CHAPTER
Gilbert Chan
114
Surgical Treatment of Flaccid
Neuromuscular Scoliosis
INTRODUCTION
Progressive spinal deformities are common in patients with
flaccid paralysis, and result from progressive truncal weakness/
hypotonia, compounded by the effects of gravity and normal
growth of the immature spine. Although the etiology is most
often a heritable condition such as spinal muscular atrophy
(SMA), the muscular dystrophies, and Friedreichs ataxia,
acquired conditions such as poliomyelitis and traumatic paraly-
sis are also causes. Progressive spinal deformities result in a loss
of sitting balance, often exacerbating pulmonary impairment.
Collapse of the chest wall reduces the space available for the
lungs, and loss of trunk height may impair diaphragmatic func-
tion. As such, maintaining sitting height and chest wall integrity
helps to stabilize pulmonary function should theoretically delay
the progression of pulmonary decline expected in many of
these diseases. Treatment is based upon the magnitude and
progression of the curvature, the age of the patient, and func-
tional considerations. Bracing plays a limited role for selected
diagnoses, and surgical instrumentation and fusion is required
for progressive curvatures. The timing of intervention is based
upon the natural history of each underlying diagnosis. The sur-
gery should achieve a stable, well-balanced spine, allowing for
optimal seating, comfort, and ease of care.
SPECIFIC CONDITIONS
DYSTROPHINOPATHIES
The dystrophinopathies are a group of x-linked recessive condi-
tions caused by mutations in the Duchennes muscular dystro-
phy (DMD) gene, which codes for dystrophin. Although skeletal
muscle involvement results in a progressive muscle weakness,
dystrophin is also found in the brain, heart, and smooth mus-
cles, explaining associated findings such as decreased intelli-
gence, cardiomyopathy, intestinal hypomotility, and abnormal
bleeding during spinal surgery. The various mutations in the
DMD gene lead to a variable deficiency in dystrophin, while
90% of children with DMD eventually have complete deficiency
of dystrophin, 85% of Becker muscular dystrophy (BMD) cases
have an abnormal dystrophin molecule. While DMD has an
overall reported incidence of 1 in every 3300 to 3500 live births,
BMD has a reported incidence of about 1 in 18 to 31,000 live
LWBK836_Ch114_p1229-1243.indd 1229
David A. Spiegel
Denis S. Drummond
male births, and generally follows a similar but more benign
course when compared with DMD.
Children diagnosed with DMD typically have a general delay
in motor development, and independent ambulation is usually
achieved by 18 months (12 to 24 months). Other presenting
symptoms include gait disturbance, most notably the onset of
toe walking in patients who previously walked with the feet flat.
As the weakness progress, patients begin to ambulate with a
broad-based gait, associated with lumbar hyperlordosis and calf
pseudohypertrophy, between 3 and 6 years of age. A progres-
sive decrease in function is observed between 6 and 11 years of
age, and ambulation is usually lost before 13 years of age. Death
usually occurs in the late second to the early third decade from
progressive cardiopulmonary failure. Patients with BMD typi-
cally manifest a more benign course, with symptom onset
between 5 and 15 years of age. The pattern of weakness is simi-
lar to DMD. Patients with BMD have a preservation of neck
flexor tone, versus those with DMD who are usually unable to
hold up their head when lifted from a supine position.
Diagnostic testing of DMD will often reveal a 10-fold increase in
the levels of creatine phosphokinase (CPK). On muscle biopsy, in
addition to an absence of dystrophin, there are dystrophic changes
including deposition of fat and connective tissue, and focal areas
of necrosis, regeneration, and hyalination. In BMD, there is a five-
fold increase in CPK, and biopsy findings include abnormalities in
size of dystrophin, in addition to the other findings noted in DMD.
At present, genetic testing may be used to establish a diagnosis
without the need for obtaining a muscle biopsy.
Scoliosis has been reported in 74% to 100% of patients with
DMD,15,41 and the onset of a spinal curvature can be correlated
with both the patients age and the onset of progressive muscle
weakness. Significant progression occurs when the capacity to
ambulate has been lost. The most common pattern of deformity
in DMD is a kyphosing scoliosis, and the vast majority of curves
will progress at 1 to 4.5 per month.41 Steroid administration
has been associated with an improvement in overall function and
a delay in the development of scoliosis.1,3 Alman et al3 reported a
prolongation of ambulation until a mean age of 12.3 years, and
only 5 out of 30 cases developed scoliosis by 16 years of age.
Loss of pulmonary function is anticipated and may be acceler-
ated by the presence or progression of a spinal deformity. A rapid
decline in pulmonary function is often seen in the second decade
of life25,47 and may be exacerbated by a spinal curvature. Kurz
et al25 documented a decline in respiratory function of 4% for
1229
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 1230 Section X Paralytic Deformity
each 10 of progression. Hsu22 reported a 12% to 16% decline in
forced vital capacity (FVC) annually with curves >40.
Nonoperative strategies such as bracing or seating modifica-
tions do not alter the natural history; while these strategies may
potentially delay the progression of scoliosis, delaying defini-
tive spinal fusion increases the risks of this surgery, given the
progressive decline in cardiopulmonary function. Although
rigid spinal orthoses restrict chest wall motion and have a nega-
tive impact on pulmonary function, a soft spinal orthosis may
be considered to support sitting balance over the short term
(while planning definitive treatment), or in selected patients
whose overall function precludes surgical treatment. Overall,
