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Orthopaedic Advances

“Growth Friendly” Spine Surgery:


Management Options for the
Young Child With Scoliosis

Abstract
Jaime A. Gomez, MD The natural history of early onset scoliosis is dismal and
Joseph K. Lee, MD associated with poor pulmonary function and increased mortality.
Although limited in situ fusion may be appropriate for certain types
Paul D. Kim, MD
of congenital scoliosis deformities, spinal deformity that affects
David P. Roye, MD young children often requires a “growth friendly” surgical approach
Michael G. Vitale, MD that allows for curve control while maintaining growth of the spine
and thorax. Growth-friendly surgical management of early onset
scoliosis can follow a distraction-based (ie, growth rods, vertical
expandable prosthetic titanium rib [Synthes, West Chester, PA]),
guided-growth (ie, Luque trolley technique, Shilla technique), or
compression-based (ie, tethers, staples) strategy.

B ecause of both the dismal natu-


ral history of early onset scolio-
sis and the unfavorable effects of
30% of the adult size by 5 years of
age and to only 50% of the adult
size by 10 years of age.1,2 Thoracic
From the Department of Orthopedic early fusion, clinicians who treat volume can be limited by curve pro-
Surgery, Columbia University
Medical Center, New York, NY.
young children with scoliosis use a gression or by early fusion.3,4
variety of surgical techniques in an Pehrsson et al5 noted a statistically
Dr. Roye or an immediate family
attempt to avoid, delay, or limit spi- significant increased risk of mortality
member has received research or
institutional support from Synthes. nal fusion. These “growth friendly” related to respiratory failure in chil-
Dr. Vitale or an immediate family techniques and implants allow curve dren with infantile or juvenile scolio-
member has received royalties from control and limit early spinal fusion. sis compared with children with ado-
Biomet; serves as a paid consultant
to Biomet and Stryker; has received
The growth rate of the spinal col- lescent scoliosis. Vitale et al6 and
research or institutional support from umn varies bimodally with age, with others7 have demonstrated untoward
Synthes; and serves as a board the most rapid growth taking place effects of early fusion on pulmonary
member, owner, officer, or from birth to age 2 years, and fur-
committee member of the Chest
function. Avoidance of this iatro-
Wall and Spinal Deformity Study ther rapid growth occurring again at genic thoracic insufficiency is a guid-
Group, Scoliosis Research Society, adolescence. The volumetric growth ing principle of treatment in the
and Pediatric Orthopaedic Society of of the thorax as a three-dimensional young patient with scoliosis.8
North America. None of the following
structure is of major importance; un-
authors or any immediate family
member has received anything of derstanding has improved regarding
value from or owns stock in a the complex relationships between Early Onset and
commercial company or institution the structure and function of the Congenital Scoliosis
related directly or indirectly to the
spine, thorax, and lungs. Although
subject of this article: Dr. Gomez,
Dr. Lee, and Dr. Kim. the spine has a major effect on lung Early onset scoliosis is a heteroge-
and thoracic cavity development, neous condition. Prognosis and natu-
J Am Acad Orthop Surg 2011;19:
722-727 cross-sectional volume also depends ral history vary widely depending on
on the growth of the ribs, both in whether the etiology is congenital,
Copyright 2011 by the American
Academy of Orthopaedic Surgeons. length and the degree of rib obliq- idiopathic, syndromic, or neuromus-
uity. Thoracic volume increases to cular.9 The natural history of congen-

