Spine I: Staying Out of Trouble with Scoliosis in Children

Ovid: Staying Out of Trouble in Pediatric Orthopaedics

Authors: Skaggs, David L.; Flynn, John M.
Title: Staying Out of Trouble in Pediatric Orthopaedics, 1st Edition
> Table of Contents > Section 4 – Spine and Hip > 19 – Spine I: Staying Out of Trouble with Scoliosis in Children

Spine I: Staying Out of Trouble with Scoliosis in Children
David L. Skaggs
John M. Flynn
John B. Emans
Lawrence G. Lenke
Robert M. Campbell Jr.
Guest Guru



Complications in children are serious matters;
complications in spine surgery in particular can be catastrophic for
the patient and family.
Idiopathic scoliosis is a diagnosis of exclusion; the
doctor’s primary job at the initial consultation is to find out if
there is an underlying cause of the scoliosis. There are many clues on
history, physical examination, and radiographs that scoliosis may not
be idiopathic (see Red Flags box). The authors have seen a child with each one of the listed clues in which the scoliosis was not idiopathic.
Asking why the child came to see you often brings out
important information— that a family member may have scoliosis, or the
child is complaining of back pain, etc. Often the referring doctor’s
diagnosis of scoliosis is not what is bothering the patient. If a
family member is said to have scoliosis, a quick Adams forward bending
test of the family member provides useful information. It gives the
surgeon more confidence that the patient’s scoliosis is more likely to
be idiopathic, though Dr. Emans points out the pitfall of incorrectly
assuming the child has AIS. We still must look for other causes of
scoliosis in the child. Looking at the parent is useful for explaining
the consequences of mild disease and usually makes the family member
feel better when you rule out significant scoliosis. Epidemiologic
studies tell us that back pain in early adolescents begins to approach
that seen in adults, so this should not raise a red flag unless it is
abnormal pain (positive finger test, night pain, pain which is not
activity related, etc.; see Chapter 20 on back
pain). Of adolescents with known idiopathic scoliosis, 23% present with
back pain, and 9% complain of back pain during the course of

FIGURE 19-1 (A)
Twenty-two degrees of scoliosis in a 12-year-old girl with constant
back pain and popliteal angles of 90 degrees, suggesting tight
hamstrings and nerve root irritation. (B) MRI reveals increased signal, which proved to be a osteoblastoma at biopsy.
FIGURE 19-2 (A)
AP radiograph of this 12-year-old girl may raise a suspicion of her
condition being slightly atypical for idiopathic scoliosis as the apex
is a bit low. (B) The lateral view of the
girl shows 58 degrees of kyphosis. While this much kyphosis in an
otherwise normal child is not alarming, in the setting of scoliosis
this raises a red flag, suggesting the scoliosis may not be idiopathic.
An MRI is indicated in that circumstance. MRI revealed a Chiari
malformation and a large syrinx. (C) A
lateral radiograph of a patient with adolescent idiopathic scoliosis.
In contrast to the “normal” sagittal profile, a complete lack of
kyphosis, or even slight thoracic lordosis, is not uncommon in
idiopathic scoliosis, and is generally not considered a red flag for an
underlying disorder.
FIGURE 19-3 (A,B) Tight hamstrings. The popliteal angle may be considered positive if the angle is >50-60 degrees.

In addition to the multiple congenital vertebral anomalies, note that
the pedicles are wider at L1 than L5. Widened pedicles suggest
diastematomyelia, which was confirmed with MRI and CT. A bony spicule
is not always appreciated on radiographs as the diastematomyelia may be
predominantly cartilaginous, especially in young children. Prior to any
surgery to correct spinal deformity, removal of the diastematomyelia
should be considered to prevent tethering of a split cord or nerve
roots over the diastematomyelia, which may lead to neurologic injury.
This 16-month-old child with congenital scoliosis and fused ribs is
being considered for a vertical expandable rib prosthesis.
FIGURE 19-5 (A)
Radiograph of a 14-year-old girl. The 26-degree left thoracic scoliosis
is an atypical curve pattern for idiopathic scoliosis. The lack of
significant vertebral rotation on this radiograph also suggests that
this curve may not be idiopathic. On physical examination, a lack of
rotatory asymmetry and an asymmetric umbilicus reflex were present as
well, both suggesting that the curve may not be idiopathic. (B) MRI demonstrated a significant syrinx of the cervical and thoracic spine.
Age 10 year old boy with 49 degree scoliosis. Note there is no rotation
of the vertebrae, and an atypical right thoracolumbar apex. MRI
revealed a significant syrinx the entire length of the thoracic spine.

