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CLINICAL

Paediatric scoliosis
Update on assessment and treatment

Adam Parr, Geoffrey Askin SCOLIOSIS is a common paediatric investigation with magnetic resonance
condition with a prevalence of 0.47–5.2%.1 imaging (MRI) is mandatory for patients
It is a three-dimensional deformity with with painful curves.4
This article is the first in a commissioned
a coronal plane Cobb angle >10° and The hallmark of a structural curve is
series on paediatric orthopaedics.
rotation evident at the apex of the curve.2 axial rotation, which clinically manifests
Background Males and females are equally affected for as a rib prominence (Figures 1 and 2).
Paediatric scoliosis is a common small curves. However, females are seven Structural causes of scoliosis include
condition seen by general practitioners. times more likely to have a curve >40°.1 idiopathic (75%), neuromuscular
Structural scoliosis is characterised by
Scoliosis can be divided on the basis of (10%), congenital (10%) or other (5%).5
axial rotation at the apex. Several new
operative treatments have recently age of onset: early onset (≤10 years of age; Neurological (upper and lower motor
been developed. further divided into infantile [0–3 years neuron) or myopathic conditions can give
of age] and juvenile [4–10 years age]) and rise to scoliosis.6 Patients commonly will
Objective
adolescent (>10 years of age).3 Concerning have a long, C-shaped curve with pelvic
The aim of this article is to give an
curves include early onset scoliosis, obliquity and truncal imbalance.
overview of scoliosis diagnosis,
assessment and management. premenarchal scoliosis with a curve >25° Congenital scoliosis arises from an
and mature patients with curves >50°. error in vertebral development resulting in
Discussion The role of the primary care provider is failure of formation and/or segmentation.7
Scoliosis assessment should identify
to identify significant curves and decide Failure of formation causes wedge/
structural curves, underlying causes,
severity and growth potential. Atypical
which patients require imaging and when butterfly vertebrae or hemivertebrae.
curves and red flags must be excluded. to refer. Failure of segmentation results in the
Observation is appropriate for curves fusion of spinal elements causing block
<20° in patients with high growth vertebrae or vertebral bar. Prognosis
potential (Risser 0–2) and curves <40° in Aetiology is highly dependent on the vertebral
patients with minimal growth potential Scoliosis can be classified as structural or malformation. The highest progression
(Risser 3–5). Bracing is appropriate for
non-structural (Table 1). Non-structural rate occurs with hemivertebrae and a
patients with a curve of 20–40° with high
growth potential. Indications for surgery lateral curvature can masquerade as contralateral bar.7 Non-vertebral skeletal,
vary depending on patient and curve scoliosis. Common causes include leg intra-spinal, cardiac and genitourinary
factors; however, surgery can be indicated length discrepancy, pain, poor posture abnormalities are common and require
when the curve is >40°. Surgery can be and spondylolisthesis. A leg length appropriate investigation.
divided into three groups: growth discrepancy can cause the spine to develop Other causes of scoliosis include
modulation, instrumentation without
a compensatory curve to improve coronal neurofibromatosis, skeletal
fusion and instrumentation with fusion.
balance. Pain from infection, tumour, dysplasia, connective tissue disorders
Early diagnosis and referral to a paediatric
spine service can improve outcomes. trauma or nerve irritation can result in and inflammatory conditions.
a curve from muscle spasm or relieving Neurofibromatosis curves can be
pressure on nerve compression. Advanced dystrophic or non-dystrophic.8

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PAEDIATRIC SCOLIOSIS CLINICAL

