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Seminar

Adolescent idiopathic scoliosis


Stuart L Weinstein, Lori A Dolan, Jack C Y Cheng, Aina Danielsson, Jose A Morcuende

Adolescent idiopathic scoliosis (AIS) affects 1–3% of children in the at-risk population of those aged 10–16 years. The Lancet 2008; 371: 1527–37
aetiopathogensis of this disorder remains unknown, with misinformation about its natural history. Non-surgical Department of Orthopaedic
treatments are aimed to reduce the number of operations by preventing curve progression. Although bracing and Surgery and Rehabilitation,
University of Iowa, Iowa City,
physiotherapy are common treatments in much of the world, their effectiveness has never been rigorously assessed.
Iowa, IA, USA (S L Weinstein MD,
Technological advances have much improved the ability of surgeons to safely correct the deformity while maintaining L A Dolan PhD,
sagittal and coronal balance. However, we do not have long-term results of these changing surgical treatments. Much J A Morcuende MD);
has yet to be learned about the general health, quality of life, and self-image of both treated and untreated patients Department of Orthopaedics
and Traumatology, Chinese
with AIS.
University of Hong Kong, Hong
Kong SAR, China
Introduction measurement of the degree of curvature in each affected (J C Y Cheng MD); and
Adolescent idiopathic scoliosis (AIS) is a structural, individual and identification of any scoliosis in the family Department of Orthopaedics,
Sahlgrenska University
lateral, rotated curvature of the spine that arises in are of paramount importance for future genetic studies. Hospital, Göteborg University,
otherwise healthy children at or around puberty. The Several investigators have reported candidate-gene Gothenburg, Sweden
diagnosis is one of exclusion, and is made only when analyses of connective tissue genes. Results of these (A Danielsson MD)
other causes of scoliosis, such as vertebral malformation, studies have excluded genes for fibrillin 1 (FBN1) and Correspondence to:
neuromuscular disorder, and syndromic disorders, have 2 (FBN2); collagen type I (COLα1) and II (COLα2); elastin Dr Stuart L Weinstein,
Department of Orthopaedic
been ruled out. Patients are generally screened with (ELN); aggrecan (ACAN); and heparan sulfotransferases
Surgery and Rehabilitation,
Adams’ forward bend test and a scoliometer, although a (HS3ST3A1 and HS3ST3B1) as causes of AIS.8,12–14 Inoue University of Iowa, Hospitals and
definitive diagnosis cannot be made without measuring and colleagues15 studied polymorphisms in the genes for Clinics, 200 Hawkins Drive, Iowa
the Cobb angle on a standing coronal radiograph vitamin D receptor (MED4), oestrogen receptor (ESR1), City, Iowa, IA 52242, USA
stuart-weinstein@uiowa.edu
(figure 1). When defined as a Cobb angle of at least 10°, and CYP17A1 in relation to curve progression. The results
epidemiological studies estimate that 1–3% of the at-risk suggested that XbaJ site polymorphism in ESR1 was
population (children aged 10–16 years) will have some associated with curve progression.15,16 However, Tang and
degree of spinal curvature, although most curves will colleagues17 did not show an association between ESR1
need no intervention.1,2 In this Seminar we discuss and AIS.
present notions about aetiopathogenesis, natural history, On the basis of the hypothesis that AIS is related to an
non-operative treatment, and surgery. abnormality in melatonin metabolism, Morcuende and
colleagues18 investigated several known receptors, such as
Aetiopathogenesis hMel1A, hMel1B, and ROR-α, but reported no evidence
Despite much clinical, epidemiological, and basic science of mutations in the coding regions of these genes.
research, the aetiopathogenesis of AIS remains Gao and colleagues19 discovered a potentially functional
unknown.3,4 AIS is often seen in multiple members of polymorphism in CHD7 that is over-transmitted to affected
one family, which strongly suggests that it has a genetic offspring and predicts disruption of a caudal-type (Cdx)
component. A meta-analysis of studies of twins showed transcription-factor binding site. They suggest that this gene
concordance for AIS in 73% of monozygotic twins and is associated with the susceptibility of AIS and propose an
36% of dizygotic twins.2 Several studies that assessed overlap of causes between the rare, early onset CHARGE
large pedigrees showed different methods of inheritance,
such as autosomal dominance, maternal factors, multiple
gene inheritance, multifactorial inheritance, and X-linked Search strategy and selection criteria
dominance.3,5 However, Axenovich and colleagues6 We searched the Cochrane Library and Medline from 1996
analysed pedigrees of 101 families (778 individuals) and to 2006 for relevant literature and entered the following
undertook a complex segregation analysis. They noted search terms: “adolescent idiopathic scoliosis”, “late onset
that genetic control of severe forms of AIS could be scoliosis”, “scoliosis” AND “bracing” or “orthotic treatment”,
attributed to an autosomal dominant, major gene diallele “natural history”, “aetiology”, “pathogenesis”, “operative
model with incomplete sex-dependent penetrance of the treatment”, “spinal fusion”, “surgical stapling”, “surgical
genotypes. Moreover, four genome-wide screens of AIS stapler” or “thoracoscopy” or “video-assisted thoracic
have been reported. surgery” or “pedicle screw”. We mainly selected literature
Overall, results from these studies are disappointing from the past 10 years, but did not exclude commonly
since no one locus has been identified.7–11 This absence referenced and highly regarded older studies. We also
can be explained by phenotypic or genotypic heterogeneity, searched the reference lists of articles identified by this search
incomplete penetrance, and variable expressivity. strategy and selected those we judged relevant. Pertinent
Multiple genes could be implicated in the disorder and a review articles and book chapters were also included.
phenocopy could also arise in some families. Precise

