Early Onset Idiopathic

Scoliosis

Bruce L. Gillingham, MD, Abstract

CAPT, MC, USN
Ryan A. Fan, MD, LT, MC,
USNR
Behrooz A. Akbarnia, MD
Dr. Gillingham is Director, Surgical
Services, Naval Medical Center, San
Diego, CA, and Assistant Professor of
Surgery, Uniformed Services University
of the Health Sciences, Bethesda, MD.
Dr. Fan is Resident, Orthopaedic
Surgery, Naval Medical Center, San
Diego. Dr. Akbarnia is Clinical Professor
of Orthopaedic Surgery, University of
California, San Diego, and Medical
Children with early onset scoliosis typically present before age 5
years. Radiographic criteria help to distinguish progressive cases
from those that will spontaneously resolve. Severe
cardiopulmonary problems may occur in untreated progressive
cases. A comprehensive evaluation should be performed to identify
commonly associated conditions, such as plagiocephaly, congenital
heart disease, inguinal hernia, and hip dysplasia. For curves >20°,
magnetic resonance imaging of the neural axis is indicated to rule
out occult central nervous system lesions. Surgical management
should be considered when nonsurgical measures, including
bracing and casting, fail to arrest progression. Surgical methods
continue to evolve and are primarily directed at obtaining and
maintaining curve correction while simultaneously preserving
spinal and trunk growth.

Director, San Diego Center for Spinal
Disorders, La Jolla, CA.
None of the following authors or the
departments with which they are
affiliated has received anything of value
from or owns stock in a commercial
company or institution related directly or
indirectly to the subject of this article:
Dr. Gillingham and Dr. Fan. Dr. Akbarnia
or the departments with which he is
affiliated has received royalties from
DePuy Spine. Dr. Akbarnia or the
departments with which he is affiliated
serves as a consultant to or is an
employee of DePuy Spine.
Reprint requests: Dr. Akbarnia, San
Diego Center for Spinal Disorders, Suite
300, 4130 La Jolla Village Drive, La
Jolla, CA 92037-1481.
J Am Acad Orthop Surg 2006;14:101-
112
Copyright 2006 by the American
Academy of Orthopaedic Surgeons.
T
reatment of children with pro-
gressive scoliosis occurring be-
fore age 5 years is challenging. Left
untreated, progressive curves may
produce significant thoracic defor-
mity, leading to deleterious effects
on the cardiopulmonary system. As
James et al1 observed in 1959, pro-
gressive early onset scoliosis “devel-
ops rapidly and relentlessly, causing
the severest form of orthopaedic
cripple with dreadful deformity,
marked dwarfing and shortening of
life.” Standard treatment options for
adolescent scoliosis, including brac-
ing and spinal fusion, have limited
use in much younger children be-
cause of potential adverse effects on
the developing spine, chest wall, and
lungs. Successful management of
idiopathic scoliosis requires under-
standing the etiology, natural his-
tory, evaluation, and available non-
surgical and surgical management
options for these patients.
In 1954, James2 described three
categories of idiopathic scoliosis ac-
cording to the age of onset: infantile,
with curves present before age 3
years; juvenile, with curves appear-
ing between the ages of 4 and 9
years; and adolescent, in which
curves present from age 10 years un-
til the end of growth. These three pe-
riods were thought to correspond to
distinct periods of increased growth
velocity during childhood and ado-
lescence. In fact, during the juvenile
period, there is a deceleration in spi-
nal growth, and onset of scoliosis is
uncommon.3,4 More recently, the
term “early onset” has been used to
reflect the presence of scoliosis of all
etiologies by age 5 years; “late on-
set” is used to describe the appear-
ance of scoliosis after age 5 years.5
These terms more accurately reflect
the physiologic basis of and clinical
experience with this condition. The
distinction between early and late

