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Management of Paralytic
Neuromuscular Disorders with
an Emphasis on Pelvic Obliquity
REVIEW OF BACKGROUND DATA scoliosis has been considered as mainly coronal plane tilt on
radiographs. However, pelvic obliquity should be thought of as
Paralytic deformities of the spine secondary to neuromuscular pelvic deformity in three planes. Surgical management of pel-
disorders are frequently associated with pelvic obliquity. In vic obliquity necessitates balancing the three-dimensional
these patients, pelvic obliquity can be clinically defined as an deformity in the coronal plane, sagittal plane, and axial (rota-
imbalance of pelvic pressures and position when sitting. Pelvic tional) plane (Fig. 116.3). Clinical assessment of pelvic obliq-
obliquity can range from mild in a patient with good truncal uity includes not only sitting pressures but also an evaluation of
control to severe in patients with global spine deformity and the muscle forces, or lack thereof, which controls the pelvis.
total body involvement cerebral palsy. Patients with an unbal- Clinically, patients with pelvic obliquity can be classified as
anced pelvis may present with difficulty in wheelchair fitting, either neurogenic (flaccid or spastic) or myopathic. The most
pressure sores due to improper weight distribution, or loss of common patient with pelvic obliquity seen by the spine sur-
functional abilities secondary to the need for truncal support geon is the patient with whole body involvement cerebral palsy.
with the upper extremities (Fig. 116.1). Spastic muscle forces across the hips and pelvis, in concert with
The driving force of pelvic obliquity can be multifactorial with scoliosis, frequently create a global spine deformity. In the past,
a main factor being the patients underlying neurogenic disorder. spastic curves have been classified based on whether there was
Patients with muscular dystrophy have lost truncal and pelvic sup- a fractional lumbosacral curve separate from the curve above
port due to their underlying myopathy. Patients with total body or whether the curve continued into the pelvis. If the pelvis was
involvement cerebral palsy have spastic forces, which cause under- part of the lower fractional curve then consideration was given
lying muscle imbalance with secondary obliquity, while patients to fusing short of the pelvis (Fig. 116.4). Clinically, however, the
with myelodysplasia have a variable mixture of motor and sensory decision to fuse to the pelvis is more complex and multifacto-
loss, which contributes to the pelvic obliquity. rial in these patients. Truncal strength and head support need
The goal of spinal instrumentation and arthrodesis in patients to be clinically evaluated. Patients with poor head and truncal
with paralytic deformity and pelvic obliquity is an improvement control almost always have some degree of pelvic deformity,
in sitting balance. Sitting balance enables the patient to interact which should be controlled with a spine fusion and instrumen-
with the environment, assists with wheelchair functionality, has a tation to the pelvis. Spasticity around the hips with resultant
beneficial effect on the clearing of pulmonary secretions, hip subluxation or dislocation also contribute to pelvic defor-
improves perineal hygiene, and prevents ischial skin break- mity in patients with cerebral palsy.5 Adduction deformities
down.11 Instrumentation and arthrodesis to the pelvis occasion- lead to pelvic obliquity and when present necessitate fusion to
ally is not needed. If stopping the fusion in the lumbar spine is the pelvis. Occasionally, hip surgery, such as adductor and/or
complemented, careful consideration must be given to the psoas releases, at the time of spinal surgery is indicated to con-
underlying disorder of the patient, the clinical and radiographic trol pelvic tilt and rotation. In patients with significant hip
parameters of the pelvic obliquity, and the likelihood of residual abnormalities, fusing short of the pelvis is unlikely to control
spinal deformity and clinical problems1,3 (Fig. 116.2). their obliquity as they have continued abnormal hip forces,
which will drive the pelvis through the unfused lumbosacral
junction.
