirds of the cervical spine axial load, their more
important function is resistance to tension. Pathology affecting
the facets, posterior musculature, and ligamentous structures
Figure 119.8. (A and B) Postopera-
tive radiographs from Case 119.1. The
sagittal plane deformity was corrected
through structural anterior interbody
grafting, and stabilized with a posterior
instrumented fusion. Although ade-
quate decompression is sometimes pos-
sible through anterior discectomy and
interspace decompression, laminec-
tomy was also performed to ensure
A B
adequate spinal cord decompression.
LWBK836_Ch119_p1281-1294.indd 1287
1287
weakens this tension band and transfers greater than physio-
logic loads to the anterior structures, which may then fail in
compression. Numerous anatomic studies have shown that the
facets play a significant role in this balance. Nowinski et al
showed that removal of as little as 25% of the facet may lead to
increased motion in laminoplasty and laminectomy patients.
Numerous studies by Zdeblick et al have similar results with a
reported compromise of shear strength after removal of 50% of
facets bilaterally and hypermobility at a segment from mere vio-
lation of the facet capsule.19 Several authors have concluded
from these and other studies that such weakening of the poste-
rior elements transfers load to the anterior columns that ulti-
mately leads to compression and anterior shift of the sagittal
vertical axis resulting in progressive kyphosis.1,17
Once kyphosis secondary to posterior ligamentous insuffi-
ciency is present, progression is likely. As the deformity wors-
ens, the spinal cord shifts anteriorly and eventually becomes
stretched over the posterior aspect of the vertebral bodies and
intervertebral discs. Compression of vascular supply to the cord
combined with increased tension within the cord itself, second-
ary to the tethering effect of the dentate ligaments, leads to
neuronal injury. Myelopathy develops and may be rapidly pro-
gressive and irreversible.
ETIOLOGIES
A common cause of cervical kyphosis is iatrogenic.1 Specifically,
postlaminectomy kyphosis may result from neglect of preopera-
tive kyphosis, disruption of posterior restraints in the cervical
spine, or inadequacy of posterior restraints secondary to radia-
tion treatment for tumors.8,9 As discussed earlier in this chap-
ter, the posterior neural arch bears the majority of load
transmission in the cervical spine, and the loss of integrity of
the archfacet complex can cause instability. When this occurs,
the weight-bearing axis of the spine shifts anteriorly and sagittal
balance is lost. The cervical musculature must then compen-
sate for this imbalance but, over time, fatigue and pain occur as
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 1288 Section XI Kyphosis and Postlaminectomy Deformities
progression of the kyphosis causes most weight to be trans-
ferred to the anterior spinal elements.
Patients with postlaminectomy kyphosis typically present with
a history of a period of improvement or even complete resolu-
tion of symptoms following their index procedure. After this
honeymoon period, they begin experiencing worsening neck
pain, forward decompensation of the head on the torso, and
progressive neurological deficits. Important points to elucidate
in the patient interview include presence, extent, and rate of
worsening of gait disturbance; sexual dysfunction or loss of bowel
or bladder function; and diminution of fine motor skills. A thor-
ough neurological examination is essential both to document
current dysfunction and to establish a baseline for future com-
parisons. Imaging studies including plain radiographs, CT, and
MRI should be obtained to fully evaluate the nature of the defor-
mity. Alignment and mobility of the spine are assessed with
anteroposterior (AP), lateral, and flexionextension views. MRI
can readily demonstrate the degree of cord compression and the
extent of disc degeneration and collapse. Finally, CT can identify
any bony ankylosis as well as assist in the assessment of pedicle
anatomy, if pedicle screw fixation is planned, and can identify
the presence of any vertebral artery anomalies. Figure 119.9 is a
basic algorithm for the treatment of postlaminectomy kyphosis.
When planning a cervical laminectomy, the surgeon must
take several factors into account to minimize the likelihood of
causing a postoperative kyphosis. Fusion in addition to the
decompression should be strongly considered in the setting of
multilevel decompression, any amount of facet resection,
preoperative instability, or skeletal immaturity. Posterior
decompression should not be considered in patients without a
neutral or lordotic spine on preoperative lateral radiographs as
Postlaminectomy
Kyphosis
FlexionExtension
Radiographs
No
Anterior Cord Fixed
Compression? Deformity?
No Yes
Number of
Posterior Fusion
Levels
Only
Involved
Single 2 or more
Disc
ACDF Space
(Anterior Only) Pathology
Only?
LWBK836_Ch119_p1281-1294.indd 1288
the spinal cord is unable to move away from the pathologic
area in these cases. Patients with kyphotic and, in many cases,
neutral sagittal balance should undergo fusion in addition to
decompression to restore anatomic alignment, which will indi-
rectly contribute to the decompression.
Numerous congenital defects include kyphosis as an occa-
sional, if not a characteristic, finding. Among these are Larsens
syndrome, KlippelFeil, myotonic dystrophy, osteogenesis
imperfecta, and neurofibromatosis. The etiology itself has little
bearing upon the treatment of kyphotic deformity, but it is
important to understand the more common of these congeni-
tal conditions as their associated anomalies may carry grave
prognoses if not identified and, oftentimes, spinal manifesta-
tions may be the first clue to the presence of the disease.
Larsens syndrome is a rare congenital disorder character-
ized by short stature, abnormal facies, cleft lip and palate, and
tracheomalacia. Associated vertebral anomalies include cervi-
cal kyphosis, hypoplastic vertebrae, and anteriorposterior dis-
sociations. Kyphosis has a variable natural history in this disease
and numerous published case series report variable results. The
series published by Francis and Noble in 1988 concluded that
surgical stabilization is typically required to prevent progres-
sion of the deformity. A more recent series published by Katz et
al suggested that nonoperative treatment may be attempted
early in life but agreed that fusion will usually be necessary.
KlippelFeil syndrome was originally described in 1912.
Patients with this disorder typically have a short, webbed neck;
decreased ROM in the cervical spine; and a low hairline. Torticollis,
facial asymmetry, and craniofacial anomalies may occur as well.
Patients with KlippelFeil syndrome usually present with the dis-
ease during childhood but may also present later in life. The chal-
Yes PosteriorAnterior
Posterior Approach
with Osteotomies
Yes Multilevel ACDFs
Plus Posterior
Fusion
Corpectomies +/
Diskectomies Plus Figure 119.9. Algorithm for
No Posterior Fusion surgical management of postlamine-
ctomy kyphosis.
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 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others) 1289

