REVIEW ARTICLE
Early Onset Scoliosis: Modern Treatment and Results
John E. Tis, MD,* Lawrence I. Karlin, MD,w Behrooz A. Akbarnia, MD,z
Laurel C. Blakemore, MD,y George H. Thompson, MD,8 Richard E. McCarthy, MD,z#
Carlos A. Tello, MD,** Michael J. Mendelow, MD,ww Edward P. Southern, MD,zz
and the Growing Spine Committee of the Scoliosis Research Society
Background: Early onset scoliosis (EOS) is a potentially fatal,
challenging group of diseases the management of which has
markedly changed in the last decade. The purpose of this review
is to provide the reader with a brief description of each of these
new therapeutic modalities, their indications for use, and early
clinical results.
Methods: A systematic review of peer-reviewed publications and
abstracts related to the treatment of EOS in the last decade was
carried out and synthesized into a review of modern treatment
methods.
Results: Recent advances in techniques and understanding of
preserving the thoracic space have improved the morbidity and
mortality of children with progressive EOS. Derotational cast-
ing may be used in younger patients with curves between 25 and
60 degrees. The vertical expandable prosthetic titanium rib is
best suited for patients with thoracic insufficiency syndrome.
Single or dual growing rods may be used alone or in combina-
tion with vertical expandable prosthetic titanium rib to treat
patients with progressive EOS who are not candidates for
casting. Shilla technique is an alternative to growing rods that
avoids the morbidity of repeated lengthenings but is not as well
proven as the techniques described above. Other methods such
as automatic growing rods and growth modulation techniques
From the *Department of Orthopaedic Surgery, Johns Hopkins Uni-
versity School of Medicine, Bloomberg Children’s Center, Baltimore,
MD; wDepartment of Orthopaedic Surgery, Harvard Medical
School, Children’s Hospital, Boston, MA; zDepartment of Ortho-
paedic Surgery, University of California, San Diego Center for
Spinal Disorders, La Jolla, CA; yDepartment of Orthopaedic Sur-
gery, George Washington University, Orthopaedics and Sports
Medicine, Children’s National Medical Center, Washington, DC;
8Department of Orthopaedic Surgery, Case-Western Reserve Uni-
versity, Rainbow Babies and Children’s Hospital, Cleveland, OH;
zDepartments of Orthopaedics and Neurosurgery, University of
Arkansas; #Spinal Deformities, Arkansas Children’s Hospital, Little
Rock, AR; wwShriners Hospitals for Children, Greenville, South
Carolina; zzDepartment of Orthopaedic Surgery, Louisiana State
University Health Science Center, Childrens Hospital of New
Orleans, LA; and **Hospital de Pediatria Garrahan, Universidad de
Buenos Aires, Buenos Aires, Argentina.
Thompson GH is the Co-Editor for the Journal of Pediatric Orthopae-
dics. The other authors declare no conflicts of interest with the
present publication.
None of the authors received financial support for this study.
Reprints: John E. Tis, MD, Department of Orthopaedic Surgery, Johns
Hopkins University, Bloomberg Children’s Center, 1800 Orleans
Street/7356, Baltimore, MD 21287. E-mail: jtis1@jhmi.edu.
Copyright r 2012 by Lippincott Williams & Wilkins
J Pediatr Orthop  Volume 32, Number 7, October/November 2012
are still investigational, and their role needs to be defined after
further study.
Conclusions: Recent advances have improved the treatment of
children with EOS. Treatment continues to be challenging with
complication rates higher than treatment of idiopathic scoliosis.
Level of Evidence: Level V.
Key Words: early onset scoliosis, growing rods, VEPTR, hybrid
growing rods, Shilla, vertebral stapling, derotation body cast
(J Pediatr Orthop 2012;32:647–657)
T he management of severe, progressive early onset
scoliosis (EOS) is challenging. The natural history can
be severe deformity, restrictive pulmonary disease, car-
diac disease, and early mortality.1–5 In the past, the
standard of care for these children was early definitive
anterior and posterior spinal fusion (PSF) and spinal in-
strumentation. The belief was that a short and straight
spine was superior to a long and deformed spine despite
the negative effects of a short trunk and disproportionate
body habitus. The principles of EOS treatment have
changed with the appreciation that early fusion of the
thoracic spine limits the growth of the spine and lungs,
and eventually leads to respiratory failure and increased
mortality.3,6,7 The focus has shifted from the spine alone
to the spine, chest wall, and lungs; and the goals of treatment
to a well-aligned spine and a thoracic cavity sufficiently de-
veloped to support adequate pulmonary development and
function. Recognizing the importance of pulmonary func-
tion, a few developmental principles should be understood.
