Idiopathic
Scoliosis
History of the
Procedure
Scoliosis is an
ancient disease that remains incompletely understood
despite a collective medical experience that approaches
4000 years. This is a sad commentary on the learning
curve of medical practitioners. Nevertheless, the
history of the recognition and treatment of scoliosis is
rich with important lessons for the modern
practitioner.
Ancient Hindu religious literature (circa 3500-1800
BC) describes the treatment of spinal deformity rather
clearly. The story is told of a woman who was "deformed
in three places" and how Lord Krishna straightened her
back.3 This was
accomplished by pressing down on her feet and pulling up
on her chin. The orthopedic trappings of the story are
unmistakable, including excellent immediate
posttreatment results and no long-term follow-up.
Hippocrates (circa 400 BC) stated, "there are many
varieties of curvature of the spine even in persons who
are in good health; for it takes place from natural
conformation and from habit." He also stated that
"lateral curvatures also occur, the proximate cause of
which is the attitudes in which these patients
lie."4 The postural and
muscular theory of scoliosis thus stated has persisted
for thousands of years and remains firmly embraced by
some.
Hippocratic scoliosis treatment methods focused
primarily on spinal manipulation and traction.5 He used an elaborate
traction table called the scamnum. Medical practitioners
used slight variations of the Hippocratic scamnum well
into the 1500s. Another treatment approach that
Hippocrates discussed involved attempting to diminish
spinal deformity with a method called succussion. This
involved strapping the patient (often upside down) to a
ladder, which was then hoisted into the air and dropped
from a height. Hippocrates thought that this method was
occasionally useful, but it was largely performed by
charlatans to impress the public.6
Ambroise Pare has been described as the "most
celebrated surgeon of the Renaissance."7 Pare is recognized
as the first physician to treat scoliosis with a brace.
He also recognized that once a patient with scoliosis
had reached maturity, bracing was not useful. Pare's
orthosis consisted of a metal corset (fashioned in a
village smithy setting) with many holes in it to help
diminish its significant weight. The record also makes
it quite clear that Ambroise Pare espoused the postural
theory of scoliosis.
Nicholas Andry was a French pediatrician who hated
the brutal barber surgeons of his day.8. At the age of 83 (a
year before his death) he wrote a short book entitled
Orthopaedia. Thus, in 1741 this name combined
the root words for straight (orthos) and child
(paedia) to create the name still used for the
broad musculoskeletal field, orthopedics.
Andry
believed that scoliosis was caused by asymmetric muscle
tightness and, thus, helped foster the French belief in
"convulsive muscular contraction" as the cause of spinal
deformity.6 Andry stated, "It is
well worth while to remark that the crookedness of the
spine does not always proceed from a fault of the spine
itself, but is sometimes owing to muscles of the
forepart of the body being too short, whereby the spine
is rendered crooked, just in the same manner as a bow is
made more crooked by tying its cord tighter.9 Andry used rest,
suspension, postural approaches, and padded corsets in
his treatment of scoliosis.
Jacques Mathieu Delpech was a successful and skilled
surgeon, yet he focused a great deal of his attention on
nonsurgical approaches to orthopedic problems. The
highlight of this focus was his orthopedic institute at
Montpellier, in the south of France. This facility
included elaborate gardens, a heated winter gymnasium,
and an outdoor gymnasium for the treatment of various
musculoskeletal problems.
For the treatment of scoliosis, Delpech devised
graded exercises for strengthening muscles of the trunk
in the belief that the deformity was due to a weak axial
musculature. This belief was almost certainly due to the
influence of Andry. Delpech also used stretching and
traction techniques but did not believe in braces. His
patients usually stayed for 1 or 2 years at the
institute, and they would wear uniforms while they
performed their exercises. Similar elaborate
efforts to treat scoliosis still exist in the physical
therapy outpatient setting.10 Delpech's life and
that of his institute came to an abrupt end in 1832 when
a disgruntled patient shot him to death as he was riding
back to Montpellier in an open carriage.4
An important event of the 1800s was the advent of
surgical treatment of scoliosis by the French orthopedic
surgeon Jules Guerin. He was very enthusiastic about
subcutaneous tenotomy and myotomy and first reported
their use in his patients with scoliosis in 1839. When
he later published the results of treatment of 1,349
patients with this technique, tremendous controversy was
ignited.4 Guerin's harshest
critic was Joseph Malgaigne, who described Guerin's work
as "some orthopedic illusion."4 This led to one of
the most famous orthopedic lawsuits in history: Guerin
versus Malgaigne. This defamation trial ended in
Malgaigne's favor and helped to establish an important
precedent for open criticism of scientific papers.
Another important tool in the treatment of scoliosis
was the plaster body jacket (ie, body cast). The
American orthopedic surgeon Lewis Sayre popularized its
use in the mid 1800s. Sayre's technique involved a large
tripod that allowed the patient to be suspended while
the corrective plaster cast was applied. Sayre was said
to be "a brusque, forceful and therefore controversial
personality" but also "an eloquent speaker" who toured
internationally demonstrating his casting
techniques.6 He also used a "jury
mast" extension from some of his casts in order to
provide constant head traction—a clear predecessor to
halo traction.
The early 1900s saw what is arguably the most
important advance in scoliosis treatment in more than
3000 years: posterior spinal fusion. Russell Hibbs first
performed his "fusion operation" for tuberculous spinal
deformity in 1911, but by 1914 he also was applying his
technique to patients with scoliosis.11 The Hibbs approach
focused on achieving maximum deformity correction via a
variety of plaster jackets before surgery. Hibbs's 1924
description of his own technique is eloquent, as
follows:
The dissection is carried farther and
farther forward upon each vertebra in turn, until the
spinous processes, the posterior surfaces of the
laminae, and the base of the transverse processes are
bared...[and] with a bone gouge, a substantial piece
of bone is elevated from the adjacent edges of each
lamina, of half its thickness and of half its width.
The free end of the piece from above is turned down to
make contact with the lamina below, and the free end
of the piece from the lamina below is turned up to
make contact with the lamina above...Each spinous
process is then partially divided with bone forceps
and broken down, forcing the tip to come into contact
with the bare bone of the vertebra
below.
In the postoperative period, Hibbs typically allowed
2 weeks of bedrest for wound healing, followed by a
final traction plaster jacket. The patient would
continue to be confined to bed while wearing the
corrective cast for another 6 weeks. Following this, the
patient would wear a removable brace during the day for
an additional 6-12 months. It was clear to Hibbs that
with his technique, he could at least partially correct
and, more important than this, prevent progression of
the curves he was treating.
By 1941, such spinal fusion operations for idiopathic
scoliosis were common enough that Shands (of the Alfred
I duPont Institute) and his fellow researchers could
assess more than 400 cases.12 Hibbs-type fusion
procedures were performed in all cases, but most
surgeons (60%) used supplemental bone graft (often from
the tibia). An approximately 25% final curve correction
was achieved, and an overall 28% pseudarthrosis rate was
noted.12 It would be
another 20 years before Paul Harrington would introduce
the spinal instrumentation system that would further
refine scoliosis surgery.13 Although
Harrington's original concept was instrumentation
without fusion, persons such as John Moe would convince
him of the value of spinal fusion in concert with
Harrington rods.14
Further refinement in surgical technique and
instrumentation has led to the greater than 50%
correction and single-digit pseudarthrosis rates to
which contemporary orthopedists have become
accustomed.
Problem
Scoliosis represents a disturbance of an otherwise
well-organized 25-member intercalated series of spinal
segments. It is, at times, grossly oversimplified as
mere lateral deviation of the spine, when in reality, it
is a complex 3-dimensional deformity.15, 16 In fact, some have
used the term rotoscoliosis to help emphasize this very
point. Two-dimensional imaging systems (plain
radiographs) remain somewhat limiting, and scoliosis is
commonly defined as greater than 10° of lateral
deviation of the spine from its central axis.
In the past, terminology such as kyphoscoliosis was
inappropriately used to describe certain patients with
idiopathic scoliosis. Idiopathic scoliosis has a strong
tendency to flatten the normal kyphosis of the thoracic
spine.17 Robert Winter teaches
that idiopathic scoliosis is a hypokyphotic
disease.18, 19 In most cases,
diagnoses of kyphoscoliosis were clinical
misinterpretations of the rib hump associated with an
otherwise hypokyphotic thoracic spine. Idiopathic
scoliosis may present as a true kyphoscoliosis, but this
occurs relatively rarely.
J.I.P. James is credited with classifying idiopathic
scoliosis according to the age of the patient at the
time of diagnosis.20 Using his
classification system, children diagnosed when they are
younger than 3 years have infantile idiopathic
scoliosis. Children diagnosed when they are aged 3-10
years have juvenile idiopathic scoliosis, and those
older than 10 years have adolescent idiopathic
scoliosis. These age distinctions, though seemingly
arbitrary, have prognostic significance. For instance,
Robinson and McMaster reviewed 109 patients with
juvenile idiopathic scoliosis and found that nearly 90%
of curves progressed, and almost 70% of these patients
went on to require surgery.21 These rates are
much higher than the rates associated with other
categories of idiopathic scoliosis. The real challenge
is to predict which curves will progress significantly
and which ones will not.22 This is discussed
in greater detail later in this article.
Frequency
Scoliosis is almost always
discussed in terms of its prevalence (ie, the total
number of existing cases within a defined population at
risk). Rates may vary quite significantly based on what
particular definition of scoliosis is used and what
patient population is being studied. Several important
studies are included below.
Stirling and his coauthors studied almost 16,000
patients aged 6-14 years in England and found the point
prevalence of idiopathic scoliosis (Cobb angle >10°)
to be 0.5% (76 of 15,799 patients).23 The prevalence of
scoliosis was highest (1.2%) in patients aged 12-14
years.23 Data such as these
have helped reiterate the idea that the focus of
screening efforts should be on children in this age
group. When smaller Cobb angle measurements have been
accepted (eg, 6° or greater), a significantly higher
scoliotic rate may be identified, such as the 4.5% rate
reported by Rogala et al.24 Other studies using
the 10° definition of scoliosis have placed the overall
prevalence in the 1.9-3.0% range.25
Scoliosis has been suggested to develop more
frequently in children born to mothers who are aged 27
years or older.26 One might
hypothesize that gene fragility might be involved (eg,
higher rate of infants with Down
syndrome born to older mothers). The precise
explanation as to why this might be the case has not
been elucidated. In addition to this, no other authors
have duplicated these results.
As mentioned previously, most patients with
idiopathic scoliosis are female, and the vast majority
of research has focused on females. One of the only
articles written on idiopathic scoliosis in males is
that by Karol et al, from the Texas Scottish Rite
Hospital. These authors showed that boys with scoliosis
are at risk for curve progression for a longer period
than girls. They also suggested that efforts to screen
for boys with scoliosis should be performed a little
later than similar screenings for girls.27
Etiology
The precise etiology of idiopathic scoliosis remains
unknown, but several intriguing research avenues exist.
A primary muscle disorder has been postulated as a
possible etiology of idiopathic scoliosis. The
contractile proteins of platelets resemble those of
skeletal muscle, and calmodulin is an important mediator
of calcium-induced contractility. Kindsfater and his
colleagues from Denver studied the level of platelet
calmodulin in 27 patients with adolescent idiopathic
scoliosis.28 Using indirect
measurement methods, these researchers had conducted
previous work indicating that increased levels of
platelet calmodulin were associated with increasingly
severe idiopathic scoliosis. Using a direct measurement
technique, they showed that patients with a progressive
curve (>10° progression) had statistically higher
platelet calmodulin levels (3.83 ng/mcg vs 0.60 ng/mcg,
P <.01).28 If these data are
reproduced in larger studies, they hold the potential to
allow clinicians to identify patients at higher risk of
curve progression.
An elastic fiber system defect (abnormal fibrillin
metabolism) has been offered as one potential etiologic
explanation for idiopathic scoliosis.29 Such abnormal
connective tissue has not been found universally in
patients with idiopathic scoliosis. No clear
cause-and-effect relationship has been established.
Further research in this area is clearly warranted.
Disorganized skeletal growth, probably with its root
cause at a gene locus or group of loci, has been
discussed as a possible etiologic explanation for
idiopathic scoliosis. This theory is simply that a
rather localized primary growth dysplasia leads to a
cascading Hueter-Volkmann effect on a much larger
portion of the spine.30 The Hueter-Volkmann
principle states that compressive forces tend to stunt
skeletal growth and that distractive forces tend to
accelerate skeletal growth. A possible, yet unproven,
association with such a growth disturbance is the
osteopenia that has been identified in patients with
idiopathic scoliosis.31
David Aronsson has conducted a series of experiments
that have explored this mechanical modulation of growth.
Using two different animal models (rats and calves), he
showed that the force exerted by external ring fixators
were quite capable of producing vertebral segment
wedging akin to that seen in human idiopathic
scoliosis.32, 33 Correlation of his
laboratory information with the clinical setting has
drawn attention to the fact that wedging occurs both
from the vertebral bodies themselves and from the disk
spaces, with a greater amount of thoracic wedging coming
from the vertebral bodies.34 The asymmetric
mechanical forces have also been associated with
elevated synthetic activity in the convex side of
scoliotic curves.35
Bylski-Austrow and Wall led a group of Cincinnati
Children's Hospital researchers who further analyzed the
mechanical modulation of spinal growth. Using a porcine
model, they successfully induced growth changes by means
of an endoscopically implanted spinal staple.36, 37, 38 Within the context
of 8 weeks' follow-up, they were able to create 35-40°
of scoliotic curvature in growing pigs. Histologic
analysis of vertebral specimens revealed increased
paraphyseal density and disorganized chondrocyte
development in the region of the staple blades.