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intervertebral discs and vertebral body growth plates


A B
creating mechanical microinsults, vascular damage, and
platelet activation with changes in calmodulin, leading to
the release of growth factors that affect the compromised
vertebral endplate. The end result of this postulated chain
of events is a relative anterior spinal overgrowth and
curve progression. However, the key questions of how
and why the initial small curve develops have not been
answered.
Several MRI studies have renewed interest in abnormal
neuroanatomy associated with AIS. Chiari type-I
malformations and spinal cord syrinx have been noted in
cases of AIS; and in younger patients, drainage of the
syrinx leads to resolution of the deformity.28–31 Additionally,
the relation between impaired autonomous nervous
system function and aetiopathogenesis of AIS has
Figure 1: Screening and diagnosis of scoliosis by scoliometer and radiography
received some attention. Sympathectomy of intercostal
(A) Measurement of trunk rotation with a scoliometer with patient in forward bend position; and
(B) posterior-anterior standing film of a 13-year-old boy, Risser 2, presenting with a Cobb angle of 30°. nerves in growing rabbits results in hypervascularity of
the soft tissues, increased rib growth on the side of the
syndrome (coloboma of the eye; heart defects; atresia of operation, and thoracic scoliosis convex to the opposite
the choanae; retardation of growth and development, or side.32,33
both; genital and urinary abnormalities, or both; and ear The hypothesis that an abnormality of the paravertebral
abnormalities and deafness) and AIS.19 muscles contributes to the development of AIS has been
Szappanos and colleagues20 reported a family with discussed for many years. Several have been recorded,
multiple familial occurrences of AIS coupled with the including decreased number of type-II fibres
same anomaly of the karyotype [inv(10)(p11q26)]. (fast-twitched), fibre splitting, tubular bodies, myofilament
However, analysis of restriction fragment length disarray consistent with myopathy, and generalised
polymorphism showed no abnormality. Bashiardes and membrane defect (namely, impaired calcium pump).
colleagues21 investigated another family in whom a However, no definitive conclusion can be reached about
pericentric inversion of chromosome 8 seemed to these abnormalities, though they are likely to be
cosegregate with AIS in three generations. They used secondary to the deformity itself.
fluorescent in-situ hybridisation and established that the Progressive AIS is attributed to relative anterior spinal
p-arm break did not interrupt any known gene, but the overgrowth during the adolescent growth spurt. MRI
q-arm break took place between exons 10 and 11 of the studies of vertebral thoracic morphometry show that
syntrophin, gamma-1 (SNTG1) gene. Mutational analysis longitudinal growth of the vertebral bodies in patients
of SNTG1 exons revealed a 6-bp deletion in exon 10, but with AIS is disproportionate and faster than age-matched
this deletion did not consistently cosegregate with the and sex-matched controls, and takes place mainly by
disease in this family.21 endochondral ossification. By contrast, the circumfer-
What is the role of melatonin in the development of AIS? ential growth by membranous ossification is slower in
Several investigators have shown that pinealectomy in both the vertebral bodies and pedicles.34–38 However, the
chickens, rats, and hamsters leads to scoliosis, and have mechanisms of this growth asymmetry are not well
attributed this effect to decreased melatonin production. understood.
However, other reports have failed to show this development
of scoliosis.22–26 Additionally, patients with AIS do not have Natural history
an inability to form melatonin, or impaired sleep or The natural history of scoliosis varies with the
immune function. Moreover, several diseases associated aetiopathogenesis and curve pattern. Early long-term
with an abnormal melatonin rhythm do not have an studies of so-called idiopathic scoliosis presented a grim
obvious effect on the development of scoliosis. Thus prognosis, perpetuating the common misperception that
scoliosis is unlikely to result from a simple absence of all types of idiopathic scoliosis inevitably lead to disability
melatonin; rather, it might be the result of melatonin’s from back pain and cardiopulmonary compromise.39–42
effect on other unknown growth mechanisms.3 Shortcomings of these earlier studies were (1) the
Abnormalities in the structure and function of platelets inclusion of patients with other causes of scoliosis or of
have been noted in patients with AIS. Evidence suggests early onset idiopathic scoliosis; and (2) failure to assess
that raised concentrations of calmodulin result in altered the outcomes related to the location of the curvature.
skeletal muscle activity and subsequent progressive Patients with AIS and their families are often unneces-
curvatures.27 Burwell4 formulated an integrative sarily upset by the long-term picture painted by these
platelet-skeletal hypothesis whereby a small curve loads early studies. Although the natural history of scoliosis

