Focus On

Management of
early-onset scoliosis
Early-onset scoliosis is defined as scoliosis diagnosed at the age
of five years or less (Fig. 1).1 These patients may have congenital
spine malformations and thoracic malformations, such as fused
or absent ribs, but they may also have normal vertebral formation.
Early-onset scoliosis is uncommon. The incidence of idiopathic
scoliosis has been reported to be 1.5% and infantile scoliosis
represents only 4% of this population.2 However, early-onset
scoliosis is an important health issue. These children develop
spinal deformities at young ages and if left untreated, are at
risk for rapid deformity progression, cosmetic disfigurement and
pulmonary insufficiency.3-6
Pehrsson et al7,8 reported that in patients with untreated
scoliosis, the mortality rate increased in infantile-onset scoliosis
followed by juvenile-onset scoliosis mainly due to respiratory
failure (40%). The average age at death was 54 years but some
patients died as young as 16 years.7-9 Postmortem findings
of these children would show very small lungs with markedly
decreased numbers of alveoli.10
Bracing
Early-onset scoliosis commonly leads to severe deformities
at an early age. Most are double major curves, and some
have substantial thoracolumbar kyphosis.11 There is no strict
guideline for treatment but most clinicians support observation
in children with mild deformities and bracing in children who
have progressive noncongenital curves. Unfortunately, bracing
is known to fail frequently in treatment of scoliosis in young
children, especially the congenital or neuromuscular types.12-15
This is especially true for those patients with a rib-vertebral angle
> 20, indicative of a progressive curve.16 Spinal bracing is now
commonly only used for minor curves in the range of 20 to
30.17,18 Bracing requires prolonged use, especially in patients
with early-onset scoliosis, and effective bracing is dependent on
patients compliance and proper fitting. Patients with proximal
thoracic curves require the use of a Milwaukee Brace, which is
usually not well tolerated by patients.12
Fusion Surgery
For early-onset scoliosis, early definitive fusion is advocated in
the past for children with severe curves, deteriorating congenital
spinal deformities, or progression despite bracing.19,20 However,
there is a growing consensus that early spinal fusion results in
negative pulmonary consequences and most surgeons now use
fusion as the last resort in the young patient.21
The technique of fusion surgery usually requires an anterior
approach to be effective due to the crankshaft phenomenon
or worsening rotational deformity in young patients with solid
posterior fusions resulting from persistent anterior growth of
2013 The British Editorial Society of Bone and Joint Surgery

the vertebral bodies as recognised by Dubousset,22 Goldberg

et al23, Karol et al24 and Vitale et al,25 who all looked into longterm results from fusion in patients with early-onset scoliosis
and found that 37.2% to 39.3% underwent reoperation due
to progression of deformity. Even some patients treated with
anterior and posterior fusion to theoretically eliminate crankshaft
progression required subsequent revision surgery.23,24 Achieving
circumferential fusion of the spine in young children may still fail
due to continued growth.26
Besides failure of the fusion to control the deformity, fusion
can have drastic consequences on overall growth of the child. In
DiMeglios27,28 studies on spinal growth, the most rapid period
of spinal growth occurred in the first five years of life, when the
spine increased 50% of its length. It was also found that between
the ages of five and ten years, the spine continued to grow, albeit
at a slower rate.27,28 In summary, the height of the thoracic spine
averaged 11 cm in the newborn, 18 cm at age five years and
22 cm at age ten years.27,28 Translating this knowledge to
treatment of early-onset scoliosis, spinal fusion in a five-year-old
child can result in a 12.5 cm loss of spinal growth.27
In addition, fused spines in growing children may lead to
arrested pulmonary development.24,25,29 This leads to thoracic
insufficiency syndrome or inability of the thorax to support normal
respiration or lung growth.12-14 Campbell et al30 showed that
immobile or nonfunctional chest wall caused by small thoracic
height and multiple congenital rib malformations interfered with
normal respiration. Poor pulmonary function was also seen in
patients who had undergone early spinal fusion for scoliosis,
inhibiting normal thoracic growth.30 Normal development of the
lungs in the growing child had been well established.10,31,32 The
greatest increase in the number of alveoli in normal children
occurred in the first two years of life.32 Although lung volumes
continued to increase into adolescence, alveolar growth was
complete by eight years of age.32 For this reason, surgery at
this age could create the greatest disturbance in pulmonary
development.33,34 Pulmonary volumes tend to decline with
normal aging and the decline was expected to begin in the
mid-30s.35 Patients who have poor pulmonary reserve as a
baseline would expect more significant loss of volume with early
definitive fusions. Goldberg et al29 showed that fusion performed
in patients who were younger than ten years of age had a mean
forced vital capacity (FVC) of 41% of the normal at maturity as
compared to 68% of normal FVC in patients fused older than ten
years of age.
Fusionless Surgery
Various methods without the need of fusion are being designed
to control growth, to delay definitive fusion surgery, and to
1