FIGURE 19-7 (A)
Scoliosis may be compensatory for a leg length discrepancy. This
patient was referred for consultation for her scoliosis with the
radiograph shown in Fig. 19-5A. (B)
By placing one’s fingers on the top of each iliac crest a limb length
discrepancy was suggested, and was radiographically confirmed at 1.2 cm
by scanogram.
FIGURE 19-8 This curve has sharp angulation, which provides a clue to the patient’s underlying diagnosis of neurofibromatosis.
Remember to look beyond the spine on each radiograph. The need for this
is aptly demonstrated here: the child’s right hip was noted to be at
risk (arrow). This is easy to overlook during a scoliosis evaluation, but may be made to appear obvious to a jury years later.

Significant scoliosis may not be appreciated when the
patient is standing. This is particularly true in double major curves,
in which curves over 50 degrees may add up to a rather straight torso (Fig. 19-10).
The rotational asymmetry of the spine, however, will be easily
appreciated in the Adam’s forward bending position. Stay out of trouble
by never considering an examination of the spine to have been done
unless rotational asymmetry has been evaluated. If on Adam’s forward
bending test the prominence is on the “wrong” side (curve concavity)
the curve is usually not structural, but compensatory for a leg length
discrepancy. A quick way to test for pelvic obliquity, which is most
often caused by a leg length discrepancy, is by placing the examiner’s
fingers on the iliac crests (see Fig. 19-7B).
Pelvic asymmetry may result from causes other than a leg length
discrepancy such as a dislocated hip, hip abduction or adduction
contractures, or previous Salter osteotomy. For significant pelvic
obliquity in the standing patient, a scanogram may provide more
information. And, of course, the importance of a neurologic examination
in evaluation of the patient with scoliosis cannot be overemphasized
(see Chapter 2, 60 Second Neuro Exam).
FIGURE 19-10 Shortly after this photograph (A),
this young lady was runner-up Miss California. Note that her back does
not appear grossly deformed when viewed from behind despite a 47-degree
primary right thoracic scoliosis (B). In the Adams forward bending test her scoliosis is more easily appreciated (C).
Standing radiographs show a greater magnitude of
scoliosis than supine radiographs. Do not mistake this for progression.
You can verify the patient is standing by the air bubble in the
stomach, which is flat on the bottom (see Figs. 19-2, 19-5A, and 19-8).
When lateral radiographs are taken, if patients hold their arms
straight out in front of them (parallel to the floor) as appears to be
the standard


for radiology technicians, this results in a sagittal vertical axis
that is at least 3 to 4 cm more posterior than normal, and likely
changes the kyphosis and lordosis as well.2
By having the patients place their hands on their shoulders, with their
elbows pointing forwards, the arms will not be superimposed on the
spine, and the effect on sagittal contour will be minimalized. Bending
films can produce highly variable results depending on how they are
done (Fig. 19-11). Bending radiographs taken
supine or over a bolster will most likely demonstrate much less
curvature than standing radiographs. Explain to parents at the first
visit that there is up to 7 degrees of measurement error in
determination of the Cobb angle, so that parental focus on minor
changes of Cobb angle, and many minutes of discussion, could be avoided
in the future. In children ≤10 years of age with scoliosis in the range
of 20 degrees or greater, an MRI of the cervical, thoracic, and lumbar
spine is indicated to rule out intraspinal pathology.3,4