Bronchial tree and alveolar complement are maximally de-
veloped by 8 years of age, and the thoracic volume at 10
years of age is 50% of expected adult volume.8–12
The T1-S1 length increases most dramatically in the
first 5 years of life (2.2 cm/y), is slower during the next 5
years (1 cm/y), and increases again at onset of puberty
(1.8 cm/y).13,14 Achieving a T1-T12 length of at least 18 cm
at maturity is associated with better pulmonary function.7,15
At present, a number of spinal and chest wall
growth-sparing techniques have been developed and re-
fined. Serial corrective cast treatment has resurfaced as an
effective treatment, and recent studies document excellent
results with derotational methods.16,17 The list of growth-
sparing surgical techniques continues to increase, and
these are being classified to facilitate treatment decisions
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TABLE 1. Classification of Growth-sparing Surgical Techniques

Distraction Based Guided Growth Convex Compression Growth Inhibition Other
Single rod Luque trolley Shape memory staples Rib lengthening/shortening
Dual rod Shilla Tethers Neurocentral synchodrosis hemiepiphyseodesis
VEPTR
Hybrid growth rods
Phenix/MAGEC
VEPTR indicates vertical expandable prosthetic titanium rib.
Modified from Skaggs.18

(D.L. Skaggs, 2010, personal oral communication) Clinical Results

(Table 1). The indications and effectiveness of each Several modern series have documented the positive
technique remain controversial. It is worthwhile to note results of derotational cast treatment.16,17,23,24 In Mehta’s
that the instrumentation used in the following techniques series of 136 patients, results were related to age and curve
is being used “off label” as defined by the Food and Drug severity at the time of the initiation of treatment.
Administration. The challenges associated with the use In children with a mean age of 1.6 years and a mean
of these systems include obtaining and maintaining de-
formity correction, achieving adequate spinal growth,
allowing adequate lung development, and decreasing the
high incidence of complications. The purpose of this
manuscript is to review the current options for growth-
sparing treatment of EOS.

DEROTATIONAL CASTING
Indications
A patient with documented progression (progression
of 10 to 20 degrees or progression past 25 degrees) but
low magnitude (< 60 degrees) coronal deformity, or an-
ticipated progression (rib-vertebral angle difference of >20
degrees, rib phase 2) in whom no surgical methods have
been attempted are common indications for serial casting
technique.

Concept
Until recently, cast treatment, the standard of care
for all scoliosis treatment before the advent of effective
spinal instrumentation, was associated with many com-
plications, including superior mesenteric artery syndrome,
pressure sores, and rib or mandibular deformities.19–22
These problems have been addressed by the replacement
of the Risser cast, which was based on a 3-point bending
principle, with the derotational technique introduced in
Europe in the 1960s and recently popularized by Mehta.
The cast is applied to the intubated, anesthetized child
using a specially designed table (Fig. 1) that permits the
application of proximal and distal traction, supports the
head, arms and legs, and exposes the trunk for the cast
placement. The ribs are not pushed towards the spine, but
are instead rotated using an anteriorly directed force on 1
side and posteriorly directed force on the other. Anterior
and posterior windows are made. Casts are changed every
2 to 4 months and discontinued or replaced by orthotic
treatment when the curve is <10 to 20 degrees. Casts may FIGURE 1. Graphic (A) and photograph (B) depicting the
not be tolerated in children with poor pulmonary func- position of the infant on the special casting table, the position of
tion or children with sensory integration abnormalities the surgeon’s hands as seen in the mirror, and the derotational
that render them unable to tolerate cast immobilization. forces applied to the chest wall (from Sanders et al.23).