Genetic roots of the disease referred to as
idiopathic scoliosis have been rather strongly suggested
by several avenues of research. An X-linked inheritance
pattern (with variable penetrance and heterogeneity) has
been suggested by several authors.39 Studies of
twins with scoliosis have pointed in a similar
direction.40, 41. More than 90% of
monozygotic twins and more than 60% of dizygotic twins
demonstrate concordance regarding their idiopathic
scoliosis.40 Some evidence has
also directed attention to portions of chromosomes 6,
10, and 18 as possible scoliosis-related loci.42
Pathophysiology
Much has been written regarding the potential
influence of melatonin on the development of idiopathic
scoliosis.43 This has largely
originated from studies in which the pineal gland was
removed in chickens and scoliosis developed. These same
studies suggested that the melatonin deficiency
following pinealectomy might be the underlying reason
for the development of scoliosis. Bagnall and his
coauthors studied pinealectomized chickens to which they
administered therapeutic doses of melatonin.44 They were unable to
demonstrate any ability of the melatonin to prevent the
development of scoliosis. It is fair to say that no
final answer is yet available.
Some authors have suggested that a posterior column
lesion within the central nervous system might be
present in patients who have idiopathic
scoliosis.45, 46 Such central
nervous system dysfunction was hypothesized to be
manifested as decreased vibratory sensation. McInnes and
her fellow researchers later pointed out that the
vibration device used in earlier studies (a
Bio-Thesiometer) did not demonstrate sufficient
reliability characteristics to allow valid
conclusions.47 This line of
research might be attractive to those who feel that a
postural disturbance is the root cause of scoliosis.
Clinical
The vast majority of patients initially present due
to perceived deformity. This may be patient
or family perception of asymmetry about the shoulders,
waist, or rib cage. A primary care physician or
school-screening nurse may perceive similar findings.
Adams forward-bending test (in conjunction with the use
of a scoliometer) has been found to be an effective
screening tool.
Highlights of the patient's history include
information relative to other family members with spinal
deformity, assessment of physiologic maturity (eg,
menarche), and presence or absence of pain.
Traditionally, scoliosis has been described as a
nonpainful condition, and aggressive workup has been
recommended for patients in whom this rule is
violated.48 Ramirez and his
coworkers from the Texas Scottish Rite Hospital studied
more than 2400 patients with scoliosis and found that a
full 23% (560 of 2442 patients) had back pain at the
time of presentation.49 An underlying
pathologic condition was identified in 9% (48 of 560) of
the patients with back pain, including mainly
spondylolysis and spondylolisthesis but also intraspinal
tumor in one instance. Thus, it would seem that pain is
not associated with scoliosis as rarely as previously
thought.
Physical examination should include a baseline
assessment of posture and body contour. Shoulder
unleveling and protruding scapulae are common. In the
most common curve pattern (right thoracic), the right
shoulder is consistently rotated forward and the medial
border of the right scapula protrudes posteriorly.
Assessment of lower (and often upper) extremity reflexes
should be performed. Abdominal reflex patterns should
also be assessed. The presence or absence of hamstring
tightness should be investigated, and screening should
be performed for ataxia and/or poor balance or
proprioception (ie, Romberg test). One or two different
methods of measuring leg length will prove valuable, as
a significant percentage of patients presenting with
scoliosis have several centimeters of limb-length
discrepancy.
An extensive yet incomplete understanding of the
natural history of idiopathic scoliosis remains a
reality. Thus, more than a modicum of uncertainty
remains associated with selection of recommended
treatments for idiopathic scoliosis. The main treatment
options for idiopathic scoliosis may be summarized as
"the 3 O's": (1) observation, (2) orthosis, and (3)
operative intervention. When to choose each of these
treatments is a complicated matter.
The risk of curve progression varies based on the
idiopathic scoliosis group in which a patient belongs
(ie, infantile, juvenile, adolescent).
Infantile idiopathic scoliosis
Although defined by a seemingly arbitrary age limit
(<3 y at the time of diagnosis), infantile idiopathic scoliosis
demonstrates marked differences that distinguish it from
the other 2 categories of idiopathic scoliosis.
Infantile idiopathic scoliosis is the only type of
idiopathic scoliosis whose most common curve pattern is
left thoracic. Infantile idiopathic scoliosis is the
only type of scoliosis that is more common in boys. It
is more common in European patients or those of
immediate European descent. In the past, infantile
idiopathic scoliosis may have constituted up to 41% of
all idiopathic scoliosis cases in parts of Europe,
but the rate would appear to be closer to 4%. This
is still dramatically higher than the estimated 0.5%
rate in North America.50
Infantile idiopathic scoliosis is also the only type
of idiopathic scoliosis with any significant reputation
for spontaneous resolution. Reported spontaneous
resolution rates range from 20-92%.20, 51 Ceballos et al
studied 113 Spanish patients with infantile idiopathic
scoliosis. They found a 92% rate of associated
plagiocephaly and an almost 25% rate of congenital hip
dysplasia.52 These same
researchers found that nearly 74% of their patients'
curves were of the resolving variety (mainly left
thoracic curves) and the other 26% were progressive
curves (mainly double primary type curves).52
Prediction of curve progression in infantile
idiopathic scoliosis has been tied to assessment of the
rib vertebral angle difference (RVAD) originally
described by Mehta in 1972.53 As described by
Mehta, this measurement is carried out at the apical
vertebra of the curve. In instances in which the curves
resolved spontaneously, the RVAD was less than 20° in
about 80% of cases, and in those instances in which the
curves were progressive, the RVAD exceeded 20° in about
80% of cases.53 Other authors have
confirmed the prognostic value of the RVAD, as well as
its reliable application.52, 54
Nonoperative treatment of progressive infantile
idiopathic scoliosis predominates and may involve the
use of conventional thoracolumbosacral orthosis
(TLSO)–type braces, Milwaukee-type braces, and even
intermittent Risser casting. Some have questioned the
value of bracing in infantile idiopathic scoliosis and
have stated, "a curve that resolves in a brace would
probably have resolved without treatment."50
If surgical treatment becomes necessary, anterior
release and fusion followed by posterior spinal fusion
with instrumentation is considered to be the functional
treatment. Every effort should be made to delay such
surgical intervention as long as possible to optimize
spinal growth, but relentless curve progression should
not be accepted or tolerated while awaiting some
arbitrary chronologic age. Although intuitively
attractive, convex spinal epiphysiodesis (which has been
shown to be quite effective in the management of
congenital scoliosis) has not been shown to be as
reliable in the setting of infantile idiopathic
scoliosis.55 Addition of some
type of posterior instrumentation may improve the
results of epiphysiodesis.56
A treatment outline for infantile idiopathic
scoliosis may be as follows:
- Curves less than 25° with an RVAD less than 20°
are preferentially observed and monitored with spinal
radiographs at regular intervals.
- Curves exceeding these parameters are typically
braced, with some consideration given to the value of
intermittent Risser casting.
- Surgery is considered for curves not adequately
controlled with nonoperative measures.
Juvenile idiopathic scoliosis
Juvenile idiopathic scoliosis
most closely mimics the epidemiology and demographics of
the adolescent version of the disease. It is more common
in females, and its most common curve pattern is a right
thoracic curve.21 In fact, due to its
demographic similarities, high rate of progression, and
need for surgery, juvenile idiopathic scoliosis might be
considered to be a malignant subtype of adolescent
idiopathic scoliosis. Robinson and McMaster studied 109
patients with juvenile idiopathic scoliosis in Scotland
and found that 95% (104 of 109 patients) demonstrated
curve progression and 64% (70 of 109 patients)
progressed to require a spinal fusion.21 This spinal
fusion rate is similar to that reported by J.I.P. James
15 years earlier.57
A study from Washington University found a 50% rate
of neural axis abnormalities in young children (<10
y) with idiopathic scoliosis.58 These findings
included Chiari type I malformations and dural ectasia.
At least one case report also exists in which a spinal
intraosseous arteriovenous malformation was found in
association with juvenile scoliosis.59
One potential treatment algorithm for juvenile
idiopathic scoliosis is as follows:
- Observation for curves less than 25° with
follow-up radiographs at regular intervals
- Bracing for curves that range from 25-40° and at
least consideration of bracing (based on curve
flexibility) for curves from 40-50°
- Bracing for smaller curves that demonstrate rapid
progression to the 20-25° range
- Surgical intervention for inflexible curves that
exceed 40° or virtually any curve that exceeds
50°.
Bracing and casting may be used outside the
above-mentioned parameters in an effort to help control
a large curve in a young child for whom the surgeon is
attempting to optimize spinal growth. Similar
recommendations exist regarding the value of MRI in
juvenile idiopathic scoliosis due to a significant rate
of neural axis abnormalities.58
Adolescent idiopathic scoliosis
Adolescent idiopathic scoliosis
is the most common type of idiopathic scoliosis and the
most common type of scoliosis overall. Progressive
curvature may be predicted by a combination of
physiologic and skeletal maturity factors and curve
magnitude. Small curves in more mature patients have a
substantially lower risk of progression (about 2%) than
larger curves in more immature patients, in whom the
risk is much higher (risk may approach or exceed
70%).
Treatment recommendations for adolescent idiopathic
scoliosis are driven almost totally by curve magnitude
(the only caveat being that brace treatment is thought
to be effective only in patients who are still growing).
It is thus somewhat ironic to note that stated
recommendations urge observation for curves less than
30°, bracing of curves that reach the 30-40° range, and
consideration of surgery for curves that exceed 40°.
This amounts to a 10° window between observation and
major spinal surgery. It is even more ironic to note
that 10° is a commonly discussed margin of error for
measuring such scoliotic curves. Additional patient
factors may also influence some orthopedic surgeons to
brace patients with curves measuring less than 30° or in
excess of 40°. For instance, a rapidly progressive curve
in a 12-year-old child that suddenly goes from 16-26°
may easily prompt bracing.
When it comes to surgical considerations, patients
with adolescent idiopathic scoliosis may be functionally
subdivided into those patients in whom significant
anterior spinal growth is a concern and those in whom it
is not. This amounts to a quantification of risk of
development of the complication known as crankshaft
phenomenon.60 This can have a
major impact on the surgical treatment plan in that a
child at significant risk for crankshaft phenomenon will
require an anterior spinal fusion procedure.
Much effort has been devoted to predicting which
patients may suffer from this continued anterior spinal
growth that results in progressive angulation and
rotation of the spine.60, 61, 62, 63, 64. In fact, a
hierarchy of risk can be constructed in which
progressively more precise estimates can be made. In
this hierarchy, the presence of a radiographic Risser
sign and reaching menarche are somewhat predictive but
less so than closure of the triradiate cartilage, and
reaching one's peak height velocity is perhaps the most
powerful predictor of being at rather low risk for the
crankshaft phenomenon.
The anatomy relevant to idiopathic scoliosis is that
of the thoracic and lumbar spine. Key points regarding
developmental anatomy of the spine are outlined below.
Scoliosis surgery is usually still performed via a
posterior approach to the spine; thus, significant
discussion of posterior anatomy is provided. A growing
appreciation and need for anterior surgical procedures
for scoliosis also demands additional discussion of
retroperitoneal anatomy and intrathoracic anatomy,
especially as it relates to video-assisted thoracoscopic
surgery (VATS).
Developmental anatomy
Significant growth, development, and differentiation
occur as a single-celled zygote progresses to become an
approximately 100 trillion–celled adult human.
Identifiable spine development has begun by the third
week of gestation. First, the neural tube forms. Later,
paired somites appear (at 4.5 weeks' gestation) and
spinal nerves are present by the sixth gestational week.
A discernible cartilage model of the spine is present by
the seventh week of gestation. The bone and cartilage of
the spine are mesodermal derivatives, as are significant
portions of the cardiovascular and urogenital systems.
This explains the frequent coexistence of congenital
spine anomalies with congenital cardiac and kidney
defects. Thus, gestational weeks 3-7 are very important
in the development of all of these major body
systems.
Postnatal spinal growth also must be understood and
appreciated. Alain Dimeglio has shown that the majority
of spinal canal diameter (about 90%) has been achieved
by age 5 years.65 By age 10 years,
approximately 80% of sitting height has also been
achieved.66 During adolescence,
radiographic evidence of ossification of the growth
cartilage of the vertebral bodies occurs. Prior to this,
these completely cartilaginous growth plates remained
nestled between their respective vertebral bodies and
intervertebral disks.
Posterior anatomy
The major superficial muscles of the back are not
often directly visualized during posterior surgical
approaches for scoliosis, but they must not be
forgotten. These muscles include the trapezius, rhomboid
major, rhomboid minor, and latissimus dorsi. Using an
animal model, Kawaguchi et al showed that significant
posterior muscle injury can be induced by the pressure
exerted by surgical retractors.67 This certainly
makes a case for intermittent removal and replacement of
such retractors during the course of posterior spinal
surgery.
The route for exposure of the posterior spinal
elements passes through the cartilaginous apophyses of
the spinous processes. These structures, often referred
to as the cartilaginous caps, are systematically split
in the midline to allow sequential subperiosteal
dissection of the spinous processes, laminae, facet
joints, and transverse processes. The laminae of the
thoracic vertebrae spread out from the midline like
wings and flow upwards (cranially) in the direction of
the transverse processes. The facet joints of the
thoracic spine are shingled in a coronal plane such that
the inferior facet that contributes to each joint is
located posteriorly and the superior facet is located
anteriorly. The thickness of the interior and superior
facets of the thoracic spine range from about 3-5
mm.68 The thoracic facet
joints are located a mere 7-11 mm from the midline of
the posterior spine.68
Progressing from the thoracic to the lumbar spine,
important differences are noted. The V-shaped laminae of
the thoracic spine give way to the butterfly-shaped
laminae of the lumbar spine. This orientation change is
important for the surgeon to remember when exposing
these bony elements. The facet joints of the thoracic
spine, which are oriented in more of a coronal plane,
transition into the more sagittally oriented facet
joints of the lumbar spine. The transverse processes of
the thoracic spine, which seem to flow directly up and
away from the laminae, change significantly in the
lumbar spine such that they are no longer in close
proximity to the laminae and are located anterior and
inferior to the lumbar facet joints.