lenge to the clinician is to recognize the associated anomalies that

can occur with KlippelFeil syndrome and to perform the appro-
priate workup for diagnosis. Renal and cardiac anomalies often
accompany the musculoskeletal manifestations of this disorder
and, therefore, patients diagnosed with the syndrome should
undergo cardiac and renal evaluation. A progressive kyphotic
deformity secondary to this disorder may lead to spinal cord com-
pression and subsequent myelopathy. Complete imaging studies
including CT and MRI must be obtained in these patients to
ensure proper preoperative planning including neurosurgical
consultation if needed for intradural anomalies that are occasion-
ally present. Surgical intervention has become the standard treat-
ment for these patients. Nonoperative modalities do not seem to
halt the progression of deformity or improve the degree of insta-
bility in KlippelFeil syndrome.
Other congenital etiologies of cervical kyphosis include
myotonic dystrophy, osteogenesis imperfecta, and neurofibro-
matosis. Kyphosis is a rare but reported sequelae of these dis-
eases. Medical work-up of the more serious anomalies associ-
ated with these syndromes is important and surgical treatment
is typically dictated by the degree of functional disability associ-
ated with the kyphosis.

SURGICAL DECISION MAKING AND

PREOPERATIVE PLANNING FOR CERVICAL
KYPHOSIS
Cervical kyphosis most commonly occurs in the setting of
degenerative disease. In this situation, patients will present with Figure 119.10. The single-level anterior cervical discectomy and
interbody fusion, which may be adequate for correction of mild cases
diminished disc height at one or more levels with intact poste-
of kyphosis. ( Eric Harris.)
rior elements and posterior ligamentous structures. Generally
these patients present because of anterior compressive pathol-
ogy causing cervical radiculopathy or myelopathy. In most
cases, the kyphosis can be corrected in combination with an
anterior decompression procedure with anterior discectomy
and interbody fusion (Fig. 119.10). With appropriate preopera-
tive planning, intraoperative positioning, and graft size selec-
tion the surgeon should be able to correct the patients
deformity to at least a neutral posture that will further facilitate
decompression of the spinal cord.
In the setting of a previous neck injury or trauma, there may
be a ligamentous injury. In most of these patients who present
with chronic mechanical neck pain, there is no significant ante-
rior compressive pathology. In general, these patients will be
identified due to their kyphosis noted on X-ray. Specifically
patients with this disorder will generally be noted on flexion/
extension X-rays to have a fixed gapping between the spinous
processes at the affected level(s). An MRI may be helpful in iden-
tifying a ligamentous disruption, although clinical judgment is
necessary in determining appropriate patients for surgical treat-
ment. Because these patients do not generally have anterior
compressive pathology, and because they generally have pre-
served anterior disc height, a posterior stabilization procedure
with restoration of normal alignment and posterior instrumenta-
tion and fusion, as shown in Figure 119.11, should be sufficient.
There are several situations in which an anterior or com-
bined anteriorposterior reconstruction will be necessary in
the setting of cervical kyphosis. The first, and perhaps most
common, is kyphosis in the setting of degenerative disease caus-
ing multilevel spinal cord compression in the cervical spine. Figure 119.11. Posterior instrumented cervical fusion. This pro-
These patients will require both correction of their defor- cedure is capable of restoring cervical lordosis and providing excel-
mity as well as a formal decompression. In the event that the lent stability. ( Eric Harris.)

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 1290 Section XI Kyphosis and Postlaminectomy Deformities
Figure 119.12. Anterior and posterior cervical fusion that can
provide excellent sagittal correction and stability even in patients with
poor bone quality. ( Eric Harris.)
compression is predominantly anterior and mostly at the level
of the disc space and end plates these patients can often be cor-
rected through multiple level anterior cervical discectomy graft
and fusion, although they will also require a posterior cervical
fusion to compress the posterior elements and fix the patients
lordosis (Fig. 119.12). In the absence of such posterior fixation,
patients are at high risk for nonunion as well as risk of recur-
rent kyphosis. In a patient with degenerative cervical kyphosis
and congenital stenosis or retrovertebral compression, a poste-
