Jurnal Scoliosis

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Early-Onset Scoliosis: A Review

of History, Current Treatment,


and Future Directions
Scott Yang, MD,a,b Lindsay M Andras, MD,a Gregory J Redding, MD,c David L Skaggs, MD, MMMa

Early-onset scoliosis (EOS) is defined as curvature of the spine in children abstract


>10° with onset before age 10 years. Young children with EOS are at risk
for impaired pulmonary function because of the high risk of progressive
spinal deformity and thoracic constraints during a critical time of lung
development. The treatment of EOS is very challenging because the
population is inhomogeneous, often medically complex, and often needs
multiple surgeries. In the past, early spinal fusion was performed in
children with severe progressive EOS, which corrected scoliosis but limited
spine and thoracic growth and resulted in poor pulmonary outcomes.
The current goal in treatment of EOS is to maximize growth of the spine
and thorax by controlling the spinal deformity, with the aim of promoting
normal lung development and pulmonary function. Bracing and casting
may improve on the natural history of progression of spinal deformity
and are often used to delay surgical intervention or in some cases obviate
surgery. Recent advances in surgical implants and techniques have led to
the development of growth-friendly implants, which have replaced early
spine fusion as the surgical treatment of choice. Treatment with growth-
friendly implants usually requires multiple surgeries and is associated with
frequent complications. However, growth-friendly spine surgery has been
shown to correct spinal deformity while allowing growth of the spine and
subsequently lung growth.

The treatment of early-onset scoliosis etiology of the spinal deformity


(EOS) remains a challenging and may be idiopathic, associated with
rapidly evolving area of pediatric underlying systemic syndromes, aChildren’s Orthopaedic Center, Children’s Hospital
orthopedics. EOS is defined as secondary to a neuromuscular Los Angeles, Los Angeles, California; bDepartment of
a curvature of the spine ≥10° condition, or caused by a structural Orthopaedic Surgery, University of Virginia Health System,
Charlottesville, Virginia; and cDepartment of Pediatrics,
in the frontal plane with onset congenital spinal deformity (Table Division of Pulmonary and Sleep Medicine, Seattle
before 10 years of age (Fig 1).1,2 1).3 The true prevalence of EOS is Children’s Hospital, Seattle, Washington
The management of EOS requires unknown, although idiopathic EOS
Dr Yang drafted the initial manuscript and edited
consideration of the interrelated accounts for <1% of all scoliosis the final manuscript; Drs Andras and Redding
growth of the spine and thorax and cases.4 Congenital scoliosis results provided critical revisions to the manuscript; Dr
their impact on lung development. from abnormalities of vertebral Skaggs provided overall supervision and critical
In addition, EOS is often associated development in utero and may include revisions to the manuscript; and all authors
approved the final manuscript as submitted.
with other comorbid conditions that single or multiple hemivertebrae or
increase the complexity of managing segmentation defects with or without
the spinal deformity. associated rib fusion. Congenital To cite: Yang S, Andras LM, Redding GJ, et al.
scoliosis is often progressive and may Early-Onset Scoliosis: A Review of History, Current
Treatment, and Future Directions. Pediatrics.
EOS includes an inhomogeneous necessitate early, more aggressive
2016;137(1):e20150709
grouping of patients, because the treatment. Idiopathic EOS in infants