The ribs are also obviously absent in the lumbar
vertebrae. What some consider a rib remnant does persist
and is referred to as a mamillary body or mamillary
process. It is most pronounced near the thoracolumbar
junction but may be identified on nearly all of the
lumbar segments. In the sagittal plane, one must also
appreciate that the normal gentle kyphosis of the
thoracic spine reaches its apex at about the T7 through
T9 region. Below this, a rather definite transition to
lumbar lordosis occurs, with an apex around the L3
level. Thoracic kyphosis typically ranges from 20-40°
(Cobb measurements usually taken from the top of T3 to
the bottom of T12). Some authors have stated that up to
50° of thoracic kyphosis should be considered
normal.69 Normal lumbar
lordosis is considered by some to range from 35-55°
(Cobb measurements usually taken from the top of L1 to
the top of L5).
Anterior anatomy
Anterior scoliosis surgery involves 3 main
strategies, as follows:
- Anterior lumbar or thoracolumbar surgery through a
retroperitoneal approach that may or may not involve a
diaphragmatic incision
- Anterior thoracic surgery via traditional open
thoracotomy
- Anterior thoracic surgery via VATS
Various factors relative to skeletal maturity, curve
location, and curve flexibility help determine which (if
any) of these anterior surgeries may be appropriate.
The most common reason to use the retroperitoneal
approach is for an instrumented anterior thoracolumbar
spinal fusion. The most common curve pattern in that
particular type of scoliosis is an apex left curve
pattern, and as such, the patient is usually positioned
lying on the right side. This position is advantageous
in that it provides the best access to the scoliotic
spine and it also places the thick-walled aorta closer
to the surgical field (as opposed to the thin-walled
inferior vena cava). After superficial muscle
dissection, the surgeon approach proceeds through the
bed of the rib that corresponds with the highest
vertebrae in which instrumentation is planned. This is
often either the ninth or tenth rib, with the rib itself
being harvested for later use as a bone graft.
Careful dissection is then performed to mobilize the
peritoneum (with its contents) in an anterior direction;
it is peeled off of the undersurface of the diaphragm.
Posterior division of the diaphragm (leaving about a
2-cm cuff for repair) helps to avoid damage to the
phrenic nerve. Diaphragmatic division begins with
splitting of the costal cartilage and proceeds in a
posterior direction with intermittently placed tagging
sutures to aid in closure.
The remainder of the retroperitoneal approach to the
thoracolumbar spine requires careful superior retraction
of the lung, anterior retraction of the peritoneum (with
associated aorta and ureter), and posterior retraction
of the iliopsoas musculature. Careful identification and
division of the segmental vessels (overlying the
vertebral bodies) is carried out with either
electrocautery or ligatures. Small sympathetic nerve
branches in this same area are sacrificed during this
stage of the exposure. This results in at least a
transient period in which the left foot (for a
left-sided approach) will be both pinker and warmer than
the contralateral foot. At times, this may result in
nursing personnel notifying the surgeon that the
contralateral foot is pale and cold, but in reality, the
foot ipsilateral to the exposure has changed.
Open thoracotomy might be performed either for
anterior thoracic spine release followed by posterior
fusion or for anterior thoracic spine fusion with
instrumentation. The most common curve pattern to
address with this approach would be a right thoracic
curve, and as such, the patient would be positioned with
the right side upward.
A similar rib selection and resection technique may
be used if desired. From the interior of the chest, the
intercostalis musculature (located between each of the
ribs) can be seen. Identification of the azygous vein
(anteriorly oriented along the vertebral bodies) is
necessary. Further medial (ie, central) and running
parallel to the azygous vein is the thoracic duct.
Several portions of the sympathetic chain may be
sacrificed as the segmental vessels overlying the
thoracic vertebral bodies are divided and mobilized
anteriorly and posteriorly. Blood flow changes similar
to those noted in the retroperitoneal approach may be
noted in the right foot (for a right thoracotomy).
In addition to this, thoracic surgical dissection
carries with it the possibility of sacrificing branches
to the greater splanchnic nerve, which would
theoretically decrease visceral referred pain that one
might feel from an inflamed gallbladder or similar
condition.
Thoracoscopic appreciation of the anatomy of the
thoracic spine is becoming more common as endoscopic
anterior release and fusion is rapidly moving from being
considered an innovation to standard practice. Just as
arthroscopic knee surgeons enjoyed an expansion in
visualized anatomy compared to that visible with knee
arthrotomies, the endoscopic spine surgeon benefits from
much greater intrathoracic latitude. Most VATS also
involve the right thoracic cavity, and this discussion
focusses on that particular side.
Proper rib counting and visualization of the superior
intercostal vein (formed by the confluence of the
second, third, and fourth intercostal veins) as it
empties into the azygous vein are necessary steps to
orient the surgeon. Beyond this, one also notes the
mounds and valleys of the thoracic spine, with the
mounds being the disks and the valleys being the
vertebral bodies with the segmental vessels that overly
them.70
The same anatomy outlined in the thoracotomy
discussion still clearly applies, but further endoscopic
fine points are needed. Specifically, the thoracic spine
may be considered to be composed of 3 separate fields
with important anatomic nuances.71 The upper field may
be considered to be T2-T5, the middle field may be
considered to be T6-T9, and the lower field may be
considered to be T10-L1.71 The upper field is
dominated by the superior intercostal vein, and it is
characterized by the fact that the rib heads tend to
completely span their respective disk spaces and
articulate with 2 vertebral bodies. This results in a
rib such as the third rib coming directly into the
region of the T2-T3 disk space such that it will
articulate with both the T2 and T3 vertebral bodies. In
the middle field, the rib head once again comes directly
in toward the disk space, but now, it rather firmly
attaches itself only to the disk space proper.
In the lower field, the rib head articulates directly
with its corresponding vertebral body. Thus, in the
lower field, the 11th rib is traced to its corresponding
vertebral body and then moves directly cephalad to reach
the T10-11 disk or directly caudad to reach the T11-12
disk. Once the vertebral bodies have been exposed in a
skeletally immature patient, the growth cartilage
of the vertebral endplate can be visualized. It has an
odd tendency to appear green in color (a quirk of
endoscopic optics) and is colloquially referred to as a
Wolf line in honor or Randall K. Wolf.
Few, if any, absolute contraindications exist
regarding scoliosis care, just as few, if any, absolute
indications for intervention exist. Accepted
contraindications for bracing include skeletal maturity
and excessive curve magnitude. Thoracic lordosis and
certain curve patterns such as double thoracic curves
also have been offered as at least relative
contraindications to bracing.
The main contraindication to posterior scoliosis
surgery would be medical instability and inability to
survive surgery. Anterior scoliosis surgery would also
be contraindicated in these patients, as well as in
those with a precarious pulmonary status.
Lab
Studies
- Laboratory workup for patients with scoliosis
consists primarily of preoperative testing. Most, if
not all, patients undergo preoperative assessment of
hemoglobin and hematocrit levels. Autologous blood
predonation is also a common practice.
Imaging
Studies
- Multiple authors have cited the value of bending
radiographs, including those over a fulcrum.72 Klepps and Lenke
et al found that thoracic fulcrum bending radiographs
worked best for them when dealing with isolated main
thoracic curves.73
-
- The thoracic curve patterns found in adolescent
idiopathic scoliosis are still most commonly
classified using the King classification
system.74 Significant
questions have been raised regarding the reliability
and reproducibility of the King classification
system.75, 76 In
addition to this, the King classification alone (in
its original form) does not allow comprehensive
curve classification (eg, lumbar and thoracolumbar
curve patterns).77
- Multiple authors have analyzed the ability of
orthopedic surgeons to reliably measure scoliosis
radiographs. Morrissy and his colleagues used 50
radiographs and 4 examiners (2 experienced
orthopedic surgeons, 1 fellow, 1 senior resident) to
study their ability to make Cobb angle measurements.
With the examiners choosing end vertebrae and
measuring scoliotic curves accordingly,
intraobserver variability was 4.9°.78 Carman
and her coworkers used 8 scoliosis radiographs
measured by 5 examiners (4 orthopedic surgeons, 1
physical therapist) to evaluate interobserver and
intraobserver variation. They found that a 10°
measurement difference is necessary before there is
a 95% confidence level that one Cobb angle measurement is
truly different from another.79
- MRI has been suggested to be primarily indicated
in patients with idiopathic scoliosis with unusual
complaints such as severe unexplained headaches and
when clinical findings such as ataxia or cavus feet
are present.80 Routine MRI
evaluation of all patients with adolescent idiopathic
scoliosis is not recommended.
Other
Tests
- Pulmonary function studies have been used rather
extensively in the evaluation of patients with
idiopathic scoliosis.19, 81, 83
-
- In general, patients whose scoliosis surgery
does not involve disruption of their chest wall can
be expected to experience improved postoperative
pulmonary function.84 Other authors
have suggested that an impairment in respiratory
mechanics may persist following successful scoliosis
surgery.85
- Preoperative pulmonary function testing is of
questionable value in patients with moderate
deformity (average Cobb angle 48°), as most of these
patients can be expected to have normal or only
mildly abnormal results.86
- Efforts at screening for scoliosis (most often in
school populations) have met with mixed success. A
2-year evaluation of more than 80,000 Greek 9- to
14-year-old students screened by their schools using
the Adams forward-bending test was conducted by
Soucacos et al. Overall, they found school screening
to be simple and effective. These authors found that
they identified 181 new children with scoliosis
requiring treatment (11 surgically, 170 with
bracing).87
- Peak height velocity has been studied rather
extensively as a predictor of curve
progression.88
Histologic
Findings
Scoliosis is clearly a disease that is strongly
influenced by, if not completely rooted in, spinal
growth. It has even been referred to by some as "an
unsynchronized growth."89
Hsu and coworkers from Vanderbilt studied muscle
biopsies from 27 patients with idiopathic scoliosis who were
undergoing posterior spinal fusion. Specimens were
obtained from the paraspinal musculature of both the
convex and concave side in all patients. All patients
had thoracic curves that ranged from 37-81°.90 Sixty-eight
percent of the patients demonstrated abnormalities in
muscle fiber distribution. The abnormalities were
similar on the convex and concave sides, the most
notable being a reversal of the normal type 2-fiber
ratio such that type 2-A fibers predominated over type
2-B fibers in the study subjects. These changes are
similar to those seen in endurance training and might be
due to the extra work of trying to maintain posture in
the setting of scoliosis.90
Medical
therapy
Nonoperative management consists of either mere
observation or orthosis use. Observation is watchful
waiting with appropriate intermittent radiographs to
check for the presence or absence of curve progression.
Orthosis use for scoliosis is discussed extensively
below. No other treatments, including electrical muscle
stimulation, physical therapy, spinal manipulation, and
nutritional therapies, have been shown to be effective
for managing the spinal deformity associated with
idiopathic scoliosis. The lack of demonstrated
effectiveness in this context means that scientifically
valid studies have either been done that do not support
the treatment or no such studies have yet been published
that would allow an evidence-based evaluation.
The first widely used scoliosis brace with proven
effectiveness was the Milwaukee brace. This brace was
developed by Walter Blount and Albert Schmitt and
introduced at a meeting of the American Academy of
Orthopaedic Surgeons in 1946.91 The brace was
originally designed to be used as part of the surgical
treatment of scoliosis and only later evolved into a
stand-alone nonoperative treatment.
Lonstein and Winter studied 1020 patients with
adolescent idiopathic scoliosis treated with the
Milwaukee brace. They reported that this orthosis was
effective in preventing significant curve progression in
patients with 20-39° curves.92 These same authors
recommended that adolescents with a curve of 25° and a
Risser sign of 0 be braced immediately and not wait for
evidence of curve progression.92 Other authors have
shown that an average curve correction of 20% in the
brace (Milwaukee brace) is associated with bracing
success.93, 94
Rowe and his colleagues performed a meta-analysis
aimed at evaluating the efficacy of nonoperative
treatments for idiopathic scoliosis.95 They calculated the
weighted mean proportion of success for 3 nonoperative
treatments: observation, electrical stimulation, and
bracing. They were able to successfully combine data on
1910 patients from 20 different studies for purposes of
meta-analysis. Their main results are as follows
(treatment, success rate):
- Observation, 49%
- Electrical stimulation, 39%
- Bracing 8 hours per day, 60%
- Bracing 16 hours per day, 62%
- Bracing 23 hours per day, 93%
In a prospective, multicenter study from the Scoliosis Research Society,
Nachemson and his coworkers found brace treatment (an
underarm plastic brace worn for at least 16 h/d) to be
successful 74% of the time (95% confidence interval
[CI], 52-84%).96 Some authors have
not been able to identify a major difference between
full-time bracing (23 h/d) and part-time bracing (12-16
h/d).97
The psychological stress associated with scoliosis
has been documented,98 and this does not
improve compliance with brace wear. MacLean and his
coauthors from Vanderbilt studied 31 adolescent and
preadolescent females who were undergoing part-time
brace treatment for their idiopathic
scoliosis.99 Part-time bracing
was defined as 13-16 hours per day. Eighty-four percent
of their patients described the initial period of
bracing in "stressful terms" and experienced lower
levels of self-esteem.99 A reassuring
finding is that overt psychopathology was not identified
in MacLean's study.