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clearly varies according to the aetiopathogenesis and


10–12 years 13–15 years 16 years
curve pattern,43–48 screening and treatment policy for AIS
has been formed on the basis of this misinformation. <19° 25% 10% 0%

Treatment of any disorder is an attempt to alter its 20–29° 60% 40% 10%
natural history; therefore, long-term studies of both the 30–39° 90% 70% 30%
natural history and treatment outcomes are necessary. >40° 100% 90% 70%
Treatment should be proven to prevent the negative *Data reproduced from reference 56 with permission of authors.
outcomes of natural history without introducing
iatrogenic complications. The outcomes of untreated AIS Table 1: Percentage of curve progression: magnitude of curve at initial
detection vs age*
through adulthood have been studied in Britain,43 Italy,44
Sweden,47 and the USA.45,49–52 The most frequently noted
long-term sequelae of untreated AIS are curve more frequent shortness of breath, but rarely, severe
progression, back pain, cardiopulmonary problems, and cardiopulmonary compromise.44,48,52,62
psychosocial concerns. Although present in most Most adults have back pain in some form during their
untreated patients, the severity of these sequelae and lifetime. About 50% of adults have an episode of low
their effect on overall health and function is very back pain in any particular year and 15% report frequent
variable. back pain or pain lasting for more than 2 weeks in a
The size of the curve tends to increase over the entire year.63 Ascani and others44 reported the frequency of pain
lifetime, but the degree of progression over a lifetime and in adults with AIS (61%) to be similar to that of the
the time-at-risk varies with many factors. Clinicians and general population, whereas another report52 showed
patients need to be aware of the risk of curve progression that chronic pain was more frequent and of greater
as they make treatment decisions. Factors predicting intensity and duration in scoliotics than in the general
curve progression include maturity (age at diagnosis, population. However, both groups of investigators agreed
menarchal status, and the amount of skeletal growth that back pain does not seem to cause excessive disability
remaining), curve size, and position of the curve apex. and, overall, patients work and undertake everyday
Many investigators agree that curves with a thoracic apex activities similarly to their peers.44,52 Over a lifetime, most
have the highest prevalence of progression, ranging patients with AIS, as well as the general population, will
from 58%53 to 100%.54 The more skeletally and sexually develop clinically important radiographic osteoarthritic
immature the patient is, the greater the probability of changes.52 However, history of backache seems to be
curve progression.44,49,54–56 Likewise, the larger the curve at unrelated to the presence or absence of osteoarthritis or
presentation, the higher the likelihood of progression curve severity. Back tenderness on palpation has not
both before54 and after maturity.49 Table 1 summarises the been related to the curve type or severity of osteoarthritis
risk of progression that results from the combined effects on radiography except for areas of lateral listhesis in
of maturity and curve size.56 Progression equations have lumbar and thoracolumbar curves. These curves,
also been developed to quantify the risk of progression. especially those with lateral listheses at the caudal end,
Peterson and Nachemson57 include Risser sign, level of tend to cause a higher frequency of back pain than do
apex, presence of trunk imbalance, and chronological other curve patterns.44,48
age; the equation developed by Lonstein and Carlson55 Untreated AIS, and its possible sequelae such as back
includes the Cobb angle, Risser sign, and chronological pain and pulmonary limitations, affects overall function
age. These methods could be helpful to clinicians and and self-esteem. In this respect, publications are sparse
patients who want to base their treatment decisions on and research is conflicting. Some studies show that
the risk of progression. patients perceive themselves to be less healthy and
The goal of non-operative treatment during adolescence restricted in physical and social activities. Ascani and
is to prevent curve progression; the goal of surgical colleagues44 reported “real psychological disturbances”
treatment is curve correction and maintenance. However, in 19% of their sample, 94% of whom had curves greater
the only negative outcome of AIS strongly and than 40°. Other studies have noted no significant
consistently associated with curve size is pulmonary difference between people with AIS and controls in their
function. Besides the degree of lateral curvature, other ability to undertake activities or in quality of life.64 With
factors such as high degrees of thoracic lordosis and respect to psychosocial aspects of AIS and presence of
vertebral rotation and decreased respiratory muscle clinical depression, scoliosis patients fare as well as
strength affect pulmonary function.58–60 Unlike early age-matched and sex-matched controls.65 However older,
onset (age 0–8 years) idiopathic scoliosis,41,43,46,47,61 in which untreated AIS patients are much less satisfied than are
substantial loss of vital capacity and forced expiratory controls with their body image, and appearance in clothes
volume in 1 s could cause pulmonary hypertension, right and swimsuits. About a third of patients believe that their
heart failure, and death, these problems rarely arise in curvature has restricted their life in some way, such as
AIS. Large curves (greater than 50°) with a thoracic apex difficulty in purchasing clothes, reduced physical ability,
have been associated with reduced vital capacity and and self-consciousness.52,65

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As they age, most patients with untreated AIS can have


Total sample size Surgical rate (%)
back pain and cosmetic concerns. Patients with untreated
AIS can function well as young adults, become employed, Wilmington84 brace 147 31%