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J Pediatr Orthop  Volume 32, Number 7, October/November 2012
(coronal) deformity of 32 degrees (group 1), the scoliosis
resolved by 4 years of age. Further treatment was not
needed. Children who were treated at a mean age of 2.5
years and a mean deformity of 52 degrees (group 2) had
progression of their scoliosis and 15 of these children
required definitive spinal fusion. In this and earlier stud-
ies, the prognosis was improved when the rib-vertebral
angle difference was <20 degrees.25 The study also
identified a phenotypic subtype of slender and liga-
mentously lax children in whom the deformity was rap-
idly progressive. Similar results were reported by Sanders
et al.16 In 55 patients with documented or anticipated
progression, treatment begun at a younger age, curve
magnitude <60 degrees, and an idiopathic etiology were
all seen to improve the prognosis. In a separate study
comparing brace, cast, and vertical expandable prosthetic
titanium rib (VEPTR) treatment, bracing was found to be
ineffective, cast treatment effective for curvatures <52
degrees, and VEPTR best for larger curvatures.24
DISTRACTION-BASED SURGICAL TECHNIQUES
Indications
These techniques are considered for progressive
deformities when cast or brace treatment has failed or
is contraindicated. In general, the rods are lengthened
at 6-month intervals, making this modality problematic
in children with high comorbidities that can compli-
cate the numerous anesthetics, surgical procedures, and
hospitalizations.
SINGLE GROWING RODS
Concept
The principle in distraction-based implants is to
anchor instrumentation to the spine proximally and to the
FIGURE 2. A, Posteroanterior radiograph of a 3-year-old boy with idiopathic early onset scoliosis with a progressive curve
measuring >50 degrees. B, Posteroanterior radiograph of the same patient 6 months later after the derotational cast has been
applied. C, Frontal photograph of the same patient showing the cutouts and molding of the cast.
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Treatment of Early Onset Scoliosis
spine or pelvis distally and avoid exposure of the inter-
vening spinal segment. Growth occurs through this cen-
tral segment and the deformity is controlled by serial
lengthening procedures that produce the spinal “growth.”
There are a number of variations of this theme. The
concept of a long subcutaneous rod spanning an unfused
spine was first introduced in 1962 by Harrington, and
later developed by other authors.26–30 The results were
associated with a high complication rate including im-
plant failure, loss of fixation at the anchor points, and
spontaneous fusion of the spine resulting in limited spinal
and thoracic growth.31 The results improved with the
advent of more secure anchor points. Morin32 was the
first to use segmental spinal instrumentation with a
“claw” foundation consisting of a downgoing supra-
laminar or transverse process hook and an upgoing sub-
laminar hook placed 1 or 2 segments distal. A single
growing rod employs either “claw” or pedicle screw
foundations33,34 with a limited fusion performed about
the foundation sites. A single pediatric (4.5 mm) or adult
(6.2 mm) rod is left long (4 to 5 cm) above or below the
proximal or distal foundation depending on the curve
location, and is used for periodic lengthenings. Two rods
overlapping in the middle and connected by side-by-side
connectors may also be used. A thoracic-lumbar-spinal
orthosis is sometimes used after surgery. Periodic
lengthenings are performed. Once the child has reached
sufficient age and size, a definitive PSF, or alternatively,
an anterior spinal fusion and PSF with segmental spinal
instrumentation, can be performed (Fig. 2).
Clinical Experience
Because single growing rods have the longest his-
tory, there are numerous series evaluating the results and
complications associated with their use. One of the ear-
liest large series was a group of 67 children with EOS
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FIGURE 3. A and B, A 2 year and 8 month old girl with infantile idiopathic scoliosis was not a candidate for cast treatment and
had dramatic progression of her deformity despite treatment with an orthotic. C, Dual growing rods provided excellent cor-
rection. D and E, Five years later, there has been continued correction of the deformity and 6.4 cm T1-S1 growth with serial
6-month lengthenings. The most recent procedures have produced less length as is consistent with what we now understand to
be diminishing returns over time. The stiffness and perhaps premature fusion provide a compelling argument for nonoperative
solutions in the very young.

secondary to a variety of etiologies between 1973
and1993.30 The deformity improved a mean of 20 degrees
from 67 to 47 degrees at the time of definitive fusion. The
rods were lengthened a mean of 31 mm over the mean
treatment time of 3.1 years. The authors found the proce-
dure to be safe; however, there was 1 death related to a rod
exchange through a subfascial tunnel. There were numer-
ous implant-related complications and more difficulty in
lengthening the rod with each subsequent procedure. Other
series have similar results with predominant infection and
implant-related complication rates, of 24% to 59%.34,35
DUAL GROWING RODS
Concept
Dual growing rods, popularized by Akbarnia and
colleagues,33,36–39 follow the same principle of single
growing rods but provide improved stability and de-
formity correction. Each rod is composed of 2 sections
that are connected by an end-to-end tandem connector
through which the lengthenings are performed. The
foundations are either pedicle screws or hook “claws” and
these anchor points are fused.40 In neuromuscular pa-
tients the surgeon should consider pelvic fixation for the
distal anchor. Sponseller et al41 demonstrated sig-
nificantly better correction of pelvic obliquity and coronal
deformity using dual rods and iliac screws compared with
other types of pelvic fixation. The rods are lengthened
every 6 months regardless of curve progression (Fig. 3).