Compliance with prescribed brace-wear regimens has
been shown to be poor. DiRaimondo and Green found that,
on average, patients only wore their braces 65% of the
prescribed amount of time.100 Patients
prescribed part-time bracing (16 h/d) actually
demonstrated worse compliance (58%) than those
prescribed full-time (24 h/d) bracing (71%).100 Overall, only 15%
of patients demonstrated a highly compliant
(>90%) brace-wear routine.100
Questions have also been raised regarding the
consistency of strap tension in thoracolumbosacral
orthosis (TLSO) bracing.101 Using an
instrumented load cell to measure strap tension, Aubin
et al studied 34 of their patients with braces in
Quebec. They found marked variability in tension, with
the greatest change occurring while patients were
recumbent.101
In part due to the aforementioned psychological and
brace-wear compliance issues, new approaches to bracing
are being developed.102, 103 One such approach
is that developed by Dr. Christine Coillard and Dr.
Charles Rivard of the St. Justine Hospital in Montreal,
Canada. Their dynamic bracing approach is referred to as
the SpineCor Brace or as the St. Justine
Brace.104 It involves
elastic straps that are anchored on a pelvic corset, and
based on curve morphology, these straps are tensioned to
exert corrective forces. The brace is a radical
departure from traditional plastic and metal orthoses.
Early results with the St. Justine Brace are rather
encouraging, with success rates comparable to those of
traditional bracing. Continued follow-up of their
growing international cohort of patients is
necessary.
Surgical
therapy
Even in the setting of adequate correction and solid
fusion, up to 38% of patients still have occasional back
pain.76, 105
The primary goal of scoliosis surgery is to achieve a
solid bony fusion. The surgical technique used to
achieve such an arthrodesis is vastly more important
than the instrumentation system that the surgeon needs
to use, if any.18, 106
Modern instrumentation systems have been shown to
allow for adequate curve correction but with little or
no ability to diminish associated rib humps.107 Despite claims of
certain instrumentation systems to derotate the spine,
little actual derotation has been documented. Derotation
of the instrumented curve also has been shown to
possibly occur at the expense of creation of new
rotation in uninstrumented portions of the
spine.108
Previously, much attention was paid to the
ability of certain spinal instrumentation systems (eg,
Cotrel-Dubousset to derotate the spine during scoliosis
correction. Jarvis and Greene showed that use of the
Wisconsin segmental spinal instrumentation (a system
traditionally thought to not be associated with
significant spinal derotation) was associated with
spinal derotation equal to or greater than that
of the Cotrel-Dubousset–type systems.109
Since 1993, VATS has been used in the anterior
treatment of pediatric spinal deformity at Cincinnati
Children's Hospital Medical Center.110 This minimally
invasive surgical technique is aimed at decreasing
operative morbidity and optimizing patient recovery from
surgery. Over 100 of these procedures have been
performed at this institution. Initial biomechanical
studies in animal models have correctly predicted what
clinical practice has now borne out—that endoscopic
anterior release and diskectomy is as effective as the
open version of the operation.70, 96 111, 112 Endoscopic spinal
instrumentation techniques have also been introduced and
continue to evolve.113
Preoperative
details
Preoperative evaluation focuses on specifics of curve location, magnitude, and
flexibility. These parameters are used in conjunction
with patient maturity factors to determine optimal
treatment choice, but definitive studies are not yet
available that dictate specific surgical tactics.
However, the scoliosis surgeon is aided by commonly
applied clinical guidelines that have evolved over time.
The goal is always to fuse as little of the spine as
possible while adequately treating existing major
curvature.
For a thoracic curve (with adequate flexibility)
without any significant associated lumbar curvature, the
most common surgical approach has not changed since the
days of Paul Harrington: posterior spinal fusion with
instrumentation. Surgeons may choose from a diverse
array of anchors to secure large-diameter rods (usually
in the 0.25-in range) to the spine. These anchors
include sublaminar hooks, pedicle hooks, transverse
process hooks, sublaminar wires (Luque wires), spinous
process wires (Drummond wires), and pedicle screws. Some
surgeons have advocated anterior spinal fusion and
instrumentation for such isolated thoracic curves. These
have included both open (thoracotomy) and
limited-incision (thoracoscopic) techniques.
When the primary problem is a large, stiff thoracic
curve (usually not bending less than 50°), a different
surgical tactic is usually undertaken in which an
anterior release (usually including diskectomy and bone
grafting) is performed prior to posterior spinal fusion
and instrumentation. Anterior spinal fusion and
instrumentation has also been advocated in this
situation, provided the patient does not have excessive
kyphosis associated with a large thoracic
curve.
Large curve patterns that include both thoracic and
lumbar deformity continue to challenge scoliosis
surgeons. If adequate flexibility and balancing of the
lumbar spine is possible, then selective fusion of the
thoracic curve is possible. When this is not the case,
extensive fusion (at times down to the fourth lumbar
segment) may become necessary.
The Scoliosis Research Society has a rather specific
definition of thoracolumbar scoliosis: a curve whose
apex lies at the body of T-12 or L-1 or at the
T12-L1 interspace. These curves are most commonly
left-sided curves and they present one of the most
common scenarios in which anterior spinal fusion and
instrumentation is utilized. Anterior approaches to this
area of the spine were pioneered by Hodgson (Hong Kong),
Dwyer (Australia), and Zielke (Germany). Current
approaches represent further refinement of these
original techniques, such as modern large rod-and-screw
systems and the John Hall short anterior segment
overcorrection technique. The value of such techniques
lies in their ability to powerfully correct large
thoracolumbar curvatures while minimizing fused segments
within the lumbar spine.
There is little debate regarding the fixation of the
rods used for anterior instrumentation. Large bone
screws are almost always the anchor of choice. For
posterior instrumentation procedures, the surgeon has
more options. Multiple hooks are the most commonly used
anchors. They offer simplicity, strength, and near
complete visualization during insertion. Their main
drawbacks relate to size mismatch between hooks and
associated bony elements, as well as the absence of
appropriate hook sites (such as might be the case in
myelomeningocele, tumor cases, or revision
surgeries).
Sublaminar wires offer the power of segmental
fixation and firm bony purchase, but with the drawback
of possible dural and/or spinal cord trauma. As a
result, either very selective use of or no use at all of
sublaminar wires is usually the case in the setting of
idiopathic scoliosis. A reasonable compromise was
achieved when Denis Drummond introduced his spinous
process wires (also known as Wisconsin wires). These
devices still offer the power of segmental fixation with
virtually none of the nerve injury risks of sublaminar
wires.
Pedicle screws have also become a popular anchor for
the rods used in posterior scoliosis fusion
procedures.114 They offer the
potential advantage of increased strength (and possibly
power of correction) while at the same time introducing
added insertion-technique complexity and different
neurologic complication risks. A very real and major
increase in the overall cost of instrumentation
constructs that include many pedicle screws is the case
when comparing them to similar constructs that may
include hooks and wires. At this time, evidence is not
conclusive to support a commensurate improvement in
clinical outcomes to support the routine use of such
pedicle screw constructs in the treatment of idiopathic
scoliosis.
Pulmonary function testing is commonly used in the
preoperative evaluation of patients with idiopathic
scoliosis who are slated to undergo surgery. Such
testing may influence the surgeon's enthusiasm for
related procedures, such as costoplasty (thoracoplasty).
Pulmonary function testing may also uncover previously
unrecognized tobacco use (an independent risk factor for
pseudarthrosis) or undiagnosed (subclinical) pulmonary
disease.
Predonation of several units of donor-directed blood
is considered standard for most patients. Certain
commercially available intraoperative blood recovery
devices may also be used at times.
Intraoperative
details
Hoppenfeld described an ankle clonus test for
intraoperative assessment of the integrity of the spinal
cord during scoliosis surgery. In more than 1000
patients, the test was noted to have no false-negative
results and 3 false-positive results. This translated
into 100% sensitivity and 99.7% specificity.115
Postoperative
details
Postoperative patient management involves close
monitoring, which often occurs initially in an intensive
care unit setting. Patients have monitoring devices,
such as arterial lines, and closed suction devices, such
as chest tubes, that also require special nursing
attention. The use of certain special spine-specific
hospital beds, such as the Stryker frame, may also aid
in patient care and comfort (change from supine to prone
position) during the initial postoperative period.
The use of postoperative bracing varies from surgeon
to surgeon. As outlined in History
of the Procedure, the roots of scoliosis surgery
involved immobilization in a body cast. Following the
development of initial instrumentation systems (eg,
Harrington instrumentation), external immobilization was
still used routinely. With the advent of large-rod
multiple-hook constructs, such as the Cotrel-Dubousset
system and its direct decendents, bracing has been
deemphasized a bit. Thus, it is almost as likely
that a patient will not receive a postoperative brace as
receive one, whereas, previously, bracing was much more
widespread. In certain specific circumstances,
postoperative bracing is still almost always used, such
as anterior thoracic or thoracolumbar instrumentation
procedures or surprisingly weak bone stock.
When a brace is used, it is typically to be worn
full-time for at least 6 weeks, followed by a period in
which the brace may be taken off for bathing, with
subsequent progressive weaning. As a rule of thumb,
patients may also miss up to 6 weeks of school (if their
procedure is done at such time of the year), and up to 6
months may be required before they resume most of their
normal activities. Vigorous sports may be restricted for
at least a year or, in some instances, permanently
(based on risk-versus-benefit discussions between
patients, families, and their surgeons).
Follow-up
At an average of 21 years following posterior spinal
fusion with Harrington instrumentation (performed by
Paul Harrington himself), about 21% of patients
experienced significant interscapular pain.116
Complications are of great concern to parents,
patients, and surgeons. Thankfully, complications are
rare with modern scoliosis surgery, despite the
magnitude of these spinal deformity procedures. Several
important intraoperative, early postoperative, and late
postoperative complications are discussed here.
McKie and Herzenberg described coagulopathy as a
complication of intraoperative blood salvage during
scoliosis surgery.117 These authors
suggested that thrombin and Gelfoam that may have been
aspirated along with salvaged blood may have contributed
to the disseminated intravascular coagulation
experienced by their 17-year-old patient. This effect of
the thrombin and Gelfoam would have been in addition to
that of hemodilution (hemodilution-induced platelet and
leukocyte activation syndrome).117
The
importance of appropriate intraoperative spinal cord
monitoring during scoliosis surgery is hardly debatable.
Such monitoring can allow early recognition and
treatment of spinal cord dysfunction.118 Somatosensory and
motor evoked potentials are commonly used to monitor
spinal cord function. A Stagnara wake-up test may also
still be employed if the surgeon desires. Current
efforts at monitoring have helped achieve and maintain a
very low rate of spinal cord injury (less than one half
of one percent).
Some concern exists regarding postoperative activity
level and the possible hazards of trauma. Neyt and
Weinstein have reported a case of lumbar spine fracture
dislocation in a teenage boy 3 years after
successful scoliosis surgery.119 The boy's fusion
extended from the second thoracic vertebra to the first
lumbar vertebra, and his subsequent fracture dislocation
occurred at the L2-3 level.119
Delayed infections following posterior spinal fusion
with Texas Scottish Rite Hospital instrumentation has
been reported. Richards reported on 10 such patients who
presented with infections at an average of about 2 years
following successful spinal fusion.120 Low-virulence
organisms such as Propionibacterium acnes were
the main cause, and instrumentation removal was
successful in eradicating the infections. Richards
hypothesized that the infections might be related to the
amount of hardware (eg, hooks, rods) used and suggested
that efforts at minimizing such hardware might help
prevent such infections.120
Much has been written regarding a particular
complication called crankshaft phenomenon. It may occur
following posterior spinal fusion of idiopathic scoliosis in
patients who have significant anterior spinal growth
remaining. Sanders and coauthors reported that the risk
of the crankshaft phenomenon was highest in patients
younger than 10 years and in patients with a Risser sign
of 0 with an open triradiate cartilage.121
Significant concern exists regarding the inferior
(caudad) extent of a patient's spinal fusion and its
potential relationship with future low back
pain.122 Connolly led a
group of researchers at the Toronto Hospital for Sick
Children who studied this question in 83 patients fused
with Harrington instrumentation to the second, third,
fourth, or fifth lumbar vertebra. At an average of
12 years (range 10-16 y) following their surgery, these
patients were found to have a statistically higher rate
(76%) of low back pain than a control group (50%).
Connolly's patients were from an era in which the
predominant instrumentation system was noncontoured
Harrington rods, which were notoriously associated with
low back pain when applied to the lumbar
spine.122 The results of
this study almost certainly cannot be generalized to
current scoliosis patients, who are treated with very
different instrumentation systems.
Some complications have been associated with
particular surgical approaches to scoliosis. For
instance, chylothorax and tension pneumothorax have both
been reported in association with video-assisted
thoracoscopic surgery (VATS) procedures.123, 124
Pseudarthrosis is a complication that represents a
basic failure of the central intention of scoliosis
surgery: bone fusion. Luckily, pseudarthrosis is very
rare in modern scoliosis surgery. This is in small part
due to excellent stable internal fixation (scoliosis
instrumentation systems) and in large part due to proper
attention to fusion technique. Pseudarthrosis may be
suggested by persistent pain, progressive deformity, or
broken hardware. Previously tomographic plain x-rays
(tomograms) were commonly used to image suspected
pseudarthrosis. This is no longer the case, as such
tomography equipment is on the endangered species list
of imaging devices. As such, computed tomography may be
helpful, but clinical suspicion and fusion mass
exploration (a rare case for modern-day exploratory
surgery) remain the cornerstones of pseudarthrosis
diagnosis and treatment.
Clinical outcomes following treatment of idiopathic
scoliosis are strongly linked to curve magnitude.
Unrealistic presurgical expectations have been shown to
correlate with a decreased likelihood of postsurgical
satisfaction.125 More long-term
follow-up studies of surgically treated patients with
scoliosis are becoming available. This section outlines
some of these data.
