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FIGURE 2. Image comparison. A, Standard digital radiograph. B, Standard dose EOS-Imaging (EOS
Imaging). Image resolution is maintained while diminishing radiation exposure.
deferred until the patient is scheduled for surgery. Low dose, age- this population. Although potential effects on brain development
appropriate protocols are utilized. after exposing children to single or multiple episodes of sedation
Imaging of the neural axis is most commonly achieved are unknown, current animal studies suggest an elevated risk
with magnetic resonance imaging (MRI). Subtle findings in of neurodevelopmental disorders in children exposed to general
physical examination, such as absent or asymmetrical abdominal anesthesia before 3 yr of age, but relatively minimal risk if exposed
reflexes, have been proven to be highly suggestive of neural axis to a single general anesthesia event after 3 yr.36-38 To minimize
pathology.28,29 Curves that reach 20◦ before 10 years of age have these risks, we generally wait until after 3 yr of age to obtain these
been shown to have a prevalence of neural axis abnormalities of studies (if possible) and try to coordinate multiple imaging studies
approximately 20% (range 17.6%-26%).14,32 Due to this high under a single anesthetic exposure.
incidence, a screening spine MRI is recommended in patients
with EOS and curves greater than 20◦ , even in the absence IMAGING GUIDANCE
of abnormal neurological findings on physical examination.14
Routine MRI evaluation for the AIS population, however, still The use of pedicle screws for posterior instrumented spinal
remains controversial. This is primarily because of the more fusion is considered the preferred method for deformity
limited availability of MRI (compared with CT and radiographs) correction in AIS. Pedicle screws have been shown to be a
and the relatively low incidence of neural axis anomalies requiring safe and reliable method of instrumentation in pediatric spine
surgical intervention.33 It is generally accepted that MRI is most deformity.39-42 A review of over 19 000 pediatric scoliosis cases
important for AIS patients with atypical curve patterns, such as from the Scoliosis Research Society morbidity and mortality
left thoracic curves, excessive kyphosis, rapid curve progression, database demonstrated a 0.8% rate of neurological deficit for
associated back pain, male gender, neurological deficits, and/or AIS.43 Another systematic review on complications of pedicle
abnormal reflexes.33-35 screw placement in scoliosis surgery revealed that approximately
Substantial debate exists regarding the neurodevelopmental 4.2% of the pedicle screws are malpositioned.44 This percentage
effects of sedation on young children. General anesthesia or increased to 15.7% in studies where postoperative CT scans were
sedation is typically required to obtain CT or MRI studies in obtained. Although free hand technique remains the predominant
FIGURE 3. Sanders Skeletal Maturity System: Stage 1: All of the digital epiphyses are not covered. Stage 2:
All the digital epiphyses are as wide as the metaphysis, “covered”. Stage 3: The majority of the epiphyses bend
over the metaphyseal edge, or are “capped”. Stage 4: Beginning of closure of the distal phalangeal physes.
Stage 5: All of the distal phalangeal physes are closed while the remaining physes are open and “capped”.
Stage 6: Proximal and middle phalangeal physes are closing. Stage 7: All of the physes, except for the distal
radius and ulna, are closed. Stage 8: All of the physes, including the distal radius, are closed. Reproduced with
permission from Sanders JO, Khoury JG, Kishan S, Browne RH et al. Predicting scoliosis progression from
skeletal maturity: a simplified classification during adolescence. J Bone Joint Surg Am 2008; 90(3):540-
53.25
method for pedicle screw insertion, CT image guidance may vention. Factors influencing decision making for each treatment
improve accuracy in placement of thoracic pedicle screws, thereby option include age, skeletal maturity and growth potential,
potentially diminishing vascular or neurological complications. magnitude of curvature and overall balance, underlying diagnosis,
A recent systematic review demonstrated moderate evidence pulmonary status, and concurrent medical comorbidities among
of lower breach rate with CT guidance (7.9%) vs free hand others.