J. P. Y CHEUNG, D. SAMARTZIS, K. M. C. CHEUNG

increase the thoracic volume. Applying principles of managing

limb length discrepancies and gradual limb deformity corrections
to the spine, epiphysiodesis on the convex side of the deformity
with or without instrumentation is a technique to provide gradual
progressive correction and to arrest the deterioration of the
curves. Hemiepiphysiodesis, hemivertebral resection, and short
segment fusions can be used in selected patients to allow for
gradual correction of these curves. Staples used are usually
biocompatible shape-memory metal alloy staples. However,
these techniques are still controversial with mixed results at
short-term follow-up. One study by Marks et al36 found that
anterior and posterior growth arrest alone was not effective
for preventing progression of deformity in infantile scoliosis. In
contrast, Betz et al37 showed that stapling the anterior vertebral
spinal growth plates could control curve progression. In their
study, six out of ten patients with a mean curvature of 35 were
controlled and stabilised during a one year follow-up period.
Growing Rods
To address the limitations of spinal bracing or fusion for earlyonset scoliosis, growing rods (distractible spinal implants)
were developed.38-40 Techniques of instrumentation include
Harrington, Cotrel-Dubousset, or Luque rods.41 More recently,
Akbarnia et al39 developed a technique using Isola dual rod
instrumentation. These growing rods are inserted across a
segment of spinal deformity where no fusion is performed.
Cranial and caudal anchoring foundations are made using hooks
or pedicle screws. Each foundation is connected to a rod, and
the rods are connected by cross-links. Distraction of the growing
rods is performed usually every six months in which the surgical
incision site must be reopened for the distraction procedure.
Once maximum spinal growth or skeletal maturity is reached,
definitive final fusion is performed. By this approach, growing
rods could control progression of spinal deformity as well as
gradually correct the deformity.15,21,38,39,42-44
In addition to gradual correction of spinal deformity and
allowing continuous spinal growth, thoracic insufficiency
syndrome could also be treated by the Vertical Expandable
Prosthetic Titanium Rib (VEPTRTM)45 device (Fig. 2). After an
opening-wedge thoracostomy, the acute correction can be
stabilised by this device. The VEPTR is extended from the cephalad
rib to the caudal rib, the lumbar spine, or to the posterior iliac
crest.46 These devices are also distracted at scheduled intervals
of six months. At a mean follow-up of 5.7 years, the vital capacity
was found in one study to have significantly increased with mean
coronal deformity correction of 74 to 49 at the latest followup.30 Technically, single growing rod instrumentation could be
placed on the concave side of the spine to expand the smaller
hemithorax.47 Some studies report better radiographic correction
and fewer complications with bilateral constructs.21
Despite the advantages of growing rod surgery, the
need for repeated surgeries under general anesthesia is a
major drawback due to increased anaesthetic and wound
complications.5,6 Other complications include proximal migration
of devices with rib fractures, and skin problems secondary to
recurrent surgery and hardware.41,45,46,48 The complication rates
ranged from 8% to 50%.41,45-48 Instrumentation failure included

Fig. 1. Posterior external view of a five-year-old

girl with early-onset scoliosis, noted with coronal
imbalance and prominent right scapular hump.

Fig. 2. Postero-anterior thoracic radiograph of a three-year-old boy with

spondylo-thoracic-dysplasia with thoracic insufficiency syndrome who
underwent bilateral implantation of a VEPTR device for correction of the
spinal deformity and enlargement of the thoracic cage.