722 Journal of the American Academy of Orthopaedic Surgeons


Jaime A. Gomez, MD, et al

ital scoliosis is unpredictable, and of a specialized casting table, moder- cations. Traditional growth rods and
patients require close follow-up. ate traction, and emphasis on derota- the VEPTR provide similar options
Those at highest risk of progression tion of the thorax and spine.19 Skin for the management of young chil-
include young patients with a failure complications have been reported, dren with scoliosis.
of segmentation, especially when ac- and attention to meticulous tech- Although Bess et al22 and Akbarnia
companied by a contralateral unseg- nique is essential. et al23 demonstrated increased curve
mented hemivertebra.10 Growth-friendly implants are used correction and overall T1-S1 growth
Resection and/or early limited fusion to control thoracic spinal deformity with frequent lengthening, more re-
may be the best option in cases of iso- and minimize the adverse impact on cent studies have demonstrated an
lated anomalies that affect short spinal growth and development of the spine increased risk of complications with
segments before progression and and thorax. Skaggs et al20 have pro- each procedure as well as less length
the development of compensatory posed a classification of growth- gained with each subsequent length-
curves.11 However, young patients with friendly implants that describes im- ening. Controversy exists regarding
more complex, multisegment deformi- plants as distraction-based (ie, the optimum timing for implantation
ties may benefit from a growth-friendly growth rods, vertical expandable of a growth rod as well as optimum
approach.6,12,13 Although indications prosthetic titanium rib [VEPTR; Syn- lengthening intervals.
for surgical intervention vary,14 most thes, West Chester, PA]), guided-
surgeons prefer to intervene with a growth (ie, Luque trolley, Shilla), Growing Rods
growth-friendly construct before ro- and compression-based techniques
Originally described by Harrington
tation results in a windswept thorax, (ie, tethers, staples).
and modified by Moe, the growing
intrusion into the hemithorax, and In large curves, preoperative halo
rod technique has undergone several
subsequent restriction of pulmonary traction is sometimes used before in-
modifications that allow for control
function.15 strumentation in an effort to de-
of the deformity while minimizing
crease neurologic risk, obtain better
complications. Although no absolute
correction, and improve pulmonary
Management indications exist for the use of grow-
function before surgery.21 Recent
ing rods, generally accepted indica-
publications have described the use
Successful management of early on- tions include significant remaining
of traction in early onset scoliosis for
set scoliosis intends to improve or axial growth, progressive deformity
curves >80° and when associated
prevent progression of spinal defor- >50°, and flexible spinal defor-
with kyphosis before growth-friendly
mity and chest wall constriction, mity.9,24-27 Typical results include
instrumentation.19
avoid or limit early spinal fusion, those reported by Akbarnia et al,9
and minimize surgical complications who reported on 23 children with
and negative effects of treatment on Distraction-based progressive early onset scoliosis who
quality of life. Implants underwent dual growing rod treat-
Casting techniques were commonly ment. The Cobb angle improved
used for the treatment of scoliosis Distraction-based implants correct from 82° preoperatively to 36° at
before the introduction of spinal in- and maintain spinal deformity via time of fusion. T1-S1 length in-
strumentation.16,17 Mehta18 reported spinal distraction, not unlike the creased from an average of 23 cm
extensive experience with serial cast- manner in which the original Har- preoperatively to 32.6 cm at the time
ing in patients with infantile idio- rington rods functioned. These of fusion. Complications occurred in
pathic scoliosis, which resulted in a distraction-based implants can be at- 11 of 23 patients (48%). Innovations
resurgence of interest in this method. tached to the spine, ribs, or pelvis de- such as low profile designs, growing
Curves with a rib vertebral angle dif- pending on patient age, characteris- connectors, dual rod application,
ference >20° were considered to have tics of the curve, and available bone and the use of rib fixation and/or
a high propensity for progression stock. In our experience, proximal pedicle screws have enabled surgeons
and were serially casted every 8 to rib fixation is generally more appro- to control deformity. Allowing con-
16 weeks. In one study, 94 of 136 priate in younger children in whom tinued spinal column growth though
patients (69%) had full curve correc- we are trying to avoid or delay spinal these systems could benefit from fur-
tion, with greater success in children fusion and in whom spinal fixation is ther technological improvements;
treated before age 2 years.16 Princi- limited by an immature spine, in however, this is a challenge given the
ples of Mehta casting include the use which implants may lead to compli- “orphan nature” of this small patient