FIGURE 19-11 (A)
The degree of effort a patient puts into bending during bending
radiographs can make a significant difference in straightening of the
curve (arrow). These two radiographs were taken minutes apart. The radiology technician gave routine instructions for a bending film. (B)
The surgeon encouraged to bend as far as possible, and the film was
repeated. Note that when there is maximal bending effort the lower ribs
almost touch the iliac crest (arrow).
Growth Remaining
The amount of growth that remains in patients is
important in predicting curve progression and length of bracing, but is
also highly variable. Hoppenfeld, et al.5
has recently shown that capping of the iliac apophysis is not the final
indicator for the end of spinal growth. After capping of the iliac
apophysis (Risser 4) there was continued growth in overall height of
75% of patients, with a mean of 1.8 cm in girls and 2.5 cm in boys. No
growth occurred in patients after iliac apophysis fusion (Risser 5) or
closure of either the rib epiphysis or proximal humerus growth plates.
Keep your patients’ families out of trouble by warning
them that there is a lot of misinformation in places such as the
Internet. Some of these “secret” oral formulas have led to metabolic
imbalances requiring emergent hospitalization for the child. More
commonly, though, the only negative consequences of unproven therapies
are a monetary loss for the families (tens of thousands of dollars for
some) and delay of standard treatment.

This subject is a minefield of troubles. First off, be sensitive—this is a time of insecurity about one’s body for adolescents without
a disease. One way to stay out of trouble is to make certain you offer
bracing to patients, and document that you offered it. There have been
lawsuits taking the position that if bracing had been used, surgery
could have been prevented. When a patient’s curve warrants observation
or bracing, it is important to gain the family’s confidence that you
are “doing all that can be done,” but the curve may progress anyway.
Make certain the family understands that efficacy of a brace is quite
controversial, and usage in no way guarantees cessation or improvement
of curve. A reasonable approach is to say that, at best, the curve will
not progress, and that lasting improvement is highly unlikely. A
discussion of rebound effect6 (that
the curve will worsen after brace treatment is discontinued) best
occurs early in the course of brace treatment to prevent later feelings
of failure.
In general, bracing candidates have between 20 and 45
degrees of scoliosis, with growth remaining, and usually documented
progression of at least 5 degrees, though the last requirement may be
overlooked for immature patients with open triradiate cartilage or if a
strong family history is present. Take a radiograph in the brace to
evaluate correction, as a poorly-made brace might not be doing anything
of value.
It is important that physicians who prescribe braces
understand enough about bracing to make suggestions. In patients with
thoracic hypokyphosis or lordosis, bracing may worsen the sagittal
contour. Pads should be placed laterally, to eliminate any anterior
force on the spine. Lateral radiographs of such patients should be
taken in the brace to make certain the thoracic spine is not pushed
more anteriorly in the brace.
One of the most important ways a scoliosis surgeon can
stay out of trouble is a family conference in which the surgeon sets
expectations, completes an explicit and exhaustive consent process, and
shows models for optimum understanding. Complete correction of
deformity should never be guaranteed, and it is important to clarify
that the child’s back will never be completely normal. While it is our
experience, and that of others7 that
scoliosis surgery often relieves back pain, this outcome is
unpredictable and should never be an expectation. Families should
understand that a second operation is quite possible, and we suspect is
more likely than most surgeons admit to themselves.
Surgical indications for AIS are controversial, but are generally considered to include patients with:
  • Thoracic curve ≥50 degrees, or slightly smaller curves in patients with significant growth remaining.
  • Lumbar or thoracolumbar curves ≥45 to 50 degrees.
There are exceptions for significant body deformity.
Choice of Surgery
Surgical approach, method of instrumentation and
correction, and selection of levels to be fused are shrouded in
controversy and mysticism. We will attempt to share a few basic
principles and pitfalls.
  • Remember that improved curve correction does not correlate to improved outcome.7 Do not let surgeon ego drive you to a surgical technique you are not


    comfortable with; the most likely modest improvement in curve correction is unlikely to benefit the patient long term.