Dystrophic curves are short, sharp increased shortness of breath when patient bends forward from the waist, and
curves characterised by rib pencilling, >80°.11 Scoliosis in children under the examiner uses a scoliometer to measure
wedge vertebrae and dural ectasia. 10 years of age may result in pulmonary symmetry between sides. A smartphone
Non-dystrophic curves behave similarly hypoplasia and long-term respiratory can be used as a substitute for a scoliometer
to idiopathic curves. Skeletal dysplasia failure.12 for measuring scoliosis parameters
and osteogenesis imperfecta are Pain is not a significant feature of (Figure 1).14,15 A patient with a measurement
characterised by a rapidly progressing scoliosis.4 Patients with untreated ≥7° requires imaging and referral to a
curve and osteopenia. Marfan syndrome idiopathic scoliosis have increased specialist paediatric spine surgeon.16
is characterised by ligamentous laxity, prevalence of pain, but do not appear A detailed neurological examination is
aortic enlargement, tall stature, lens to experience pain of longer duration or required to assess tone, power, reflexes
dislocation, high arched palate and stronger intensity than their peers and and sensation of upper and lower limbs.
arachnodactyly. generally do not require analgesia.11
The diagnosis of idiopathic scoliosis Long-term studies have shown that
is dependent on excluding the function in patients with curves of Radiological assessment
aforementioned underlying causes.9 >40° was only marginally worse than in A standing whole-spine plain
A typical adolescent idiopathic scoliosis age-matched controls with no scoliosis.11 posteroanterior and lateral radiograph
patient is a female with a convex right Scoliosis almost never causes paralysis, should be obtained for patients with
thoracic curve or convex left lumbar irrespective of curve size. structural scoliosis. The Cobb angle
curve, right shoulder elevated, right rib measures the most significant magnitude
prominence, left lumbar loin bolster, no of the curve from the superior endplate
abnormal neurology and no significant Clinical assessment of the upper vertebral body to the inferior
pain. Deviation from typical features should The goal of the clinical assessment is to endplate of the lower vertebral body
prompt a search for underlying causes. identify any underlying cause, assess involved in the curve. Measurements are
the severity and determine if the curve subject to high intra- and inter-observer
is typical or atypical (Box 1). Critical error.14 The lateral radiograph can be used
Natural history elements to determine during history- to identify sagittal plane deformities such
Patients with untreated idiopathic taking include family history, pre-existing as hypokyphosis and spondylolisthesis.
scoliosis have increased dissatisfaction conditions, pain and neurological
with their appearance. One-third of symptoms.
patients feel their lives are limited as General inspection should look for Box 1. Red flags for atypical curve
a result of scoliosis causing decreased findings associated with the aforementioned
physical activity and self-consciousness.10 clinical syndromes. It is important to • Structural scoliosis in a male patient
Psychological therapy may be beneficial. document curve location as well as • Significant pain, particularly night pain
While scoliosis has cosmetic shoulder and pelvis symmetry. Leg length • Left-sided thoracic curves
implications, its clinical significance discrepancy can be assessed by palpating • Abnormal neurological signs/symptoms
is related to growth potential and both iliac crests while the patient is standing. • Rapidly progressive curves
respiratory function. Thoracic idiopathic Rib prominence, resulting from rotational
• Onset in childhood rather than
curves may be associated with decreased deformity, is measured by the Adam’s adolescence
respiratory function when >50° and forward bend test.13 In this test, the standing

Table 1. Non-exhaustive list of causes of spinal curves

Non-structural Structural (rotation)


(nil rotation)
Idiopathic (75%) Neuromuscular (10%) Congenital (10%) Other (5%)

• Poor posture • Early onset ≤10 years Neuropathic • Failure of • Neurofibromatosis


• Leg length discrepancy • Adolescent >10 years • Cerebral palsy formation • Skeletal dysplasia
• Trauma • Friedreich’s ataxia • Failure of • Metabolic disorders
segmentation
• Tumour • Polio • Collagen disorders
• Mixed
• Infection • Spinomuscular atrophy • Irradiation
• Nerve irritation • Spina bifida
Myopathic
Muscular dystrophy

© The Royal Australian College of General Practitioners 2020 REPRINTED FROM AJGP VOL. 49, NO. 12, DECEMBER 2020 | 833
CLINICAL PAEDIATRIC SCOLIOSIS

Where available, EOS imaging is Computed tomography is rarely required Scoliosis progression
increasingly being used for the assessment for initial scoliosis assessment. Given Curve progression is primarily related to
of scoliosis deformities. MRI of the radiation risks, these authors recommend curve magnitude and growth potential.17
neuroaxis (brain and whole spine) should that it be ordered by the paediatric spinal Presentation Cobb angle is the most
be obtained for patients with atypical surgeon as pre-operative work-up for predictive factor of scoliosis progression.18
curves or those undergoing surgery. severe congenital deformities. Cobb angle <25° is unlikely to progress,

A C A B

Figure 1. Clinical photographs of right-sided thoracic scoliosis Figure 2. Erect plain posteroanterior radiographs of the spine
a. Patient standing; b. & c. Adam’s forward bending test measuring a. Non-structural scoliosis secondary to spondylolisthesis; note
thoracic rotation and rib prominence using inclinometer application symmetrical pedicles over the apex of the curve; b. Structural
on the smartphone thoracic scoliosis with rotation at the apex of the curve; note
asymmetrical pedicles and lateral deviation of the spinous process
over the apex of the curve