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PEDIATRICS Volume 137, number 1, January 2016:e20150709 STATE-OF-THE-ART REVIEW ARTICLE
occurs in children ≤3 years of age children dying of EOS report similar comparing expected population
and has a variable course over time. alveolar features and pulmonary death rates demonstrated more than
A unique feature of idiopathic EOS vascular remodeling associated with twice the mortality rate by age 40
in infants is that it often improves pulmonary hypertension.14 Lung in patients with EOS compared with
spontaneously.5,6 Idiopathic EOS in function studies of children with EOS that of the general population.18
juveniles occurs in children aged 4 demonstrate a variable severity of Consequently, the fundamental
to 10 years. Among children with restrictive lung disease caused by principle of treating EOS is to foster
neuromuscular disorders, scoliosis small lung volumes, reduced chest normal respiratory development
is common and compounds the wall compliance, and respiratory and maximize spinal growth while
restrictive lung disease produced muscle dysfunction.15 The concept preventing additional deformity that
by respiratory muscle weakness. of thoracic insufficiency syndrome can lead to TIS.
Treatment strategies and duration (TIS), popularized by Campbell et
differ significantly based on al,16 is defined as the inability of the
both etiology and the amount of thorax to support normal respiratory HISTORY OF TREATMENT STRATEGIES:
anticipated growth remaining. The function and lung development in WHAT WE HAVE LEARNED
younger the child, the greater the growing children. Because of scoliosis
risk that the spinal deformity will progression early in life, patients Before the introduction of spinal
affect pulmonary development and with severe EOS can potentially implants, the historical treatment of
function. develop TIS. TIS has been associated EOS consisted of casting or bracing
with poorer quality of life scores than the spine and thorax. Although
those of childhood epilepsy, heart casting for scoliosis has been
GROWTH OF THE SPINE AND LUNG disease, and cancer.17 performed for centuries, it fell out
DEVELOPMENT of favor as the primary treatment
The natural history of untreated of scoliosis because of concerns
The spine grows most rapidly in EOS is associated with significant that casting deformed the ribs. Paul
the first 5 years, with an average morbidity and often profound Harrington introduced a spinal
T1 to S1 segment length increase cardiopulmonary compromise, implant for scoliosis in the 1960s
of 10 cm during this time (2 cm/ including respiratory failure and that provided a surgical alternative.
year). After the first 5 years, there cor pulmonale. A Swedish study In these cases, the rod was used to
is a slower T1 to S1 growth from
age 5 to 10 years of ~5 cm until
adolescence (1 cm/year). From
age 10 years to adulthood, T1 to
S1 grows an additional 10 cm; this
includes the adolescent growth spurt
(2 cm/year).7–9 Because growth
can promote the progression of the
deformity, patients with EOS are at
greatest risk for progression of spinal
deformity in the first few years of life
and during the adolescent growth
spurt.
In EOS, the progressive spinal
deformity occurs during a critical
time of lung development. The
number of alveoli and lung volume
increase most rapidly in the first
several years and continue to
increase at a lower rate during
adolescence and adulthood (Fig
2).10–12 Animal models of EOS
produced early in life demonstrate
alveolar simplification and reduced FIGURE 1
Posteroanterior (A) and lateral (B) radiographs of a 4-year-old boy whose untreated scoliosis had
number, producing an example of rapidly progressed to 110° and who developed severe kyphosis. Reproduced with permission of
postnatal hypoplasia.13 Autopsies of Children’s Orthopedic Center, Los Angeles, California.