  • Any surgery that disrupts the chest wall,
    including anterior surgery in the chest and posterior thoracoplasties,
    is detrimental to short-term pulmonary function, and may have long-term
    implications as well.
  • If there is a history of pulmonary
    problems, pulmonary consultation and pulmonary function tests should be
    considered for curves greater than 90 degrees or with significant thoracic lordosis. Pulmonary compromise in the postop period, which is most severe on postop day 3, can be predicted by preoperative PFTs.8
  • Early reports of success with new
    techniques are often written by surgeons with a financial and/or
    profession stake in the success of the technique or
    instrumentation—waiting for confirmation by other groups may be
Staying Out of Trouble during Surgery
The points below are subjects of much debate, but some recommendations to stay out of trouble are warranted.
  • For anterior instrumentation of the
    thoracic spine, expect continued posterior growth to contribute to
    about 15 degrees of increased kyphosis over time in 60% of patients who
    are Risser 0 and 27% of patients Risser 1-5.9
  • For anterior instrumentation, single rods smaller than ¼-inch have a high incidence of breakage and should be avoided.
  • For posterior instrumentation there is
    little place for a single rod, which has been shown to have an
    unacceptably high failure rate.10,11
  • For selective anterior fusion of
    thoracolumbar/lumbar curves in adolescents, the associated thoracic
    curve can probably be left unfused if:
    • The thoracic curve is less than 55 degrees, and bends out to 20 degrees or less
    • The thoracolumbar Cobb angle is at least 25% greater than that of the thoracic curve
  • For anterior instrumentation in the
    lumbar or thoracolumbar spine, place screws as posterior as possible to
    prevent kyphosis during compression. Also consider use of a structural
    graft or cage, which will maintain lordosis and decrease stress on
  • In patients with open triradiate
    cartilages, surgery performed before or during the peak height velocity
    is a strong predictor of the crankshaft phenomenon.12
  • Posterior sublaminar wires are safe and
    strong in the treatment of idiopathic scoliosis. They are useful as
    primary instrumentation or as a bailout if other fixation was not
  • Consider including an upper left thoracic
    curve in a posterior spinal fusion if T1 is tilted high on the left on
    the preoperative standing film, the left shoulder is high
    preoperatively, the curve is greater than 35 degrees, bends out to less
    than 25 degrees or has greater than 20 degrees of kyphosis from T2-T5.
  • The use of pedicle screws instead of
    hooks in the lowest level(s) may help improve correction and/or save a
    level, with a smaller likelihood of pullout.14
  • When preparing a pedicle for screw
    insertion, if it doesn’t feel just right, don’t use a pedicle screw.
    Other anchors have been used effectively for years, and a misplaced
    pedicle screw can cause lots of harm (Figs. 19-12, 19-13 and 19-14).
  • Use only a pedicle hook finder with a
    “stop” to prevent inadvertent entrance into the spinal canal. Paralysis
    and lawsuits have been associated with pedicle hook finders without
    stops (Fig. 19-15).
  • P.268
  • In verifying vertebral level
    intraoperatively, an AP image with the transverse process marked avoids
    the pitfall of thinking you are on the more caudal vertebrae, as the
    lower lamina shingles caudally over the next vertebra (Fig. 19-16).
  • While conclusive, long-term data is
    lacking, it is generally accepted that avoiding fusing down to L5, and
    if possible L4, is beneficial long term in minimizing back pain.
  • Similarly, maintenance of lumbar lordosis within and below instrumentation is likely to be beneficial long term.
FIGURE 19-12
Although penetration of pedicle screws are usually benign, in this case
the child had nerve-root pain and paresthesias. Removal of screw was
accompanied by immediate improvement of nerve root SSEPs and complete
relief of symptoms. In the lumbar spine, palpating the pedicle from
inside the canal with a Penfield #4 may help identify such a protrusion
if you are uncertain when palpating from within the pedicle.
FIGURE 19-13
Following surgery for idiopathic scoliosis this child had discomfort
deep in the chest. The screw was removed and a bypass graft of the
aorta was placed across the damaged part of the aorta.