get married, have children, and become active older Wilmington94 brace 79 11%
adults. Unfortunately, patients can develop substantial Providence75 brace 102 18%
deformity, and the cosmetic aspect of this disorder cannot Boston86 brace 212 13%
be disregarded. Treatment recommendations—watchful TLSO89 brace 54 26%
waiting, physiotherapy, bracing, and surgery—should be Boston vs Charleston95 brace 319 22%
made on an individual basis, and the patient and family TLSO96 brace 24 13%
should be well informed about the natural history of the Boston or Charleston97 brace 120 43%
disease. Boston98 brace 276 30%
Charleston99 brace 95 18%
Non-operative treatment Rosenberger100 brace 71 31%
Although AIS is diagnosed and treated worldwide, Charleston93 brace 42 7%
treatment approaches vary internationally. In patients Boston101 brace 151 13%
who still have growth remaining, watchful waiting Boston102 brace 50 30%
(observation), followed by bracing if the curve progresses
TLSO=thoracolumbosacral orthosis.
to greater than 25°, is the general course of care in
North America.1,2 Physical therapy (outpatient and Table 2: Surgery rates after TLSO treatment
inpatient rehabilitation) has been recommended as the
first line of treatment for small curves and those with a Blount first used the Milwaukee brace (originally a
low risk of progression by various, mostly European, postoperative orthotic) in 1958.73 Thoracolumbosacral
clinicians.66,67 No definite evidence has shown that orthoses (TLSO) were then developed, which improved
physical therapy or bracing reduces the risk of curve on the Milwaukee brace by removal of the cervical
progression, corrects the existing deformity, or component; use of lighter materials; and customisation
decreases the need for surgery. Patients should be aware to improve comfort, cosmesis, and compliance.74 Some
of the absence of evidence for these treatments, and be braces have been developed for use only part-time or at
provided with the opportunity to decide for themselves night-time,75,76 whereas most others are worn for 18 or
which treatment, if any, they believe will best fit with more hours per day.
their own personal assessment of risk, side-effects, and Modifications to the typical rigid TLSO include the
benefits. Cheneau bracing programme and the SpineCor dynamic
The popularity of physical therapy for AIS has waxed brace (SpineCorporation, Chesterfield, UK). The Cheneau
and waned over time, but it continues to be favoured in brace77,78 delivers a higher degree of initial correction
France, Germany, and Spain. The aim of physiotherapy through the use of a hypercorrected mould and pads to
is to prevent aggravation of the deformity in mild scoliosis provide derotational forces throughout the trunk than
(ie, curves less than 25°) and to enhance the effect of a did the original braces. Novel to this brace is the addition
brace and counteract its side-effects in moderate scoliosis of expansion room to allow for active correction by
(ie, curves between 25° and 45°). These aims are met, respiratory movements.79 The SpineCor and TrIAC
theoretically, by a combination of prescribed and (Boston Brace International, Avon, MA, USA) are
monitored sports activities and kinesitherapy to increase non-rigid braces for AIS treatment. Straps are placed to
coordination, spinal proprioception, and movement coincide with a specific correcting movement for each
control.68 kind of curve, producing a progressive positional change,
The expectation of treatment with bracing is to dynamic curve correction, and more appropriate muscle
prevent progression of the curve until the patient balance.80,81 Neither of these braces have been widely
reaches skeletal maturity, at which time the risk of tested, but the inclusion of dynamic forces in addition to
curve progression (and hence the risk of surgery) the traditional three-point control shows a more
greatly diminishes. Over the years, the medical sophisticated understanding of the growing spine.
community has arrived at two different conclusions. The nature of the dose–response relation between
On the one hand, some believe that the weight of brace wear and outcome is not well established, most
evidence favours a positive effect of bracing on the probably because of previous inability to accurately and
natural history of AIS. On the other hand, the US reliably measure time that a brace is worn. On the basis
Preventive Services Task Force,69 its Canadian counter- solely of review of records and other subjective
part,70 and many individual practitioners worldwide indicators of compliance, in a meta-analysis Rowe and
believe that evidence to support bracing is poor or colleagues82 reported that a 23-h per day protocol was
inconclusive. In fact, the effectiveness of bracing is more successful than 16-h or night-time protocols.
being tested in two randomised trials in North America Mechanical devices—mainly measuring temperature,
and the Netherlands.71,72 pressure, and proximity sensors—to measure brace

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wear are now available. These devices create the Surgery