(9.7%-17.1%).45 Conflicting evidence was found regarding the Successful management of scoliosis attempts to correct or
complication rate between CT navigation vs free hand pedicle prevent progression of spinal deformity. This is particularly
screw insertion. Our preferred method continues to be freehand important in young children with EOS, where significant chest
technique but we find CT guidance extremely helpful in cases wall and pulmonary development is taking place. When nonop-
with complex spinal anatomy and in teaching residents safe erative management fails in the AIS population, surgical inter-
techniques for pedicle screw placement, although the potential vention for deformity correction with instrumentation and
adverse effects of radiation exposure with the CT guided definitive fusion is most often recommended. In children with
technique need to be taken into consideration. New technology EOS, however, early fusion across the thoracic spine must
in spine surgery, such as robotic assisted pedicle screw placement, be avoided in order to minimize chest wall constriction and
continues to evolve, but further studies about its efficacy and impairment of cardiopulmonary development during growth.
safety in pediatric spine deformity are needed. When surgery is indicated, surgical implants that permit some
spinal growth are preferred. Growth friendly implants for patients
with EOS were classified by Skaggs et al5 into 3 distinct categories,
TREATMENT OPTIONS based on the correction strategy or force exerted by the implants
on the spine: (1) distraction-based, (2) compression-based, and
Treatment options for AIS and EOS include observation, (3) guided growth systems. The goal of each of these implants is to
physical therapy, orthotic management, and surgical inter- provide adequate correction of the spine deformity without spinal
fusion, thereby permitting lung development as well as chest wall Bracing or cast placement in EOS may allow chest wall and
and spinal growth. spine growth to continue while maintaining or improving spinal
balance. The effectiveness of bracing alone in the treatment of
Nonoperative Treatment EOS is still under debate. In contrast, derotational casting for
Observation is reserved for skeletally mature patients with infantile idiopathic scoliosis has been shown by multiple studies
nonprogressive and balanced spinal curves. In the EOS to be potentially curative, although its effect and utility for
population there are certain pathologies that are less likely to non-idiopathic scoliosis still remains in question.51-55 Mehta55
deteriorate, such as an isolated block vertebra or a hemimetameric described progressive and nonprogressive infantile scoliosis based
shift, among others. Suggested protocol for follow-up in both on the rib-vertebral angle difference (RVAD) and the phase of the
EOS and AIS patients includes obtaining plain radiographs every convex ribs in the apex of the deformity. RVAD is obtained by
6 to 12 month, depending on the phase of growth, and serial measuring the difference in the angles formed by the attachment
assessment of curve magnitude using Cobb angle and other of the concave and convex rib in relation to the apical vertebra.
standard measurements. Rib phase is determined by observing the relationship of the rib
Spinal deformity often remains relatively stable during slow head and vertebral body in the apex of the deformity (Phase
periods of growth, but may show signs of progression during 1: Rib heads do not overlap the vertebral body. Phase 2: There
accelerated growth phases, such as in infancy or puberty. Obser- is an overlap of the convex rib head and the vertebral body).
vation is typically pursued until the coronal deformity exceeds 25◦ Progressive curves presented with an initial RVAD ≥20◦ or a
with more than 10◦ of documented progression.46,47 After this Phase 2 rib-vertebra relation. The majority of the patients with
point, bracing or derotational casting is considered depending on and initial RVAD <20◦ and a Phase 1 rib-vertebra relation (83%)
the patient’s age. had resolution of their spinal deformity.55 In a study of 55 pts
Physical therapy has not been shown to have any influence on with progressive infantile scoliosis, Sanders et al53 demonstrated
spinal deformity in the EOS population.6 However, in children excellent response to derotational casting, with 89% of these
with AIS, scoliosis specific exercises, such as the Schroth method patients showing a decrease in curve magnitude. Initiation of
of physical therapy, are increasingly used alone or in combination derotational casting at a younger age, curve size <60◦ and an
with brace treatment to potentially improve spinal deformity idiopathic etiology carried a better prognosis53 (Figure 4). The
by promoting muscle strengthening and trunk elongation. It is nonidiopathic population in EOS can present with diverse spinal
designed only for flexible curves and limited by the necessity deformities and structural anomalies which can be less amenable
of patient participation. These exercises can be used in adjunct to bracing or casting.