A large cohort (nearly 2000 subjects) of patients
with idiopathic scoliosis in Montreal, Canada, referred
to as the St. Justine Cohort Study, has been monitored
for 10-20 years. These patients were compared to a
population-based control group drawn from the general
Quebec population. Compared to the general population
and regardless of whether their scoliosis was treated
surgically or nonsurgically, patients with scoliosis
were found to have a higher self-reported rate of
arthritis and poorer perceptions of their overall
health, body image, and ability to participate in
vigorous activities.126, 127
A subset of the cohort (700-1500 patients) was
analyzed further regarding low back pain.128, 129 These Canadian
researchers found a higher overall rate of significant
back pain reported within the last year (75% of patients
with scoliosis versus 56% of control
subjects).128 Patients with
scoliosis who were treated surgically also reported a
high rate (73%) of back pain within the last year, but
it did not correlate with the distal extent of the
spinal fusion. The St. Justine authors went on to state
that their study "does not provide any evidence that
extending the level of fusion down even as far as L4
will increase the prevalence of back pain in
adulthood."129
References:
- Lonstein
JE. Idiopathic scoliosis. In: Lonstein JE,
Bradfordn DS, Winter RB, Ogilvie J, eds. Moe's
Textbook of Scoliosis and Other Spinal
Deformities. 3rd ed. Philadelphia,
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of the study. Spine. Jul
15 1994;19(14):1551-61. [Medline].
- Goldberg MS, Mayo NE,
Poitras B, et al. The Ste-Justine Adolescent
Idiopathic Scoliosis Cohort Study. Part II: Perception
of health, self and body image, and participation in
physical activities. Spine. Jul
15 1994;19(14):1562-72. [Medline].
- Mayo NE, Goldberg MS,
Poitras B, et al. The Ste-Justine Adolescent
Idiopathic Scoliosis Cohort Study. Part III: Back
pain. Spine. Jul
15 1994;19(14):1573-81. [Medline].
- Poitras B, Mayo NE,
Goldberg MS, et al. The Ste-Justine Adolescent
Idiopathic Scoliosis Cohort Study. Part IV: Surgical
correction and back pain. Spine. Jul
15 1994;19(14):1582-8. [Medline].
- Puzas JE, O''Keefe
RJ, Lieberman JR. The orthopaedic genome: what
does the future hold and are we ready?. J Bone
Joint Surg
Am. Jan 2002;84-A(1):133-41. [Medline].
Idiopathic Scoliosis
History of the Procedure
Scoliosis is an ancient disease that remains incompletely understood despite a collective medical experience that approaches 4000 years. This is a sad commentary on the learning curve of medical practitioners. Nevertheless, the history of the recognition and treatment of scoliosis is rich with important lessons for the modern practitioner.
Ancient Hindu religious literature (circa 3500-1800 BC) describes the treatment of spinal deformity rather clearly. The story is told of a woman who was "deformed in three places" and how Lord Krishna straightened her back.3 This was accomplished by pressing down on her feet and pulling up on her chin. The orthopedic trappings of the story are unmistakable, including excellent immediate posttreatment results and no long-term follow-up.
Hippocrates (circa 400 BC) stated, "there are many varieties of curvature of the spine even in persons who are in good health; for it takes place from natural conformation and from habit." He also stated that "lateral curvatures also occur, the proximate cause of which is the attitudes in which these patients lie."4 The postural and muscular theory of scoliosis thus stated has persisted for thousands of years and remains firmly embraced by some.
Hippocratic scoliosis treatment methods focused primarily on spinal manipulation and traction.5 He used an elaborate traction table called the scamnum. Medical practitioners used slight variations of the Hippocratic scamnum well into the 1500s. Another treatment approach that Hippocrates discussed involved attempting to diminish spinal deformity with a method called succussion. This involved strapping the patient (often upside down) to a ladder, which was then hoisted into the air and dropped from a height. Hippocrates thought that this method was occasionally useful, but it was largely performed by charlatans to impress the public.6
Ambroise Pare has been described as the "most celebrated surgeon of the Renaissance."7 Pare is recognized as the first physician to treat scoliosis with a brace. He also recognized that once a patient with scoliosis had reached maturity, bracing was not useful. Pare's orthosis consisted of a metal corset (fashioned in a village smithy setting) with many holes in it to help diminish its significant weight. The record also makes it quite clear that Ambroise Pare espoused the postural theory of scoliosis.
Nicholas Andry was a French pediatrician who hated
the brutal barber surgeons of his day.8. At the age of 83 (a
year before his death) he wrote a short book entitled
Orthopaedia. Thus, in 1741 this name combined
the root words for straight (orthos) and child
(paedia) to create the name still used for the
broad musculoskeletal field, orthopedics.
Andry
believed that scoliosis was caused by asymmetric muscle
tightness and, thus, helped foster the French belief in
"convulsive muscular contraction" as the cause of spinal
deformity.6 Andry stated, "It is
well worth while to remark that the crookedness of the
spine does not always proceed from a fault of the spine
itself, but is sometimes owing to muscles of the
forepart of the body being too short, whereby the spine
is rendered crooked, just in the same manner as a bow is
made more crooked by tying its cord tighter.9 Andry used rest,
suspension, postural approaches, and padded corsets in
his treatment of scoliosis.
Jacques Mathieu Delpech was a successful and skilled surgeon, yet he focused a great deal of his attention on nonsurgical approaches to orthopedic problems. The highlight of this focus was his orthopedic institute at Montpellier, in the south of France. This facility included elaborate gardens, a heated winter gymnasium, and an outdoor gymnasium for the treatment of various musculoskeletal problems.
For the treatment of scoliosis, Delpech devised graded exercises for strengthening muscles of the trunk in the belief that the deformity was due to a weak axial musculature. This belief was almost certainly due to the influence of Andry. Delpech also used stretching and traction techniques but did not believe in braces. His patients usually stayed for 1 or 2 years at the institute, and they would wear uniforms while they performed their exercises. Similar elaborate efforts to treat scoliosis still exist in the physical therapy outpatient setting.10 Delpech's life and that of his institute came to an abrupt end in 1832 when a disgruntled patient shot him to death as he was riding back to Montpellier in an open carriage.4
An important event of the 1800s was the advent of surgical treatment of scoliosis by the French orthopedic surgeon Jules Guerin. He was very enthusiastic about subcutaneous tenotomy and myotomy and first reported their use in his patients with scoliosis in 1839. When he later published the results of treatment of 1,349 patients with this technique, tremendous controversy was ignited.4 Guerin's harshest critic was Joseph Malgaigne, who described Guerin's work as "some orthopedic illusion."4 This led to one of the most famous orthopedic lawsuits in history: Guerin versus Malgaigne. This defamation trial ended in Malgaigne's favor and helped to establish an important precedent for open criticism of scientific papers.
Another important tool in the treatment of scoliosis was the plaster body jacket (ie, body cast). The American orthopedic surgeon Lewis Sayre popularized its use in the mid 1800s. Sayre's technique involved a large tripod that allowed the patient to be suspended while the corrective plaster cast was applied. Sayre was said to be "a brusque, forceful and therefore controversial personality" but also "an eloquent speaker" who toured internationally demonstrating his casting techniques.6 He also used a "jury mast" extension from some of his casts in order to provide constant head traction—a clear predecessor to halo traction.
The early 1900s saw what is arguably the most
important advance in scoliosis treatment in more than
3000 years: posterior spinal fusion. Russell Hibbs first
performed his "fusion operation" for tuberculous spinal
deformity in 1911, but by 1914 he also was applying his
technique to patients with scoliosis.11 The Hibbs approach
focused on achieving maximum deformity correction via a
variety of plaster jackets before surgery. Hibbs's 1924
description of his own technique is eloquent, as
follows:
The dissection is carried farther and farther forward upon each vertebra in turn, until the spinous processes, the posterior surfaces of the laminae, and the base of the transverse processes are bared...[and] with a bone gouge, a substantial piece of bone is elevated from the adjacent edges of each lamina, of half its thickness and of half its width. The free end of the piece from above is turned down to make contact with the lamina below, and the free end of the piece from the lamina below is turned up to make contact with the lamina above...Each spinous process is then partially divided with bone forceps and broken down, forcing the tip to come into contact with the bare bone of the vertebra below.
In the postoperative period, Hibbs typically allowed 2 weeks of bedrest for wound healing, followed by a final traction plaster jacket. The patient would continue to be confined to bed while wearing the corrective cast for another 6 weeks. Following this, the patient would wear a removable brace during the day for an additional 6-12 months. It was clear to Hibbs that with his technique, he could at least partially correct and, more important than this, prevent progression of the curves he was treating.
By 1941, such spinal fusion operations for idiopathic scoliosis were common enough that Shands (of the Alfred I duPont Institute) and his fellow researchers could assess more than 400 cases.12 Hibbs-type fusion procedures were performed in all cases, but most surgeons (60%) used supplemental bone graft (often from the tibia). An approximately 25% final curve correction was achieved, and an overall 28% pseudarthrosis rate was noted.12 It would be another 20 years before Paul Harrington would introduce the spinal instrumentation system that would further refine scoliosis surgery.13 Although Harrington's original concept was instrumentation without fusion, persons such as John Moe would convince him of the value of spinal fusion in concert with Harrington rods.14
Further refinement in surgical technique and instrumentation has led to the greater than 50% correction and single-digit pseudarthrosis rates to which contemporary orthopedists have become accustomed.
Problem
Scoliosis represents a disturbance of an otherwise well-organized 25-member intercalated series of spinal segments. It is, at times, grossly oversimplified as mere lateral deviation of the spine, when in reality, it is a complex 3-dimensional deformity.15, 16 In fact, some have used the term rotoscoliosis to help emphasize this very point. Two-dimensional imaging systems (plain radiographs) remain somewhat limiting, and scoliosis is commonly defined as greater than 10° of lateral deviation of the spine from its central axis.
In the past, terminology such as kyphoscoliosis was inappropriately used to describe certain patients with idiopathic scoliosis. Idiopathic scoliosis has a strong tendency to flatten the normal kyphosis of the thoracic spine.17 Robert Winter teaches that idiopathic scoliosis is a hypokyphotic disease.18, 19 In most cases, diagnoses of kyphoscoliosis were clinical misinterpretations of the rib hump associated with an otherwise hypokyphotic thoracic spine. Idiopathic scoliosis may present as a true kyphoscoliosis, but this occurs relatively rarely.
J.I.P. James is credited with classifying idiopathic scoliosis according to the age of the patient at the time of diagnosis.20 Using his classification system, children diagnosed when they are younger than 3 years have infantile idiopathic scoliosis. Children diagnosed when they are aged 3-10 years have juvenile idiopathic scoliosis, and those older than 10 years have adolescent idiopathic scoliosis. These age distinctions, though seemingly arbitrary, have prognostic significance. For instance, Robinson and McMaster reviewed 109 patients with juvenile idiopathic scoliosis and found that nearly 90% of curves progressed, and almost 70% of these patients went on to require surgery.21 These rates are much higher than the rates associated with other categories of idiopathic scoliosis. The real challenge is to predict which curves will progress significantly and which ones will not.22 This is discussed in greater detail later in this article.
Frequency
Scoliosis is almost always discussed in terms of its prevalence (ie, the total number of existing cases within a defined population at risk). Rates may vary quite significantly based on what particular definition of scoliosis is used and what patient population is being studied. Several important studies are included below.
Stirling and his coauthors studied almost 16,000 patients aged 6-14 years in England and found the point prevalence of idiopathic scoliosis (Cobb angle >10°) to be 0.5% (76 of 15,799 patients).23 The prevalence of scoliosis was highest (1.2%) in patients aged 12-14 years.23 Data such as these have helped reiterate the idea that the focus of screening efforts should be on children in this age group. When smaller Cobb angle measurements have been accepted (eg, 6° or greater), a significantly higher scoliotic rate may be identified, such as the 4.5% rate reported by Rogala et al.24 Other studies using the 10° definition of scoliosis have placed the overall prevalence in the 1.9-3.0% range.25
Scoliosis has been suggested to develop more frequently in children born to mothers who are aged 27 years or older.26 One might hypothesize that gene fragility might be involved (eg, higher rate of infants with Down syndrome born to older mothers). The precise explanation as to why this might be the case has not been elucidated. In addition to this, no other authors have duplicated these results.
As mentioned previously, most patients with idiopathic scoliosis are female, and the vast majority of research has focused on females. One of the only articles written on idiopathic scoliosis in males is that by Karol et al, from the Texas Scottish Rite Hospital. These authors showed that boys with scoliosis are at risk for curve progression for a longer period than girls. They also suggested that efforts to screen for boys with scoliosis should be performed a little later than similar screenings for girls.27
Etiology
The precise etiology of idiopathic scoliosis remains unknown, but several intriguing research avenues exist.
A primary muscle disorder has been postulated as a possible etiology of idiopathic scoliosis. The contractile proteins of platelets resemble those of skeletal muscle, and calmodulin is an important mediator of calcium-induced contractility. Kindsfater and his colleagues from Denver studied the level of platelet calmodulin in 27 patients with adolescent idiopathic scoliosis.28 Using indirect measurement methods, these researchers had conducted previous work indicating that increased levels of platelet calmodulin were associated with increasingly severe idiopathic scoliosis. Using a direct measurement technique, they showed that patients with a progressive curve (>10° progression) had statistically higher platelet calmodulin levels (3.83 ng/mcg vs 0.60 ng/mcg, P <.01).28 If these data are reproduced in larger studies, they hold the potential to allow clinicians to identify patients at higher risk of curve progression.
An elastic fiber system defect (abnormal fibrillin metabolism) has been offered as one potential etiologic explanation for idiopathic scoliosis.29 Such abnormal connective tissue has not been found universally in patients with idiopathic scoliosis. No clear cause-and-effect relationship has been established. Further research in this area is clearly warranted.