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2 YANG et al
surgically correct scoliosis without demonstrated that early spinal reduction in FVC ≥5 years after spine
fusion by applying distraction fusion, which prevents continued fusion directly correlated with the
across the concavity of the curve.19 spinal growth of the fused region, number of thoracic spinal segments
Harrington rods improved curves limits intrathoracic volume and fused.21 Early posterior spinal fusion
in a 2-dimensional plane, although hence lung volume. As a result, techniques have often led to the
this technique often led to a flat children developed severe restrictive crankshaft phenomenon, in which
back deformity. Implant failure and lung disease with continued growth. the anterior column of the immature
dislodgment with this method were Patients with idiopathic EOS who spine continues to grow, leading to
high, and its use was limited. underwent spinal fusion at a mean progressive deformity. Consequently,
age of 4.1 years demonstrated mean new treatment strategies have been
Early spinal fusion to halt deformity forced vital capacity (FVC) of 41% of developed that allow or promote
in EOS then became the preferred normal when evaluated at skeletal spinal growth, usually referred to
treatment, because a short and maturity, whereas patients who as growth-friendly techniques.22
straight spine was thought to underwent fusion at a mean age of At worst these techniques may be
be superior to a progressively 12.9 years demonstrated mean FVC thought of as delaying the need
crooked spine. Subsequent studies of 68% of normal.20 In 1 study, the for a spine fusion to allow spinal
growth, and at best they may cure
TABLE 1 Summary of the Different Types of EOS and Their Unique Features the scoliosis or avoid the need for a
spinal fusion.
Types of EOS Characteristics Associated Diagnoses Treatment Considerations
Congenital Structural abnormality of Cardiac, renal Hemivertebrae excision. One exception to avoiding early
the spine or thorax present abnormalities Short-segment early spinal fusion is congenital scoliosis in
at birth. spinal fusion in select which the spine deformity is limited
cases in this group
may be the exception to
to a small number of vertebrae.
growth-friendly spine The classic example of congenital
surgery. scoliosis that requires early fusion is
Other musculoskeletal the case of a hemivertebra causing
Failure of formation (eg,
abnormalities (upper progressive scoliosis (Fig 3). In such
hemivertebra).
limb, club foot)
Failure of segmentation (eg, Associated with VATER/
cases early fusion (with or without
fused vertebra or ribs). VACTERL syndromes excision of the hemivertebrae)
Intraspinal abnormalities can often correct the scoliosis in
(ie, diastematomyelia, 1 surgery, with a fusion of only
tethered cord) 2 vertebrae. This procedure is
Neuromuscular Abnormalities in muscular Examples: Cerebral palsy, Generally higher-risk
tone lead to scoliosis. muscular dystrophies, surgical patients with
generally performed around the age
myopathies, spinal cord medical comorbidities of 3 to 6 years.
injuries (eg, respiratory,
gastrointestinal).
Often a long, sweeping CURRENT TREATMENT STRATEGIES:
scoliosis curve.
NONOPERATIVE
Syndromic Includes any other syndrome Examples: Connective Each syndrome has
associated scoliosis tissue disorders, unique considerations Nonoperative treatment of EOS
(excluding neuromuscular Marfan syndrome, (eg, neurofibromatosis
consists of bracing or casting.
or congenital scoliosis neurofibromatosis, may have dural ectasia,
syndromes). skeletal dysplasias, making spinal implants Bracing can be considered for mild
Prader–Willi syndrome more challenging to progressive curves, although its
place. efficacy remains unproven in EOS,23
Idiopathic Scoliosis without a known Higher incidence Generally healthier and ensuring compliance with brace
attributable cause. of Arnold–Chiari children.
wear can be difficult in young children.
malformation and
syringomyelia compared Commonly used braces are variants of
with adolescent idiopathic a custom-molded thoracolumbosacral
scoliosis orthosis. Braces are often used to
Infantile (diagnosed <3 y): Casting shown to resolve maintain correction obtained from
Many milder curves will some infantile curves.
serial casting or delay surgical
resolve but need observation.
Juvenile (diagnosed 4–10 y): Surgery often needed intervention. Several case series have
Often left sided curves. Slight despite often long-term shown that 74% to 92% of idiopathic
male predominance. casting or brace wear. EOS in infants spontaneously
Reproduced with permission of Children’s Orthopedic Center, Los Angeles, California. resolves.6,24 For idiopathic EOS in

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PEDIATRICS Volume 137, number 1, January 2016 3
infants with unresolved progressive 12 weeks. For curves that resolved was controlled sufficiently to delay
curves, Mehta25 demonstrated that or stabilized in a cast, bracing is often spine surgery for at least 2 years.
casting may be effective in completely used to help maintain the correction Normal longitudinal growth of
resolving some curves, especially through skeletal maturity. If there the spine was observed while the
those of lower magnitude. Because is progression despite bracing, patient was in the cast.28 Based on
casting has proven to be a safe method additional casting may be used to current evidence, a trial of casting in
to manage idiopathic EOS, there attempt to regain the correction. EOS regardless of curve etiology is
has been a resurgence of interest in In other cases, growth-friendly considered a treatment option. The
expanding traditional cast methods implants or fusion may be the most specific indications for the threshold
to treat multiple subtypes of EOS to appropriate step depending on the to institute cast treatment continue
avoid the risks of surgery and early patient’s age and curve severity. to vary between institutions but are
spine fusion (Fig 4). The cast is applied Serial casting applied to young generally considered for EOS curves
to the torso under anesthesia while children with nonidiopathic EOS has >25°, with >10° of documented
the child is in traction, elongating been shown to be an effective way progression.
the spine. The cast is molded while to delay surgical treatment.26–28 In 1
the child’s torso is derotated and study, curve resolution was rare with
flexed away from the concavity of the serial casting in the nonidiopathic CURRENT TREATMENT STRATEGIES:
curve. Casts are changed every 8 to EOS, but progression of the curve SURGICAL
The surgical treatment strategy for
EOS has evolved significantly over
the past decade with the use of
modern growth-friendly implants.
These implants attempt to maximize
the growth of the spine and thorax
while controlling curve progression
to preserve normal lung volume.
Growth-friendly implants can be
classified into 3 distinct subtypes:
distraction-based, guided growth,
and compression-based strategies.22