opportunity to critically assess the relation between The primary objectives of surgical treatment with
brace dose (ie, wear time) and curve response. instrumentation are to (1) arrest progression, (2) achieve
Investigators used objective measures to show maximum permanent correction of the deformity in
compliance to be much lower than that reported by the three dimensions, (3) improve appearance by balancing
patient. However, a positive correlation between the the trunk, and (4) keep short-term and long-term
time that a brace is worn and outcome is partly evident. complications to a minimum. The generally agreed
In Rahman and colleagues’ study,83 34 patients had indication for surgery in adolescents is a primary curve
temperature monitors mounted in their braces for the greater than a Cobb angle of 45°. In adults, the surgical
duration of treatment. Of those patients who were indications are pain related to the curvature that is
highly compliant, 11% had progressive curves compared unresponsive to non-operative management, curve
with 56% of those patients who were less compliant. progression that has exacerbated symptoms, and
Many investigators have examined radiographic functional capabilities.103 Although adolescent patients
outcomes of TLSOs for AIS over the past half-century. can choose to delay surgery until they are adults, adults
Many regard a progression of greater than 5° to suggest a often have less flexible curves that than seen in children,
true change in curvature status, and often use this which could need anterior and posterior (staged or
radiographic benchmark as their definition of treatment sequential) procedures. Additionally, the rate of
failure. This outcome has been used in many case series complications in adults is much higher for the same
reports,84–88 although a few studies used improved designs procedures than it is in the adolescent population.
such as case–control comparison89–91 and one prospective Complications in adults are pseudarthrosis, proximal
cohort study92 has been reported. The only prospective and distal junctional kyphosis, and extended recovery.104
trial92 showed a lower rate of curve progression in the Improved preoperative assessments supplemented by
group with braces as compared with the non-treated intraoperative neurophysiological monitoring and
group. Although the most rigorous so far, this study was blood salvage techniques have made modern scoliosis
non-randomised, non-blinded, with baseline differences surgery safer than previously. Enhancement of solid
between the groups not statistically adjusted for, and the bony fusion over the surgical instrumentation can now
results have not been replicated. be achieved through autogenous bone graft, allograft,
Another definition of brace treatment failure is the demineralised bone matrix, or the latest biological bone
number of surgical procedures required despite treatment. substitutes.
The results of studies tracking surgical rates vary widely, Posterior instrumentation remains the mainstay of
most probably caused by different sample characteristics. treatment for most idiopathic curves. From the first
Table 2 gives an overview of the studies of patients who generation of Harrington instrumentation introduced in
meet present indications for bracing treatment with the the 1960s105 to modern third-generation instrumentation
rate of surgery despite bracing. The surgical rate ranges evolved from the Cotrel-Dubousset system in the 1980s,106
from 7%93 to 43%.97 much progress has been achieved by improved multi-

A B C D

Figure 2: Radiographs of a teenager with progressive AIS treated by posterior instrumentation by hybrid (rods, hooks, and screws)