December 2011, Vol 19, No 12 723


“Growth Friendly” Spine Surgery: Management Options for the Young Child With Scoliosis

Figure 1 which generally need to be placed at


the thoracolumbar junction.
Several studies demonstrate the ef-
ficacy of the VEPTR in controlling
curve magnitude and promoting
spine growth (Figure 2). Campbell
and Hell-Vocke29 reviewed 27 chil-
dren with congenital scoliosis and
fused ribs who underwent expansion
thoracostomy and VEPTR insertion.
At a mean follow-up of 5.7 years,
scoliosis had decreased from a mean
of 74° preoperatively to 49°. Mean
thoracic spine growth per year was
0.80 cm. The presence of an unseg-
mented bar seemingly did not pre-
vent spine growth; expansion thora-
coplasty led to an average of 7.3%
increase in length of the bar at a 4.2-
In an 8-year-old girl with tetralogy of Fallot with progressive thoracic
scoliosis, the curve continued to progress despite the use of year follow-up.29
thoracolumbosacral orthosis bracing. A, Preoperative PA radiograph Complications are similar to those
demonstrating a 65° thoracic curve. B, The patient underwent implantation of of traditional growing rods, includ-
a growing rod distraction system. Immediate postoperative PA radiograph
demonstrating a Cobb angle of 29°. C, PA radiograph made after 3-year ing wound problems, rib fracture,
follow-up demonstrating that curve correction is maintained after multiple and creeping fusion. Iliac S-hook mi-
lengthenings. The white arrow demonstrates axial lengthening along the gration has also been reported, and
growing rod. debate continues regarding the ap-
propriate means of anchoring to the
population (Figure 1). screws. At a follow-up of 40 months, pelvis in growing constructs.30 Tradi-
Pedicle screws should be used with iliac screws achieved statistically sig- tional growing rods are currently
caution in the upper thoracic spine of nificantly better Cobb and pelvic employed in a “physician directed”
the very young child; other types of obliquity correction than did sacral use, whereas the VEPTR has been
proximal instrumentation (ie, hooks, fixation (47% versus 29% and 66% FDA approved for the management
rib cradles) should be considered in- versus 40%, respectively). However, of progressive scoliosis with “tho-
stead. In our experience, when screws there were five broken iliac screws, racic insufficiency” under a humani-
are used in the proximal thoracic spine whereas the other fixation tech- tarian device exemption.31
of the young child, it is advisable to niques had no breakage. Limited data exist regarding the ef-
have at least four screws sharing load. fect of distraction instrumentation
Also, to avoid convergence, the start- on lung function, in part because of
Vertical Expandable
ing point should be positioned on the the difficulty in measuring pulmo-
Prosthetic Titanium Rib
medial edge of the pedicle aiming di- nary function in young children. Mo-
rectly down the pedicle. This technique VEPTR placement was originally in- toyama et al32 measured forced vital
may prevent devastating neurologic dicated for patients with rib fusions, capacity in 10 children with thoracic
complications if proximal control of but currently the VEPTR functions insufficiency syndrome and scoliosis.
the deformity is lost or if screws pull very much like the traditional grow- At a mean follow-up of 33 months,
out posteriorly. ing rod. Compared with the growing forced vital capacity increased signif-
Pelvic fixation for growing rods in- rod, the VEPTR features circumfer- icantly, averaging 26.8% per year.
cludes sacral screws, iliac screws, or ential rib anchors, telescopic length- Using hemoglobin as a surrogate
iliac hook fixation. Sponseller et al28 ening allowing twice more lengthen- marker for pulmonary function,
compared four different types of pel- ing, and a lengthening mechanism Caubet et al33 reviewed hemoglobin
vic fixation in 36 children with pro- that allows expansion in the ky- levels in 138 children with early on-
gressive scoliosis, including iliac photic segment of the thoracic spine, set scoliosis preoperatively and at 6
screws, iliac rods, S-rods, and sacral as opposed to axial connectors, to 24 months postoperatively follow-