to 1. There were a total of 13 complications (56%) in 11
patients. Another, more recent, series showed a slightly
better curve correction and more length gained when
lengthenings were performed at intervals of r6 months.38
In a study comparing single rods, single rods with short
apical fusion and dual rods, patients who underwent the
short apical fusion had the poorest curve correction and
length gained, whereas the dual rod group had the best
initial correction, maintenance of correction, spinal
growth per year, and the percentage of expected T1-S1
growth.33
Complications are frequent and related to the pro-
longed treatment required of distraction-based techniques.
A comprehensive analysis of complications from single and
dual rod constructs reports 58% of the 140 patients eval-
uated had at least 1 complication.42 The complication rate
increased by 24% for each additional procedure performed,
and complication rate decreased by 13% for each year of
increased patient age at treatment initiation. There were less
instrumentation complications in dual as opposed to single
rods, and patients with subcutaneous rods had more wound
complications, prominent implants, and unplanned proce-
dures than those with submuscular rods (Table 2). Wound
complications are more common when lengthenings are
performed at more frequent intervals, whereas implant-re-
lated complications occur more often when lengthenings
are performed at longer intervals.43 Regarding neurological
safety, the insertion and lengthening of both single and dual

Clinical Experience
The largest clinical series involving dual growing TABLE 2. Complications With Single/Dual Growing Rods
rods was published by Akbarnia et al39 in 2005. The mean Unplanned procedure for implant complications
curve magnitude improved from 82 degrees pre- Single rod 27%
operatively to 38 degrees after the first surgery and 36 Dual rod 10%
degrees at final follow-up. Patients were lengthened a Wound complications
mean of 6.6 times for a mean T1-S1 length increase Subcutaneous rod placement 26%
Submuscular rod placement 10%
of 1.2 cm/y and an increase in lung space ratio from 0.87

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J Pediatr Orthop  Volume 32, Number 7, October/November 2012
growing rods appears to be extremely safe with an overall
incidence of neuromonitoring changes of 0.9%, and only 1
reported clinically detectable transient neurological deficit
(0.1%)44 in a patient with a known intradural tumor.
Finally, there appears to be a “Law of Diminishing Re-
turns” for repeated lengthenings of growing rods. Sankar
et al,45 reporting on 38 patients, noted the mean T1-S1
length gain from the first lengthening was 1.04 cm and
progressively less with subsequent procedures.
VEPTR
Concept
The VEPTR (VEPTR-Synthes Spine, West Chester,
PA) is a titanium alloy longitudinal rib distraction device.
As with growth rods, repeated lengthenings are required.
It was recently approved by the Food and Drug Ad-
ministration under Humanitarian Device Exemption for
treatment of skeletally immature patients with thoracic
insufficiency syndrome (TIS).46–48 Approved anatomic
diagnoses of TIS include flail chest syndrome, constrictive
chest wall syndrome that includes rib fusion and scoliosis,
hypoplastic thorax syndrome, which includes Jeunes
syndrome, achondroplasia, Jarcho-Levin syndrome, and
Ellis van Creveld syndrome, and progressive scoliosis of
congenital or neurogenic origin without rib anomaly.49
FIGURE 4. A, A 7-month-old child had a progressive scoliosis due to fused ribs and congenital spinal deformities. B–D, The initial
rib resection and vertical expandable prosthetic titanium rib improved the chest size, but failed to halt the progressive deformity.
E–G, Here the technique was adapted to the situation by adding an additional rib-to-spine rod to control the chest as well as the
spine and an excellent result obtained (courtesy: John Emans, MD).