Distraction-Based Implants
Distraction-based implants are the
most common devices used in EOS.
They apply traction to the spinal
column between proximal and
distal anchors joined by expandable
rods. The rods are periodically
FIGURE 2
Composite plots of total alveolar number, T1–S1 segment growth from birth, and mean lung volume lengthened as the child grows to
with age. The number of alveoli, mean lung volume, and spinal growth increase rapidly in the first few maintain spine curve correction.
years of life. Reproduced with permission of Children’s Orthopedic Center, Los Angeles, California. Four types of implants have been
used: the traditional growing rod
(TGR), vertical expandable prosthetic
titanium rib (VEPTR) device, hybrid
systems, and magnetically controlled
growing rod (MCGR).

Growing Rod
The TGR incorporates proximal and
distal hook or screw anchors on the
spine, joined by rods with connectors
that allow serial distractions between
FIGURE 3 the rods (Fig 5). Limited fusion is
Preoperative radiograph (A) and CT scan (B) of a patient with a hemivertebrae (red arrow).
Intraoperative images demonstrating correction of the deformity with a hemivertebrectomy (C). performed at the proximal and distal
Reproduced with permission of Children’s Orthopedic Center, Los Angeles, California. anchor sites on the spine to provide

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4 YANG et al
solid sites for spine distraction. uses traditional spinal implants, along the ribs (Fig 7). As in the
The area between the anchors is as in the TGR system. Traditional TGR strategy, the distal anchor
intentionally not fused, allowing spinal hooks are placed proximally site is incorporated by a fusion,
motion and growth through this
region. Lengthenings are typically
performed at ~6-month intervals.
Akbarnia et al29 reported on the
use of TGRs for EOS in 24 patients,
resulting in improvement of coronal
plane major curves from 82° to
36°, with 1.2 cm growth in T1 to
S1 length per year at mean 4-year
follow-up. Akbarnia et al30 also
demonstrated that patients whose
spines were lengthened at ≤6-month
intervals had significantly higher
annual T1 to S1 growth rate of 1.8
cm/year, compared with 1.0 cm/
year in patients whose spines were
lengthened less frequently. This
finding has led many to believe that
distraction may actually promote
vertebral growth.

VEPTR

Developed by Bob Campbell, VEPTRs


use ribs as anchors, and sometimes
the spine and pelvis as well. VEPTRs
are generally thought of as primarily
providing thoracic expansion,
compared with growing rods,
which primarily provide control
of scoliosis. In reality, thoracic and
spinal deformities are closely linked.
Similar to other distraction-based
systems, these constructs undergo
recurrent surgical expansion (Fig 6).
Original descriptions of the VEPTR
technique recommended incising
between ribs to maximize thoracic
expansion, but concerns of scarring
and stiffening of the chest wall
led most surgeons to cut between
the ribs only in cases of multiple
fused ribs. VEPTR treatment has
demonstrated continued spinal
growth with serial expansions (mean
71 mm over 4 lengthenings) while
improving the coronal curve.31,32

Hybrid FIGURE 4
A, Radiograph of a 29-month-old girl with idiopathic EOS and a curve of 47°. B, In-cast radiograph
A hybrid distraction-based strategy
shows curve corrected to 18° with the initial cast. C, Like many children she continued to be quite
incorporates the VEPTR concept of active despite the cast. Reproduced with permission of Children’s Orthopedic Center, Los Angeles,
using ribs as anchor sites but also California.