rior decompressive laminectomy is also recommended. In gen-
eral, much more sagittal plane correction can be obtained
through a discectomy and interbody grafting than with a cor-
pectomy and strut grafting. The benefits of corpectomy and
strut grafting with regard to a higher likelihood of successful
fusion are negated by the supplemental posterior fixation and
bone grafting. In addition, restoration of lordosis through
appropriate sizing of interbody grafts should also provide an
indirect decompression to the spinal cord, which can be capi-
talized upon by the performance of concomitant laminectomy.
In general, the patient who presents with a postlaminectomy
kyphosis would also best be treated through an anterior/poste-
rior reconstruction. This typically involves an anterior decom-
pression through discectomy or corpectomy and strut grafting.
As has been reported previously20 these patients generally should
also undergo a supplemental posterior stabilization procedure.
The posterior stabilization procedure should, in general, extend
beyond the laminectomy both proximally and distally. Once
again the method of anterior grafting should depend primarily
upon the location of any focal compressive pathology.
LWBK836_Ch119_p1281-1294.indd 1290
There are also certain situations in which a patient with cer-
vical kyphosis may require an osteotomy. The most well known
of these is in the setting of ankylosing spondylitis. In this situa-
tion, the osteotomy is generally performed through a posterior
approach through a pedicle subtraction type of procedure at
the C7-T1 disc space as depicted in Figure 119.13. Generally,
patients who have ankylosing spondylitis do not have any sig-
nificant spinal cord compression. The procedure is typically
performed in a sitting position, and many surgeons will per-
form the procedure in an awake patient under local anesthesia.
It is the preference of the authors to perform the procedure in
this position but under general anesthesia with the use of tran-
scranial motor-evoked potential monitoring.
Another indication for cervical osteotomy is in the setting
of a patient who has a postlaminectomy kyphosis, which is
fixed secondary to an anterior cervical fusion. The anterior
cervical fusion may have developed spontaneously or from a
previous attempt to correct the deformity. In this situation, an
anterior osteotomy is necessary. It is possible that such patients
may also require a supplemental posterior osteotomy with
pedicle subtraction. Again, if a pedicle subtraction osteotomy
is performed, this is most safely done at C7-T1 because the
vertebral arteries lie anterior to the spinal column at this level.
With or without a posterior osteotomy the anterior osteotomy
is on its own a much more dangerous procedure with signifi-
cant risk to the vertebral artery, which may occur through the
lengthening of the anterior column and through the wide dis-
section needed to work laterally beyond the margins of the
uncovertebral joints.
One other indication for consideration of osteotomy is in
the setting of the patient with severe cervical myopathy causing
a postural kyphosis. These patients will generally require rela-
tively extensive anterior and posterior reconstructions. Through
multiple levels of interbody grafting throughout the lower cer-
vical spine significant correction of kyphosis can be obtained
although a supplemental posterior fusion will be needed. In
the setting of a fixed deformity that cannot be corrected
through anterior grafting alone, a posterior osteotomy is again
a reasonable option. In this case, it may be necessary to per-
form osteotomies at the apex of the deformity and care must be
taken to avoid injury to the vertebral arteries. Table 119.2 sum-
marizes key considerations in treating cervical kyphosis.
Considerations in Treating
TABLE 119.2
Cervical Kyphosis
Important Considerations in Treating Cervical Kyphosis
Iatrogenic kyphosis can usually be avoided by including a
posterior instrumented fusion when performing cervical
laminectomy.
Laminectomy alone will not allow for decompression of the
spinal cord in the kyphotic cervical spine.
Restoration of neutral or lordotic alignment usually necessitates
anterior reconstructive procedures.
Posterior stabilization should be included in cases where
anterior reconstruction of cervical kyphosis involves more than
two motion segments.
Multiple interbody grafts are more effective at restoring normal
lordosis than multilevel corpectomy and strut grafting.
Osteotomies of the cervical spine are most safely performed at
C7-T1 due to the anterior location of the vertebral arteries at
this level.
8/26/11 10:07:59 PM
 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others) 1291