Volume 14, Number 2, February 2006 101

Early Onset Idiopathic Scoliosis
Figure 1
2.5
2 2
2
1.5
1.25
cm/year
1.25
1
0.75 0.75
0.5
0 .25
Age 1 Age 5
T1-L5
Spinal growth velocity from ages 1 through 15 years. (Adapted with permission
from Dimeglio A: Growth of the spine before age 5 years. J Pediatr Orthop B
1993;1:102-107.)
onset is important because of the po-
tential for serious cardiopulmonary
compromise in patients in whom
scoliosis appears before age 5 years.
Basic Science
Anatomy, Growth, and
Development
Knowledge of the normal growth
of the chest, spine, and pulmonary
system is essential in understanding
the effect of scoliosis on these struc-
tures. The number of alveoli in the
terminal respiratory unit rapidly in-
creases in number and volume dur-
ing the first year of life. The number
of alveoli increases more than 10-
fold between birth and adulthood,
primarily during the first 8 years of
life.6 In addition, the number of re-
spiratory branches increases from 21
at age 3 months to 23 at age 8
years.7
The significant increase in
growth of the lung parenchyma is
paralleled by corresponding growth
of the spine and chest. Two thirds of
the final sitting height is achieved by
age 5 years.3 The growth velocity of
the T1 to L5 segment is greatest
from birth to age 5 years, averaging
>2 cm per year, with marked decel-
eration between ages 5 and 10 years.
Growth velocity increases again at
102
1.75
1.25
1 sis he termed “infantile idiopathic
0.75 0.5
Age 10 Age 15
T1-T12 L1-L5
age 10 years and after but does not
equal the velocity occurring before
age 5 years3 (Figure 1). Overall, the
height of the thoracic spine doubles
between birth and skeletal maturity.
Thoracic volume is approximately
6% of adult volume at birth, reach-
ing roughly 30% of adult volume by
age 5 years and 50% by age 10 years.
Final thoracic volume is achieved by
age 15 years in both boys and girls.3
Etiology
Several causes have been identi-
fied for early onset scoliosis, includ-
ing congenital vertebral anomalies,
neuromuscular conditions (eg, cere-
bral palsy, myelomeningocele, mus-
cular dystrophies), associated syn-
dromes (eg, neurofibromatosis), and
structural lesions of the central ner-
vous system (eg, diastematomyelia,
syrinx, tethered cord). Patients with
no obvious associated abnormalities
are considered to have early onset id-
iopathic scoliosis.
Natural History
In contrast with late-onset dis-
ease, early onset idiopathic scoliosis,
specifically infantile idiopathic
scoliosis, spontaneously resolves in
a large number of patients. Although
early series reported a low incidence
of resolution, later reports demon-
Journal of the American Academy of Orthopaedic Surgeons
strate a much more favorable
outcome.8-11 Magnetic resonance im-
aging (MRI) evaluation of the neural
axis was unavailable at the time of
these reports, however. In 1951,
James8 identified a pattern of scolio-
scoliosis,” involving primarily in-
fant boys with a left mid or lower
thoracic curve. Only 4 of 33 patients
(12%) resolved. In contrast, Lloyd-
Roberts and Pilcher10 reported on
100 patients with structural curves
diagnosed within the first year of
life. Ninety-two percent of the
curves resolved spontaneously. In
their study of 99 infants, Ceballos et
al11 reported progression in 26%.
The original hypothesis that in-
fantile cases were caused by intra-
uterine molding was refuted by the
absence of scoliosis at birth. Scott
and Morgan9 compared the inci-
dence of idiopathic scoliosis in En-
gland and the United States, as re-
ported by the Research Committee
of the American Orthopaedic Asso-
ciation in 1941. Twenty-eight of 218
patients from England presented
with idiopathic scoliosis before age 2
years, compared with only 1 of 404
patients in the United States. Mau12
subsequently proposed that postna-
tal pressure caused by constant ob-
lique supine positioning in European
infants (compared with prone posi-
tioning in North American babies)
was responsible for this difference in
incidence. He also noted associated
ipsilateral plagiocephaly (an asym-
metric and twisted head in reference
to the spine), pelvic flattening and
obliquity, and hip adduction. This
proposed correlation between infant
positioning and early onset scoliotic
deformities is disquieting and war-
rants further research in light of the
current trend in American pediatrics
to recommend positioning infants
supine to reduce the risk of sudden
infant death syndrome.13
Several factors, such as age of on-
set; location, type and magnitude of
the scoliotic curve; associated devel-
opmental anomalies; sex; and fami-
 Bruce L. Gillingham, MD, CAPT, MC, USN, et al

Figure 2 develop when the vital capacity re-

mains >40% of the predicted normal
value.
Clinically, there is a far greater
risk of cardiorespiratory complica-
tion when scoliosis is apparent be-
fore age 5 years. Even in the absence
of associated disease, disabling or
life-threatening respiratory failure is
relatively common and is likely to
present at or before late middle
age.17,19