brace treatment is never definitive.
An instrumented spinal arthrodesis has been offered for
progressive curves beyond 20 to 30. Early treatment of smaller,
more flexible curves improves the outcome while decreasing
the risks and should be performed before cardiac and pulmo-
nary functions have declined substantially. Recent evidence
suggests that it is safe to carry out corrective surgery even with
FVC between 20% and 30%.16 The effect of spinal surgery on
pulmonary function remains undetermined, especially as a
progressive decline in function (up to 8% per year) is antici-
pated from the disease. Although a reduction in the rate of pul-
monary decline after spinal fusion has been reported,44 other
studies have failed to corroborate this finding.24,32 Anesthetic
complications include acute rhabdomyolysis (with inhalational
agents), producing a picture very similar to malignant hyper-
thermia, as well as hyperkalemia and myocardial depression.19
Significant intraoperative blood loss must be anticipated (mean
as high as 4000 cc). Cardiac involvement is common, usually a
progressive cardiomyopathy (50% diagnosed by 18 years), and
cardiac complications include tachyarrhythmias and sudden
cardiac arrest.
Several studies have analyzed quality of life following spinal
stabilization. While acknowledging the anticipated decline in
quality of life in DMD, Bridwell et al8 found a high level of
patient satisfaction, and improved quality of life and cosmesis,
at nearly 8 years follow-up. However, overall function was
decreased in nearly 40%.8 Ramirez et al37 administered a ques-
tionnaire to parents and found that quality of life was improved
in 15 out of 21 families, including better sitting ability (and
posture), less discomfort, and greater self-esteem.37 A recent
systematic review of the literature found weak evidence to sup-
port improvements in quality of life, due to the uncontrolled
nature of surgical series and other factors.30
SPINAL MUSCULAR ATROPHY
SMA is a recessively inherited condition resulting from a defect
in the survival motor neuron (SMN) gene on chromosome 5.
TABLE 114.1 Modified SMA Classification45
Type Age at Onset
1 (Severe) 06 mo
2 (Intermediate) 718 mo
3 (Mild) >18 mo
4 (Adult) Second or third decade
Adapted from Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal
muscular atrophy. J Child Neurol 2007;22(8):10271049.
LWBK836_Ch114_p1229-1243.indd 1230
The condition affects the anterior horn cells, resulting in pro-
gressive degeneration of motor neurons and progressive mus-
cle weakness. The incidence is estimated to be between 10 and
16 per 100,000 live births. The clinical manifestations are vari-
able, and disease severity correlates with the age at diagnosis;
patients diagnosed in the first few months of life have a poor
prognosis with limited survival, while those diagnosed in child-
hood or adolescence have a milder form of the disease. Clinical
features include hypotonia and symmetrical muscle weakness
of the trunk and extremities. The magnitude of weakness is
greater in proximal muscle groups, and the lower extremities
are more involved than the upper extremities. The sensory
examination is normal, and deep tendon reflexes are dimin-
ished or absent. Common orthopedic concerns include scolio-
sis, contractures, and hip dysplasia. With significant intercostal
weakness, many patients exhibit a characteristic diaphragmatic
breathing pattern and develop a bell-shaped thorax. There is
an initial onset of weakness that plateaus, and residual deficits
remain stable for a prolonged period of time. Although muscle
strength may be maintained, overall function may decline over
time.
The diagnosis of SMA was previously dependent on clinical
findings: symmetrical weakness affecting the trunk and limbs,
denervation demonstrated on muscle biopsy, neurophysiologic
abnormalities including denervation on electromyogram
(EMG) and a decrease in amplitude on nerve conduction
velocity (NCV) studies, and exclusion of other conditions (dis-
orders of the central or peripheral nervous system, myopathies,
neuromuscular junction). These diagnostic criteria have been
largely replaced by genetic testing, and the SMN gene deletion
test has a sensitivity of 95% and a specificity of 100%.
SMA has been classified using the age at presentation, the
functional level, and a combination of these. The temporal
classification includes three clinical types: type 1 (Werdnig
Hoffman) presents in infancy and usually is fatal within the first
few years, type 2 (chronic infantile form) presents from 6 to
18 months, and type 3 (KugelbergWelander), usually presents
after the second year of life and has a more benign course. The
functional classification proposed by Evans et al14 includes four
types: type 1 (never achieve the ability to sit), type 2 (develops
head control and sits independently), type 3 (pull up, stand,
and ambulate with support), and type 4 (ambulate before the
onset of weakness). A classification combining elements of each
of these is shown in Table 114.1.45
Patients diagnosed within the first 6 months of life have the
most severe form, which is usually fatal during the first few
years, although recent advances in respiratory care may enhance
long-term survival. Chung et al12 showed a 30% probability of
survival at 10 years of age. These children never achieve the
ability to maintain an upright posture, and up to 95% will not
Highest Function Survival
Never sit <2 yr
Sit but do not stand >2 yr
Stands and walks Adult
Walks through adult years Adult
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 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis
survive beyond their second year of life. Children with type 2
(infantile) are diagnosed after 6 months of age, and while they
usually achieve the ability to sit independently, ambulation is
rare. Survival rate to 5 years of age is approximately 98.5%, and