A B C

Figure 4. Erect plain posteroanterior radiographs of the spine of


a patient with Arnold Chiari malformation type 1 and syrinx and
atypical scoliosis treated with growing rods. Five lengthenings
were performed, allowing a 13.2 cm increase in trunk length prior to
Figure 3. Pelvic radiograph showing Risser grade. A Risser grade of definitive fusion.
5 is a fully fused iliac apophysis; 0–2 indicates high growth potential; a. Prior to treatment; b. Post–growing rod insertion c. Post–definitive
3–5 indicates low growth potential. posterior spinal instrumented fusion

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PAEDIATRIC SCOLIOSIS CLINICAL

25–50° will likely progress during skeletal When brace treatment ceases at the end mean Cobb angle decreases by 40° and
immaturity and >50° will likely progress of growth, the curve will often return to total spine length increases by 11.3 cm.25
even after skeletal maturity.19 Assessment its initial Cobb angle. Bracing reduces Vertebral body tethering has been
of growth potential is important in clinical curve progression to <50° in 72% of developed recently to treat young patients
decision making. Pubertal status correlates patients, compared with 48% of patients with scoliosis (Figure 5).26 This surgery can
with peak growth velocity. Menses whose treatment involved observation be considered if the patient has a single
generally coincides with a slowing of alone.21 Brace compliance correlates major thoracic curve of 30–65° and a Risser
growth, with most girls reaching skeletal with treatment success.22 In adolescent grade of 0–2.27 This fusionless procedure
maturity within 18 months. Peak growth patients with idiopathic scoliosis, 51% uses thoracoscopically placed vertebral
velocity (growth of 5–6 cm in six months) of untreated patients had progression, body screws with a tape tensioned between
usually occurs 6–12 months before compared with 7% of patients wearing connecting screws on the convexity of the
menses. This growth spurt represents a brace 23 hours per day.23 As a result, curve. Only partial correction of the curve
the period of highest risk of curve bracing is often prescribed 23 hours per occurs intra-operatively. Spinal growth
progression.17 Bone age is assessed by day. Symptomatic treatment includes an occurs asymmetrically, according to the
Risser grade on the iliac apophysis. This is exercise regimen for core strengthening Hueter-Volkmann principle, leading to
graded from 0–5 on the basis of increasing and posture control. Physiotherapy further curve correction. Long-term data
ossification (Figure 3). A Risser grade of can help obtain and maintain muscle are still pending.
0–2 represents skeletal immaturity, high condition. In addition, physiotherapy
growth potential and increased scoliosis scoliosis-specific exercises may be Instrumentation without fusion
growth rates. beneficial when used in conjunction with Bipolar instrumentation is a fusionless
bracing. However, physiotherapy and technique used for severe neuromuscular
manipulative treatments do not reverse scoliosis (Figure 6).28 This recently
Treatment structural scoliosis.24 developed technique uses proximal hooks,
Treatment of paediatric scoliosis is distal iliosacral screws and submuscular
dependent on the aetiology, magnitude of Operative rods to distract and straighten scoliosis.
curve and growth potential. The goal of Indications for the surgical management Long-term data are still pending.
management is to enter skeletal maturity of scoliosis vary considerably depending
with a balanced spine that will not on patient and curve factors; however, Instrumentation with fusion
progress. If the curve is >40–50° degrees surgery can be indicated when the curve Spinal correction and fusion is generally
at skeletal maturity, progression will likely is >40–50°.20 Surgery can be divided reserved until patients are aged
occur. The treatment chosen should be the into three groups: growth modulation, >10–12 years. Fusion can be anterior or
most minimally invasive option available instrumentation without fusion and posterior depending on curve and patient
that can achieve these goals for a specific instrumentation with fusion. characteristics. The posterior approach
patient. Referral to a paediatric spinal is the most commonly used method
specialist is appropriate for immature Growth modulation for scoliosis correction (Figure 7). The
patients with curves >20°, significant Thoracic growth is essential for lung thoracoabdominal approach is indicated in
rotation (>7° on scoliometer) or red flags. development. Delaying spinal fusion is primary lumbar curves. The thoracoscopic
desirable until the patient is aged 10–12 approach is indicated for a single main
Non-operative years to allow chest and lung development.12 thoracic curve (Figure 8).29
Non-operative treatments consist of This can be achieved by using growing rods
observation, bracing and symptomatic or vertebral body tethering.
management. Observation is appropriate Growing rods are expandable rods Conclusion
for curves <20° in patients with high attached to the spine proximally and Scoliosis assessment should identify
growth potential (Risser 0–2) and curves distally, allowing the spine to grow structural curves, underlying causes,
<40° in patients with minimal growth (Figure 4). They are inserted using a severity and growth potential. Red flags
potential (Risser 3–5).20 If the curve is minimally invasively procedure with no for atypical curves must be excluded.
<20° without rotation, it is appropriate for fusion in the primary curve. At regular Observation is appropriate for curves
the general practitioner to repeat plain time intervals (often six monthly), the rods <20° in patients with high growth potential
radiograph imaging in six months’ time to are expanded, surgically or magnetically, (Risser 0–2) and curves <40° in patients
assess progression. At each consultation, to straighten the spine and allow thoracic with minimal growth potential (Risser 3–5).
the patient should perform an Adam’s development. When chest growth is Bracing is appropriate for patients with
forward bend test. Bracing is appropriate sufficient, the construct can be replaced curves of 20–40° with high growth
for patients with a curve of 20–40° with with a posterior spinal instrumented potential. Indications for surgery vary
high growth potential.21 A brace works fusion. Comparing spinal parameters pre– depending on patient and curve factors.
by holding the deformity during growth. growing rods to post–definitive fusion, the Surgery can be indicated when the curve