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PEDIATRICS Volume 137, number 1, January 2016 5
and lengthening is performed at a a decrease in the incidence of rod external remote control (Fig 8).
connector between the rods. The breakage.33 Because of the noninvasive nature
advantage of this technique is that of lengthening, it is also possible
it avoids fusion of the proximal MCGR to distract at shorter intervals.
anchor site of the thoracic spine, Recently the US Food and Drug Preliminary studies of MCGRs
potentially allowing more total Administration cleared the use have been met with optimism
growth of the thorax. Furthermore, of MCGRs, which can lengthen with respect to achieving the same
rib anchors without traditional rigid nonsurgically without anesthesia results of TGRs.34–36 Dannawi et
fusion at the proximal anchor site after the initial implantation.34,35 al34 demonstrated that the mean
allow some motion of the spinal This implant is similar to other coronal Cobb angle improved from
implant construct. This feature may growing rod constructs with distal 69° to 41° after MCGRs with a mean
reduce the stress and rigidity of the spine anchors and proximal rib or of 4.8 distractions per patient were
distraction system across an unfused spine anchors that are connected by used over 15 months. The T1 to S1
mobile spine. Consequently, use of telescoping rods. This telescoping length increased a mean of 3.5 cm
a hybrid system with proximal rib portion contains an internal magnet during this time period. A study
anchors has been associated with that can be lengthened from an comparing 12 MCGR- and TGR-
treated patients demonstrated no
significant difference in spine length
gains, but there were 57 fewer
surgical procedures in the MCGR
group.37 Although MCGR avoids the
need for repeat surgical intervention
for routine lengthenings, long-term
data are not yet available for this
technique. Similar to growth-friendly
implants, the complication rate is
high: 33% of patients treated with
MCGR within 2 years of follow-up.37

FIGURE 5
Preoperative (A) and postoperative (B) radiographs of patient with traditional spine-to-spine growing
rods. Radiographs obtained 5 years after the initial placement of growing rods (C) show that the
scoliosis continues to be well controlled. Reproduced with permission of Children’s Orthopedic
Center, Los Angeles, California.

FIGURE 6
Postoperative radiograph of an 8-year-old
boy with VATER syndrome with congenital
FIGURE 7 scoliosis, multiple rib fusions, and thoracic
A, Preoperative posteroanterior and lateral radiograph of a 4-year-old boy with severe progressive insufficiency syndrome that was treated with a
scoliosis and an 85° curve. He was not a casting candidate because of his restrictive lung disease. B, VEPTR construct. Reproduced with permission
Postoperative radiograph showing a hybrid growing rod construct (rib to spine) with improvement of Children’s Orthopedic Center, Los Angeles,
to 37°. Reproduced with permission of Children’s Orthopedic Center, Los Angeles, California. California.

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6 YANG et al
Complications of Distraction-Based with 29% in proximal spine- Repeated general anesthesia in
Implants anchored growing rods at a mean children may cause detrimental
56-month follow-up. neurocognitive effects, based on
Regardless of the implant used for animal and preclinical studies,
distraction-based treatment of the although this remains an area of
The multiple surgeries needed for
growing spine, all strategies are controversy.48–50 New technologies,
treatment with distraction-based
associated with a high complication such as MCGRs that obviate surgical
implants are associated with adverse
rate.29,30,38–43 Implant complications lengthenings, will probably help
outcomes. Each lengthening surgery
such as anchor malfunction (pullout minimize the total number of
has been shown to increase the
from the spine, erosion through the exposures to anesthesia in EOS.
risk of deep infection 3.3 times in
rib) and rod breakage are common
EOS.41 The length gained from serial
(Fig 9). Distraction-based posterior
lengthening has also been shown to Guided Growth Implants
implants often produce kyphosis,
follow a law of diminishing returns, In guided growth techniques, the
which may result in an unfavorable with decreased spinal length gained
overall sagittal plane balance. Wound spine is straightened with spinal
after each lengthening because of implants that allow the vertebrae
complications are also common increased stiffness of the spine.45,46
because of the prominence of to grow along the path of the spinal
Autofusion of the spine has been implants. The original guided growth
implants under the skin and poor also described after repeated
healing potential in small, thin, and system used Luque wires wrapped
lengthenings.47 Ultimately, the utility around the lamina of each vertebrae,
often chronically malnourished of lengthening may be minimal after
patients with EOS (Fig 10). At least 1 which were wrapped around straight
the sixth or seventh lengthening rods that corrected scoliosis and then
complication of treatment has been procedure, limiting the potential
reported to occur in 58% to 86% permitted guided growth as the wires
spinal growth to 4 to 5 years after slid along the rods. This technique
of patients undergoing distraction- initial surgery.
based treatment, leading to multiple was found to lead to spontaneous
unplanned surgical procedures.38,43,44 In addition to the physical effects fusion and limited spinal growth. A
Among patients with complications, on the spine, there are significant more recent type of guided growth
1 study demonstrated a mean of psychological effects from implant, called the Shilla technique,
2.2 complications per patient.38 distraction-based treatment. Patients has been developed by Richard
Application of stiff implants on an with repeated surgery in EOS McCarthy. In this technique, screws
unfused spine that continues to have demonstrate abnormal psychosocial are placed into vertebrae with
scores, with a positive correlation minimal dissection in the hopes of
motion ultimately leads to fatigue
between behavioral problems and avoiding spontaneous fusion and
failure of the implants. Risk factors
the number of repetitive surgeries.42 allowing 3-dimensional correction of
for implant failures include severe
thoracic kyphosis that produces
proximal anchor pullout and
increased number of lengthening
procedures.40 Implantation
strategies are being critically
evaluated to decrease the incidence
of implant-related complications. A
comparison study of complications
in TGRs, hybrid proximal rib anchor
systems, and VEPTR treatments
in EOS demonstrated a trend
toward decreased implant-related
complications in the hybrid system.43
Hooks are not as rigidly fixed to
the spine compared with screws,
theoretically allowing some motion
and dispersion of stress, decreasing
fatigue-related implant failures. FIGURE 8
A, Preoperative anteroposterior radiograph of a 7-year-old girl with spinal muscular atrophy and
Yamaguchi et al33 demonstrated
100° thoracolumbar curve. B, Postoperative posteroanterior radiograph demonstrating a construct
6% rod breakage in proximal rib- including the MCGR device. C, Model demonstrating an MCGR device. Reproduced with permission of
anchored growing rods, compared Children’s Orthopedic Center, Los Angeles, California.