PATIENT EVALUATION WITH RESPECT Classically myopathic disorders present with a collapsing
TO PELVIC OBLIQUITY kyphoscoliosis, which continues down to include the pelvis. In
general, many of these patients (i.e., muscular dystrophy) have
Pelvic obliquity can be measured both clinically and located and mobile hips; however, their overall collapsing
radiographically. Classically, pelvic obliquity in neuromuscular deformity and muscle weakness frequently necessitates
1252
A B C
Figure 116.2. (A) Plain radiograph of a patient with Friedreichs ataxia demonstrating a collapsing curve
with pelvic obliquity in this patient with no truncal support. (B) Postoperative radiograph after a posterior
instrumented fusion to L5. The spinopelvic balance is excellent in the immediate postoperative period.
(C) Follow-up radiograph at 2-year follow-up. Note the development of deformity at the lumbosacral junction
and pelvis obliquity. The black lines represent the intercristal line of the pelvis.
CASE EXAMPLE
The patient is a 16-year-old girl who presents with a progressive
spine deformity and hip stiffness related to a spastic dislocation
of her hip (Fig. 116.7). According to her caregivers she has had
progressive sitting difficulties related to her lumbar truncal
deformity. Her examination revealed a stiff spastic lumbar sco-
Figure 116.3. Clinical photograph demonstrating significant sag- liosis as well as a dislocated hip with 20 of abduction and 30
ittal plane deformity of the pelvis in a patient with spastic quadripa-
to 90 of motion in flexionextension plane. External examina-
retic cerebral palsy.
tion of her pelvis revealed a rotational deformity and a lordotic
posture secondary to spastic muscle forces. Radiographs
revealed a scoliosis with pelvic inequality. Hip radiographs
lumbar kyphoscoliosis (Fig. 116.6). This is evident both radio- revealed a chronically dislocated femoral head with resultant
graphically and clinically. However, pelvic rotation is difficult to deformity. To improve her sitting balance as well as to arrest
classify radiographically. Clinical evaluation of the patient her curvature, she underwent a posterior instrumented fusion
should include an assessment of the axial or rotational defor- to the pelvis. Postoperatively, she has a balanced spine and pel-
mity of the pelvis in these patients. Any significant rotational vis and greatly improved sitting balance. This case highlights
deformity of the pelvis will likely make fusion to the pelvis man- the importance of fusion to the pelvis in a patient with spastic
datory or decompensation may occur (see Table 116.1). scoliosis and a dislocated hip.
A B
Figure 116.4. (A) Radiograph demonstrating a fractional lumbosacral curve, evident by the rotation of
the spinous processes turning back as a separate curve. In a patient with reasonable truncal support and head
control, stopping short of the pelvis with a fusion is an option. The arrow points to the fractional curve.
(B) Radiograph demonstrating that the pelvis and sacrum are part of the primary curve, in this instance
fusing to the pelvis is clinically necessary.
PELVIC FIXATION
GALVESTON FIXATION
Figure 116.5. Clinical photograph demonstrating ischial skin Galveston fixation relies in the strength of the iliac bone to
breakdown in a patient with pelvic obliquity and neurogenic scoliosis. afford a stable base of fixation.2,6,9,15 It was originally described
with segmental Luque wiring, which provides segmental sub-
laminar fixation and remains a common method of fixation to
the pelvis. Galveston fixation requires the surgeon create a
complex three-dimensional contour of the rod to allow a
ACCEPTED FORMS OF SURGICAL smooth transition into the pelvis from the thoracolumbar
TREATMENT spine. For experienced surgeons, the rod contouring is not as
challenging; however, for surgeons unfamiliar with the rod
GLOBAL INSTRUMENTATION
contouring technique can be technically demanding. In this
There are multiple fixation options to the pelvis in paralytic technique, the rod is driven into the iliac crest and the thora-
deformity, and in many instances the mode of fixation is sur- columbar spine is translated to the precontoured rod by way of
geon dependent. The fixation options in the lumbar spine and sublaminar wires. The unit rod is made precontoured rod and
thoracic spine are varied as well and are discussed elsewhere in relies on Galveston technique into the pelvis and in the thora-
this book. Segmental pedicle screw fixation optimizes the columbar spine with sublaminar wires. The advantages of this
strength of the construct and in the lumbar spine helps share technique are the ability to obtain balance of the global spine
the load of the pelvic fixation construct. Sublaminar wires by segmental sublaminar wire to translate to the precontoured
remain an often used construct in the spine for neuromuscular rod. The disadvantages remain the challenging two-dimen-
disease and have been shown to offer excellent stability.2,6 The sional rod bend, as discussed above, and the potential for
addition of pedicle screws in the lumbar spine has been shown implant loosening ilia, since the rods cross the sacroiliac
to improve pelvis construct stability and thus hybrid fixation in joint.