© The Royal Australian College of General Practitioners 2020 REPRINTED FROM AJGP VOL. 49, NO. 12, DECEMBER 2020 | 835
CLINICAL PAEDIATRIC SCOLIOSIS

is >40°. Early discussion with the local • Underlying causes for scoliosis (Table 1) using pubertal status and Risser grade.
paediatric spine service can be considered. must be excluded. • Early referral to a paediatric spinal
• Atypical curves and red flags need to be specialist is recommended, especially
investigated (Box 1). for skeletally immature patients with
Key points • Practitioners can assess the curve curves >20°, patients with significant
• It is necessary to determine if the curve is severity by examining radiographs, rotation or those with red flags.
structural; this can be done by measuring rib prominence and alignment. • Management is based on growth
rotation on the forward bend test. • Growth potential can be determined potential and curve character.

A B C

Figure 5. Errect plain posteroanterior radiographs of the spine of a female A B


aged 13 years. The patient had a Risser grade of 0 with idiopathic scoliosis
treated with vertebral body tethering. Figure 6. Sitting plain posteroanterior radiographs of the spine
a. Prior to treatment; b. Post-operative partial curve correction; c. Further of a female aged 10 years with neuromuscular scoliosis (pelvic
improvement at six months post-operatively obliquity 27°) treated with bipolar posterior instrumentation.
a. Prior to treatment; b. Post-operative radiograph shows
well‑balanced spine and minimal pelvic obliquity

A B C
A B

Figure 8. Female aged 16 years with idiopathic scoliosis treated with


Figure 7. Erect plain posteroanterior radiographs of the spine thoracoscopic instrumented correction and fusion.
of an adolescent with idiopathic scoliosis treated with open a. Radiograph prior to treatment; b. Six month post-operative radiograph;
posterior spinal instrumented fusion. c. Clinical photograph shows improved curve with minimal scars and
a. Prior to treatment; b. Post-operative radiograph rib prominence

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PAEDIATRIC SCOLIOSIS CLINICAL

Authors in scoliosis. Eur Spine J 2012;21(6):1062–68.


Adam Parr MBBS, FRACS (Ortho), Clinical doi: 10.1007/s00586-011-2059-0.
Researcher, Biomechanics and Spine Research 15. Izatt MT, Bateman GR, Adam CJ. Evaluation
Group, Institute of Health and Biomedical Innovation, of the iPhone with an acrylic sleeve versus the
QUT, Qld; Adult and Paediatric Spine Surgeon, Scoliometer for rib hump measurement in
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Hospital, Greenslopes Private Hospital and The 7161-7-14.
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Hospital, Qld girls. J Bone Joint Surg Am 2000;82(5):685–93.
Competing interests: None. doi: 10.2106/00004623-200005000-00009.
Funding: None. 18. Tan KJ, Moe MM, Vaithinathan R, Wong HK.
Curve progression in idiopathic scoliosis:
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