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PEDIATRICS Volume 137, number 1, January 2016 7
the spinal deformity and permitting side of the scoliosis. Although there challenging, complicated by the fact
growth along the rods (Fig 11). is 1 device approved by the US Food that many of these patients start
The major advantage of guided and Drug Administration, tethers treatment of EOS before they are old
growth techniques over growing and staples are most commonly enough to undergo formal pulmonary
rods is that children avoid multiple used off label. Several case series on function tests (PFTs). Much of the
surgical lengthenings. A recent study compression-based implants have current literature evaluating the
comparing the Shilla technique demonstrated curve correction with pulmonary outcomes after growth-
with growing rods demonstrated growth in patients who underwent friendly spine surgery has been
that patients treated with the Shilla surgery at age <10 years.52,53 There based on VEPTR in children >6
technique had fewer surgeries (2.8) have been cases of overcorrection years old. PFTs have been measured
compared with growing rods (7.4) with compression-based implants, in both awake and anesthetized
in >4-year mean follow-up. Shilla in which the curve corrects and then patients. Computed tomography has
resulted in less spinal growth and develops in the opposite direction. also been used to measure thoracic
less correction of scoliosis, with Therefore, this technique is generally volumes as a surrogate for PFTs.
similar complication rates to growing reserved for patients with limited Studies of lung function in EOS have
rods.51 growth remaining, such as 9- to not compared treatment strategies
10-year-olds. Additional concerns or different devices and are often
Compression-Based Implants include the potential pulmonary small, descriptive case series. In 1
impact of ≥1 transthoracic surgeries. such series, 10 children with EOS
Compression-based implants involve Serial measures of lung function in (median age 4.3 years) demonstrated
correcting scoliosis by stopping older children with scoliosis treated increased mean annual absolute
the growth of the convex side of with transthoracic spine surgery FVC of 27% of predicted norms and
the scoliosis without fusion while have shown greater loss of function maintenance of FVC as a percentage
allowing growth of the concave postoperatively when the thorax is of normal after VEPTR treatment at
side of the curve. This correction opened.54 a mean 22 months of follow-up.55
is accomplished by placing staples, With longer periods of follow-up
tethers, or other devices across the (mean 6 years) during and at the
growth plates of the vertebrae from PULMONARY OUTCOMES OF RECENT
EOS TREATMENT completion of growing construct
an anterior approach on the convex surgery, FVC as a percentage of
The study of pulmonary function in normal declined by an average of
this patient population is extremely 28%.56,57 There are no untreated
control groups to assess what loss of
lung function might have occurred in
the natural progression of the spine
deformities. The implication of these
studies is that lung function is not
normalized or predictably improved
after treatments for EOS but that
progressive loss of lung function may
be reduced with treatment.
Surrogate pulmonary outcomes
that do not require voluntary
effort by young children have also
been reported. Several studies
used weight gain as an indirect
marker of improved pulmonary
function and found that up to 50%
of patients with EOS demonstrated
a mean 24- to 26-percentage-
FIGURE 9 point improvement after VEPTR
Radiograph demonstrating a broken rod FIGURE 10 or growing rod treatment.58,59
with traditional spine-to-spine growing rods, Clinical photo showing marked hardware
Overnight polysomnography in
identified by the red arrow. Reproduced with prominence in a thin child with EOS. Reproduced
permission of Children’s Orthopedic Center, Los with permission of Children’s Orthopedic children with EOS demonstrates an
Angeles, California. Center, Los Angeles, California. increased Apnea–Hypopnea Index