A B

Figure 119.13. (A) Significant focal kyphosis of C7. The shaded area represents the margins of resection for
a pedicle subtraction osteotomy. This procedure can provide significant correction over a single segment and is
most safely performed at the C7-T1 level where the vertebral artery lies anterior to the spinal column. (B) The
same spine after closure of the osteotomy and combined anterior and posterior fixation. ( Eric Harris.)

CASE 119.2 Degenerative Cervical Kyphosis

A 64-year-old woman presented with a chief complaint of
gait abnormality and loss of fine finger dexterity. She noted
a gradual onset of increasingly frequent episodes of drop-
ping objects, decreasing bilateral upper extremity strength,
and numerous falls secondary to losing her balance. She
denied fecal incontinence but noted having to strain to
start her urinary stream and never feeling that her bladder
was completely empty. She had bilateral radicular pain in
the C6 and C7 nerve root distributions.
Physical examination demonstrated a clinically evident
swan neck deformity by acceptable overall sagittal and coro-
nal balance. She had difficulty walking with a heel to toe
gait and had a positive Romberg sign, positive Hofmanns
sign bilaterally, and was globally hyperreflexic. Muscle
strength testing revealed decreased strength in bilateral del-
toids, biceps, triceps, and wrist extensors. Sensation was
grossly intact in all dermatomes but the patient remarked
that nothing felt normal.
On plain radiography, shown in Figure 119.14, she was
found to have a swan neck type deformity with spondylolis-
thesis of C3 on C4 and C4 on C5, with significant kyphosis,
fixed spondylotic collapse at C5-6 and C6-7. MRI showed sig-
nificant spinal cord compression secondary to the deformity
and multiple disc herniations with additional spinal cord
compression at C7-T1. Because of the degree of myelopathy,
the severe deformity, and the number of levels involved, we
recommended an anterior and posterior procedure with an
anterior decompression and reconstruction and a posterior
stabilization and fusion with iliac crest autograft to maintain
the reduction. Figure 119.15 shows postoperative radio-
graphs demonstrating restoration of sagittal alignment and
stabilization of the previously unstable segments.