approximately 69% will survive to 25 years of age.49 Type 3
(juvenile type or KugelbergWelander disease) has a variable
age at presentation. Patients are able to ambulate indepen-
dently; however, a decline in ambulatory function may be
observed in older children and adults. Maintenance of ambula-
tion depends upon the age at presentation, and life expectancy
approaches those of the normal populace.49 Development of
musculoskeletal problems (scoliosis, contractures) is directly
linked to functional capacity and is more commonly seen in
those who have lost ambulatory capacity. Type 4 SMA has a very
mild course with life expectancy approaching or near the nor-
mal population. Patients are highly functional with onset of
weakness typically seen between the second and third decades
of life.
Scoliosis is very common in the SMA population, and
patients with a greater severity of disease develop a spinal defor-
mity earlier, and these tend to be progressive.4,14,38 The mean
age at diagnosis is between 6 and 8 years.4 The most common
pattern of deformity is a thoracolumbar curvature (90%), often
right sided, and 20% to 30% will have a significant kyphosis
associated with the scoliosis.38 Other curve patterns have been
reported, including single thoracic curves, lumbar curves, and
double curve patterns.4 Patients with SMA develop a chronic
restrictive form of respiratory insufficiency (progressive weak-
ness of the intercostals and abdominal muscles), which may be
worsened by the development of a spinal deformity.23
Most children with a scoliosis will require treatment. Studies
have shown that orthotic treatment does not prevent progres-
sion of the spinal deformity, thus the role of bracing is to pro-
vide postural support for activities of daily living, and ideally to
delay the need for surgical intervention.4,38 As more rigid
orthoses may produce ribcage deformity and have a detrimen-
tal effect on pulmonary function, a soft spinal orthosis is the
most appropriate design.
Spinal fusion is both safe and effective in children with
SMA.4,14,17 Bridwell et al8 demonstrated an improvement in
function, quality of life, pulmonary function, and cosmesis.
Although definitive spinal fusion with instrumentation best
achieves these goals, a major concern is the timing of interven-
tion as many curvatures present during infancy or childhood,
and spinal fusion is best delayed until after at least 5 to 6 years
of age, to allow for growth of the chest wall (and rib cage) and
maturation of the lungs, and ideally until adolescence to allow
for maximal trunk growth. Other surgical options are available
when definitive fusion is undesirable, including growing rods
and the vertical expandable prosthetic titanium rib (VEPTR).
POLIOMYELITIS
Thousands of residual cases of polio are present worldwide,
mostly in developing nations. The incidence of scoliosis in
poliomyelitis remains unclear, and the pattern and severity of
the curvature is largely dependent on the levels and severity of
weakness. Leong and colleagues27 attributed the spinal defor-
mity to two different mechanisms, a milder asymmetric form
and a more severe collapsing form due to extensive symmetri-
cal paralysis. As for other forms of flaccid weakness or paralysis,
progressive deformities require definitive spinal fusion. Bracing
LWBK836_Ch114_p1229-1243.indd 1231
1231
may serve as a temporizing measure, and anecdotal evidence
suggests that these curves often remain flexible into adoles-
cence. Surgical treatment is recommended for progressive
curves greater than 40, especially if upper extremity function
is compromised.27 The treatment approach depends upon the
magnitude and flexibility of the curve, and usually involves an
instrumented posterior spinal fusion.
FRIEDREICHS ATAXIA
Friedreichs ataxia is an autosomal recessive condition resulting
from a defect in Friedreichs ataxia-frataxin gene (FRDA)
mapped on chromosome 9q13. The diagnosis is established
during the first or second decades, and clinical findings include
ataxia, absent deep tendon reflexes, and an extensor plantar
response. Dysarthria is observed within 5 years of the onset of
symptoms, and because of ataxia ambulation becomes increas-
ingly difficult as the disease progresses. Beauchamp et al5 found
that use of a wheelchair began at a mean age of 18.2 years, and
all patients were wheelchair bound by 20.5 years. Medical
comorbidities include cardiomyopathy and type 1 diabetes mel-
litus (10%). Musculoskeletal manifestations include scoliosis
and foot deformities (pes cavus, pes equinovarus, claw toes).
The incidence of scoliosis is 63% to 100%,10,26,31 and curve
patterns are often similar to those observed in idiopathic scoli-
osis, double major curves in 33% and thoracolumbar curves in
21%, and 22% of curves were left sided.31 Labelle and col-
leagues found that 28% were nonprogressive, and one third of
cases progressed to more than 60. As there was no correlation
between curve progression and the severity of muscle weakness,
they postulated that the scoliosis was due to ataxia and distur-
bance in equilibrium. They also found that curve progression
was more likely with an early age at diagnosis of both the dis-
ease and the scoliosis.26 Surgical intervention is considered for
curves exceeding 40 to 60.26 Ambulatory patients with
Friedreichs ataxia have curve patterns similar to idiopathic
scoliosis and are treated using the same principles. Treatment
of scoliosis with segmental spinal instrumentation has been
shown to be effective and yield good results.26,31 Milbrandt
et al31 recommend fusion to the pelvis in patients with pelvic
obliquity. Their results demonstrated a loss of correction after
posterior spinal instrumentation (51% to 39%), particularly in
lumbar curvatures. The role of neurophysiologic monitoring in
this condition is unclear, as studies have largely shown the
inability to achieve adequate potentials.31 The need for an