Physical Examination
To measure the rib-vertebra angle difference, a line is drawn perpendicular to the
end plate of the apical vertebrae (a). Next, a line is drawn from the midpoint of the The evaluation of a child with sus-
neck of the rib through the midpoint of the head of the rib to the perpendicular on pected spinal deformity should begin
the convex side (b). The resultant angle is calculated. The angle on the concave side with a comprehensive history and
is calculated in a similar manner. Concave − convex = rib-vertebra angle difference. physical examination. Given that
(Adapted with permission from Mehta MH: The rib-vertebra angle in the early
the presence of cognitive delay has
diagnosis between resolving and progressive infantile scoliosis. J Bone Joint Surg
been shown to correlate with curve
Br 1972;54:230-243.)
progression, particular attention
should be paid to whether the child
ly history, have been proposed as This effect is directly related to the has appropriately reached develop-
predictors of curve progression. The age of onset. The most hypoplastic mental milestones.20,21 It is also im-
most reliable indicator, however, is lungs are found in patients with the portant to obtain a birth history; ear-
the rib-vertebra angle difference earliest onset of scoliosis.17 ly onset idiopathic scoliosis has been
(RVAD), reported by Mehta14 in 1972 Patients with scoliosis demon- noted to occur more frequently with
(Figure 2). strate a restrictive pattern of lung breech presentation and in prema-
Progressive scoliosis during the disease, with reduction in vital ca- ture, low-birth-weight males.21
first 5 years of life may negatively af- pacity and total lung capacity along A thorough physical examination
fect the normal development of the with increased residual volume. should be performed, beginning with
lungs, chest wall, and spine. The pri- This reflects decreased compliance a search for cutaneous markers of
mary effect of scoliosis on the devel- of both the lung and chest wall. In systemic disorders, such as the café
oping lung is its inhibition of the children with early onset curves, the au lait spots and axillary freckling
growth of both alveoli and pulmo- amount by which vital capacity is observed in neurofibromatosis and
nary arterioles. This incomplete reduced depends on the severity of the hairy patch associated with oc-
maturation of the lung and pulmo- the deformity. This severity has lit- cult spinal dysraphism. Evidence of
nary vasculature is the primary tle or no effect on vital capacity in chest wall and shoulder height
cause of the ventilation defect seen patients whose curves begin in ado- asymmetry, trunk imbalance, and
in patients with early onset scolio- lescence.15 Ultimately, severe re- pelvic obliquity are sought. Flexibil-
sis.15 Although progressive distor- strictive lung disease causes alveolar ity of the scoliosis should be as-
tion of the pulmonary architecture hypoventilation, hypoxic vasocon- sessed: the patient is laid horizontal-
by increasing deformity of the spine striction, and, eventually, pulmo- ly over the examiner’s knee with the
and thorax would be thought to nary arterial hypertension and cor convex side downward, or by hold-
compress the developing alveoli, pulmonale. Arterial hypoxemia in ing the infant suspended under the
this has not been observed. Histolog- scoliosis patients is primarily caused arms, looking for correction of cur-
ic studies of lung tissue in patients by a decrease in minute ventilation vature with lateral pressure. Curves
with early onset scoliosis demon- secondary to small tidal volumes that correct are considered to be flex-
strate alveoli that are normal in rather than an impairment in gas ex- ible; those that do not are termed
shape and outline but diminished in change.18 The smallest tidal vol- rigid. The relative rigidity of the
number. In addition, the number of umes and greatest decreases in curve is an approximate clinical in-
alveoli for a given lung volume was minute ventilation are seen in pa- dicator of the likelihood of scoliosis
diminished more than could be ac- tients with the highest Cobb angles. progression and should be correlated
counted for by limitation of space.16 Cardiorespiratory failure does not with the RVAD and Cobb angle.22