A B C
A B C
Figure 116.8. (A) Sitting radiograph of a patient with scoliosis and pelvic obliquity. (B) Traction radio-
graph showing inability to obtain a balanced pelvis with traction. (C) Postoperative radiograph demonstrating
excellent spinopelvic balance after a posterior fusion using hybrid instrumentation.
fixation. The Colorado plate system (Medtronic, Memphis, TN) rod, which is fixed distally into the plate with any residual pelvic
allows for screws to be placed through a plate into S1 and S2 imbalance corrected by distracting the rod on the side of the
(Fig. 116.9). The fixation points are standard S1 screws, which elevated hemipelvis. Another method of correction is to perform
should be placed, if possible, bicortically into the S1 end plate. a convex cantilever method (proximal to distal) by capturing the
The S2 screws are angled laterally and cranially allowing fixation plate distally while correcting the lumbar curve and resultant
into the sacral alae, with optimally bicortical fixation, which pelvic obliquity. Fixation strength has been shown to be improved
improves pullout strength. The rod can subsequently be fixed to by 50% by adding a pair of L1 pedicle screws to the construct.4
the plate allowing for solid fixation into the sacrum. The pelvis The advantages of sacral plate fixation is the avoidance of cross-
is balanced by either translating the spine to the precontoured ing the sacroiliac joint and the potential loosening seen with iliac
fixation methods. The disadvantages are suboptimal pullout The biomechanical forces across the L5-S1 motion segment are
strength due to small or osteopenic sacrum. Significant pelvic tilt significant and will overcome a pair of S1 screws, especially in
or deformity may also make screw placement challenging and patients with pelvic imbalance and osteopenia. The rate of
suboptimal. Biomechanically, this construct has been shown to implant dislodgement, implant loosening, and pseudarthrosis
be as stable as Galveston fixation.4 would be unacceptably high and we do not recommend ending
constructs in this patient population with unprotected S1
screws. If this form of fixation is used, it is better applied in a
SACRAL SCREWS
larger patient with stronger bone stock (Fig. 116.10) and is best
The use of a pair of S1 screws alone as distal anchors for long supplemented by segmental fixation to the lumbar spine via
constructs in neuromuscular scoliosis is not recommended. pedicle screws.
A B C
Figure 116.10. (A and B) Sitting anteroposterior and lateral radiographs of a patient with
neuromuscular scoliosis. (C and D) Radiographs after instrumented fusion to the pelvis. The
distal end of the construct is a pair of S1 screws, which is usually not recommended without
D
anterior column support at L5-S1.