FIGURE 19-14
The smallest pedicle screw in the set was 25 mm, which was too long for
this 3-year-old, which resulted in impingement on the right mainstem
bronchus. Either the screw could have been cut shorter with a bolt
cutter or an alternative fixation could have been used.
FIGURE 19-15 Pedicle hook finders with stop (A) prevents excessive entrance into the spinal canal. Pedicle hook finder without stop (B).
FIGURE 19-16 (A)
In this case, the surgeon planned instrumentation to L3, and the
intraoperative radiograph was misinterpreted as ending at L3. (B)
This model demonstrates that when the lumbar spine with lordosis is
viewed in an AP direction, the spinous process and the inferior lamina
of the superior vertebra may appear superimposed on the inferior
vertebrae. (C) Intraoperative marking of the transverse process rather than the spinous process or lamina may help avoid this error.

If generalized bleeding becomes worse during surgery,
make certain the patient’s abdomen is hanging free, as a shifted
bolster may increase venous pressure. When significant forces arise
during correction of large or stiff curves, waiting a few minutes
before the next maneuver takes advantage of the viscoelasticity of the
spine and surrounding structures, permitting stress on the implants to
decrease over time. Prior to beginning closure, ask yourself if you
have forgotten to do anything, and double-check final tightening of
implants (Fig. 19-17).
FIGURE 19-17 A systematic final tightening of all implants prior to closure may have prevented this implant failure.
While there is a legitimate concern that thoracoplasty
has a negative pulmonary effect, the cosmetic and body-image
improvement can be substantial. This procedure may be explained to the
family as a way to get bone graft, as it has to come from somewhere,
with subsequent cosmetic improvement. It is important to set
expectations for this procedure that the rib prominence will improve,
but the back will never be “normal.”
FIGURE 19-18
This 16-year-old girl had a chest tube placed as an infant, with the
resulting breast deformity. (Reprinted with permission from Rainer C,
Gardetto A, Fruhwirth et al. Breast deformity in adolescence as a
result of pneumothorax drainage during neonatal intensive care. Pediatrics. 2003;111(1):80-86.
The ribs are easiest to approach in the plane between
paraspinal muscles and latissimus in lumbar spine. Try to cut the ribs
as medially as possible, adjacent to the transverse process, or the
remaining rib will be prominent. Following rib resections, the
operative site may be filled with warm saline, and a Valsalva


performed by anesthesia, with bubbles signifying entrance into the
chest. If so, make certain that a drain is place in the chest. Consider
placing a drain at the site, as the postoperative hematoma could lead
to a pleural effusion.

The indications for a combined anterior and posterior
fusion are evolving. Many studies on crankshaft phenomenon are limited
in that they generally rely on Cobb angle, while clinically rotation is
difficult to quantify. With this limitation in mind, it appears that
the best indication from the literature is to do an anterior fusion in
addition to a posteror fusion to prevent crankshaft if surgery is
performed before the peak height velocity12 (Fig. 19-19).
Practically, many times there is not sufficient data to determine peak
height velocity, so the triradiate cartilage becomes the second choice.
To stay out of trouble, discuss with family members ahead of time that
there is about a 40% chance of crankshaft if a posterior fusion is
performed before closure of the triradiate cartilage, and let them
participate in the decision.12,17 There is a potential role for pedicle
screw constructs in preventing crankshaft, but is not yet conclusively
FIGURE 19-19
This patient had a posterior fusion only at age 6 for a progressive
scoliosis. Her shoulders are now 90 degrees rotated to her pelvis due
to crankshaft. A concomitant anterior fusion in skeletally immature
children may play a role in minimizing crankshaft.
In terms of curve size and stiffness as indications for
a combined procedure, the traditional recommendations of curves >70
degrees or >40 degrees on bending as indications for an anterior
procedure are no longer valid. With current techniques, larger and
stiffer curves can be successfully treated with posterior surgery
alone, though the limits of this approach have yet to be defined and
are probably somewhat surgeon dependent. Stay out of trouble by doing
what you are comfortable with, and evolving to doing bigger and stiffer
curves posterior only in an incremental fashion. Thoracoscopic release
and fusion may be less of a pulmonary hit to the patient, but this is
still an invasive procedure whose risks must be weighed against the
While a wake-up test remains the gold standard, spinal
cord monitoring should be employed as well as an early warning, and
thus allow corrective steps earlier. SSEPs monitor primarily the
posterior columns, and changes may occur up to 20 minutes after an