to other nonoperative treatments although compliance in this
age group can limit its effectiveness. Currently more evidence is
needed to recommend the use of this therapy as a sole treatment Halo-Gravity Traction
for mild to moderate AIS.48,49 In cases of severe spinal deformity, both in the adolescent
and early onset population, a period of perioperative halo-
Bracing/Derotational Casting gravity traction may be beneficial in decreasing the magnitude
Bracing in the AIS population is currently recommended of the deformity. This period of traction is also favorable in
in skeletally immature patients with curves from 25◦ to 40◦ . improving respiratory mechanics and nutritional status, as well
Results of brace treatment in the AIS population have been well as making the patient a more suitable candidate for surgery.56-58
documented in the Bracing in Adolescent Idiopathic Scoliosis Length of traction, generally between 2 and 12 weeks, depends
Trial.50 In this prospective multicenter study, skeletally immature on the overall medical status and the presence of radiographic
patients from 10 to 15 yr of age with an initial Cobb angle from evidence of curve improvement. During this period 30% to
20◦ to 40◦ were randomized to brace treatment with a thora- 44% of deformity correction in both sagittal and coronal
columbosacral orthosis (TLSO) vs observation. Due to preference planes can be achieved before growing rod implantation or
of treatment, significant crossover occurred between groups. This definitive fusion57-61 (Figure 5). The most common compli-
crossover altered the randomization of the study and an intention- cations associated with halo-gravity traction include pin site
to-treat as well as an as-treated analysis was conducted. Overall, infection and pin loosening,61 which can be reduced by increasing
the rate of treatment success after bracing (defined as reaching the number of pins during initial application. Patients typically
skeletal maturity without the need for surgical intervention) was complain of neck and trapezial soreness that occurs due to
72%, compared with only 48% after observation. They also traction weight transmission to the neck musculature. This can be
demonstrated a brace-dose response with similar success rates decreased by progressing slowly with the addition of weights. Our
between the observation group (48%) and patients who wore current protocol is to add 2 pounds of traction in the morning
the brace from 0 to 6 h daily (41%), but significantly higher and 1 pound in the afternoon daily until the traction goal is
success rates in those who wore the TLSO for an average of at least reached. Traction goal fluctuates between 30% and 50% total
12.9 h per day (90%-93%).50 The trial had to be stopped before body weight as tolerated by the patient, with daily neurochecks
completion due to the efficacy demonstrated by TLSO treatment. as each additional weight is added.
FIGURE 4. A, PA radiograph of a 19-mo-old male patient with idiopathic early onset scoliosis treated with serial derotational casting for 9 mo and
subsequent bracing. Coronal Cobb angle of 37◦ , RVAD >20◦ , and phase 2 ribs. B, Three months after initial cast placement. C, Nine months after initial
cast placement. D, At 3 yr after initiating derotational casting the coronal curve has remained stable.
FIGURE 5. A, PA radiograph of a 4-yr-old female patient with Syndromic EOS. B, Four weeks after
halo-gravity traction demonstrating a 38% improvement on her coronal Cobb angle.
then, this technique has undergone several modifications but the or allograft is used to augment fusion at both cranial and
underlying principle has remained the same. caudal foundation sites. Rods are contoured for the sagittal plane
Klemme et al74 published their experience on patients treated and placed submuscularly without periosteum violation over the
with posterior instrumentation without fusion and sequential noninstrumented segments. This can be performed by carefully
distraction. At the time of definite fusion, the curves had tunneling the rods underneath the fascia or through direct visual-
improved an average of 20◦ (67◦ -47◦ ) and the measured growth ization. In line or side to side tandem connectors are placed
of the instrumented but unfused segments averaged 3.1 cm over a around the straight thoracolumbar junction and cranial and
mean treatment period of 3 years. Akbarnia et al75 described the caudal rods are attached using these connectors. Periodical length-
dual rod technique which is the preferred method today. A single ening is typically performed every 6 to 12 months depending on
or dual midline skin incision is performed. Meticulous dissection the age, although it has been demonstrated that patients with
is performed with subperiosteal dissection limited to the proximal dual growing rod constructs who are lengthened frequently (≤ 6
and distal anchoring points. Fixation points are obtained at each months) may have greater curve correction and overall T1-S1
end with pedicle screws or hooks. Biomechanical studies have growth.80
demonstrated the superior stability and fewer implant related The incidence of neuromonitoring changes has been reported
complications of all screw constructs when compared to screw- to be 0.9% during primary growing rod placement or exchange
hook or hook-hook fixation in dual growing rods.76-78 Selection and 0.5% during lengthening, therefore, routine use of intraop-
of foundation levels should take into consideration the presence of erative neuromonitoring during primary surgery or exchange is
pelvic obliquity as well as the magnitude and location of the curve. currently recommended.81 The complication rate for the growing
Obtaining a solid foundation at the caudal end of the construct is rod techniques is high.75,80-82 Bess et al82 reported the Growing
of utmost importance. Distally, four pedicle screws are generally Spine Study Group data on 140 patients who underwent 897
placed. Depending on the patient’s age, size, and anatomic varia- growing rods procedures. In their study, 58% of the patients had
tions, hooks vs pedicle screws are chosen for proximal fixation. at least one complication. They demonstrated that complication
In our experience, adequate proximal fixation should be obtained rates could be reduced by delaying initial implantation (13%
over three levels with at least five fixation points.79 Bone autograft decrease for every additional year in patient age at the beginning
FIGURE 7. A, PA radiograph of a 7-yr-old patient with early onset idiopathic scoliosis. B, Two years postop
PA radiograph after 6 lengthenings with magnetically controlled growing rods.