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8 YANG et al
and hypoxemia associated with survey of 14 pediatric spine surgeons VEPTR has potential advantages in
hypopneic events.60 Serial measures found that the majority considered cases that require direct expansion
of breathing during sleep before curves with recent progression and of the thorax, such as cases of
and after treatment of EOS may also a magnitude of ≥60° an indication thoracic dystrophy. Compression-
prove useful as an indirect measure for distraction-based implants.63 The based therapies need more data in
of lung function. indications for any of the distraction- the treatment of EOS, although they
based implants are similar, although appear to be an option in children
Two-dimensional images of the
spine, such as the Cobb angle, do
not correlate with lung function
measures and do not reflect changes
in lung function after spine curvature
has been reduced.61 However,
an encouraging study recently
demonstrated that radiographic
T1 to T12 height and T1 ro S1
height modestly correlate with
improved pulmonary function in
EOS.62 New imaging modalities,
such as diaphragm and thoracic
excursion, measured by dynamic
MRI, hold some promise in improving
assessment of spine structure–
respiratory function relations.
Persistent barriers to a high-quality FIGURE 11
literature on the topic include a A, Preoperative posteroanterior radiograph of a 7-year-old boy with a 61° curve. B, Initial postoperative
lack of control groups, because radiograph shows correction with a Shilla construct with a fusion at the apex (red arrows) and
untreated progressive EOS is known screws at the upper and lower anchors that can slide along the rod (yellow arrows). C, Images
2.5 years later show how the child’s growth has been guided by the rods, as demonstrated by the
to have a poor outcome, and a lack shorter distance beyond the anchors that the rods extend (blue arrow). Reproduced with permission
of standardization and difficulty in of Children’s Orthopedic Center, Los Angeles, California.
evaluating respiratory function in
young children. TABLE 2 Summary of Treatment Types for EOS and Some of Their Advantages and Disadvantages
Treatment Pros Cons
Bracing May help delay need for surgery in very Standard thoracolumbosacral orthosis
CURRENT TREATMENT
young patients. brace cannot control curves with apex
RECOMMENDATIONS above midthoracic spine (around T7).
Helpful for idiopathic EOS curves in Brace wear compliance may be difficult.
Management of EOS involves a juvenile patients near adolescent age.
diverse patient population, variable Not much literature about bracing in EOS.
spinal and thoracic deformities, and Casting Maximizes spinal growth before surgery. Some children may not tolerate full-time
multiple treatment options (Table body cast well.
Some idiopathic curves may resolve. Is not a definitive treatment in most cases.
2). Optimizing the treatment of each
Distraction Effective method to correct curve and Requires multiple periodic lengthening
child is a process in evolution. In lengthen the spine before final spinal surgeries (exception: magnetically
many cases, a trial of serial casting fusion. controlled rods).
can help control the scoliosis and Holds the most clinical experience and High complication rates (eg, implant failure,
allow growth while delaying surgery. literature in the surgical treatment of infection).
EOS.
In some idiopathic cases, these
Guided growth Initial apical fusion procedure guides Requires larger anatomy to allow
curves may resolve with casting subsequent spinal growth. instrumentation of apex (avoid in very small
alone. Many children may not be able children).
to tolerate casting or demonstrate No scheduled repeated lengthening
progression of the scoliosis despite procedures.
Compression Fusionless procedure. Limited data for use in EOS.
casting necessitating the initiation
Requires a thoracic approach (potentially
of growth-friendly spinal surgery. detrimental to pulmonary function).
There is considerable variation Risk of overcorrection when used for young
with regard to the optimal timing children.
and indication of surgery. A recent Reproduced with permission of Children’s Orthopedic Center, Los Angeles, California.