While false negative readings are quite rare, they have
occurred and could prove disastrous if SSEPs are used alone.
Motor-evoked potentials (MEPs) provide almost immediate feedback on
neurologic changes, but have been found to be more difficult to perform
in many centers. In terms of staying out of trouble, more monitoring is
In children ≤10 years of age with scoliosis, there is a
greater likelihood than in adolescents that the scoliosis is not
idiopathic. In one center, of children ≤10 years of age believed to
have idiopathic scoliosis with curves >20 degrees, an MRI of the
entire spine revealed intraspinal anomalies in 20% of children 3 to 10
years of age and up to 50% of children <3 years of age.3
Even though young children may require anesthesia or sedation to lie
still during an MRI, we suggest staying out of trouble by getting an
MRI in young children with presumed idiopathic scoliosis in curves
Unlike adolescents with idiopathic scoliosis, children
with early onset scoliosis have quite significantly increased
mortality, which becomes apparent at age 40 to 50, primarily due to
respiratory and cardiovascular causes.20
Treating the chest wall deformity, and maximizing the space available
for the lungs should be the primary goal in the treatment of young
children with scoliosis, with correction of spinal deformity as the
secondary goal. Unfortunately, treatment options (including bracing,
growing rods, and titanium ribs) are all controversial, thus not great
solutions. Keeping parents informed of all options (including referral
to centers with large populations of young children with scoliosis) and
not missing underlying problems is the best way to keep out of trouble.
If using a growing rod, do not use a single pedicle
screw as an anchor, as it is possible to erode distally over time with
potential damage to nerve roots. One may also want to consider use of
titanium instrumentation for future imaging, including CTs to assess
implant location.

FIGURE 19-20 (A) Radiograph shortly after birth of an infant with congenital scoliosis with a Cobb angle of 75 degrees. (B)
A spinal fusion with a Harrington rod was successful at improving the
Cobb angle, as demonstrated by this radiograph at 4 years of age. (C)
At age 21 this patient’s scoliosis correction had been maintained, but
an inherent part of the “successful” spine fusion was a cessation of
growth of the involved spine. The patient had a functional vital
capacity of 28% at age 21. At age 25, the patient died after catching

FIGURE 19-21
A 2-year-old boy with a progressive 92-degree congenital scoliosis with
multiple anomalies, including a hemivertebra opposite a multilevel
unilateral bar. (A) Preoperative AP radiograph. (B) Three-dimensional CT. (C)
Surgery included an opening wedge thoracostomy and placement of
vertical expandable prosthetic titanium rib (VEPTR) devices. Note that
the scoliosis is improved and the bilateral lung fields appear more
symmetrical. The child gained 3 inches in height from the initial
procedure. (D) At 3-year followup,
following multiple lengthening procedures and an exchange of the hybrid
device (rib-to-spine device), improvement of the scoliosis and deformed
thorax is maintained. The scoliosis measures 51 degrees, and the child
is playing on a soccer team.
For children with congenital scoliosis, keep out of
trouble by looking for associated abnormalities. The prevalence of an
intraspinal anomaly is approximately 40%. An MRI is warranted in a
child with congenital scoliosis who has signs or symptoms of a
neurologic problem, shows significant curve progression, or will be
undergoing surgery. Unlike children with infantile “idiopathic”
scoliosis, an MRI is not necessarily automatic as there is already a
known cause of the scoliosis—the congenital anomaly. Sixty one percent
of patients in one center had associated abnormalities affecting seven
systems. The most common abnormalities associated with vertebral
malformation were cranial nerve palsy, radial hypoplasia, club feet,
dislocated hip, Sprengel deformity, imperforate anus, and hemifacial
microsomia.27 Renal anomalies (about
20%) may be evaluated with an ultrasound; cardiac anomalies (about 12%)
may be evaluated at the discretion of the primary care physician, who
may order an echocardiogram.
In general, documentation of curve progression over time should be noted before surgery is recommended, with three exceptions:
  • Unilateral bar opposite a hemivertebra
    (or a large unilateral bar without a contralateral hemivertebra) will
    progress, so early treatment is indicated.
  • Significant deformity from hemivertebrae amenable to resection, e.g., hemivertebrae at L5-S1.
  • Very large curve in a young child with resultant thoracic insufficiency.
Traditional treatment for exceptions 1 and 2 is a
hemiepiphysiodesis, with the hope that improvement may occur with
growth. A posterior-only fusion has been