of treatment), utilizing dual rods, submuscular rod implantation, achieved by radiographs or ultrasound, which has recently been
and reducing the number of surgical lengthenings. described as an accurate tool reducing the amount of radiation
exposure in the EOS population.83,84 A multicenter study by
Magnetically Controlled Growing Rods Akbarnia et al85 comparing MCGR (n = 12) to TGR (n = 12)
Magnetically controlled growing rods allow lengthening proce- demonstrated similar curve correction (32% in MCGR vs 31% in
dures without the need of anesthesia or surgical intervention TGR; p = .95), incidence of unplanned revisions, T1-S1 growth
(Figure 7). The surgical technique and indications for MCGR (8.1 mm/yr vs 9.7 mm/yr respectively; p = .73) and T1-T12
and TGR placement are similar. The MCGR device consists of an growth (1.5 mm/yr vs 2.3 mm/yr respectively; p = .83). The
implantable rod containing a magnetic actuator that couples with MCGR cohort had a total of 16 surgeries and 137 noninvasive
external magnets on a remote controller converting rotational lengthenings whereas the TGR cohort had 73 total surgeries
motion to longitudinal growth of the rod. Lengthening can including 56 surgical lengthenings.
be done in the office setting with no sedation, reducing the Reported complication rate for growing spinal implants in the
amount of surgical interventions and anesthesia exposure, while treatment of patients with early onset scoliosis ranges from 29% to
maintaining the benefits of deformity correction and growth more than 70%, the most common being implant related compli-
of the spine. Confirmation of the desired lengthening can be cations.75,81,82,87–89 Even though the use of MCGR may reduce
the amount of surgical interventions when compared to TGR, its Thoracic curves <35◦ had a 77% success rate while those ≥35◦
use does not appear to prevent implant related complications such only a 25%. Lumbar curves demonstrated a success rate of
as proximal junctional kyphosis, anchor failure, rod breakage, and 86.7%. Thoracic curves who reached ≤20◦ of coronal Cobb angle
failure of device lengthening, among others. In a recent multi- measurement on the first standing x-ray had an 85.7% success
center retrospective review, Choi et al90 reported on 54 patients rate. Suggested criteria for vertebral body stapling (VBS) include
with early onset scoliosis who were treated with MCGR. Twenty- age (<13 yr for girls and <15 yr for boys), Risser grade 0 to 1, at
three patients (42%) had at least one complication. A total of least 1 yr of remaining growth by wrist bone age or Sanders grade
11% of the patients had at least 1 broken rod while 13% experi- ≤4; flexible thoracic curves 25 to 35◦ (lumbar coronal curves
enced either proximal or distal implant related complications. In <45◦ ); minimal rotation along the apex of the deformity and
six patients (11%), the rod did not lengthen at least in 1 episode, a thoracic kyphosis <40◦ .85 Bumpass et al93 recently published
but four of these patients lengthened at subsequent office visits their results on 31 patients with curves from 25◦ to 40◦ (Risser
with repeated magnetically controlled noninvasive distraction. 0-2) who were treated with VBS. Success in curve control was
As in many other aspects of medicine, there is typically defined as per Betz et al.92 At a minimum of 2 yr of follow-up,
an initial higher cost associated with new technology and the overall success of curve control in their group was 61%. When
implants. MCGR is not an exception. However, given the groups were subdivided based on the preoperative coronal curve
reduction in the number of surgical interventions and anesthetic magnitude of <35◦ vs ≥35◦ , those with a preoperative Cobb
episodes associated with MCGR, it is likely that the long term angle <35◦ had a 75% control rate vs only a 22% in curves
economic outcome with this new device will be to reduce ≥35◦ . Thoracic curves ≥35◦ at initial treatment, progressed to
costs while maintaining safety and clinical results. A recent cost more than 50◦ in 83% of the cases.