intraoperative wake-up test must be anticipated and discussed
with the patient and family preoperatively.
PREOPERATIVE EVALUATION
A complete and detailed history and physical examination are
required, including an assessment of the patients functional
level. The orthopedic assessment focuses on the degree of
weakness, the presence and magnitude of coexisting contrac-
tures in the upper and/or lower extremities, and a detailed
spinal examination including the location (and flexibility) of
the curvature, the coronal and sagittal spinal balance, the
degree of pelvic obliquity, and the overall sitting balance. When
evaluating pelvic obliquity, one needs to examine the hip. In
the sitting position, a hip flexion contracture creates an ante-
rior tilt of the pelvis, which is compensated for by an increase
8/26/11 5:44:21 PM
 1232 Section X Paralytic Deformity
in lumbar lordosis. Contracture of the hamstring muscles (pos-
terior pelvic tilt) is compensated for by a decrease in lumbar
lordosis. Patients with fixed infrapelvic obliquity are at risk for
sagittal decompensation when lumbar lordosis is corrected to
within the normal range following surgery. Such imbalances
must be anticipated preoperatively and may need to be
addressed postoperatively. Similarly, adduction or abduction
deformities (or a windswept hip deformity) must be docu-
mented, as positioning of the lower extremities may change
significantly with correction of the spinal deformity.
The patients overall function should be evaluated, focusing
not only on how the spinal deformity impacts function, but also
how the patient has adapted to the deformity. While correcting
the deformity may have a positive effect on seating balance,
there may be a negative effect on other activities such as walk-
ing and crawling. Minimal ambulators may lose the capacity to
walk following spinal fusion. It is important to plan for aggres-
sive postoperative physical and occupational therapy to maxi-
mize function.
An assessment of nutritional status is extremely important,
as many of these children may be malnourished or undernour-
ished (often due to problems such as gastroesophageal reflux),
predisposing them to complications such as infection or wound
dehiscence. Methods to improve nutritional status preopera-
tively include enhancing oral intake, placement of a temporary
feeding tube, or even insertion of a gastrostomy tube. We rou-
tinely ask for a nutritional consult preoperatively.
A pulmonary consultation is also advised in patients with
DMD and SMA. Although recent evidence suggests that it is
possible to perform surgery safely even if the FVC is between
20% and 30%,16 surgical intervention will ideally be performed
prior to the development of significant pulmonary dysfunction.
Pulmonary complications have been correlated with preopera-
tive pulmonary function.34 Yuan et al48 showed that a forced
expiratory volume at 1 second (FEV1) of less than 40% pre-
dicted, vital capacity (VC) of less than 60% predicted, inspira-
tory capacity (IC) of less than 30 mL/kg, total lung capacity
(TLC) of less than 60% predicted, maximal inspiratory pres-
sure (MIP) of less than 60 cm H2O, a diagnosis of neuromuscu-
lar disease, and older age all correlated with the need for pro-
longed postoperative pulmonary ventilation and pulmonary
toilet. Padman and McNamara34 also showed that pulmonary
complications developed in patients with VC below 50%. A pre-
operative tracheostomy may be considered for selected patients
with a limited ability to expectorate, to maximize pulmonary
toilette. Early mobilization, chest physiotherapy, and noninva-
sive methods to assist ventilation (continuous positive airway
pressure [CPAP], bilevel positive airway pressure [BIPAP]) may
reduce the risk of pulmonary complications. Despite treatment
of the deformity, pulmonary function will continue to deterio-
rate because of progressive weakness associated with the under-
lying condition, although there is some evidence to suggest
that spinal fusion might slow the pulmonary decline.44 A car-
diac evaluation is suggested for patients with dystrophinopa-
thies and Friedreichs ataxia.
With regard to imaging, upright (standing or sitting) poster-
oanterior (PA) and lateral radiographs are obtained, in addi-
tion to bending films. In addition to determining curve magni-
tude, a careful evaluation of coronal and sagittal balance is
required. The flexibility of the spine may be assessed by bend-
ing films, and techniques include side bending, bolster bend-
ing, traction films, and push prone films. A supine traction film
LWBK836_Ch114_p1229-1243.indd 1232
may be used to evaluate the relationship between the trunk and
the pelvis, as a major goal is to achieve balance between the
trunk and pelvis rather than absolute curve correction. Bending
films may also be used to follow curve flexibility during routine
clinic visits in selected patients whose curvatures have met the
indications for surgical intervention, but in whom a delay in
surgery might be advisable due to age, nutritional status, or the
treatment of coexisting medical problems.
MANAGEMENT
NONOPERATIVE MANAGEMENT
Observation alone is recommended for small curves and when
there is no functional impairment. While bracing does not
alter the natural history, even when used prophylactically,
orthoses may improve sitting balance and may possibly delay
curve progression.13,31 Delaying the need for definitive arthro-
desis facilitates growth of the chest wall and maturation of the
lungs. Rigid orthoses are generally not well tolerated in this
patient population, and concerns include further compromis-
ing pulmonary function by restriction of chest movement, cre-
ating a chest wall deformity (especially in SMA), and skin
irritation or breakdown. As such, when bracing is contem-
plated, a soft spinal orthosis is typically recommended. The
brace is worn only when upright and left off during sleep.