ILIAC SCREW FIXATION groups of pediatric neuromuscular patients treated with instru-
mentation to the pelvis with either Galveston fixation or iliac
Iliac screw fixation has become a common method of fixation screw fixation.9 The groups had similar preoperative radio-
to the pelvis in neuromuscular deformities. Iliac screws provide graphic measures of the coronal and sagittal plane deformities
an additional plane of fixation (inferior and lateral orienta- and similar postoperative correction. However, the group
tion), which protect the S1 screws from pulling out. It allows for treated with iliac screws had greater improvement of their pel-
an easier capture of the rod into lumbar pedicle screws. Biome- vic obliquity, less implant failures, and require no revisions
chanically, iliac screw fixation has been shown to be a consis- when compared with the Galveston group at 3-year follow-up.
tently strong method of fixation. Clinically, the rate of Biomechanically as well as clinically, the addition of S1 screws
pseudarthrosis and implant loosening is less for this form of significantly improves construct strength in long constructs
fixation than in Galveston fixation. Peele et al evaluated two (Fig. 116.11). Rigid fixation to the pelvis is especially important
A B C
INTRAOPERATIVE METHODS
ANTERIOR RELEASE
The decision regarding whether or not to perform an anterior
release on a patient with neuromuscular scoliosis to obtain pel-
vic balance can be difficult.11,15,16 The answer to the question
revolves around whether or not a posterior-only procedure will
be able to meet the goal of obtaining a balanced spine and
horizontal pelvis. Determining flexibility in a spastic patient
can be difficult with standard flexibility films. We routinely use
Figure 116.12. Computed tomography scan in the immediate a traction view of these patients to assess flexibility.16 Larger
postoperative period documenting the tip of an iliac screw impinging thoracolumbar curves and large lumbar curves especially when
into the hip joint.
associated with fixed lordosis may benefit from an anterior api-
cal release with thorough discectomies. The sagittal and three-
dimensional deformity of the pelvis also may aid in this decision.
in paralytic deformities where it is uncommon to add anterior Patients with lordoscoliosis of the lumbar spine frequently have
column support at L5-S1. a mixed coronal and sagittal plane deformity of the pelvis. In
Technically, screws should be placed under direct vision of these patients, the lumbar spine must be flexible enough to
the outer table of the pelvis. This is accomplished by exposing correct the pelvic deformity in both planes. Frequently, more
the outer table of the crest and by visualizing the sciatic notch. spastic patients benefit from anterior releases whereas a patient
This permits the screw to be directed just superior to the sciatic with a more myopathic and hypotonic curve can be managed
notch and minimizes the chance of lateral penetration of the posteriorly. Although complete curve correction is not neces-
screw. Schwend et al studied the anatomy of the ilium with sary to obtain pelvic balance, fixed and rigid curves based on
regard to the optimum bone available for screw purchase.12 clinical examination and radiographs may signify that an ante-
This study showed that iliac screws need to be placed in the rior release is beneficial. In patients with deficient posterior
bony pelvic area, which consists of straight columns of bone elements such as myelodysplasia, an anterior release and fusion
extending from 2 cm below the posterior iliac spine, traversing are paramount when fusing to the pelvis to obtain an adequate
above the sciatic notch, and ending at the anterior iliac spine. arthrodesis (Fig. 116.13).
Potential problems with iliac screws are misplacement outside
of the tables of the ilium, with resultant poor biomechanical
TRACTION
strength, and the potential for screws to be misguided into the
sciatic notch or the hip joint (Fig. 116.12). Traction can be used to obtain some balance through ligamen-
Strength of fixation into the ilium may be increased by plac- totaxis. This technique has been described in patients with neu-
ing two screws on each side. Phillips et al in their study demon- romuscular scoliosis.7,14 Halo-femoral traction is applied with a
strated a clinically significant increase in pullout strength with four- or six-pin halo and a femoral pin on the high pelvis side
this technique.10 In their series of 50 patients comparing the and applied at the time of positioning in the operating room.
use of a single screw in each ilium to two screws in each ilium, This technique will be discussed elsewhere in depth in this text-
they reported a high complication rate in the single screw book and is mentioned here for completeness.