LWBK836_Ch119_p1281-1294.indd 1291 8/26/11 10:07:59 PM

 1292 Section XI Kyphosis and Postlaminectomy Deformities

A B

Figure 119.14. (A and B) (Case 119.2) Plain radiographs of a patient with a severe swan neck deformity
and moderate, progressive cervical myelopathy. There is significant anterolisthesis of C3 on C4 and a marked
kyphosis from C4 to C7 with significant spinal cord compression and myelomalacia.

A B

Figure 119.15. (A and B). (Case 119.2) Postoperative radiographs demonstrating excellent coronal and
sagittal alignment achieved through multiple level anterior discectomy and placement of interbody grafts,
which were crafted into a lordotic shape. Posterior stabilization and fusion with a lateral mass and pedicle
screw construct was necessary given the number of levels addressed anteriorly and to prevent recurrence of
the kyphotic deformity, especially in the setting of the C3-C6 laminectomy.

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 Chapter 119 Cervical Instability and Kyphosis (Rheumatoid, Dwarfism, Degenerative, Others)
CASE 119.3 Cervical Kyphosis Secondary to Neurofibromatosis Type 1
A 17-year-old girl with neurofibromatosis type 1 (NF-1) was
referred for a severe chin-on-chest deformity and deteriorat-
ing neurologic status. She had previously undergone mul-
tiple resections of neurofibromas. After the first
neurofibroma resection, the patient developed a progres-
sive kyphotic deformity. Multiple subsequent cervical proce-
dures including a C2 laminoplasty and a C3-C6 posterior
instrumented fusion had been performed. Despite these
interventions, her deformity progressed to the point where
she could no longer hold her head erect.
Physical examination revealed a 4-ft tall 64-lb female who
was wheelchair bound. Neck motion was severely limited.
Strength was 4/5 in deltoids, biceps, and triceps; 2/5 in wrist
extensors; 3/5 in grip, and 1/5 in hand intrinsics. With the
exception of iliopsoas that was 2/5, lower extremity strength
was 4/5. Sensation was globally intact. Deep tendon reflexes
were 2 in the lower and 1 in the upper extremities. The
patient had no findings suggestive of myelopathy.
A lateral radiograph and sagittal MRI cut, seen in Fig-
ures 119.16 and 119.17, show a severe kyphoscoliosis,
Figure 119.16. (Case 119.3) A severe chin-on-chest deformity
with previously placed instrumentation. Despite this intervention, this
neurofibromatosis patient had progression of her deformity.
LWBK836_Ch119_p1281-1294.indd 1293
1293
intact instrumentation, and multiple recurrent neurofi-
bromas. Following careful medical optimization, the
patient was admitted to the hospital and had a halo
placed under anesthesia the day prior to surgery. The
following day, she underwent removal of her existing
spinal implants and revision posterior fusion from the
occiput to T3. Postoperative radiographs are shown in
Figure 119.18. Three years after surgery, the patient
continues to do well with a solid fusion and mainte-
nance of her correction.
Diagnosis and surgical management of cervical instabil-
ity and kyphosis can be challenging. Careful clinical
workup and appropriate referral for related abnormalities
are of paramount importance in caring for these patients.
As always in spine surgery, thoughtful workup, thorough
preoperative planning, and meticulous surgical technique
lead to superior outcomes. Once a diagnosis is made,
however, the results can be quite rewarding with most
patients returning to active and satisfying lifestyles.
Figure 119.17. In addition to her remarkable cervical kyphosis,
this patient has several recurrent neurofibromas and severe compres-
sion of the spinal cord.
8/26/11 10:08:00 PM
 1294 Section XI Kyphosis and Postlaminectomy Deformities

Figure 119.18. (A) Postoperative

radiographs from Case 119.3 demon-
strate instrumented fusion from
occiput to T3. In this setting, the
patients deformity was quite rigid.
Correction would have required ante-
rior and posterior osteotomies, which
would have been very high risk in light
of the patients underlying syndromic
morbidity. As a result, the goals of sur-
gery were removal of the instrumenta-
tion and correction of deformity
through a long instrumented fusion
from the occiput to the thoracic spine.
As shown in (B), overall sagittal bal-
A B
ance was greatly improved.

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