Disorganized skeletal growth, probably with its root cause at a gene locus or group of loci, has been discussed as a possible etiologic explanation for idiopathic scoliosis. This theory is simply that a rather localized primary growth dysplasia leads to a cascading Hueter-Volkmann effect on a much larger portion of the spine.30 The Hueter-Volkmann principle states that compressive forces tend to stunt skeletal growth and that distractive forces tend to accelerate skeletal growth. A possible, yet unproven, association with such a growth disturbance is the osteopenia that has been identified in patients with idiopathic scoliosis.31
David Aronsson has conducted a series of experiments that have explored this mechanical modulation of growth. Using two different animal models (rats and calves), he showed that the force exerted by external ring fixators were quite capable of producing vertebral segment wedging akin to that seen in human idiopathic scoliosis.32, 33 Correlation of his laboratory information with the clinical setting has drawn attention to the fact that wedging occurs both from the vertebral bodies themselves and from the disk spaces, with a greater amount of thoracic wedging coming from the vertebral bodies.34 The asymmetric mechanical forces have also been associated with elevated synthetic activity in the convex side of scoliotic curves.35
Bylski-Austrow and Wall led a group of Cincinnati Children's Hospital researchers who further analyzed the mechanical modulation of spinal growth. Using a porcine model, they successfully induced growth changes by means of an endoscopically implanted spinal staple.36, 37, 38 Within the context of 8 weeks' follow-up, they were able to create 35-40° of scoliotic curvature in growing pigs. Histologic analysis of vertebral specimens revealed increased paraphyseal density and disorganized chondrocyte development in the region of the staple blades.
Genetic roots of the disease referred to as idiopathic scoliosis have been rather strongly suggested by several avenues of research. An X-linked inheritance pattern (with variable penetrance and heterogeneity) has been suggested by several authors.39 Studies of twins with scoliosis have pointed in a similar direction.40, 41. More than 90% of monozygotic twins and more than 60% of dizygotic twins demonstrate concordance regarding their idiopathic scoliosis.40 Some evidence has also directed attention to portions of chromosomes 6, 10, and 18 as possible scoliosis-related loci.42
Pathophysiology
Much has been written regarding the potential influence of melatonin on the development of idiopathic scoliosis.43 This has largely originated from studies in which the pineal gland was removed in chickens and scoliosis developed. These same studies suggested that the melatonin deficiency following pinealectomy might be the underlying reason for the development of scoliosis. Bagnall and his coauthors studied pinealectomized chickens to which they administered therapeutic doses of melatonin.44 They were unable to demonstrate any ability of the melatonin to prevent the development of scoliosis. It is fair to say that no final answer is yet available.
Some authors have suggested that a posterior column lesion within the central nervous system might be present in patients who have idiopathic scoliosis.45, 46 Such central nervous system dysfunction was hypothesized to be manifested as decreased vibratory sensation. McInnes and her fellow researchers later pointed out that the vibration device used in earlier studies (a Bio-Thesiometer) did not demonstrate sufficient reliability characteristics to allow valid conclusions.47 This line of research might be attractive to those who feel that a postural disturbance is the root cause of scoliosis.
Clinical
The vast majority of patients initially present due to perceived deformity. This may be patient or family perception of asymmetry about the shoulders, waist, or rib cage. A primary care physician or school-screening nurse may perceive similar findings. Adams forward-bending test (in conjunction with the use of a scoliometer) has been found to be an effective screening tool.
Highlights of the patient's history include information relative to other family members with spinal deformity, assessment of physiologic maturity (eg, menarche), and presence or absence of pain.
Traditionally, scoliosis has been described as a nonpainful condition, and aggressive workup has been recommended for patients in whom this rule is violated.48 Ramirez and his coworkers from the Texas Scottish Rite Hospital studied more than 2400 patients with scoliosis and found that a full 23% (560 of 2442 patients) had back pain at the time of presentation.49 An underlying pathologic condition was identified in 9% (48 of 560) of the patients with back pain, including mainly spondylolysis and spondylolisthesis but also intraspinal tumor in one instance. Thus, it would seem that pain is not associated with scoliosis as rarely as previously thought.
Physical examination should include a baseline assessment of posture and body contour. Shoulder unleveling and protruding scapulae are common. In the most common curve pattern (right thoracic), the right shoulder is consistently rotated forward and the medial border of the right scapula protrudes posteriorly. Assessment of lower (and often upper) extremity reflexes should be performed. Abdominal reflex patterns should also be assessed. The presence or absence of hamstring tightness should be investigated, and screening should be performed for ataxia and/or poor balance or proprioception (ie, Romberg test). One or two different methods of measuring leg length will prove valuable, as a significant percentage of patients presenting with scoliosis have several centimeters of limb-length discrepancy.
An extensive yet incomplete understanding of the natural history of idiopathic scoliosis remains a reality. Thus, more than a modicum of uncertainty remains associated with selection of recommended treatments for idiopathic scoliosis. The main treatment options for idiopathic scoliosis may be summarized as "the 3 O's": (1) observation, (2) orthosis, and (3) operative intervention. When to choose each of these treatments is a complicated matter.
The risk of curve progression varies based on the idiopathic scoliosis group in which a patient belongs (ie, infantile, juvenile, adolescent).
Infantile idiopathic scoliosis
Although defined by a seemingly arbitrary age limit (<3 y at the time of diagnosis), infantile idiopathic scoliosis demonstrates marked differences that distinguish it from the other 2 categories of idiopathic scoliosis. Infantile idiopathic scoliosis is the only type of idiopathic scoliosis whose most common curve pattern is left thoracic. Infantile idiopathic scoliosis is the only type of scoliosis that is more common in boys. It is more common in European patients or those of immediate European descent. In the past, infantile idiopathic scoliosis may have constituted up to 41% of all idiopathic scoliosis cases in parts of Europe, but the rate would appear to be closer to 4%. This is still dramatically higher than the estimated 0.5% rate in North America.50
Infantile idiopathic scoliosis is also the only type of idiopathic scoliosis with any significant reputation for spontaneous resolution. Reported spontaneous resolution rates range from 20-92%.20, 51 Ceballos et al studied 113 Spanish patients with infantile idiopathic scoliosis. They found a 92% rate of associated plagiocephaly and an almost 25% rate of congenital hip dysplasia.52 These same researchers found that nearly 74% of their patients' curves were of the resolving variety (mainly left thoracic curves) and the other 26% were progressive curves (mainly double primary type curves).52
Prediction of curve progression in infantile idiopathic scoliosis has been tied to assessment of the rib vertebral angle difference (RVAD) originally described by Mehta in 1972.53 As described by Mehta, this measurement is carried out at the apical vertebra of the curve. In instances in which the curves resolved spontaneously, the RVAD was less than 20° in about 80% of cases, and in those instances in which the curves were progressive, the RVAD exceeded 20° in about 80% of cases.53 Other authors have confirmed the prognostic value of the RVAD, as well as its reliable application.52, 54
Nonoperative treatment of progressive infantile idiopathic scoliosis predominates and may involve the use of conventional thoracolumbosacral orthosis (TLSO)–type braces, Milwaukee-type braces, and even intermittent Risser casting. Some have questioned the value of bracing in infantile idiopathic scoliosis and have stated, "a curve that resolves in a brace would probably have resolved without treatment."50
If surgical treatment becomes necessary, anterior release and fusion followed by posterior spinal fusion with instrumentation is considered to be the functional treatment. Every effort should be made to delay such surgical intervention as long as possible to optimize spinal growth, but relentless curve progression should not be accepted or tolerated while awaiting some arbitrary chronologic age. Although intuitively attractive, convex spinal epiphysiodesis (which has been shown to be quite effective in the management of congenital scoliosis) has not been shown to be as reliable in the setting of infantile idiopathic scoliosis.55 Addition of some type of posterior instrumentation may improve the results of epiphysiodesis.56
A treatment outline for infantile idiopathic
scoliosis may be as follows:
- Curves less than 25° with an RVAD less than 20° are preferentially observed and monitored with spinal radiographs at regular intervals.
- Curves exceeding these parameters are typically braced, with some consideration given to the value of intermittent Risser casting.
- Surgery is considered for curves not adequately controlled with nonoperative measures.
Juvenile idiopathic scoliosis
Juvenile idiopathic scoliosis most closely mimics the epidemiology and demographics of the adolescent version of the disease. It is more common in females, and its most common curve pattern is a right thoracic curve.21 In fact, due to its demographic similarities, high rate of progression, and need for surgery, juvenile idiopathic scoliosis might be considered to be a malignant subtype of adolescent idiopathic scoliosis. Robinson and McMaster studied 109 patients with juvenile idiopathic scoliosis in Scotland and found that 95% (104 of 109 patients) demonstrated curve progression and 64% (70 of 109 patients) progressed to require a spinal fusion.21 This spinal fusion rate is similar to that reported by J.I.P. James 15 years earlier.57
A study from Washington University found a 50% rate of neural axis abnormalities in young children (<10 y) with idiopathic scoliosis.58 These findings included Chiari type I malformations and dural ectasia. At least one case report also exists in which a spinal intraosseous arteriovenous malformation was found in association with juvenile scoliosis.59
One potential treatment algorithm for juvenile
idiopathic scoliosis is as follows:
- Observation for curves less than 25° with follow-up radiographs at regular intervals
- Bracing for curves that range from 25-40° and at least consideration of bracing (based on curve flexibility) for curves from 40-50°
- Bracing for smaller curves that demonstrate rapid progression to the 20-25° range
- Surgical intervention for inflexible curves that exceed 40° or virtually any curve that exceeds 50°.
Bracing and casting may be used outside the above-mentioned parameters in an effort to help control a large curve in a young child for whom the surgeon is attempting to optimize spinal growth. Similar recommendations exist regarding the value of MRI in juvenile idiopathic scoliosis due to a significant rate of neural axis abnormalities.58
Adolescent idiopathic scoliosis
Adolescent idiopathic scoliosis is the most common type of idiopathic scoliosis and the most common type of scoliosis overall. Progressive curvature may be predicted by a combination of physiologic and skeletal maturity factors and curve magnitude. Small curves in more mature patients have a substantially lower risk of progression (about 2%) than larger curves in more immature patients, in whom the risk is much higher (risk may approach or exceed 70%).
Treatment recommendations for adolescent idiopathic scoliosis are driven almost totally by curve magnitude (the only caveat being that brace treatment is thought to be effective only in patients who are still growing). It is thus somewhat ironic to note that stated recommendations urge observation for curves less than 30°, bracing of curves that reach the 30-40° range, and consideration of surgery for curves that exceed 40°. This amounts to a 10° window between observation and major spinal surgery. It is even more ironic to note that 10° is a commonly discussed margin of error for measuring such scoliotic curves. Additional patient factors may also influence some orthopedic surgeons to brace patients with curves measuring less than 30° or in excess of 40°. For instance, a rapidly progressive curve in a 12-year-old child that suddenly goes from 16-26° may easily prompt bracing.
When it comes to surgical considerations, patients with adolescent idiopathic scoliosis may be functionally subdivided into those patients in whom significant anterior spinal growth is a concern and those in whom it is not. This amounts to a quantification of risk of development of the complication known as crankshaft phenomenon.60 This can have a major impact on the surgical treatment plan in that a child at significant risk for crankshaft phenomenon will require an anterior spinal fusion procedure.
Much effort has been devoted to predicting which patients may suffer from this continued anterior spinal growth that results in progressive angulation and rotation of the spine.60, 61, 62, 63, 64. In fact, a hierarchy of risk can be constructed in which progressively more precise estimates can be made. In this hierarchy, the presence of a radiographic Risser sign and reaching menarche are somewhat predictive but less so than closure of the triradiate cartilage, and reaching one's peak height velocity is perhaps the most powerful predictor of being at rather low risk for the crankshaft phenomenon.
The anatomy relevant to idiopathic scoliosis is that of the thoracic and lumbar spine. Key points regarding developmental anatomy of the spine are outlined below. Scoliosis surgery is usually still performed via a posterior approach to the spine; thus, significant discussion of posterior anatomy is provided. A growing appreciation and need for anterior surgical procedures for scoliosis also demands additional discussion of retroperitoneal anatomy and intrathoracic anatomy, especially as it relates to video-assisted thoracoscopic surgery (VATS).
Developmental anatomy
Significant growth, development, and differentiation occur as a single-celled zygote progresses to become an approximately 100 trillion–celled adult human. Identifiable spine development has begun by the third week of gestation. First, the neural tube forms. Later, paired somites appear (at 4.5 weeks' gestation) and spinal nerves are present by the sixth gestational week. A discernible cartilage model of the spine is present by the seventh week of gestation. The bone and cartilage of the spine are mesodermal derivatives, as are significant portions of the cardiovascular and urogenital systems. This explains the frequent coexistence of congenital spine anomalies with congenital cardiac and kidney defects. Thus, gestational weeks 3-7 are very important in the development of all of these major body systems.
Postnatal spinal growth also must be understood and appreciated. Alain Dimeglio has shown that the majority of spinal canal diameter (about 90%) has been achieved by age 5 years.65 By age 10 years, approximately 80% of sitting height has also been achieved.66 During adolescence, radiographic evidence of ossification of the growth cartilage of the vertebral bodies occurs. Prior to this, these completely cartilaginous growth plates remained nestled between their respective vertebral bodies and intervertebral disks.
Posterior anatomy
The major superficial muscles of the back are not often directly visualized during posterior surgical approaches for scoliosis, but they must not be forgotten. These muscles include the trapezius, rhomboid major, rhomboid minor, and latissimus dorsi. Using an animal model, Kawaguchi et al showed that significant posterior muscle injury can be induced by the pressure exerted by surgical retractors.67 This certainly makes a case for intermittent removal and replacement of such retractors during the course of posterior spinal surgery.