to be relatively successful in preventing crankshaft in children with
congenital scoliosis, with only 15% of cases showing >10 degrees
progression of Cobb angle. However, children in two subsets did not
fare so well: children ≤4 years of age or with curves >50 degrees
had an incidence of crankshaft of 36% and 33%, respectively.28

Many children with congenital scoliosis do not progress
significantly and never need spine surgery. However, once progression
is confirmed, surgery should be performed before a major deformity
results. Traditional teaching is that no spinal instrumentation is
needed and that most of the curve improvement comes from the position
of the spine in the cast. With modern pediatric spinal instrumentation
systems, or cervical systems in the very small children, significant
correction of curve can be achieved. In our experience, a posterior
fusion or anterior-posterior hemiepiphysiodesis in the young child with
congenital scoliosis frequently does not prevent significant rotational
deformity from progressing despite an apparently solid fusion with
little change in Cobb angle. Though encouraging, it is too early to
tell if pedicle screw fixation may help prevent this progression of the
rotational deformity. Parents should be warned that the “hump” may get
worse with growth.
In older children, spinal fusion and instrumentation can
often achieve significant curve correction and cosmetic improvement via
movement through adjacent open discs. Brace treatment is not effective
for congenital scoliosis.
CT scans are particularly helpful in defining anatomy
and anomalies, and useful for identifying otherwise unrecognized lamina
defects preoperatively. Stay out of trouble by exposing the spine
carefully as there are frequently unrecognized osseous defects in the
posterior elements, making an inadvertent plunge into the spinal canal
an all-too-real possibility. Exposure of a child’s spine with
congenital scoliosis is best not left to unsupervised surgeons in
For nonambulators, focus on the goal of sitting up in a
wheelchair—truncal balance is more important than magnitude of curve
correction. These children have a higher likelihood of pulmonary
complications; stay out of the chest, and do not take down the
diaphragm if the goal of truncal balance can be met with posterior
instrumentation alone. Progression of the curve above or below the
primary curve over time is much more likely in neuromuscular scoliosis
than idiopathic. Think of T2-pelvis as your default plan for
neuromuscular scoliosis, and only change if special circumstances
exist. We have seen many, many more children we wished were fused to
the pelvis at the first operation than those we wished were not fused
to the pelvis initially.