Volume 14, Number 2, February 2006 103

Early Onset Idiopathic Scoliosis
Figure 3
Convex
Concave
A B
A, Phase 1 rib-vertebrae relationship demonstrating no overlap of the rib head and vertebral body. B, Phase 2 rib-vertebrae
relationship. The overlap of the rib head on the vertebral body is indicative of curve progression. (Adapted with permission from
Mehta MH: The rib-vertebra angle in the early diagnosis between resolving and progressive infantile scoliosis. J Bone Joint
Surg Br 1972;54:230-243.)
Lower extremity limb-length equal-
ity is verified to rule out nonstruc-
tural scoliosis, particularly in the
ambulatory child with a predomi-
nant lumbar curve.
Following this focused evaluation
of the scoliotic curve and its second-
ary manifestations, the patient is ex-
amined for conditions associated
with early onset idiopathic scoliosis.
Plagiocephaly, which is extremely
common, is found in most patients
with early onset idiopathic scoliosis.
Developmental dysplasia of the hip,
inguinal hernia, and congenital heart
disease are found at a higher frequen-
cy in patients with early onset idio-
pathic scoliosis than in children
without scoliosis.20,21,23
Finally, a careful neurologic ex-
amination, including assessment of
muscle tone and reflexes, is imper-
ative to detect occult central ner-
vous system abnormalities. Superfi-
cial abdominal reflex also should be
examined for abnormalities because
it may be the only clue to an under-
lying syringomyelia with an asso-
ciated Chiari I malformation. The
superficial abdominal reflex is char-
acteristically absent on the same
side as the curve convexity.24
Consideration should be given to
obtaining a pediatric pulmonology
evaluation, at least at the beginning
and again at the end of treatment. In
the presence of significant chest wall
104
Convex
and rib asymmetry and respiratory
insufficiencies, more frequent pul-
monary function tests may be re-
quired. Pulmonary function tests are
difficult to obtain before age 5 years.
Other techniques to assess lung
function and volume, such as three-
dimensional computed tomography,
provide valuable information in de-
termining the severity of the pa-
tient’s condition and the timing and
effect of surgical intervention.25
Radiographic
Evaluation
Anteroposterior and lateral radio-
graphs with full-length spinal views
should be obtained to evaluate both
the Cobb angle and the RVAD.14
These radiographs are also helpful in
ruling out other associated vertebral,
lumbosacral, and hip joint abnor-
malities. The RVAD (Figure 2) is use-
ful in predicting curve progression
when there is no overlap of the rib
heads on the apical vertebra (phase 1
relationship)14 (Figure 3, A). An
RVAD >20° indicates a high likeli-
hood of curve progression; curves
with RVAD measuring <20° are
more likely to resolve. A phase 2 re-
lationship, in which a rib head over-
laps the apical vertebra, implies that
progression is certain; thus, the
RVAD is not measured14 (Figure 3,
B). In the Mehta study,14 46 of the 86
Journal of the American Academy of Orthopaedic Surgeons
Concave
phase 1 curves resolved. Of those
that resolved, 83% had an RVAD
<20°, with a mean RVAD of 11.7°.
The progressive group demonstrated
a mean RVAD of 25.5°; 84% had an
RVAD >20°.
In a series of 132 patients, Ferrei-
ra et al26 confirmed these findings,
reporting that 67 of 68 resolving
curves had an angle difference <20°;
all were in a phase 1 relationship,
and only two had a Cobb angle >30°.
Of the progressive curves in that
study, 37 of 40 had an RVAD ≥20°,
with two of the remaining three dis-
playing an increased RVAD to >21°
by the first year.
The RVAD is also useful in de-
tecting the lumbar component of
early double curves. Detecting dou-
ble curves is important because they
are highly likely to progress.11 Meh-
ta14 noted key radiographic features
of the early double curve: the near
symmetric alignment of the apical
ribs with a resultant low RVAD of
the thoracic curve, downward obliq-
uity of the twelfth rib on the con-
cave side of the curve, and vertebral
rotation in opposite directions in the
thoracic and lumbar vertebrae.
Recent attention has focused on
defining the role of special imaging
for patients with early onset scolio-
sis. In contrast to patients with late-
onset scoliosis, the incidence of
occult central nervous system ab-
 Bruce L. Gillingham, MD, CAPT, MC, USN, et al

Figure 4 mended performing total spine MRI

in patients with early onset curves
Early onset measuring ≥20°.28
scoliosis (< age 5)

Absent abdominal Comprehensive history and Significant Management

reflexes or physical examination and nonorthopaedic
Cobb angle >20° scoliosis radiographs findings Nonsurgical
No The treatment of children with
No
Yes
Yes early onset scoliosis is based on an-
ticipated or actual curve progression
Continue with nonsurgical
(Figure 4). Mehta’s prognostic crite-
orthopaedic management; Specialty referral for
MRI of spinal cord, ria have proved to be very helpful in
No progression to Cobb angle nonorthopaedic
positive finding
>25°, RVAD >20°, conditions differentiating between resolving
or positive phase 2 rib
Yes relationship and progressive curves. Curves with
Cobb angles <25° with an RVAD
Yes <20° are at low risk for progression.
Neurosurgery Casting/bracing
specialty evaluation
These patients may be observed and
No should be reevaluated with serial ra-
Good response diographs every 4 to 6 months. Ac-
tive treatment should be initiated
No with curve progression >10°. Upon
Serial observation
Yes
curve resolution, follow-up at 1- to
every 4-6 months
Consider surgical 2-year intervals until maturity is
intervention prudent to ensure that there is no re-
Progression of currence during the adolescent
curve Growing rod ±
Yes
anterior release
growth spurt. A recent long-term
Other study of resolving curves validated
No emerging
techniques the use of the RVAD and demon-
Serial lengthenings strated that there was no advantage
every 4-6 months of supine plaster bed treatment over
Annual clinical
examination until physiotherapy in regard to either
skeletal maturity
Possible removal of time to resolution or functional out-
instrumentation and come.29
continued observation Definitive fusion
Infants with an RVAD >20° or a
phase 2 rib-vertebral relationship
and a Cobb angle between 20° and
Treatment algorithm for early onset (<5 years) idiopathic scoliosis. MRI = magnetic 35° have a high likelihood of progres-
resonance imaging, RVAD = rib-vertebra angle difference
sion. When a child presents with a
curve >35°, immediate treatment
normalities is high in otherwise an associated cervicothoracic syrinx should be seriously considered.
healthy-appearing patients with requiring surgical decompression. These patients should be followed
early onset scoliosis. In a prospec- The MRI of the third patient dis- closely at 4- to 6-month intervals,
tive study of 34 patients younger closed diffuse dural ectasia. with active treatment initiated in
than age 10 years who presented A more recent, larger study retro- the presence of >5° of Cobb angle
with curves measuring >20° and a spectively reviewed 46 patients with progression.
normal neurologic examination, a Cobb angle ≥20°, normal neuro- Traditional nonsurgical manage-
Gupta et al27 reported a 17.6% inci- logic examination, no associated ment for early onset scoliosis in-
dence of neural axis abnormalities. syndromes, and no congenital abnor- cludes casting, bracing, or a combi-
Occult abnormalities were discov- mality. The mean age at presenta- nation of both. Cast application is
ered in three of the six patients tion was 17 months (range, 2 to 37 usually done under anesthesia. The
younger than age 4 years. Two of months). Neural axis abnormality cast is changed at 6- to 12-week in-
these patients demonstrated caudal was demonstrated on MRI in 10 pa- tervals until maximum correction is
displacement of the cerebellar ton- tients (21.7%), 8 of whom required achieved. Following this, the cast
sils below the foramen magnum neurosurgical intervention. Based on may be replaced by a Milwaukee
(Chiari type I malformation), with these findings, the authors recom- brace with full-time implementa-