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Treatment of Early Onset Scoliosis
Infantile idiopathic or syndromic EOS are included in this
group if a constrictive chest wall syndrome is present
based on a windswept deformity of the thorax at the apex
of the curve on computed tomography.50,51
The operative strategy of VEPTR application varies
by diagnosis. In type II volume depletion conditions (fused
ribs and scoliosis), the goal is to maximize thoracic volume
and symmetry of the deformed thorax by lengthening the
constricted hemithorax through a transverse opening wedge
thoracostomy of the concave side,47,48 either through os-
teotomy of fused ribs or intercostal muscle lysis. The sco-
liosis is corrected indirectly by the thoracostomy, and the
thoracic reconstruction is stabilized by the addition of a rib
to spine or rib to pelvis VEPTR construct, and another rib
to rib VEPTR. No bracing is used postoperatively. The
VEPTRs are lengthened on schedule every 4 to 6 months.
As 50% of final thoracic volume depends on growth be-
tween age 10 and 15 years, final fusion is preferentially
delayed until skeletal maturity (Fig. 4). Other conditions
such as myelodysplasia, in which secondary TIS may de-
velop, can be treated with VEPTR devices placed without
expansion thoracoplasty.
Clinical Experience
Originally intended to treat children with TIS secon-
dary to fused ribs and congenital scoliosis, the indications
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Tis et al J Pediatr Orthop
have expanded to include all etiologies of progressive EOS
(congenital, neuromuscular, and syndromic with or without
fused ribs) in which the surgeon wishes to avoid or mini-
mize direct exposure of the spine. Campbell et al47 studied
27 children with congenital scoliosis associated with fused
ribs and found a mean correction of the scoliosis from 74 to
49 degrees, and thoracic spine height increased by a
mean of 7.1 mm/y. There were 52 complications (193%) in
22 patients. The most common complication was
“asymptomatic” proximal migration of the device through
ribs in 7 patients. Hasler et al52 studied 23 children with
noncongenital EOS. Cobb correction was from 68 degrees
preoperatively to 54 degrees at the final follow-up.
Although space available for the lung (SAL) significantly
increased, the percentage predicted pulmonary values were
not reported. There were 23 complications (100%) of which
16 were wound complications and 7 implant-related com-
plications. In children older than 10 years comparable
correction and a lower complication rates have been
documented.53 Use in “exotic scoliosis” has also been de-
scribed.54 A recent study of the Chest Wall Disorders Study
Group Database revealed that although there was a sig-
nificant decrease in Cobb angle and increase in SAL, ob-
jective measures of pulmonary function did not seem to
improve as expected.55 Computed tomographic examina-
tion has demonstrated no correction, but only stabilization
of the transverse plane deformity seen in TIS. All authors
caution the need for a multidisciplinary team approach
when the VEPTR is used. With proper precautions its use
appears to be relatively safe with low mortality even in the
most severely affected patients who have a poor prognosis
FIGURE 5. A and B, The hybrid technique allows excellent spinal deformity correction while avoiding the morbidity of an upper
thoracic fusion. Conventional instrumentation is used. Note the medial placement of the rib hooks (courtesy: David Skaggs, MD).
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if no treatment is undertaken. Betz et al56 studied 43 pa-
tients with either Jarcho-Levin or Jeune Syndrome, and
found a total mortality rate of 9% of which all deaths
occurred in patients with Jeune Syndrome from respira-
tory, liver, or renal failure. Another 9% had potentially
life-threatening occurrences of which half were indirectly
related to the surgery.
HYBRID DISTRACTION-BASED GROWING RODS
Concept
Fusion of the upper thorax, as is performed for the
upper anchors of growing rods, has been shown to ad-
versely affect pulmonary function.7 Hybrid systems utiliz-
ing standard hooks avoid this fusion by using ribs as the
upper anchor. In addition, soft-tissue coverage of the hook
on the rib position is usually better than when using spinal
fixation (Fig. 5).
Clinical Experience
This is a recently described technique.18 A multi-
center study has shown bilateral hybrid growing rods
produce on average 1.2 cm/y of T1-S1 growth which is
comparable with that found with dual growing rods and
superior to that of VEPTR.57 In a comparison study in
similar patient populations, the hybrid instrumentation
had a complication rate of 0.86/patient, the dual growing
rods 2.3/patient, and the VEPTR 2.37/patient.58
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LESS CONVENTIONAL TECHNIQUES/
FUTURE DIRECTIONS REMOTE CONTROL:
PHENIX, MAGEC
Concept
Both the Phenix (France) and MAGEC [(Magnetic
Expansion Control System) Ellipse Technologies, Inc.,
Irvine, CA] are distraction-based systems that can allow
growth with remote control theoretically diminishing the
complications of infection and lack of soft-tissue cover-
age. It may be especially helpful in children with co-
morbidities that make the repeated surgeries necessary
with the usual distraction methods especially difficult.