FIGURE 19-22 In children with neuromuscular and/or pulmonary problems the biggest issue we often face is nutrition.
For odd diagnoses and syndromes, follow the Boy Scout motto and be prepared!
It is really nice to know ahead of time that your patient with
camptomelic dysplasia is likely to have thin or missing lamina before
directing a Cobb in that direction. For neuromuscular children
nutritional status is overwhelmingly important; if in doubt, get a
nutrition consult or g-tube first (Fig. 19-22).
Children with neuromuscular conditions at times have
significantly increased bleeding hours into the surgical procedure.
Valproic acid, a seizure medication frequently used in children with
neuromuscular disease, has been shown to be associated with
significantly increased blood loss during spine surgery. Unfortunately,
routine laboratory tests of complete blood count, prothrombin time, and
partial thromboplastin time will not adequately screen for the
plateletmediated effects of valproic acid, so consider having the
neurologist change medications one month prior to a major elective
The hematology literature recommends caution for elective surgery, and
suggests perioperative use of DDAVP (desmopressin acetate) to increase
von Willebrand factor levels and improve platelet function as
appropriate in some cases.31
Aprotinin has been shown to safely decrease the blood
loss in children with neuromuscular disease undergoing spine surgery,
as well as the postoperative drainage.33,34 But be aware that a second exposure to aprotinin within 6 months is associated with a 5% risk of anaphylaxis.35
Although there is a lack of scientific evidence, anecdotal evidence of
many surgeons suggests that once cell saver is given, bleeding becomes
more significant. Consider not using cell saver in children with
neuromuscular conditions. Dr. Emans suggests considering administering
fresh-frozen plasma to children with neuromuscular conditions rather
than throwing out the cell saver.
At times, cessation of surgery before the procedure is
completed and returning to the OR in a few days after the coagulopathy
resolves can be a life-saving decision. For the surgeon with less
experience in neuromuscular scoliosis surgery, this is difficult yet
particularly important.

Finally, make certain families understand the magnitude
of the risks and limited benefits in this population. Unfortunately,
many family members have expressed disappointment that their
nonambulatory child did not begin walking as a result of the spinal
fusion. Quote the families a 25% or greater risk of complications for
this surgery, including medical problems postop, and a significant risk
of death in the perioperative period.
The first, and sometimes hardest, step is to recognize
that there is an infection. A small amount of drainage in the first few
days to weeks is unlikely to be an infection, though prophylactic oral
antibiotics may help prevent bacterial seeding through a draining
wound. Drainage, increasing pain, or progressive feelings of
generalized malaise months after surgery are highly suggestive of
infection. Do not be lulled into security
by a normal erythrocyte sedimentation rate (ESR) and c-reactive protein
(CRP) and lack of fever. This is common in long-standing spinal implant
If in doubt, an aspiration down to implants and/or
fusion may be performed in the office, but a negative result does not
rule out an infection. The best information is from operative
evaluation, and at times these cultures will remain negative. If the
fusion appears solid at time of surgery, consider taking out all
implants (see Dr. Lenke’s opinion). Ongoing research suggests that when
only a partial implant removal is performed; it is likely that another
surgery will be needed in the future to remove the rest of the implants
for persistent infection.36
If the fusion is not yet solid, hardware should remain
in place. A single irrigation and debridement, with closure over
multiple drains (as one may clog) is usually adequate. Pay careful
attention to nutritional needs during this period. In problem cases a
wound VAC can be used as a last resort (Fig. 19-24).

FIGURE 19-23 (A) Patient on Risser Table for application of a well-molded cast. (B) The cast is worn for 6 months. (C) Oblique views help verify significant fusion. This radiograph shows residual curve present in fused spine. (D) One year following surgery.
FIGURE 19-24 (A)
Getting out of trouble with infection. Despite exemplary care,
infections will occur at times in children with neuromuscular
scoliosis. This child with mental retardation and quadriplegic cerebral
palsy had over 90 degrees of scoliosis treated with T2-to-pelvis
fusion. Infection occurred within months of surgery, and persisted
after three attempts at irrigation and debridement. (B)
Following use of a wound VAC, granulation tissue grew over the implant,
allowing her instrumentation to remain in, and fusion to solidify. She
required implant removal for clinically evident infection approximately
3 years later.

FIGURE 19-25 (A)
A 13-year-old girl with idiopathic scoliosis (63 degrees supine)
underwent thoracoscopic discectomy and fusion, followed by selective
posterior spinal fusion with a pedicle screw construct. (B) Unfortunately, it appears the wrong fusion levels were chosen, the indication for an anterior fusion is unclear, and (C)
not one pedicle screw was contained within the pedicle and vertebral
body. New technology did not help this child. Note that when the tips
of pedicle screws cross on an AP radiograph, chances are that at least
one of the screws is in the spinal canal.

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