group. As mentioned above, adding sacral and multiple lumbar
pedicle screws to an iliac screw construct acts to improve
HARRINGTON DISTRACTOR
strength of fixation. Rod capture into the screws and the
mechanics of pelvic leveling may be done by means of spine The intraoperative use of a Harrington distractor can help
translation to the rod, cantilever correction, or distraction obtain overall spinal balance. The Harrington outrigger can be
techniques. attached to an anchor point in the upper thoracic spine and
The advantages of iliac screw fixation are the ease of inser- then to a sacral screw or a large hook over the sacral alae. Grad-
tion and ease of rod capture. Variable-angle screw heads are ual distraction is applied to the outrigger after thorough facet-
now available for most implant systems, which permit easier ectomies and ligamentum flavum resection and allows significant
rod capture in long constructs. Potential disadvantages are correction of lumbar curves. In general, the outrigger does not
screw prominence, screw loosening secondary to forces across negatively affect sagittal balance and can frequently help man-
the SI joint, and screw misplacement. Screw misplacement can age the lumbar lordoscoliosis seen in these patients with pelvic
be avoided by exposure, and screw prominence can be avoided deformity. Once the outrigger is applied and distraction
by cutting out a 1 cm 1 cm block of bone at the entry point of performed, a rod can be placed on the other side of the spine
the screw so that it can be countersunk deep enough to avoid to maintain correction. In theory, this is similar to intraopera-
prominence. Placement of these screws also entails exposure, tive halo-femoral traction. Our experience is that this is a
powerful technique given the direct attachment to the spine lumbosacral junction allowing for hematoma formation and
(Fig. 116.14). subsequent drainage.13 Wound drainage in these patients is
problematic and the best defense against this is the intraopera-
tive placement of a subfascial drain. Our practice is to keep the
drain in the first two postoperative days with removal on the
STRENGTHS AND WEAKNESSES
third day. We prophylactically place these patients on intrave-
nous antibiotics until the drain is removed given the intimate
POSTOPERATIVE MANAGEMENT
relationship to the implants. Soiling of the distal end of the
Postoperative management in these patients in the immediate wound is a potential source of wound contamination and sub-
perioperative period revolves around nutritional support and sequent infection. The distal end of the wound must be dressed
pulmonary and wound care.8 The surgical wounds in these with an occlusive, adhesive dressing and frequent inspection by
patients are large, and the dead space can be significant at the the nursing staff to prevent urine or fecal contamination. If
Is clinically
Does the patient have
evident pelvic
head control and some NO
obliquity
truncal control?
present?
YES NO YES
NO YES
YES
Clinically the patient was doing well without problems and was
scheduled for follow-up at 2 years postoperatively. He presented
back to clinic at 18 months postoperatively with fevers and an
elevated serum C-reactive protein levels. The lucencies around
the iliac screws had increased further. He was taken to the oper-
ating room after ultrasound showed a fluid collection around
the rods. All the spine implants were removed at the time of
irrigation and debridement with subsequent healing of the
infection. He currently has a balanced spine with no clinical
evidence of continuing problems.
This case highlights the need for not only iliac screws but
also distal lumbar and sacral screws. It is unclear whether or not
his lucency at the beginning was related to biomechanical fac-
tors or infection. Likely, in this patient he had progressive loos-
ening related to a poor biomechanical construct, which became
Figure 116.14. Photograph of a Harrington outrigger, which may a focus for hematologic seeding. This patient underwent com-
be used as an intraoperative means of obtaining curve correction via plete removal of implants, which is paramount in the presence
distraction. of a delayed, deep wound infection. Clinically he has done well
after implant removal.