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PEDIATRICS Volume 137, number 1, January 2016 9
nearing adolescence, with less total A better understanding of the differently with regard to the rate
growth remaining. After achieving 3-dimensional natural growth of of curve progression. Williams et
maximal correction and growth with the thorax and how it is affected by al3 developed a new classification
growth-friendly spinal implants, surgical treatment in EOS is crucial. scheme in EOS that may help
children can have their spine Distraction-based implants help establish the optimal treatment of
definitively fused at a later age if decrease the scoliosis, although each subtype of EOS. Multicenter
significant deformity remains. how this result correlates with groups such as the Growing Spine
improved pulmonary function has Study Group and the Chest Wall
not been established. Radiographic and Spine Deformity Study Group
FUTURE DIRECTIONS IN EARLY-ONSET measurements in the 2-dimensional have been developed to collaborate
SCOLIOSIS in the study of this heterogeneous
plane, such as Cobb angles, are
not reliable predictors of severity population of children.
The basic unanswered question is
how much early and late treatment of pulmonary disease. Three-
strategies for EOS maximize dimensional understanding and
respiratory function when children functional imaging of the thorax
reach maturity. This question warrant additional study to improve
ABBREVIATIONS
remains difficult to study because characterization of how the structure
of the thorax relates to function to EOS: early-onset scoliosis
it is unethical to have an untreated
predict severity of pulmonary disease FVC: forced vital capacity
natural history comparison group
in EOS. MCGR: magnetically controlled
in which the scoliosis is allowed to
growing rod
progress relentlessly. Collaboration EOS incorporates a multitude of
PFT: pulmonary function test
between pediatric pulmonologists etiologies and associated diagnoses,
TGR: traditional growing rod
and orthopaedists is essential to and greater subclassification can
TIS: thoracic insufficiency
standardize how pulmonary function help develop a framework for future
syndrome
evaluations are being performed for study. Each etiology carries different
VEPTR: vertical expandable
children who are not able to comply implications, because congenital and
prosthetic titanium rib
with traditional PFTs. idiopathic EOS may behave much

DOI: 10.1542/peds.2015-0709
Accepted for publication Jul 28, 2015
Address correspondence to David L. Skaggs, MD, MMM, Children’s Orthopaedic Center, Children’s Hospital Los Angeles, 4650 Sunset Blvd, MS#69, Los Angeles, CA
90027. E-mail: dskaggs@chla.usc.edu
PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).
Copyright © 2016 by the American Academy of Pediatrics
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
FUNDING: No external funding.
POTENTIAL CONFLICT OF INTEREST: Dr Andras owns stock in Eli Lily, receives publishing royalties from Orthobullets, and is a board or committee member of
the Pediatric Orthopaedic Society of North America and the Scoliosis Research Society. Dr Skaggs has received grants from the Pediatric Orthopaedic Society
of North America & Scoliosis Research Society, paid to Columbia University; has received consulting fees or honoraria from Biomet, Medtronic, Zipline Medical,
Inc, and Orthobullets; is a board member of the Growing Spine Study Group, Scoliosis Research Society, and Growing Spine Foundation; has received payment
for lectures including service on speakers’ bureaus from Biomet, Medtronic, and Johnson & Johnson; is a patent holder for Medtronic and Biomet; has
received royalties from Wolters Kluwer Health–Lippincott Williams & Wilkins and Biomet Spine; and has received payment for the development of educational
presentations from Stryker, Biomet, Medtronic, and Johnson & Johnson. Drs Yang and Redding have indicated they have no potential conflicts of interest to
disclose.

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12 YANG et al
Early-Onset Scoliosis: A Review of History, Current Treatment, and Future
Directions
Scott Yang, Lindsay M Andras, Gregory J Redding and David L Skaggs
Pediatrics 2016;137;
DOI: 10.1542/peds.2015-0709 originally published online December 7, 2015;

Updated Information & including high resolution figures, can be found at:
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Early-Onset Scoliosis: A Review of History, Current Treatment, and Future
Directions
Scott Yang, Lindsay M Andras, Gregory J Redding and David L Skaggs
Pediatrics 2016;137;
DOI: 10.1542/peds.2015-0709 originally published online December 7, 2015;

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pediatrics.aappublications.org/content/137/1/e20150709

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it
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