analysis comparison between MCGR and TGR using a projected
economic model demonstrated cost neutrality between implants
by eliminating TGR surgical lengthenings. This model estimated Vertebral Body Tethering
the costs for implantation, revisions, lengthenings, equipment With a similar mechanism, tethers consist of bony anchors
exchanges, and final fusion for the 2 groups over a projected 6 yr attached to flexible cords which provide compressive forces
period of care. Over this period, the use of MCGR was associated around the convexity of a curve, potentially correcting the
with a decrease of approximately 11.2 procedures per patient, 270 scoliosis. A theoretical advantage of tethering over VBS is
fewer deep surgical site infections, and 197 less revisions due to that tethering provides less restriction of motion in direc-
device failure in over 1000 simulated patients when compared to tions other than lateral bending away from the device, whereas
TGR.86 staples restrict motion in multiple planes, hence potentially
causing a less harmful effect on the overall health of interver-
Compression-Based Techniques tebral discs. Vertebral body tethering (VBT) has been studied
Based on the Hueter–Volkmann principle, the vertical growth in animal models and has shown radiographic evidence of
along the concavity of a scoliotic deformity is known to be vertebral deformity correction while maintaining flexibility of
slower than along the convexity, due to compressive forces the spine.94,95 Samdani et al96 published the results of 11
inhibiting growth.91 These techniques, which are commonly used patients with AIS and showed a 70% correction on coronal
in the treatment of limb malalignment in children, attempt to Cobb angle of the thoracic curve with no major complica-
modulate growth by applying compressive forces through the tions at 2 yr follow-up. They suggest the use of VBT in skele-
convex vertebral physis. Vertebral stapling or tethers are placed tally immature patients (Risser 0-1, Sanders ≤4) with thoracic
across the convex growth plates and concave growth proceeds with curves from 35◦ to 60◦ who demonstrate flexibility to less than
an ultimate goal of curve correction and balance. These proce- 30◦ , whereas thoracic kyphosis >40◦ and a rotational promi-
dures are generally performed through a thoracoscopic approach nence >20◦ were considered absolute contraindications for this
or mini open retroperitoneal approach if below the diaphragm. procedure. Crawford and Lenke97 demonstrated an impressive
scoliosis correction in a patient with juvenile idiopathic scoliosis
Vertebral Body Stapling treated with this technique.
Vertebral staples are made of Nitinol, a nickel-titanium alloy As previously discussed, bracing is helpful in preventing
that changes its configuration when in contact with body temper- progression of scoliosis, but in contrast to these compression-
atures. Nitinol staples are approved by the US Food & Drug based techniques, it does not provide correction of existing
Administration for upper and lower extremities use, but their deformity. Compliance has also been shown to be a signif-
use in the pediatric spine deformity is still off-label. Betz and icant problem among patients braced for idiopathic scoliosis.98
colleagues92 reported their experience with anterior vertebral Although brace treatment is still the first line treatment in
staples in a retrospective review of 28 patients with idiopathic moderate idiopathic scoliosis, compression based techniques
scoliosis treated with anterior vertebral body staples and followed offer a potential alternative for patients who refuse or are not
for 2 yr. They defined success as curves that corrected to within compliant with brace wear. The determination of appropriate
10◦ or decreased by >10◦ from the preoperative measurement. indications for these procedures as well as optimal implants
FIGURE 8. A, PA and lateral radiographs of a 6-yr-old female patient with idiopathic early onset scoliosis treated with a Shilla technique. B, Two months postop. C,
Three years postop demonstrating guided growth through the Shilla construct with conservation of the initial correction.