Volume 14, Number 2, February 2006 105

Early Onset Idiopathic Scoliosis
tion (23 h/day). The Milwaukee
brace is preferred over a thoracolum-
bar orthosis because of the rib cage
distortion and pulmonary function
reduction reported with the circum-
ferentially fitting thoracolumbar
brace. In addition, the immature rib
cage often deforms before significant
correction is transmitted to the
spine. Bracing is generally continued
for a minimum of 2 years until there
is no further evidence of progression,
as indicated by an unchanging Cobb
angle and RVAD.
Mehta and Morel22 studied 21 pa-
tients with infantile idiopathic scoli-
osis who were treated nonsurgically.
They reported that with total correc-
tion before the prepubertal growth
spurt, there is no relapse during ad-
olescence. Without full correction,
however, small relapses may occur.
These patients may require surgical
intervention if further progression
occurs during the adolescent growth
spurt. They should be followed until
skeletal maturity.
Surgical
Fusion
Several surgical procedures have
been used to manage progressive
curves in skeletally immature pa-
tients. Early procedures focused on
slowing or halting curve progression
with spinal fusion, with the ratio-
nale that a short, straight spine was
preferable to a long, crooked one.
Continued anterior growth follow-
ing posterior fusion alone, known as
the crankshaft phenomenon,30 ne-
cessitates circumferential fusion in
preadolescents. Although effective
in halting curve progression, this ap-
proach prevents future spinal growth
and has deleterious effects on the de-
veloping thorax and lungs.
The effectiveness of hemiepi-
physiodesis in correcting lower ex-
tremity axial malalignment led to
the application of this technique to
the growing spine. In 1963, Roaf31
proposed that the spinal deformity
was produced by growth asymmetry,
with overgrowth on the convex side
106
of the spine and inhibition of growth
on the concave side of the curve. He
recommended and performed con-
vex spinal epiphysiodesis to address
this imbalance. Twenty-three per-
cent of his patients showed signifi-
cant improvement. However, 40%
were stationary or showed very little
improvement (<10°).
In a recent study reviewing long-
term results, Marks et al32 found
that convex spinal epiphysiodesis
with or without Harrington instru-
mentation did not significantly re-
verse the established deformity.
Thirteen patients with infantile id-
iopathic scoliosis were treated with
anterior and posterior convex epi-
physiodesis alone (four received Har-
rington instrumentation 2 to 4 years
later); a further nine patients were
treated with convex epiphysiodesis
and concurrent Harrington instru-
mentation. Radiographic progres-
sion, mirrored by a deteriorating
clinical result, occurred in all but
one patient. The best results were
noted with epiphysiodesis and si-
multaneous Harrington instrumen-
tation placement, which controlled
but did not improve the degree of the
primary curve.
Single Rod Instrumentation
Instrumentation without arthro-
desis has been performed in an at-
tempt to preserve spinal growth, ob-
tain initial scoliosis correction, and
control ongoing deformity. Har-
rington33 first reported this tech-
nique in 27 idiopathic and post-polio
patients in 1962. Using a subperi-
osteal approach, Harrington placed
on the curve concavity a single dis-
traction rod connected to hooks at
the upper and lower end vertebrae.
These patients were a subset of the
129 patients he initially treated with
his implant. No longitudinal results
were reported. Complications in-
cluded hook dislocation and rod
breakage. Based on this early experi-
ence, Harrington believed that chil-
dren younger than age 10 years with
progressive scoliosis could be man-
Journal of the American Academy of Orthopaedic Surgeons
aged with instrumentation alone,
whereas children age 10 years and
older should be fused initially. Re-
gardless of patient age, treatment
should not be considered definitive.33
Moe et al34 subsequently modi-
fied Harrington’s technique by limit-
ing subperiosteal exposure to the
area of hook placement. The hook
sites were not fused. The rod itself
was placed subcutaneously. Moe et
al34 also modified the Harrington rod
to have a smooth, thicker central
portion to prevent scar adhesion to
threads and to allow sagittal con-
touring. Patients were placed in a
Milwaukee brace postoperatively;
the construct was lengthened when
>10° loss of correction occurred.
There was an average increase of
2.9 cm in the length of the instru-
mented portion for all 20 patients
and of 3.8 cm (compared with a pre-
dicted growth of 4.5 cm) in the 9 pa-
tients who went on to fusion. Nota-
ble decrease in curve magnitude was
reported in the two patients with
progressive early onset curves. Com-
plications occurred in 50% of pa-
tients. Rod breakage, although less
common with the modified thicker
rod, still occurred. The authors also
reported hook dislodgement from
the rod and dislocation from the
lamina.
Klemme et al35 in 1997 reported
the results of a 20-year experience
with the Moe technique. An average
of 6.1 procedures were performed
from initial instrumentation to de-
finitive fusion in 67 patients. In 44 of
the 67 patients, curve progression
was arrested or improved over the
course of treatment, with an aver-
age curve reduction of 30%. In the
remaining 23 patients, including
12 with neuromuscular scoliosis,
curves progressed an average of 33%.
The overall growth rate of instru-
mented but unfused spinal segments
was 0.08 cm per segment per year.
Implant-related problems, including
hook dislocation and rod breakage,
were reported in 33 of 402 proce-
dures (8%).