724 Journal of the American Academy of Orthopaedic Surgeons


Jaime A. Gomez, MD, et al

Figure 2

Vertical expandable prosthetic titanium rib (VEPTR [Synthes]) instrumentation in a 5-year-old girl with early onset
scoliosis as well as congenital diaphragmatic hernia and rapid curve progression. Radiographs made following surgery
to insert a bilateral rib-to-pelvis VEPTR, with a lengthening every 6 months, demonstrate adequate curve correction
and spine growth. Clinically, the patient’s lung function significantly improved, as well, at 2-year follow-up.
A, Preoperative AP scoliosis radiograph. Lateral (B) and AP (C) scoliosis radiographs made 2 months postoperatively.
D, AP scoliosis radiograph made 6 months postoperatively. E, AP scoliosis radiograph made 2 years postoperatively.

ing VEPTR (n = 85) or growing rod the instrumented region.


(n = 58) insertion. These authors The Shilla technique allows for Compression-based
noted that 23% of children with apical curve control while allowing Implants
early onset scoliosis had evidence of spine growth, without the need for Vertebral body stapling is a new
chronic hypoxia as measured by se- frequent, planned subsequent surger- technique used for adolescent and ju-
rum hemoglobin levels, with signifi- ies.35 Apical pedicle screws are venile idiopathic scoliosis. As pre-
cant improvements noted after spinal placed, effecting a limited fusion, dicted by the Hueter-Volkmann prin-
distraction using the VEPTR. while proximal and distal screws are ciple, increased pressure across a
placed under fluoroscopic guidance growth plate in the vertebral body
Guided-growth Implants with the intention of avoiding fusion. slows growth. Although this phe-
The rods are fixed to the screws at nomenon has been observed in ex-
The Luque trolley was a technique in the apex and can slide inside the perimental animal spine models,36
which rods were attached to the screw head at the proximal and dis- experience in the 1950s using sta-
spine using sublaminar wiring. Now tal end. McCarthy et al35 demon- pling with large congenital curves
largely of historical interest, this strated continued spine growth and was disappointing.37
technique required extensive subperi- no implant failure with the Shilla More recently, flexible tethers at-
osteal dissection, which increased the system in a goat model; however, sig- tached to vertebral anchors have been
risk of inadvertent fusion and, thus, nificant facet arthrosis was also used to modulate spinal growth. Use of
limited spine growth. Mardjetko found. Although these implants are tethers in animal models by Newton et
et al34 retrospectively reviewed nine currently not available for clinical al38 have demonstrated vertebral
children treated with the Luque trol- use, they will likely soon be available wedging, which could potentially
ley system; all required revision sur- under a humanitarian device exemp- correct scoliosis. Using a goat scolio-
gery and had spontaneous fusion of tion. sis model, Braun et al39 showed that

December 2011, Vol 19, No 12 725


“Growth Friendly” Spine Surgery: Management Options for the Young Child With Scoliosis

flexible bone anchor/tethers can mitment to foster these novel im- pear to be viable, notable regulatory
moderately correct deformity in the plants through the regulatory hurdles will delay widespread dis-
coronal plane but that this effect was process. Still, early work has docu- semination of such techniques. Sig-
lost over time. They concluded that mented “proof of concept.” nificant research efforts are under
rigid shape-memory alloy staples way that seek to address many of the
have better final deformity correc- gaps in our understanding of how
tion compared with the flexible liga- Complications
best to optimize management of pa-
ment tethers. tients with early onset scoliosis.
Nonfusion techniques carry with
Modern vertebral body staples
them the risk of numerous complica-
consist of shape-memory alloy (ie,
tions, with minor complication rates
nitinol) that allows the staple to
reported to be from 58% to
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726 Journal of the American Academy of Orthopaedic Surgeons


Jaime A. Gomez, MD, et al

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