Clinical Experience
The Phenix rod system, currently unavailable in the
United States, was developed by Drs Lotfi Miladi Arnaud
Soubeiran, and Jean Dubousset that uses magnets to
produce gradual lengthening without repeated surgeries.
Rib or spine fixation is possible. In a preliminary study,
there was correction of the scoliosis from 63 to 33 degrees
and a growth rate of 2 mm/mo.59 The MAGEC system is
a similar magnetic device. An animal study has shown
favorable results.60 Lengthenings can be performed in the
office without anesthesia. Longer-term results of both
systems will be needed to determine the efficacy and safety
of this technology.
GROWTH INHIBITION: CONVEX COMPRESSION
Concept
These technologies, based on the Hueter-Volkman
Law, inhibit growth by compression of the convexity of
the deformity. The idea is that the scoliosis will then
correct through growth on the concavity. Historically,
convex growth inhibition was performed through
the technique of anterior and posterior hemiepiphy-
seodesis,61–64 although this technique was primarily used
for congenital scoliosis and was not found to be effective
for other types of EOS.34 More recently, hemiepiphyse-
odesis has taken the form of shape memory alloy (SMA)
staples,65–71 tethers,72–75 and transpedicular growth mod-
ulation of the neurocentral junction.76–78 Although most
of these techniques are experimental there is a fair
amount of clinical experience with SMA staple treatment
in older children.66,69,79,80
Anterior SMA Staples
Indications
Patients considered for this procedure should have
at least 1 year of growth remaining and a deformity that
could also be considered for bracing. According to Betz
and colleagues, the thoracic and lumbar curves should
be <45 degrees with minimal rotation and flexible to
<20 degrees. The sagittal thoracic curve should be <40
degrees.81
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Treatment of Early Onset Scoliosis
Clinical Experience
Early spinal staples failed because of dislodgement.
This problem now seems to be solved with a Nitinol
(Nickel Titanium Naval Ordnance lab) staple. The prongs
of the staple are straight when cooled but change to a “C”
shape and stabilize within the bone when they heat up to
body temperature. Using these staples, Betz et al66 fol-
lowed 26 children with thoracic curves and 15 with lum-
bar curves who were a mean age of 9 when the staples
were placed. In the thoracic curvatures, 78% either sta-
bilized or improved when the curves were <35 degrees at
staple insertion. Larger curves demonstrated a 75%
progression rate past 50 degrees. In patients less than age
10 with thoracic curves of all curve magnitudes, there was
a 75% success rate. The stapling also appeared to have a
positive effect on sagittal contour in patients with hypo-
kyphosis. Lumbar curves demonstrated an 87% success
rate overall and 100% success rate in patients under the
age of 10. Complications included rupture of a previously
unrecognized diaphragmatic hernia, overcorrection of 1
curve, atelectasis, and superior mesenteric artery syn-
drome. Similar results have been reported by others69
(Fig. 6).
Anterolateral Spinal Tethering
Concept
Tethers, like staples, produce correction by convex
growth inhibition, but use flexible connections between
the vertebral anchor points. Numerous animal models
have demonstrated the ability of tethers to produce and
correct scoliosis.72–74,82,83 The tether is theoretically less
likely to damage the intervertebral disks than rigid de-
vices. Preservation of disks has been demonstrated in
animal models,84 however, both tethers and staples do
produce chemical, cellular, and vascular changes in the
disk and endplate,74,85 although the significance of these
changes is unknown.
Clinical Experience
Most of the experience is experimental. There is a
case report of an 8-year-old boy treated with vertebral
body screws connected by a polypropylene tether.86 The
40 degrees thoracic scoliosis was reduced to 25 degrees at
the time of surgery and improved to 6 degrees at the
4-year evaluation.
Growth Guidance
Indications
This technique should be considered for progressive
deformities when cast or brace treatment has failed or is
contraindicated. As there is no need for repeated open
distractions and associated anesthesia, the method seems
better suited than distraction techniques in children with
significant medical comorbidities.
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FIGURE 6. A and B, A 11 year and 9 month old boy with early onset idiopathic scoliosis would not tolerate a brace. Convex staple
hemiepiphyseodesis was chosen as treatment. C, The index procedure produced good correction of the coronal deformity. D, In
the first year after surgery the deformity progressed. E, Follow-up radiograph demonstrates that with growth, the deformity
improved. F and G, Lateral and posteroanterior radiographs 2 years after surgery demonstrate that the deformity continued to
improve nicely with continued growth (courtesy: M. Timothy Hresko, MD).