Marchetti and Faldini36 in 1978 re-
ported on what they termed the “end
fusion technique.” Fourteen patients
underwent staged procedures in
which the vertebrae at each end of the
curve were initially fused. Five to 6
months later, hook placement was
performed along with subperiosteal
rod placement. Finally, at a third pro-
cedure 6 to 8 weeks later, the upper
hook was distracted. Serial lengthen-
ing was performed until definitive fu-
sion at maturity. Four of the 14 pa-
tients had completed treatment at the
time of publication of their report,
with “most satisfactory” results.
In 1977, Luque and Cardoso37 re-
ported on their technique for segmen-
tal spinal instrumentation (SSI) with-
out arthrodesis. In 1982, Luque38
reported the results of adding sublam-
inar wiring to a Harrington rod in 47
paralytic patients. The immobilized
area grew an average of 4.6 cm, with
curve correction of 78%. Smooth,
L-shaped rods were subsequently sub-
stituted for the Harrington rod in a
construct that became known as the
Luque trolley. Initial enthusiasm
based on these perceived advantages
was tempered by reports that the sub-
periosteal exposure and sublaminar
wire passage created scar tissue and
weakened the lamina, which made
revision and later definitive fusion
difficult. In addition, premature spon-
taneous fusion was noted in several
patients. In subsequent reports by
other authors, growth preservation
was demonstrated to be substantially
less than expected.39,40
Patterson et al41 combined SSI
with anterior apical convex growth
arrest and fusion in 9 of 13 patients
who had previously undergone sur-
gery at an average age of 5 years 5
months. At 2-year follow-up, curve
correction averaged 46%. Patients
with anterior apical growth arrest
combined with SSI without fusion
had less curve deterioration than did
those who had SSI alone. No sponta-
neous fusions were reported.
Pratt et al42 performed a retrospec-
tive analysis of Luque trolley instru-
Volume 14, Number 2, February 2006
mentation with and without convex
epiphysiodesis in 26 patients. Curve
deterioration was observed in all eight
patients treated with the Luque trol-
ley alone. In curves managed with
combined convex epiphysiodesis and
Luque instrumentation, the Cobb an-
gle worsened in 7 of 13 patients, was
unchanged in 4, and improved in 2.
Growth of the instrumented spinal
segment was 49% of the curve pre-
dicted in patients treated with the
Luque trolley alone, and 32% of the
curve predicted in patients undergo-
ing the combined procedure.
More recently, Blakemore et al43
reported periodic lengthening with a
submuscular rod with and without
limited apical fusion in 29 children
with scoliosis. Ten of the curves
were idiopathic. The single rod was
placed within the muscle above the
spinal periosteum. This approach
placed the rod closer to the spine for
better contour and alignment with-
out causing spontaneous posterior
fusion. Apical fusion was performed
in curves >70° and in those that were
stiff on side bending radiographs. All
patients were placed in a Milwaukee
brace postoperatively. Curves im-
proved from a mean preoperative
Cobb angle of 66° to a mean of 38°
on initial postoperative radiographs.
Slight deterioration to a mean of 47°
was observed on the most recent ra-
diographs. Spinal growth had not
been calculated for the entire group
at the time of publication. Compli-
cations occurred in 24%, including
five hook displacements, three rod
breakages, and one superficial infec-
tion. The authors concluded that,
despite the frequent but manageable
complications, their technique is
useful for managing severe spinal de-
formities in young children who
have failed, or who have a contrain-
dication to, orthotic management.
Emerging Techniques
Dual Growing Rod
Instrumentation
Dissatisfied with the unpredict-
ability and implant-related compli-
Bruce L. Gillingham, MD, CAPT, MC, USN, et al
cations of single rod distraction
techniques, Akbarnia and Marks44
developed a dual growing rod tech-
nique, building on concepts formu-
lated by Asher.45 We currently prefer
this technique. Subperiosteal dissec-
tion is limited to upper and lower
anchor sites (foundations) (Figure 5).
Hooks or screws are placed on both
sides of the spine in so-called claw
patterns over two to three spinal lev-
els to avoid hook crowding. Pedicle
screws seem to add stability to the
construct.46 A transverse rod con-
nector is positioned adjacent to or in
the middle of the claw constructs at
both foundations. Foundation sites
may be fused with local bone graft
supplemented with synthetic graft.
Upper and lower contoured 3/16-
inch-diameter rods are placed subcu-
taneously on both sides of the spine.
The rods are joined on each side with
extended tandem connectors placed
at the thoracolumbar junction to
avoid disturbing sagittal balance.44
Lengthening is performed with a
distractor designed to fit within the
longitudinal openings in the tandem
connector. The intent of initial
lengthening during implant inser-
tion is to achieve modest correction
of the scoliotic curve without plac-
ing undue stress on the foundations.
More aggressive correction is at-
tempted at the first lengthening after
fusion of the foundation sites. Serial
lengthening occurs at approximately
6-month intervals. Somatosensory
evoked potential monitoring is per-
formed during each lengthening,
which is generally done as an outpa-
tient surgery. Bracing is performed
until the foundation sites are fused.
Encouraging results were ob-
tained from a multicenter study at
minimum 2-year follow-up (range,
24 to 111 months) of 23 patients, in-
cluding 7 with early onset idiopath-
ic curves.47 The average age at initial
surgery was 5 years 5 months; pa-
tients had an average of 6.6 length-
enings. The mean Cobb angle im-
proved from 82° to 38° following the
initial surgery; it was 36° at the lat-
107
Early Onset Idiopathic Scoliosis