Complications in this patient population are frequent.
contamination occurs, a dressing change must be performed Stopping the fusion short of the pelvis can be complicated by
with a Betadine scrub gently to the area of contamination. adding on to the deformity of the caudal unfused spine and
In the first 3 months after surgery protection of the instru- pelvis and further pelvic sitting inequality. This can be poten-
mentation is paramount and is done by first insisting that trans- tially avoided by proper preoperative planning. If there is any
fers be done by two or more people to prevent implant dis- doubt about including the pelvis in the fusion then fusing long,
lodgement during transfers of the patients unsupported lower with pelvic fixation, is usually the best operation and can pre-
extremity and pelvis. Patients with significant global body vent the adding on phenomena postoperatively.
involvement should be prevented from leaning forward and The risk of infection in patients with paralytic deformity is
placing a kyphotic stress onto the instrumentation. We also increased due to these patients poor nutritional status.
advocate that physical therapy should be judicious in the Preoperative assessment of serum total protein and lymphocyte
amount of postoperative therapy that is done for the hip flex- counts and nutritional consults is recommended if concerns
ors, hip adductors, and hamstrings. These muscle groups con- are present. Standard antibiotic prophylaxis is done with a first-
trol the pelvis and any indiscriminate therapy should be avoided generation cephalosporin antibiotic given prior to skin incision
in these patients given their osteopenia and potential for and then dosing 24 hours postoperatively; if a drain is used, we
implant dislodgement. will continue antibiotic coverage until the drain is taken out.
Instrumentation and fusion to the sacrum or pelvis creates lon-
ger operating room times and bigger surgical dissections. The
DISCUSSION OF COMPLICATIONS use of postoperative drains may help minimize the dead spacing
HOW TO MANAGE due to surgical dissection and secondary hematoma formation.
Early postoperative infections are ideally managed by aggressive
CASE PRESENTATION surgical debridement and intravenous antibiotics. Unfortunately,
delayed infections are best managed by deep wound irrigation
The patient is a 15-year-old boy with spastic quadriparesis sec- and debridement with complete implant removal.
ondary to neonatal hypoxia (Fig. 116.15). He presents to ortho- Implant prominence, especially iliac screws, can be a prob-
pedic clinic with a progressive spine deformity that has affected lematic complication in these patients. This can best be man-
his ability to sit in his wheelchair. His parents report that his aged by taking a square of bone away from the iliac crest start-
progressive deformity is making it difficult to function in his ing point for iliac screws. In addition, the use of sacral
environment at school and daily interactions; they also voice screwplate systems can minimize implant bulk in those patients
concern regarding the potential for continuing curvature. His who are instrumented to the sacrum.
spinal-based examination revealed poor head control and trun- Windshield wipering of Galveston rods or iliac screws can be
cal shift not controlled with side bolsters in his wheelchair. seen in neuromuscular patients managed with instrumentation
Pelvic examination revealed uneven sitting pressures with bilat- and arthrodesis to the pelvis. This can be seen, even in the face
eral hip flexion deformities of 20 and abduction of each hip of a fusion to the sacrum as the sacroiliac joint remains unfused.
of approximately 40. Given his spinal deformity, total body However, progressive radiolucency around screw can be a har-
involvement, and abnormal muscle forces across the pelvis he binger of pseudarthrosis or deep infection and should be cor-
underwent an instrumented fusion to his pelvis. Intraopera- related with the patients clinical course. The addition of S1
tively the patient developed a coagulopathy and thus had dom- screws and L5 screws to constructs involving iliac screws greatly
inos placed to more rapidly finish the case. His postoperative enhances stability and should be done to avoid loosening of the
radiographs demonstrated appropriate coronal and sagittal iliac screws.
balance. At 6 months postoperatively, he developed significant Implant failure can be seen as either fatigue fractures or dis-
radiographic lucencies (windshield wipering) around the lodgement. Fatigue fractures are usually related to pseudart-
iliac screws. The lucencies progressed at the 1-year follow-up. hrosis. Fatigue fractures may need to be treated with revision if
A B C
D E F
Figure 116.15. (A and B) Sitting radiographs of a patient with progressive neuromuscular scoliosis.
(C and D) Postoperative radiographs after an instrumented posterior fusion. (E) Radiograph at postoperative
follow-up showing clear radiolucency represented by the arrow and halo signs around both iliac screws.
(F) Sitting radiograph obtained 1 year after instrumentation removal for infection.