continues to evolve. Certainly, a thorough discussion with the layer with minimal disruption to the adjacent soft tissue and
families and strict selection criteria should be followed before periosteum as to minimize the possibility of ankylosis at these
suggesting compression based techniques in the early onset or AIS segments. The goal is to correct the apical deformity, rendering
population. these segments back to neutral in all planes, while permitting and
guiding growth through the remaining instrumented but unfused
Growth Guided Techniques spine segments (Figure 8). McCarthy et al102 published their
5 year follow-up on this technique. At 5 years the curves were
Growth guided techniques aim to preserve growth centers reduced from an average of 69◦ to 38.4◦ and the space available
throughout the spine while correcting the apex of the deformity. for the lung, determined by the Campbell technique,103 was
The Luque trolley consists of rods fixed segmentally to the increased by an average of 27.7% on the right and 30.5% on the
spine by sublaminar wires in the absence of a fusion. As left hemi-thorax. Complication rate was 73%, the majority being
growth continues, the rods glide through the sublaminar wires return to the OR due to implant-related problems. Concerns
maintaining spinal correction. This technique is not used about the development of metal ions in the blood and tissues
abundantly due to high rates of associated complications, sponta- surrounding these implants have recently emerged.104 Despite
neous fusion, and inadequate maintenance of spinal growth.99,100 these concerns, the enormous advantage of both of these growth
Ouellet et al101 published a small series of 5 patients treated with a guidance techniques is that they allow for the surgical treatment
modern Luque trolley technique in which the cranial and caudal of EOS without the need of repetitive surgical lengthening.
ends of the construct were instrumented and fused, while only
sliding instrumentation was placed throughout the rest of the
spine using muscle sparing techniques. They showed an average CONCLUSION
correction of the coronal Cobb angle for the primary curve from
60◦ to 21◦ and a mean spine growth of 3 cm over 4 yr which The underlying principles in the treatment of both adolescent
represented 77% of the predicted growth. and early onset idiopathic scoliosis have remained stable over
With the Shilla technique, a short apical fusion is performed decades, but technological advances in imaging as well as new
with additional proximal and distal screws that permit the rods to instrumentation and surgical techniques have provided new
slide, thereby directing the growth along the rod trajectory. The methods for the management of these conditions. As new
cranial and caudal Shilla screws are placed through the muscle technology continues to develop, our understanding on the
pathophysiology and treatment of early onset and adolescent 21. Cheh G, Lenke L, Lehman R, Jr, Kim YJ, Nunley R, Bridwell K. The reliability
scoliosis will continue to evolve. of preoperative supine radiographs to predict the amount of curve flexibility in
adolescent idiopathic scoliosis. Spine. 2007;32(24):2668-2672
22. Deschenes S, Charron G, Beaudoin G, et al. Diagnostic imaging of spinal defor-
Disclosures mities. Spine. 2010;35(9):989-994
Dr Vitale reports research grants from Pediatric Orthopaedic Society of 23. Ilharreborde B, Ferrero E, Alison M, Mazda K. EOS microdose protocol
North America, Children’s Spine Foundation; royalties from Biomet, consultant for the radiological follow-up of adolescent idiopathic scoliosis. Eur Spine J
fees from Stryker, Biomet, and Medtronic. He also holds positions in the 2016;25(2):526-531
24. Hui S, Pialasse JP, Wong JY, et al. Radiation dose of digital radiography (DR)
following organizations: Wellinks, Inc. (Chief Medical Officer), Children’s
versus micro-dose x-ray (EOS) on patients with adolescent idiopathic scoliosis:
Spine Foundation (VP, BoD), POSNA (board member), International Pediatric 2016 SOSORT- IRSSD “John Sevastic Award” winner in imaging research.
Orthopaedic Symposium (board member). He also receives travel support from Scoliosis Spinal Disord. 2016;11(1):46.
Biomet. The other authors have no personal, financial, or institutional interest in 25. Sanders JO, Khoury JG, Kishan S, et al. Predicting scoliosis progression from
any of the drugs, materials, or devices described in this article. skeletal maturity: a simplified classification during adolescence. J Bone Joint Surg
Am. 2008;90(3):540-553
26. Lenke LG, Betz RB, Harms J, et al. Adolescent idiopathic scoliosis. J Bone Joint
Surg Am. 2001;83(8):1169-1181
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T he authors have nicely summarized the management of early onset
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