Bracing is rarely considered in the treatment of scoliosis
associated with DMD, as delaying definitive surgical treatment
increases the risks of complications. A soft spinal orthosis may
be considered for the limited number of patients who cannot
be treated surgically due to prohibitive surgical risk or the
desires of the family. Patients with SMA commonly have early
onset of scoliosis, and delaying definitive surgical stabilization
is desirable. A soft spinal orthosis is commonly recommended
in this situation, prior to considering surgical treatment (grow-
ing rods, VEPTR, definitive fusion). In Friedreichs ataxia, the
treatment principles are similar to those of idiopathic scoliosis,
and standard orthoses are utilized in ambulatory patients.
While bracing is rarely successful in stabilizing progressive cur-
vatures,13,31 surgical intervention may often be delayed.
OPERATIVE MANAGEMENT
The goals of surgical treatment are to arrest curve progression,
to improve sitting balance, and to preserve or enhance overall
function, ease of care, and pulmonary function. The treatment
should achieve a well-balanced spine over a level pelvis. In gen-
eral, curves in patients with flaccid weakness or paralysis that
have progressed beyond 40 to 50 will continue to progress
even after skeletal maturity, and surgical stabilization should be
offered. Spinal fusion should be delayed until at least 6 to
7 years of age to allow for maturation of the lungs, and ideally
into adolescence to maximize trunk height.
The timing of intervention depends on the underlying diag-
nosis. Patients with DMD may be offered definitive treatment
for curves greater than 20 to 30, given the natural history and
the anticipated progressive decline in pulmonary function.
Also, with time the muscle is replaced by fibrofatty infiltration
that increases the stiffness of the deformity. SMA is commonly
associated with an early onset scoliosis, and surgery is required
when nonoperative measures fail to control the deformity. An
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 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis
Paralaytic scoliosis
PA and lateral spine radiographs
Deformity <40 degrees Deformity > 4060 degrees
Observe Younger age (inadequate
lung development and
trunk height)
Delay definitive fusion
Orthosis Growing rods
Serial casting VEPTR
followed by orthosis
Figure 114.1. Algorithm for treatment of flaccid neuromuscular scoliosis.
algorithm for the treatment of flaccid neuromuscular scoliosis
is shown in Figure 114.1.
POSTERIOR PROCEDURES
An instrumented posterior spinal fusion is the most common
procedure for neuromuscular scoliosis. Important principles in
treating patients with flaccid neuromuscular diseases include
segmental fixation, and performing long fusions (T2 to the
sacrum or pelvis) to correct pelvic obliquity and avoid progres-
sion of deformity above or below the instrumented segments in
the nonambulatory population. While the techniques and
implants continue to evolve, the ideal fixation device provides
sufficient rigidity to maintain correction and enhance arthrod-
esis, while having a low profile to avoid implant prominence
and possible soft tissue complications. Osteopenia is common
in this population, and segmental fixation is required. The
instrumentation typically extends across the lumbosacral joint
(to control pelvic obliquity), either to the sacrum or to the pel-
vis. Bone graft materials include autograft (harvested locally),
freeze-dried cancellous allograft, and often bone graft substi-
tutes. Although the basic concepts and principles have not
changed, newer strategies using the latest generation implants
have enhanced correction and have reduced the rates of pseu-
darthrosis. A better understanding of the natural history and
medical risks (appropriate timing and preparation for surgery)
has decreased the rate of complications. Newer strategies for
LWBK836_Ch114_p1229-1243.indd 1233
1233
Older age (adequate lung
development and trunk height)
Definitive spinal fusion
Bending spine radiographs to document flexibility
Ambulatory Non ambulatory
Fuse to neutral + Pelvic obliquity Pelvic obliquity
vertebrae
Fuse to pelvis Fuse to lower lumbar
spine in DMD spine
addressing early onset scoliosis, such as growing rods or the
VEPTR, have broadened the options available for this challeng-
ing patient population. Much of the literature has concerned
mixed populations of neuromuscular patients, and many of the
papers concerning flaccid paralysis focused on older genera-
tion implant systems. Our anecdotal impression as that the risk
of complications has lessened considerably with aggressive
medical management and the current surgical techniques.
The most common surgical approach in patients with a flac-
cid neuromuscular disease has been instrumentation and
fusion across the lumbosacral junction using the Luque
Galveston technique or the unit rod. Other choices for achiev-
ing distal fixation include the use of iliac screws and/or sacral
pedicle screws. The unit rod is a precontoured implant, and
the technique may be viewed as a variant of the Luque
Galveston procedure (Figs. 114.2AD).6 The technique involves
first placing the pelvic limbs of the rod into the ilium, and the
spine deformity and pelvic obliquity are then corrected using
cantilever bending forces while sequentially tightening the sub-
laminar wires to the rods. Excellent results have been reported
by multiple authors, with curve correction rates of 54% to
81%.6,28,46 Insertion of the unit rod may be difficult in the set-
ting of lumbar hyperlordosis, and in such cases the implant
may be cut at the level of the midlumbar spine. After inserting
each of the pelvis limbs separately, the upper segment of the
unit rod can be linked to the lower rods (Fig. 114.3) prior to
correction. Excellent corrections of both the curvature and
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 1234 Section X Paralytic Deformity