Shilla Procedure
Concept
The precursor to the Shilla system (Medtronic Spine,
Memphis, TN) was the Luque trolley system,87–91 which
did not appear to provide reliable spinal growth or de-
formity correction.91 In the Shilla procedure, multiplanar
correction is obtained by pedicle fixation at the apex of the
deformity. This usually involves instrumentation and fusion
of 3 to 4 segments. Four to 6 gliding pedicle screws (Shilla
growing screws-Medtronic) are then placed at each end of
the construct. These screws are placed without subperio-
steal exposure to decrease the risk of inadvertent fusion
of the proximal and distal segments. Good fluoroscopic
technique and equipment are essential for placement of the
proximal and distal anchor screws because the anatomic
landmarks are not exposed. The rods are left long at the
ends to allow the sliding screws to move along the rods with
spinal growth. Normal sagittal contours are maintained
and the anchor screws slide cranially and caudally on the
dual rods as the patient grows. With correction and stabi-
lization of the most deformed apical segment there is the-
oretically less stress on the end anchor points. The patients
are placed in a thoracic-lumbar-spinal orthosis for 3
months after the surgery (Fig. 7).
Clinical Experience
Although the effectiveness of this concept has been
proven in a caprine model,92 the Shilla technique has no

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FIGURE 7. A and B, Posteroanterior and lateral radiographs of a 3-year-old boy with scoliosis. C and D, Posteroanterior and lateral
radiographs taken 31 months after surgery using the Shilla technique reveal good correction of the deformity and continued
growth over time. There is guided growth without further surgery (courtesy: Richard McCarthy, MD).
long-term follow-up series to date. Thus far, the results of
10 patients with > 2 year follow-up from a cohort of 36
patients have been reported.93 Curve magnitude went
from a mean of 70.5 degrees preoperatively to 27 degrees
postoperatively and 34 degrees at 2 years. Truncal height
increased a mean of 12% and the SAL increased an
average of 13%.
Other Treatments
A number of technologies that address possible
etiologies of scoliosis to control scoliosis in a growth-
sparing method are under investigation. Rib shortening
and lengthening has been performed experimentally and
clinically.94–96 Asymmetric epiphyseodesis of the neuro-
central synchondrosis has shown some promise in ex-
perimental studies as well.78,97
SUMMARY
Recent advances in operative and nonoperative
techniques and understanding regarding the importance
of preserving the thoracic space have improved the mor-
bidity and mortality of children with progressive EOS. It
is important to understand the concepts behind treatment
of EOS and be aware of the range of treatments and their
indications. Current treatment is evolving as existing
techniques are refined and new methods evolve. Derota-
tional body cast treatment should be considered in chil-
dren with documented progression of a curve <60
degrees and no previous surgical treatment. In contrast,
bracing does not appear to be effective in the management
of EOS, and the role of bracing is limited to postcasting
or postsurgical maintenance of correction. When the child
has failed nonoperative treatment, there are a number of
surgical options available to decrease progression, main-
tain SAL, and delay or eliminate a definitive fusion. Both
single and dual growing rods are effective, but dual rods
seem to have better initial correction, maintenance of
correction, increased T1-S1 length gain, and decreased
implant-related problems such as rod breakage and hook
r 2012 Lippincott Williams & Wilkins
Treatment of Early Onset Scoliosis
pullout. Children who cannot tolerate repeated length-
enings because of medical comorbidities should be consid-
ered for the Shilla technique. A hybrid system offers a
promising distraction-based alternative and may be a better
alternative in children who require a low-profile proximal
anchor. VEPTR may be beneficial for those patients with
congenital scoliosis and fused ribs and TIS, although im-
provement in pulmonary function with treatment is con-
troversial.55 Growth modulation using SMA staples or
other tethers show promise for milder curvatures, but fur-
ther follow-up is needed to define their use.
Complication rates for all surgical techniques for
treatment of EOS remain high because of the repetitive
nature of the lengthening surgeries, the bulk of the in-
strumentation, and the stresses placed on instrumentation
in a mobile spine.98 Younger children, neuromuscular and
syndromic children, and higher curve magnitude appear
to be associated with higher complication rates.
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