Figure 5 in 28 patients followed through de-

finitive surgery. Five patients had
single rod with anterior and posteri-
or apical fusion, 16 had single rod
Upper
without apical fusion, and 7 had dual
foundation rod without fusion. The mean Cobb
angle respectively improved from
85° to 65°, 61° to 39°, and 92° to 26°.
Spinal growth respectively was 0.3,
1.0, and 1.7 cm per year. The authors
concluded that growing rod tech-
niques using single or dual rods are
effective in establishing and main-
Extended taining curve correction and allow-
tandem ing spinal growth. On the basis of its
connectors
greater strength and more frequent
Extended
tandem
lengthening, however, dual rod in-
connectors strumentation produced better ini-
tial correction and maintenance of
correction and allowed more growth
than did single rod instrumentation.
Lower Although the numbers were small,
foundation the least favorable outcomes were in
patients who underwent short apical
fusion. This technique appeared to
lead to stiffening of the curve, crank-
A B
shaft phenomenon, less correction,
and a higher incidence of complica-
Dual growing rod instrumentation. A, Anteroposterior view. The upper and lower tions. The authors indicated that
rods are joined at the thoracolumbar junction by extended tandem connectors. apical fusion may not be helpful.49
B, Lateral view. The rods are contoured to maintain sagittal alignment. Extended
tandem connectors are placed at the thoracolumbar junction to minimize adverse Other Emerging
effects on thoracic kyphosis and lumbar lordosis. (Reproduced with permission Techniques
from Bagheri R, Akbarnia BA: Pediatric ISOLA [DePuy Spine] instrumentation, in Although still evolving, current
Kim DH, Vaccaro AR, Fessler RG [eds]: Spinal Instrumentation: Surgical surgical techniques using instru-
Techniques. New York, NY: Thieme, 2005, pp 636-643.) mentation with minimal or no ar-
throdesis in the treatment of early
est follow-up. Growth of the T1-S1 displacements, two rod breakages, onset idiopathic scoliosis are capable
segment averaged 1.21 cm per year. two deep wound infections, four of significant initial curve correction
The seven patients who completed superficial wound problems, one and prevention of subsequent curve
treatment at an average age of 10 crankshaft, and one junctional ky- decompensation. This allows defin-
years 3 months achieved a total T1- phosis requiring an extension of in- itive fusion to be delayed until ado-
S1 length increase of 11.8 cm from strumentation. lescence. In addition, it is possible to
preoperative status to postoperative These results indicate that the preserve nearly normal growth
final fusion (1.66 cm growth per dual rod technique is safe and effec- within the area of instrumentation.
year). In 14 patients with thoracic tive and provides increased implant It is hoped that growing rod instru-
curves, the space available for lung stability. Although the complication mentation may be removed at matu-
ratio, as described by Campbell et rate is high, this procedure has few- rity in some patients, avoiding fu-
al,48 improved from 0.87 preopera- er complications compared with sin- sion completely and preserving
tively to normal (1.00) at latest gle rod systems (Figures 6 and 7). A spinal motion. The search continues,
follow-up or after final fusion.47 recent study by Thompson et al49 of- however, for methods that will
Complications occurred in 11 of 23 fers additional support for the use of achieve curve correction and prevent
patients between initial surgery and the dual growing rod technique. The subsequent curve decompensation in
final fusion. The complications in- authors compared the results of sin- a less invasive fashion and minimize
cluded three anchor (hook or screw) gle and dual growing rod techniques the need for repeat surgical proce-