A B C

Figure 114.2. (AD) This 17-year-old boy with Duchenne muscular dystro-
phy developed a progressive curvature (A and B) and was treated by a poste-
D
rior spinal fusion with unit rod instrumentation.

pelvic obliquity have been reported with variants of the Luque
Galveston technique, including the use of intrailiac screws (one
or two on each side) and iliosacral screws, with or without lum-
bar pedicle screws. These modifications attempt to improve the
quality of fixation across the lumbosacral junction.
While recognizing that extension of the instrumentation
and fusion across the lumbosacral joint is required in most
nonambulatory patients, complications are more frequent and
include fixation failure and/or pseudarthrosis, implant promi-
nence/skin irritation, and pelvic pain. In an effort to avoid
crossing the sacroiliac joint, one option in patients with more
flexible curves and minimal pelvic obliquity is to place the dis-
tal fixation in the sacrum rather than the pelvis. Anecdotally,
we have used this strategy in selected patients with relatively
flexible curves and no significant pelvic obliquity. We have uti-
lized a hybrid construct, with a base of pedicle screws extending
from the lumbar spine to S1, with sublaminar wire fixation in
the thoracic spine (Figs. 114.4AD). The intrailiac limbs of the

LWBK836_Ch114_p1229-1243.indd 1234 8/26/11 5:44:22 PM

 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis
A B
Figure 114.3. (A and B) For patients with significant lumbar
hyperlordosis, we often cut the unit rod at the level of the midlumbar
spine, and then insert each pelvic limb individually. The distal limbs
are then be linked to the proximal portion of the unit rod prior to
applying any corrective forces.
unit rod are removed, and the residual unit rod is placed into
the pedicle screws at the base of the construct. Once these have
been secured provisionally, and the lower lumbar spine is
squared off relative to the pelvis, a cantilever maneuver is then
applied to gradually place the rods into the remaining pedicle
screws, and then down to the thoracic spine, while sequentially
tightening the sublaminar wires. Segmental derotation can
then be performed in the lumbar spine, as well as compression
and distraction, to enhance correction. One potential concern
with pedicle screws in the osteopenic spine (especially the
sacrum) is inadequate screw purchase, especially with repetitive
flexion and extension loading, which might result in pull out.
We will often add a sublaminar wire or cable in the lower lum-
bar spine to resist such pullout forces. If sacral fixation is inad-
equate, or in the rare case in which a sacral screw pulls out
during the correction, then posterior iliac screws may be placed
and linked to the rods (Figs. 114.5AD). Our anecdotal impres-
sion is that the use of multiple lumbar screws affords load shar-
ing and protection for the sacral screws, although we have no
biomechanical data to corroborate this.
The placement of iliac/pelvic fixation requires a larger
exposure with longer operative times and greater blood loss.29,42
McCall and Hayes29 have compared treated selected cases of
neuromuscular scoliosis (L5 vertebrae had <15 of tilt) with a
U-rod construct including a pedicle screw anchor at L5, and
found similar outcomes to a group treated with unit rod fixa-
tion. Patients with DMD who have a small thoracic curve and
no significant pelvic obliquity may also be candidates for fusion
to L5, especially when the construct is anchored distally with
pedicle screws (Figs. 114.6AD).2,33
LWBK836_Ch114_p1229-1243.indd 1235
1235
Newer fusionless strategies have emerged that may poten-
tially play a role in the treatment of early onset neuromuscular
curves (especially SMA), including growing rod instrumenta-
tion and the VEPTR (Figs. 114.7AD). While the indications
are evolving as experience is gained, either method may
improve or preserve alignment while delaying the need for
definitive arthrodesis. Both require multiple procedures to
maintain the correction, usually at 6-month intervals. While the
growing rod procedure involves instrumenting the spine, the
VEPTR strategy facilitates indirect correction of the spine, with
distraction between the ribs and the pelvis or lower spine. At
this point, there is limited information on the long-term results
of these strategies in young patients with flaccid neuromuscular
disease. Hell et al20 reported on the VEPTR device in six chil-
dren with neuromuscular scoliosis and showed improvement in
sitting as well as in respiratory function.20 We typically recom-
mend bracing as the first option when addressing progressive
curvatures in patients who are too young to consider definitive
spinal fusion and will consider growing rod constructs or
VEPTR if bracing fails to control the deformity.
While most patients managed with modern implant systems
do not require a spinal orthosis postoperatively, bracing may be
considered. We have advised postoperative bracing to reduce
cyclic loads on the implants in ambulatory patients with
Friedreichs ataxia. We have observed several cases of implant
failure, typically screw fracture at the distal aspect of the instru-
mentation, and postulate that this is due to excessive trunk
motion during ambulation. We also offer a soft spinal orthosis
for comfort and in patients who have been instrumented to the
first sacral vertebra.
ANTERIOR PROCEDURES
Anterior spinal surgery has an extremely limited role in patients
with flaccid neuromuscular disease, given the increased pulmo-
nary risks associated with violation of the chest wall (and dia-
phragm). With an early diagnosis and appropriate timing of
intervention, this should rarely be required. Although an ante-
rior release and fusion might be considered for patients with
rigid curvatures in whom adequate balance cannot be achieved
with an instrumented posterior spinal fusion, consideration
should be given to performing posterior osteotomies (Ponte)
to improve curve flexibility and to avoid an anterior procedure.
If necessary, the anterior and posterior procedures may be per-
formed on the same day, or staged.
COMPLICATIONS
Complications following spinal fusion are frequent in patients
with neuromuscular scoliosis and may be grouped as medical
and surgical (Table 114.2). With regard to medical complica-
tions, the pulmonary system is at greatest risk due to preexist-
ing impairments including chest wall weakness/deformity and
a decreased capacity to clear secretions. Complications include
atelectasis, pneumonia, mucous plugging, and rarely the need
for prolonged mechanical ventilation. Aggressive pulmonary
care reduces the risk of such complications, and strategies
include early mobilization, chest physiotherapy, and the use of
noninvasive methods such as incentive spirometry if possible,
CPAP or BIPAP. Gastrointestinal complications include ileus,
and nutritional deficiency may increase the risk of infection
8/26/11 5:44:22 PM
 1236 Section X Paralytic Deformity

A B C

D E

Figure 114.4. (AE) Preoperative radiographs of a 17-year old boy with Duchenne muscular dystrophy
show a 73 thoracolumbar curvature (A) with 100 of kyphosis (B). Bending radiographs demonstrated a
scoliosis of 38 and a kyphosis of 51. He was treated by a posterior spinal fusion (PSF) from T2 to S1 with
hybrid instrumentation (C and D). The pelvic limbs are removed from the unit rod, and the remaining
portion is inserted into a base of pedicle screws ending at the first sacral vertebra. E demonstrates a postop-
erative anteroposterior radiograph following use of the same technique, in which the screws were placed up
to T11.