MANAGEMENT OF EARLY-ONSET SCOLIOSIS

development calls for the construction of an implant that allows

less invasive distractions. A remotely distractable, magneticallycontrolled growing rod system has been developed to facilitate
out-patient rod distractions, eliminate frequent surgeries
under general anesthesia and reduce wound complications,
psychological and socioeconomic problems, and frequent
hospitalisation in young children (Fig. 3). This procedure can
also mimic normal physiological growth and provides continuous
neurological monitoring in a conscious patient during distractions.
In addition, frequent distractions may avoid the law of diminishing
returns suggested by Sankar et al.54 The magnetically-controlled
growing rod has already been validated in animal studies.55,56
The first report of the safety and efficacy of this technology was
reported by Cheung et al.57 Their study noted that the corrective
power of the device was similar to the conventional growing
rod at two year follow-up. It was found that consistent gains in
spinal growth were obtained with each distraction. The monthly
increase also matched the predicted monthly spinal growth
in five- to ten-year-olds27 and other traditional growing rods.39
Furthermore, there were no major rod or wound complications
and there were good clinical outcome scores. Wick et al58 also
showed promising results with a similar magnetic growing rod
(Phenix rod) in two case reports. As such, the preliminary findings
of the magnetically-controlled growing rods in young children
with early-onset scoliosis have shown to be safe and effective;
however, longer and larger studies are warranted to further verify
the current findings, assess complications on long-term followup, and refine patient selection.

Fig. 3. Postero-anterior thoracic radiograph of a female

with early-onset scoliosis who underwent curve correction
management with bilateral magnetic remote-controlled growing
rods.

rod fracture or anchor failure and the overall rate was reported
to be 15%.49 Junctional kyphosis and curve decompensation
were also noted but rarely occurred.39 In addition, growing rod
surgery is associated with various socioeconomic concerns
including longer periods of hospitalisation and increased time
away from school for the child and time away from work for the
parents.50-52 Repeated operations and hospitalisation may also
affect the childs psychological well-being and appearance with
a cosmetically poor surgical scar.50-52
Recently, there were reports of decreased spinal length gain
from repeated lengthening of the growing rods.53,54 Sankar et al54
proposed a law of diminishing returns whereby the average T1S1 gain from a given surgical lengthening decreased significantly
with repeated lengthening. Forcing distraction in such cases
would result in implant failure. This phenomenon was likely due
to progressive stiffness or autofusion of the spine caused by
sudden distractions.54
Magnetically Controlled Growing Rods
With the limitations associated with the growing rod, natural

Conclusion
Early-onset scoliosis is a difficult challenge. These patients are
young with significant remaining growth potential. Thus, patients
will likely develop progressive deformities, cosmetic disfigurement
and cardiopulmonary consequences. Conventional management
tools, such as spinal bracing and fusion, have not been shown
to be effective. In addition, fusion may also cause significant
inhibition of normal spinal and thoracic growth. Growing rods
have been the mainstay treatment of early-onset scoliosis with
the ability to allow periodical distractions to accommodate
growth. However, these growing rods require repeated surgeries
for distraction and are associated with exponential increase in
complications, namely anesthetic and wound complications.
With the development of the magnetically-controlled growing rod
system, remote and non-invasive distractions can be performed,
reducing the need for frequent follow-up surgeries and the
complications associated with such treatment, quicker return
to activities, decrease psychosocial consequences for child
and parent, in the long-term are more cost-effective. However,
as with any new technology, additional studies are needed to
further reduce complications and improve outcomes in children
undergoing treatment for early-onset scoliosis.
J. P. Y Cheung MBBS(HK), MMedSc
D. Samartzis DSc
K. M. C. Cheung MBBS (UK), MD (HK), FRCS, FHKCOS, FHKAM
(Orth)

J. P. Y CHEUNG, D. SAMARTZIS, K. M. C. CHEUNG

Department of Orthopaedics & Traumatology

University of Hong Kong
Pokfulam
Hong Kong SAR
China
E-mail: cheungmc@hku.hk
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