(A) Preoperative standing posterior-anterior (PA); (B) preoperative standing lateral; (C) postoperative standing PA; and (D) postoperative standing lateral.

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planar (coronal, sagittal, and transverse plane) correction, screw system provides good rigid fixation, improved
stable fixation, reduced levels of fusion, and avoidance of correction in the sagittal plane, and minimum need for
postoperative immobilisation with a cast or brace. Such postoperative protection.122,123
progress has been possible through combinations of Anterior instrumentation has been extended to thoracic
wires, hooks, and lumbar pedicle screw constructs over scoliosis to achieve improved sagittal plane correction
contoured rods, and interconnecting systems applied and reduced number of fused levels, and to prevent
over several purchase sites posteriorly (the lamina, crankshafting (continued anterior spinal growth in the
pedicle, transverse processes, and spinous processes)107,108 face of a posterior fusion) in the immature patient
(figure 2). (figure 4).124–126 The disadvantages of anterior open
The extended use of many segmental pedicle screws in thoracotomy approach are the rate of implant breakage,
the thoracic spine, originally pioneered by Suk,109 allows pseudarthrosis, the surgical scar, and the unfavourable
even better three-column mechanical fixation, effect on lung function.124,127 Video-assisted thoracoscopic
multiplanar corrections, such as the rib prominence anterior instrumentation has been used to reduce the
reduction, and saving of fusion levels compared with surgical scar. Such surgery, however, entails a very steep
standard hook-wire constructs110–112 (figure 3). The learning curve, risk of encroaching on adjacent vital
disadvantages of thoracic pedicle screws are a steep structures, and problems related to anaesthesia in one
learning curve, increased cost, safety concerns, and lung.128–132 Hence, whether this method is useful cannot
difficulties associated with accurately placing pedicle be addressed without clearly documented advantages
screws within dysplastic pedicles.113–115At present time, no over the latest posterior instrumentation with thoracic
conclusive evidence exists about improved radiographic pedicle screws.133
outcomes in patients with AIS correlate with enhanced A novel way to modulate the growth of the anterior
function, self-image, or health.104 vertebral epiphyses through thoracoscopic stapling of the
Anterior instrumentation has been used mainly for convex apical vertebrae for scoliosis in skeletally
isolated thoracolumbar and lumbar curves. Evolving immature patients is under investigation after promising
from the early Dwyer cable to the vertebral screw preliminary work in animals (figure 5).134,135 The theoretical
system,116 Zeilke ventral derotation spondylodesis, advantages are preservation of growth, and motion and
screw-single rod system,117 and other similar systems function of the spine caused by non-fusion. However,
have been used satisfactorily with good correction of the apart from very limited experience, the need to refine the
frontal and transverse plane, restoration of truncal staple design, and other essential improvements, the
balance,118,119 and reduction of fusion levels. The main most important questions relate to indications. So, who
disadvantages are the kyphosing tendency of these is the ideal candidate? In the absence of objective
systems, higher rates of implant breakage, pseudarthrosis, measures to reliably predict curve progression, one might
and the necessity of postoperative cast or brace be unnecessarily treating patients with curves that will
protection.120,121 The latest dual-rod, multiple vertebral never progress.