LWBK836_Ch114_p1229-1243.indd 1236 8/26/11 5:44:23 PM

 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis 1237

A B C

Figure 114.5. (AD) Posteroanterior and lateral radiographs (A and B) of 12-year-old boy with spi-
nal muscular atrophy presented with a progressive kyphoscoliotic deformity, with a right thoracolumbar
curvature measuring 90. During the instrumentation and correction, the S1 screw on the right side
pulled out and was salvaged with an iliac screw, and balance was maintained at 18 months follow-up
D
(C and D).

and wound dehiscence. Pre- and postoperative nutritional sup-
plementation should reduce these risks, and options include
oral supplementation, placement of a temporary feeding tube,
or placement of a gastrostomy tube. A short period of total
parenteral nutrition is considered when enteral feedings can-
not be started within the first few postoperative days. Cardiac
complications are also seen in patients with DMD and
Friedreichs ataxia, including arrhythmias and sudden cardiac
death, and appropriate monitoring is required.
Surgical complications are also frequent. Patients with
neuromuscular diseases, especially DMD, are prone to
excessive intraoperative bleeding. In addition to the use of

LWBK836_Ch114_p1229-1243.indd 1237 8/26/11 5:44:24 PM

 1238 Section X Paralytic Deformity

A B C

Figure 114.6. (AD) This 15-year-old boy with Duchenne muscular dystrophy devel-
D oped a 73 right thoracolumbar curve (A and B) without significant pelvic obliquity. He
underwent a T3 to L5 posterior spinal fusion with a base of lumbar pedicle screws.

intraoperative recovery or cell saver, antifibrinolytic agents
(aprotinin, tranexamic acid, and aminocaproic acid) may
also be considered.43 A recent systematic review of six clinical
trials demonstrated a reduction in blood loss and blood
transfused using these agents.43 We often perform a staged
exposure of the spine in an attempt to further reduce intra-
operative bleeding. The initial exposure is performed from
the lower thoracic spine to the sacrum, followed by place-
ment of lumbar pedicle screws and the distal anchors either
at S1 or the ilium. The lumbar wound is then packed and the
fascia secured with towel clips. We then expose the thoracic
spine and place sublaminar wires. The entire wound is then
opened for placement of the rods and correction of the
deformity.
Postoperative wound infections, superficial or deep, are
more frequent in the neuromuscular population. Persistent
wound drainage (or superficial dehiscence) may signify an
infection, early wound exploration should be considered to

LWBK836_Ch114_p1229-1243.indd 1238 8/26/11 5:44:25 PM

 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis 1239

A B C

Figure 114.7. (AD) This 6-year-old girl with a diagnosis of spinal muscular atrophy devel-
oped a rapidly progressive left thoracolumbar curvature (71) (A and B), and was treated by a
D
bilateral rib to pelvis vertical expandable prosthetic titanium rib (C and D).

obtain cultures and perform irrigation and debridement
(superficial or deep) depending upon the findings. For an
established infection during the early postoperative period,
one or more irrigation and debridements (and intravenous
antibiotics) with retention of the implants may control the
infection. In the case of a deep infection, we typically remove
all of the bone graft material. A vacuum-assisted wound closure
device (VAC) should be considered postoperatively and may
limit the number of subsequent operative procedures.
The loss of spine fixation can occur because the common
finding of generalized osteopenia makes achieving stable fixa-
tion more difficult. Failure at the implant spine interface (acute
or delayed) may lead to a loss of fixation, migration of the
implants, pseudarthrosis, and/or progression of the deformity.
Progression of deformity above or below the instrumented seg-
ments may be prevented by instrumenting from T2 or T3 to the
pelvis. The use of a postoperative orthosis may decrease the
likelihood of implant failure.

LWBK836_Ch114_p1229-1243.indd 1239 8/26/11 5:44:26 PM

 1240
TABLE 114.2 Complications Associated with Flaccid Neuromuscular Scoliosis

Mean

LWBK836_Ch114_p1229-1243.indd 1240
Blood Infection/ Deformity
Loss Wound Progression/
Authors Year Condition Cases Instrumentation (mL) Healing Cardiopulmonary Pseudoarthrosis Recurrence Mortality Others
Aprin et al4 1982 SMA 22 1 No 10/22 45% 1/22 4.5% 2/22 9%
instrumentation Atelectasis and Recurrence of
15 Harrington pneumonitis pelvic
6 Dwyer obliquity 2/22
9% kyphotic
deformity
Milbrandt 2008 Friedreichs 49 (16/49 10/16 All hooks 1268 1/16 6.25% 1/16 6.25% sup. 1/16 6.25%
et al31 ataxia treated 4/16 Luque rods Respiratory Junctional Implant failure
surgically) 1/16 Hooks and distress kyphosis
screws 1/16 6.25%
1/16 Harrington Curve
rod progression
Heller 2001 DMD 31 Isola 3373 5/31 17% 4/31 13% 1/31 3% 4/31 13%
et al21 Abdominal
1/31 3%
Thrombosis
1/31 3% Bleeding
2/31 6% Hook
dislocation
Brook 1996 DMD 17 10/17 L-rod 2/17 12% 6/10 30% 1/10 10% (L-rod)
et al9 7/17 L-rod with (One in (L-rod) antibiotic-
Galveston each deformity induced colitis
group) progression 1/7 14% Sacral
or sitting nerve root
imbalance dysesthesia
Phillips 1990 SMA 10 Cases 4/10 Harrington 1/10 10% 1/10 10% 1/9 10% 1/9 10% Painful
et al35 5/10 Luque Respiratory Progression implants
1/10 Cotrel compromise (case with CD
Dubousset without
1/10 Dwyer fusion)
(1 Luque and
Dwyer
performed for
revision of one
Harrington rod)
Granata 1996 DMD 30 22/30 Luque 1/30 3% Painful 1/30 3% 1/30 3% Sacral
et al18 1/30 CD prominent pressure sore
7/30 Hartshill implants