108 Journal of the American Academy of Orthopaedic Surgeons

 Bruce L. Gillingham, MD, CAPT, MC, USN, et al

Figure 6

Radiographs and photographs of a girl aged 5 years 10 months old with infantile idiopathic scoliosis who was followed for 7
years and eventually had final fusion. Preoperative anteroposterior (A) and lateral (B) radiographs of the spine. Anteroposterior
(C) and lateral (D) radiographs taken after the initial surgery. Anteroposterior (E) and lateral (F) radiographs taken 5 years after
initial surgery. G and H, Clinical appearance at 5-year follow-up. Note the correction of scoliotic curve and the linear scar (G).
Patient kyphosis fell within normal limits (~50°) at the time of radiographs and clinical photographs.

Volume 14, Number 2, February 2006 109

Early Onset Idiopathic Scoliosis
Figure 7
Graphic representation of the improvement in length of implant, T1-S1 length, and
Cobb angle magnitude in the patient shown in Figure 6. 5 + 10 = 5 years 10
months; 13 + 6 = 13 years 6 months.
dures. The ideal implant would re-
quire minimally invasive insertion;
would be durable, rarely requiring re-
vision or replacement; would have a
minimal effect on adjacent tissue;
and, if required, would be easily in-
corporated into the definitive fusion.
One intriguing possibility is the
development of an implant that can
be lengthened by remote control. In
1998, Takaso et al50 reported on the
development of a rod containing a
direct-current motor attached to a
radio-controlled receiver. Successful
serial correction of experimental
scoliosis was achieved in beagles.
The main problems encountered
were the relatively large size of the
rod (16 mm) and the receiver, which
necessitated placement in the ab-
dominal cavity.
Recently, interest has returned to
attempts at modulating the growth of
the scoliotic spine with anterior
asymmetric growth arrest performed
with staples placed endoscopically.
Historically, this technique had some
110
failures, in part because of loss of sta-
ple fixation.51 Improved staple designs
promise better fixation, however.
Betz et al51 recently reviewed 21
patients with adolescent idiopathic
scoliosis who were treated with ver-
tebral body stapling. No patients
with either infantile or juvenile scoli-
osis were included. Six of 10 patients
with curves between 28° and 40° re-
mained stable or improved at 1-year
follow-up. Four patients progressed.
Further investigation is needed to de-
termine the efficacy of this technique
in early onset scoliosis.
The technique of expansion tho-
racoplasty for managing thoracic in-
sufficiency syndrome with the verti-
cally expandable prosthetic titanium
rib has recently been described.48,52
This device was designed to manage
thoracic deformities resulting from
absent and fused ribs in congenital
and syndromic conditions, such as
Jeune’s asphyxiating thoracic dystro-
phy and Jarcho-Levin syndrome.
This effort has resulted in a broader
Journal of the American Academy of Orthopaedic Surgeons
and deeper understanding of the cen-
tral role that the spine plays in the
architecture and function of the
chest wall and thorax. This new
awareness emphasizes the impor-
tance of evaluating thoracic volume
in addition to the standard assess-
ment of anteroposterior and sagittal
spinal alignment. Restoring this
“fourth dimension,” thereby maxi-
mizing the potential for pulmonary
development, is emerging as an im-
portant goal in the treatment of pa-
tients with congenital spine and rib
anomalies or with thoracic insuffi-
ciency syndrome.48
Summary
Treating progressive early onset idio-
pathic scoliosis is challenging. Un-
treated curves may cause significant
disturbance of normal trunk and
spine growth, with severely deleteri-
ous effects on the cardiopulmonary
system. Surgical treatment is indi-
cated in patients whose curves
progress despite nonsurgical treat-
ment. Spinal fusion at an early age
also may lead to a shorter trunk, a
smaller thorax, and resultant pulmo-
nary complications. Other tech-
niques, such as hemiepiphysiodesis,
single growing rod methods, and SSI,
have yielded satisfactory results in
this patient population. New, more
effective techniques, such as the dual
growing rod technique, anterior non-
fusion techniques, and the vertically
expandable prosthetic titanium rib,
have emerged with the goal of pre-
serving spinal growth and maintain-
ing scoliosis correction. Even though
they often require the patient to un-
dergo multiple operations through-
out childhood, these techniques
promise to be less invasive in the fu-
ture and more valuable in treating pa-
tients with early onset scoliosis.
Acknowledgment
The authors wish to acknowledge
Sarah Canale, BS, for her assistance
with the editorial process.

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