The route for exposure of the posterior spinal elements passes through the cartilaginous apophyses of the spinous processes. These structures, often referred to as the cartilaginous caps, are systematically split in the midline to allow sequential subperiosteal dissection of the spinous processes, laminae, facet joints, and transverse processes. The laminae of the thoracic vertebrae spread out from the midline like wings and flow upwards (cranially) in the direction of the transverse processes. The facet joints of the thoracic spine are shingled in a coronal plane such that the inferior facet that contributes to each joint is located posteriorly and the superior facet is located anteriorly. The thickness of the interior and superior facets of the thoracic spine range from about 3-5 mm.68 The thoracic facet joints are located a mere 7-11 mm from the midline of the posterior spine.68
Progressing from the thoracic to the lumbar spine, important differences are noted. The V-shaped laminae of the thoracic spine give way to the butterfly-shaped laminae of the lumbar spine. This orientation change is important for the surgeon to remember when exposing these bony elements. The facet joints of the thoracic spine, which are oriented in more of a coronal plane, transition into the more sagittally oriented facet joints of the lumbar spine. The transverse processes of the thoracic spine, which seem to flow directly up and away from the laminae, change significantly in the lumbar spine such that they are no longer in close proximity to the laminae and are located anterior and inferior to the lumbar facet joints.
The ribs are also obviously absent in the lumbar vertebrae. What some consider a rib remnant does persist and is referred to as a mamillary body or mamillary process. It is most pronounced near the thoracolumbar junction but may be identified on nearly all of the lumbar segments. In the sagittal plane, one must also appreciate that the normal gentle kyphosis of the thoracic spine reaches its apex at about the T7 through T9 region. Below this, a rather definite transition to lumbar lordosis occurs, with an apex around the L3 level. Thoracic kyphosis typically ranges from 20-40° (Cobb measurements usually taken from the top of T3 to the bottom of T12). Some authors have stated that up to 50° of thoracic kyphosis should be considered normal.69 Normal lumbar lordosis is considered by some to range from 35-55° (Cobb measurements usually taken from the top of L1 to the top of L5).
Anterior anatomy
Anterior scoliosis surgery involves 3 main
strategies, as follows:
- Anterior lumbar or thoracolumbar surgery through a retroperitoneal approach that may or may not involve a diaphragmatic incision
- Anterior thoracic surgery via traditional open thoracotomy
- Anterior thoracic surgery via VATS
Various factors relative to skeletal maturity, curve location, and curve flexibility help determine which (if any) of these anterior surgeries may be appropriate.
The most common reason to use the retroperitoneal approach is for an instrumented anterior thoracolumbar spinal fusion. The most common curve pattern in that particular type of scoliosis is an apex left curve pattern, and as such, the patient is usually positioned lying on the right side. This position is advantageous in that it provides the best access to the scoliotic spine and it also places the thick-walled aorta closer to the surgical field (as opposed to the thin-walled inferior vena cava). After superficial muscle dissection, the surgeon approach proceeds through the bed of the rib that corresponds with the highest vertebrae in which instrumentation is planned. This is often either the ninth or tenth rib, with the rib itself being harvested for later use as a bone graft.
Careful dissection is then performed to mobilize the peritoneum (with its contents) in an anterior direction; it is peeled off of the undersurface of the diaphragm. Posterior division of the diaphragm (leaving about a 2-cm cuff for repair) helps to avoid damage to the phrenic nerve. Diaphragmatic division begins with splitting of the costal cartilage and proceeds in a posterior direction with intermittently placed tagging sutures to aid in closure.
The remainder of the retroperitoneal approach to the thoracolumbar spine requires careful superior retraction of the lung, anterior retraction of the peritoneum (with associated aorta and ureter), and posterior retraction of the iliopsoas musculature. Careful identification and division of the segmental vessels (overlying the vertebral bodies) is carried out with either electrocautery or ligatures. Small sympathetic nerve branches in this same area are sacrificed during this stage of the exposure. This results in at least a transient period in which the left foot (for a left-sided approach) will be both pinker and warmer than the contralateral foot. At times, this may result in nursing personnel notifying the surgeon that the contralateral foot is pale and cold, but in reality, the foot ipsilateral to the exposure has changed.
Open thoracotomy might be performed either for anterior thoracic spine release followed by posterior fusion or for anterior thoracic spine fusion with instrumentation. The most common curve pattern to address with this approach would be a right thoracic curve, and as such, the patient would be positioned with the right side upward.
A similar rib selection and resection technique may be used if desired. From the interior of the chest, the intercostalis musculature (located between each of the ribs) can be seen. Identification of the azygous vein (anteriorly oriented along the vertebral bodies) is necessary. Further medial (ie, central) and running parallel to the azygous vein is the thoracic duct. Several portions of the sympathetic chain may be sacrificed as the segmental vessels overlying the thoracic vertebral bodies are divided and mobilized anteriorly and posteriorly. Blood flow changes similar to those noted in the retroperitoneal approach may be noted in the right foot (for a right thoracotomy).
In addition to this, thoracic surgical dissection carries with it the possibility of sacrificing branches to the greater splanchnic nerve, which would theoretically decrease visceral referred pain that one might feel from an inflamed gallbladder or similar condition.
Thoracoscopic appreciation of the anatomy of the thoracic spine is becoming more common as endoscopic anterior release and fusion is rapidly moving from being considered an innovation to standard practice. Just as arthroscopic knee surgeons enjoyed an expansion in visualized anatomy compared to that visible with knee arthrotomies, the endoscopic spine surgeon benefits from much greater intrathoracic latitude. Most VATS also involve the right thoracic cavity, and this discussion focusses on that particular side.
Proper rib counting and visualization of the superior intercostal vein (formed by the confluence of the second, third, and fourth intercostal veins) as it empties into the azygous vein are necessary steps to orient the surgeon. Beyond this, one also notes the mounds and valleys of the thoracic spine, with the mounds being the disks and the valleys being the vertebral bodies with the segmental vessels that overly them.70
The same anatomy outlined in the thoracotomy discussion still clearly applies, but further endoscopic fine points are needed. Specifically, the thoracic spine may be considered to be composed of 3 separate fields with important anatomic nuances.71 The upper field may be considered to be T2-T5, the middle field may be considered to be T6-T9, and the lower field may be considered to be T10-L1.71 The upper field is dominated by the superior intercostal vein, and it is characterized by the fact that the rib heads tend to completely span their respective disk spaces and articulate with 2 vertebral bodies. This results in a rib such as the third rib coming directly into the region of the T2-T3 disk space such that it will articulate with both the T2 and T3 vertebral bodies. In the middle field, the rib head once again comes directly in toward the disk space, but now, it rather firmly attaches itself only to the disk space proper.
In the lower field, the rib head articulates directly with its corresponding vertebral body. Thus, in the lower field, the 11th rib is traced to its corresponding vertebral body and then moves directly cephalad to reach the T10-11 disk or directly caudad to reach the T11-12 disk. Once the vertebral bodies have been exposed in a skeletally immature patient, the growth cartilage of the vertebral endplate can be visualized. It has an odd tendency to appear green in color (a quirk of endoscopic optics) and is colloquially referred to as a Wolf line in honor or Randall K. Wolf.
Few, if any, absolute contraindications exist regarding scoliosis care, just as few, if any, absolute indications for intervention exist. Accepted contraindications for bracing include skeletal maturity and excessive curve magnitude. Thoracic lordosis and certain curve patterns such as double thoracic curves also have been offered as at least relative contraindications to bracing.
The main contraindication to posterior scoliosis surgery would be medical instability and inability to survive surgery. Anterior scoliosis surgery would also be contraindicated in these patients, as well as in those with a precarious pulmonary status.
Lab Studies
- Laboratory workup for patients with scoliosis consists primarily of preoperative testing. Most, if not all, patients undergo preoperative assessment of hemoglobin and hematocrit levels. Autologous blood predonation is also a common practice.
Imaging Studies
- Multiple authors have cited the value of bending radiographs, including those over a fulcrum.72 Klepps and Lenke et al found that thoracic fulcrum bending radiographs worked best for them when dealing with isolated main thoracic curves.73
-
- The thoracic curve patterns found in adolescent idiopathic scoliosis are still most commonly classified using the King classification system.74 Significant questions have been raised regarding the reliability and reproducibility of the King classification system.75, 76 In addition to this, the King classification alone (in its original form) does not allow comprehensive curve classification (eg, lumbar and thoracolumbar curve patterns).77
- Multiple authors have analyzed the ability of orthopedic surgeons to reliably measure scoliosis radiographs. Morrissy and his colleagues used 50 radiographs and 4 examiners (2 experienced orthopedic surgeons, 1 fellow, 1 senior resident) to study their ability to make Cobb angle measurements. With the examiners choosing end vertebrae and measuring scoliotic curves accordingly, intraobserver variability was 4.9°.78 Carman and her coworkers used 8 scoliosis radiographs measured by 5 examiners (4 orthopedic surgeons, 1 physical therapist) to evaluate interobserver and intraobserver variation. They found that a 10° measurement difference is necessary before there is a 95% confidence level that one Cobb angle measurement is truly different from another.79
- MRI has been suggested to be primarily indicated in patients with idiopathic scoliosis with unusual complaints such as severe unexplained headaches and when clinical findings such as ataxia or cavus feet are present.80 Routine MRI evaluation of all patients with adolescent idiopathic scoliosis is not recommended.
Other Tests
- Pulmonary function studies have been used rather extensively in the evaluation of patients with idiopathic scoliosis.19, 81, 83
-
- In general, patients whose scoliosis surgery does not involve disruption of their chest wall can be expected to experience improved postoperative pulmonary function.84 Other authors have suggested that an impairment in respiratory mechanics may persist following successful scoliosis surgery.85
- Preoperative pulmonary function testing is of questionable value in patients with moderate deformity (average Cobb angle 48°), as most of these patients can be expected to have normal or only mildly abnormal results.86
- Efforts at screening for scoliosis (most often in school populations) have met with mixed success. A 2-year evaluation of more than 80,000 Greek 9- to 14-year-old students screened by their schools using the Adams forward-bending test was conducted by Soucacos et al. Overall, they found school screening to be simple and effective. These authors found that they identified 181 new children with scoliosis requiring treatment (11 surgically, 170 with bracing).87
- Peak height velocity has been studied rather extensively as a predictor of curve progression.88
Histologic Findings
Scoliosis is clearly a disease that is strongly influenced by, if not completely rooted in, spinal growth. It has even been referred to by some as "an unsynchronized growth."89
Hsu and coworkers from Vanderbilt studied muscle biopsies from 27 patients with idiopathic scoliosis who were undergoing posterior spinal fusion. Specimens were obtained from the paraspinal musculature of both the convex and concave side in all patients. All patients had thoracic curves that ranged from 37-81°.90 Sixty-eight percent of the patients demonstrated abnormalities in muscle fiber distribution. The abnormalities were similar on the convex and concave sides, the most notable being a reversal of the normal type 2-fiber ratio such that type 2-A fibers predominated over type 2-B fibers in the study subjects. These changes are similar to those seen in endurance training and might be due to the extra work of trying to maintain posture in the setting of scoliosis.90
Medical therapy
Nonoperative management consists of either mere observation or orthosis use. Observation is watchful waiting with appropriate intermittent radiographs to check for the presence or absence of curve progression. Orthosis use for scoliosis is discussed extensively below. No other treatments, including electrical muscle stimulation, physical therapy, spinal manipulation, and nutritional therapies, have been shown to be effective for managing the spinal deformity associated with idiopathic scoliosis. The lack of demonstrated effectiveness in this context means that scientifically valid studies have either been done that do not support the treatment or no such studies have yet been published that would allow an evidence-based evaluation.
The first widely used scoliosis brace with proven effectiveness was the Milwaukee brace. This brace was developed by Walter Blount and Albert Schmitt and introduced at a meeting of the American Academy of Orthopaedic Surgeons in 1946.91 The brace was originally designed to be used as part of the surgical treatment of scoliosis and only later evolved into a stand-alone nonoperative treatment.
Lonstein and Winter studied 1020 patients with adolescent idiopathic scoliosis treated with the Milwaukee brace. They reported that this orthosis was effective in preventing significant curve progression in patients with 20-39° curves.92 These same authors recommended that adolescents with a curve of 25° and a Risser sign of 0 be braced immediately and not wait for evidence of curve progression.92 Other authors have shown that an average curve correction of 20% in the brace (Milwaukee brace) is associated with bracing success.93, 94
Rowe and his colleagues performed a meta-analysis
aimed at evaluating the efficacy of nonoperative
treatments for idiopathic scoliosis.95 They calculated the
weighted mean proportion of success for 3 nonoperative
treatments: observation, electrical stimulation, and
bracing. They were able to successfully combine data on
1910 patients from 20 different studies for purposes of
meta-analysis. Their main results are as follows
(treatment, success rate):
- Observation, 49%
- Electrical stimulation, 39%
- Bracing 8 hours per day, 60%
- Bracing 16 hours per day, 62%
- Bracing 23 hours per day, 93%
In a prospective, multicenter study from the Scoliosis Research Society, Nachemson and his coworkers found brace treatment (an underarm plastic brace worn for at least 16 h/d) to be successful 74% of the time (95% confidence interval [CI], 52-84%).96 Some authors have not been able to identify a major difference between full-time bracing (23 h/d) and part-time bracing (12-16 h/d).97
The psychological stress associated with scoliosis has been documented,98 and this does not improve compliance with brace wear. MacLean and his coauthors from Vanderbilt studied 31 adolescent and preadolescent females who were undergoing part-time brace treatment for their idiopathic scoliosis.99 Part-time bracing was defined as 13-16 hours per day. Eighty-four percent of their patients described the initial period of bracing in "stressful terms" and experienced lower levels of self-esteem.99 A reassuring finding is that overt psychopathology was not identified in MacLean's study.