8/26/11 5:44:26 PM
 Piasecki 1986 SMA 19 2/19 Anterior/ 4/19 21% 5/19 26% 4/19 21% Urinary
et al36 posterior Dwyer 4 Pneumonia tract infection
and Harrington 1 Respiratory 2/19 11% Pressure
17/19 Harrington arrest sore
(14 fused to 2/19 11%
sacrum) Neurologic
1/19 5% Hepatitis

LWBK836_Ch114_p1229-1243.indd 1241
1/19 5%
Sacroiliitis
2/19 11% Motion
(Masseter
contracture and
decreased
shoulder
motion)
Ramirez 1997 DMD 30 23/30 Luque 2500 4/30 13% 3/30 10% 1/30 3% 3/30 10% 1/30 3% Expired 3/30 10% urinary
et al37 6/30 CD 1 Intraoperative (2 hardware 6 weeks tract infection
instrumentation decompensation failure, 1 postoperatively 1/30 3% Delayed
1/30 Texas 1 Pneumonia broken rod) wound healing
Scottish rite 1 Syncopal
hospital episodes
instrumentation
(20 fused to pelvis,
10 to L5)
Sengupta 2002 DMD 50 31/50 Luque 4100 Grp 1 Grp 1
et al40 fused to (grp 1) 2/31 6.5% 4/31 13%
sacropelvis grp 1 3300 Grp 2 2 Developed
19/50 Luque with (grp 2) 1/19 5% prominent
lumbar pedicle painful
screws fused to hardware
L5 grp 2 proximally, 1
loosening of
Galveston, 1
loosening and
removal of
sacral screw
Grp 2
1/19 5%
Loosening of
proximal
fixation

(continued)

1241

8/26/11 5:44:26 PM
 TABLE 114.2 Complications Associated with Flaccid Neuromuscular Scoliosis (Continued)

1242
Mean
Blood Infection/ Deformity
Loss Wound Progression/
Authors Year Condition Cases Instrumentation (mL) Healing Cardiopulmonary Pseudoarthrosis Recurrence Mortality Others

LWBK836_Ch114_p1229-1243.indd 1242
Bentley 2001 DMD/SMA 64 DMD 14 Harrington 3034 6/101 6% 16/101 16% 9/101 9% 1/101 1% Colonic
et al7 33 SMA 87 Luque DMD 3 Pneumothorax 2 Protruding perforation
4 CMD 12 Chest infection rods 28/101 28%
1 Ventilation 4 Fusion Paralytic ileus
1/101 1% Cardiac extended to 62/101 62%
arrest pelvis Neurologic
perioperatively 2 Broken rods 39 Trunk
1 Prominent hyperesthesia
wires 10 Distal numbness
13/101 13% with or without
Seating weakness
problems, hip 13 Bladder
pain dysfunction
Evans 1981 SMA 11 7 Harrington 2/11 18% 6/11 55% 3/11 27% Loss of
et al14 4 Harrington with Reexploration function
sacral bar
Cervellati 2004 DMD 20/58 19 Modified 1200 3/20 15% 1/20 5% 1/20 5%
et al11 Luque Painful Cardiac arrest Sacral decubitus
technique prolapse of probable 1/20 5%
1 CD horizontal hemodilution Neurogenic
part of the bladder
rod from the
pelvis
Schwentker 1976 SMA 9 8 Harrington 1 1/9 11% 4/9 Progression 7/9 78%
and Dwyer Decreased of deformity Decreased
Gibson39 function 1 Progressed function
above, below, postoperatively
and within 1/9 11%
the fusion Neurologic loss
mass of left arm
2 Progressed function
above and
below
1 Progressed
above the
fusion

DMD, Duchennes muscular dystrophy; SMA, spinal muscular atrophy.

8/26/11 5:44:27 PM
 Chapter 114 Surgical Treatment of Flaccid Neuromuscular Scoliosis
SUMMARY
The treatment of scoliosis in patients with flaccid neuromuscu-
lar paralysis is challenging, and complications are frequent.
While nonoperative measures will not alter the natural history
for the vast majority of patients, bracing with a soft spinal ortho-
sis may improve function and possibly delay progression of the
deformity. Progressive deformities are definitively treated by an
instrumented spinal fusion, which is ideally performed after
the lungs have matured and trunk growth has been maximized.
Newer strategies are evolving to address progressive curvatures
in juvenile patients, to delay the need for definitive spinal
fusion. Medical comorbidities must be identified and addressed
to minimize the risk of perioperative complications. This usu-
ally requires preoperative consultation with multiple services
(pulmonary, gastroenterology/nutritional services, cardiol-
ogy), and aggressive pulmonary care and nutritional supple-
mentation are required during the postoperative period. The
goal of surgery is a well-balanced spine while avoiding compli-
cations and is best achieved by an instrumented posterior spi-
nal fusion from T2 to the pelvis.
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