A B C D

Figure 3: Radiographs of a teenager with progressive right thoracic adolescent idiopathic scoliosis treated by posterior segmental pedicle screws
(A) Preoperative standing lateral; (B) preoperative standing posterior-anterior (PA); (C) postoperative standing lateral; and (D) postoperative standing PA.
Reproduced with permission of Dr Peter Newton, San Diego, CA, USA.

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A B C D

Figure 4: Radiographs of a teenager with progressive right thoracic adolescent idiopathic scoliosis treated by anterior spinal instrumentation
(A) Preoperative standing lateral; (B) preoperative standing posterior-anterior (PA); (C) postoperative standing PA; and (D) postoperative standing lateral.
Reproduced with permission of Dr Peter Newton, San Diego, CA, USA.

Treatment outcome versus natural history previously wore a brace and those who had undergone
Typically, three types of treatment outcomes are reported: surgery each with a set of age-matched controls. They
(1) radiographical (coronal and sagittal radiographic reported little evidence that either patient group was
measurements); (2) clinical (complications, pulmonary greatly impaired relative to their peers when the 36-item
function and symptoms, mobility, muscle strength, back short form health survey and Oswestry low back pain
function); and (3) self-reported outcomes (general health disability questionnaire was used. The mean curve size
and health concerns specific to scoliosis and its in both groups was greater than 30°. Another follow-up
treatment). report of more than 20 years145 showed no difference in
Several workers have reported that curve size or curve quality of life, including back pain and function, between
correction do not correlate with quality of life. Moreover AIS patients who had undergone surgery and those who
several retrospective long-term studies have established remained untreated, as inferred from several
that patients treated surgically or with a brace have nearly instruments, such as the Oswestry, Roland-Morris, and
the same quality of life, both mentally and physically, the EuroQol-5D. This finding is in contrast with that of
after treatment as do healthy controls or national norms. Mayo and colleagues’ survey.65 Their sample consisted of
However, this evidence is not conclusive. patients who were untreated, had a brace, or had
Some retrospective investigations have shown that AIS undergone surgery. The frequency of back pain did not
causes disturbances in body image and in other indicators differ by Cobb angle, and these workers concluded that
of mental health and adjustment.136–141 Each investigation
used different instruments to measure psychological A B C
indices and adherence. Despite these shortcomings,
bracing clearly causes some psychological stress to the
patient, at least at the initiation of treatment and possibly
in the long term. Tones and colleagues142 reported a
systematic review of publications about health-related
quality of life and psychosocial issues in AIS. They
concluded that adolescents can have poorer psychosocial
functioning, body image, and health-related quality of
life than have their peers, but that adults generally do not
have psychological distress. However, adults are at risk of
disability and concerns about body image. Stress as a
result of treatment needs to be considered in the
decision-to-treat equation, and the benefits of watchful
waiting, bracing, or surgery should outweigh the risk of
adverse psychological sequelae. Figure 5: Radiographs of a skeletally immature 10 year old whose right thoracic curve progressed
from 35° to 42° despite bracing
A long-term study from Sweden143,144 corroborates (A) Preoperative but after bracing standing posterior-anterior (PA); (B) 3 years’ postoperative standing PA, Cobb
studies of the natural history of back pain and function. angle 28°; and (C) 3 years’ postoperative standing lateral. He was treated with vertebral body stapling T6-L1.
Danielsson and Nachemson,143,144 compared patients who Reproduced with permission of Lippincott Williams & Wilkins from reference 134.

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