Compliance with prescribed brace-wear regimens has been shown to be poor. DiRaimondo and Green found that, on average, patients only wore their braces 65% of the prescribed amount of time.100 Patients prescribed part-time bracing (16 h/d) actually demonstrated worse compliance (58%) than those prescribed full-time (24 h/d) bracing (71%).100 Overall, only 15% of patients demonstrated a highly compliant (>90%) brace-wear routine.100
Questions have also been raised regarding the consistency of strap tension in thoracolumbosacral orthosis (TLSO) bracing.101 Using an instrumented load cell to measure strap tension, Aubin et al studied 34 of their patients with braces in Quebec. They found marked variability in tension, with the greatest change occurring while patients were recumbent.101
In part due to the aforementioned psychological and brace-wear compliance issues, new approaches to bracing are being developed.102, 103 One such approach is that developed by Dr. Christine Coillard and Dr. Charles Rivard of the St. Justine Hospital in Montreal, Canada. Their dynamic bracing approach is referred to as the SpineCor Brace or as the St. Justine Brace.104 It involves elastic straps that are anchored on a pelvic corset, and based on curve morphology, these straps are tensioned to exert corrective forces. The brace is a radical departure from traditional plastic and metal orthoses. Early results with the St. Justine Brace are rather encouraging, with success rates comparable to those of traditional bracing. Continued follow-up of their growing international cohort of patients is necessary.
Surgical therapy
Even in the setting of adequate correction and solid fusion, up to 38% of patients still have occasional back pain.76, 105
The primary goal of scoliosis surgery is to achieve a solid bony fusion. The surgical technique used to achieve such an arthrodesis is vastly more important than the instrumentation system that the surgeon needs to use, if any.18, 106
Modern instrumentation systems have been shown to allow for adequate curve correction but with little or no ability to diminish associated rib humps.107 Despite claims of certain instrumentation systems to derotate the spine, little actual derotation has been documented. Derotation of the instrumented curve also has been shown to possibly occur at the expense of creation of new rotation in uninstrumented portions of the spine.108
Previously, much attention was paid to the ability of certain spinal instrumentation systems (eg, Cotrel-Dubousset to derotate the spine during scoliosis correction. Jarvis and Greene showed that use of the Wisconsin segmental spinal instrumentation (a system traditionally thought to not be associated with significant spinal derotation) was associated with spinal derotation equal to or greater than that of the Cotrel-Dubousset–type systems.109
Since 1993, VATS has been used in the anterior treatment of pediatric spinal deformity at Cincinnati Children's Hospital Medical Center.110 This minimally invasive surgical technique is aimed at decreasing operative morbidity and optimizing patient recovery from surgery. Over 100 of these procedures have been performed at this institution. Initial biomechanical studies in animal models have correctly predicted what clinical practice has now borne out—that endoscopic anterior release and diskectomy is as effective as the open version of the operation.70, 96 111, 112 Endoscopic spinal instrumentation techniques have also been introduced and continue to evolve.113
Preoperative details
Preoperative evaluation focuses on specifics of curve location, magnitude, and flexibility. These parameters are used in conjunction with patient maturity factors to determine optimal treatment choice, but definitive studies are not yet available that dictate specific surgical tactics. However, the scoliosis surgeon is aided by commonly applied clinical guidelines that have evolved over time. The goal is always to fuse as little of the spine as possible while adequately treating existing major curvature.
For a thoracic curve (with adequate flexibility) without any significant associated lumbar curvature, the most common surgical approach has not changed since the days of Paul Harrington: posterior spinal fusion with instrumentation. Surgeons may choose from a diverse array of anchors to secure large-diameter rods (usually in the 0.25-in range) to the spine. These anchors include sublaminar hooks, pedicle hooks, transverse process hooks, sublaminar wires (Luque wires), spinous process wires (Drummond wires), and pedicle screws. Some surgeons have advocated anterior spinal fusion and instrumentation for such isolated thoracic curves. These have included both open (thoracotomy) and limited-incision (thoracoscopic) techniques.
When the primary problem is a large, stiff thoracic curve (usually not bending less than 50°), a different surgical tactic is usually undertaken in which an anterior release (usually including diskectomy and bone grafting) is performed prior to posterior spinal fusion and instrumentation. Anterior spinal fusion and instrumentation has also been advocated in this situation, provided the patient does not have excessive kyphosis associated with a large thoracic curve.
Large curve patterns that include both thoracic and lumbar deformity continue to challenge scoliosis surgeons. If adequate flexibility and balancing of the lumbar spine is possible, then selective fusion of the thoracic curve is possible. When this is not the case, extensive fusion (at times down to the fourth lumbar segment) may become necessary.
The Scoliosis Research Society has a rather specific definition of thoracolumbar scoliosis: a curve whose apex lies at the body of T-12 or L-1 or at the T12-L1 interspace. These curves are most commonly left-sided curves and they present one of the most common scenarios in which anterior spinal fusion and instrumentation is utilized. Anterior approaches to this area of the spine were pioneered by Hodgson (Hong Kong), Dwyer (Australia), and Zielke (Germany). Current approaches represent further refinement of these original techniques, such as modern large rod-and-screw systems and the John Hall short anterior segment overcorrection technique. The value of such techniques lies in their ability to powerfully correct large thoracolumbar curvatures while minimizing fused segments within the lumbar spine.
There is little debate regarding the fixation of the rods used for anterior instrumentation. Large bone screws are almost always the anchor of choice. For posterior instrumentation procedures, the surgeon has more options. Multiple hooks are the most commonly used anchors. They offer simplicity, strength, and near complete visualization during insertion. Their main drawbacks relate to size mismatch between hooks and associated bony elements, as well as the absence of appropriate hook sites (such as might be the case in myelomeningocele, tumor cases, or revision surgeries).
Sublaminar wires offer the power of segmental fixation and firm bony purchase, but with the drawback of possible dural and/or spinal cord trauma. As a result, either very selective use of or no use at all of sublaminar wires is usually the case in the setting of idiopathic scoliosis. A reasonable compromise was achieved when Denis Drummond introduced his spinous process wires (also known as Wisconsin wires). These devices still offer the power of segmental fixation with virtually none of the nerve injury risks of sublaminar wires.
Pedicle screws have also become a popular anchor for the rods used in posterior scoliosis fusion procedures.114 They offer the potential advantage of increased strength (and possibly power of correction) while at the same time introducing added insertion-technique complexity and different neurologic complication risks. A very real and major increase in the overall cost of instrumentation constructs that include many pedicle screws is the case when comparing them to similar constructs that may include hooks and wires. At this time, evidence is not conclusive to support a commensurate improvement in clinical outcomes to support the routine use of such pedicle screw constructs in the treatment of idiopathic scoliosis.
Pulmonary function testing is commonly used in the preoperative evaluation of patients with idiopathic scoliosis who are slated to undergo surgery. Such testing may influence the surgeon's enthusiasm for related procedures, such as costoplasty (thoracoplasty). Pulmonary function testing may also uncover previously unrecognized tobacco use (an independent risk factor for pseudarthrosis) or undiagnosed (subclinical) pulmonary disease.
Predonation of several units of donor-directed blood is considered standard for most patients. Certain commercially available intraoperative blood recovery devices may also be used at times.
Intraoperative details
Hoppenfeld described an ankle clonus test for intraoperative assessment of the integrity of the spinal cord during scoliosis surgery. In more than 1000 patients, the test was noted to have no false-negative results and 3 false-positive results. This translated into 100% sensitivity and 99.7% specificity.115
Postoperative details
Postoperative patient management involves close monitoring, which often occurs initially in an intensive care unit setting. Patients have monitoring devices, such as arterial lines, and closed suction devices, such as chest tubes, that also require special nursing attention. The use of certain special spine-specific hospital beds, such as the Stryker frame, may also aid in patient care and comfort (change from supine to prone position) during the initial postoperative period.
The use of postoperative bracing varies from surgeon to surgeon. As outlined in History of the Procedure, the roots of scoliosis surgery involved immobilization in a body cast. Following the development of initial instrumentation systems (eg, Harrington instrumentation), external immobilization was still used routinely. With the advent of large-rod multiple-hook constructs, such as the Cotrel-Dubousset system and its direct decendents, bracing has been deemphasized a bit. Thus, it is almost as likely that a patient will not receive a postoperative brace as receive one, whereas, previously, bracing was much more widespread. In certain specific circumstances, postoperative bracing is still almost always used, such as anterior thoracic or thoracolumbar instrumentation procedures or surprisingly weak bone stock.
When a brace is used, it is typically to be worn full-time for at least 6 weeks, followed by a period in which the brace may be taken off for bathing, with subsequent progressive weaning. As a rule of thumb, patients may also miss up to 6 weeks of school (if their procedure is done at such time of the year), and up to 6 months may be required before they resume most of their normal activities. Vigorous sports may be restricted for at least a year or, in some instances, permanently (based on risk-versus-benefit discussions between patients, families, and their surgeons).
Follow-up
At an average of 21 years following posterior spinal fusion with Harrington instrumentation (performed by Paul Harrington himself), about 21% of patients experienced significant interscapular pain.116
Complications are of great concern to parents, patients, and surgeons. Thankfully, complications are rare with modern scoliosis surgery, despite the magnitude of these spinal deformity procedures. Several important intraoperative, early postoperative, and late postoperative complications are discussed here.
McKie and Herzenberg described coagulopathy as a
complication of intraoperative blood salvage during
scoliosis surgery.117 These authors
suggested that thrombin and Gelfoam that may have been
aspirated along with salvaged blood may have contributed
to the disseminated intravascular coagulation
experienced by their 17-year-old patient. This effect of
the thrombin and Gelfoam would have been in addition to
that of hemodilution (hemodilution-induced platelet and
leukocyte activation syndrome).117
The
importance of appropriate intraoperative spinal cord
monitoring during scoliosis surgery is hardly debatable.
Such monitoring can allow early recognition and
treatment of spinal cord dysfunction.118 Somatosensory and
motor evoked potentials are commonly used to monitor
spinal cord function. A Stagnara wake-up test may also
still be employed if the surgeon desires. Current
efforts at monitoring have helped achieve and maintain a
very low rate of spinal cord injury (less than one half
of one percent).
Some concern exists regarding postoperative activity level and the possible hazards of trauma. Neyt and Weinstein have reported a case of lumbar spine fracture dislocation in a teenage boy 3 years after successful scoliosis surgery.119 The boy's fusion extended from the second thoracic vertebra to the first lumbar vertebra, and his subsequent fracture dislocation occurred at the L2-3 level.119
Delayed infections following posterior spinal fusion with Texas Scottish Rite Hospital instrumentation has been reported. Richards reported on 10 such patients who presented with infections at an average of about 2 years following successful spinal fusion.120 Low-virulence organisms such as Propionibacterium acnes were the main cause, and instrumentation removal was successful in eradicating the infections. Richards hypothesized that the infections might be related to the amount of hardware (eg, hooks, rods) used and suggested that efforts at minimizing such hardware might help prevent such infections.120
Much has been written regarding a particular complication called crankshaft phenomenon. It may occur following posterior spinal fusion of idiopathic scoliosis in patients who have significant anterior spinal growth remaining. Sanders and coauthors reported that the risk of the crankshaft phenomenon was highest in patients younger than 10 years and in patients with a Risser sign of 0 with an open triradiate cartilage.121
Significant concern exists regarding the inferior (caudad) extent of a patient's spinal fusion and its potential relationship with future low back pain.122 Connolly led a group of researchers at the Toronto Hospital for Sick Children who studied this question in 83 patients fused with Harrington instrumentation to the second, third, fourth, or fifth lumbar vertebra. At an average of 12 years (range 10-16 y) following their surgery, these patients were found to have a statistically higher rate (76%) of low back pain than a control group (50%). Connolly's patients were from an era in which the predominant instrumentation system was noncontoured Harrington rods, which were notoriously associated with low back pain when applied to the lumbar spine.122 The results of this study almost certainly cannot be generalized to current scoliosis patients, who are treated with very different instrumentation systems.
Some complications have been associated with particular surgical approaches to scoliosis. For instance, chylothorax and tension pneumothorax have both been reported in association with video-assisted thoracoscopic surgery (VATS) procedures.123, 124
Pseudarthrosis is a complication that represents a basic failure of the central intention of scoliosis surgery: bone fusion. Luckily, pseudarthrosis is very rare in modern scoliosis surgery. This is in small part due to excellent stable internal fixation (scoliosis instrumentation systems) and in large part due to proper attention to fusion technique. Pseudarthrosis may be suggested by persistent pain, progressive deformity, or broken hardware. Previously tomographic plain x-rays (tomograms) were commonly used to image suspected pseudarthrosis. This is no longer the case, as such tomography equipment is on the endangered species list of imaging devices. As such, computed tomography may be helpful, but clinical suspicion and fusion mass exploration (a rare case for modern-day exploratory surgery) remain the cornerstones of pseudarthrosis diagnosis and treatment.
Clinical outcomes following treatment of idiopathic scoliosis are strongly linked to curve magnitude. Unrealistic presurgical expectations have been shown to correlate with a decreased likelihood of postsurgical satisfaction.125 More long-term follow-up studies of surgically treated patients with scoliosis are becoming available. This section outlines some of these data.
A large cohort (nearly 2000 subjects) of patients with idiopathic scoliosis in Montreal, Canada, referred to as the St. Justine Cohort Study, has been monitored for 10-20 years. These patients were compared to a population-based control group drawn from the general Quebec population. Compared to the general population and regardless of whether their scoliosis was treated surgically or nonsurgically, patients with scoliosis were found to have a higher self-reported rate of arthritis and poorer perceptions of their overall health, body image, and ability to participate in vigorous activities.126, 127
A subset of the cohort (700-1500 patients) was analyzed further regarding low back pain.128, 129 These Canadian researchers found a higher overall rate of significant back pain reported within the last year (75% of patients with scoliosis versus 56% of control subjects).128 Patients with scoliosis who were treated surgically also reported a high rate (73%) of back pain within the last year, but it did not correlate with the distal extent of the spinal fusion. The St. Justine authors went on to state that their study "does not provide any evidence that extending the level of fusion down even as far as L4 will increase the prevalence of back pain in adulthood."129
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