Current Orthopaedics (2003) 17, 117^123

c 2003 Elsevier Science Ltd. All rights reserved.

doi:10.1054/cuor.2002.0327

MINI-SYMPOSIUM: CEREBRAL PALSY

(iv) Management of the spine in cerebral palsy

J.B.Williamson

Royal Manchester Childrens Hospital, Pendlebury, Manchester M27 8HA, UK

KEYWORDS Summary Cerebral palsyis the commonestcause of neuromuscular scoliosis.The eects

cerebral palsy, scoliosis
c 2003 Elsevier
of cerebral palsy scoliosis are to cause trunk decompensation and decrease sitting balance.
These eects can often be ameliorated by orthotic management, but a small number of pa-
tients come to surgical treatment.The objectives of surgical treatment are to give the patient
a balanced trunkover a level pelvis.In order to achieve this, in patients with large curvatures,
itis often necessary to perform sequential anterior and posterior spinal surgery.Thisis a very
major undertaking, and the multisystem eects of cerebral palsy must be taken into account
when surgery is contemplated if good results are to be achieved with safety.
Science Ltd. All rights reserved.

INTRODUCTION respects. The deformity is likely to present earlier espe-

Cerebral palsy (CP) is the commonest cause of neuro-
muscular spinal deformity. The manifestations of CP are
protean, ranging from minor hemiplegia, with little or no
functional eect and detectable only by an experienced
physician, to severe and disabling whole-body CP. Quite
plainly, the eects of one form of CP on the spine are not
the same as the other.
Prevalence
Estimates of the prevalence of spinal deformity in CP are
based largely on severely aected individuals, in whom
the prevalence is quite high.The incidence of spinal defor-
mity in patients with CP as a whole is estimated at about
25%, but the incidence in the institutionalised population
is 60 or 70%.1 It must be realised that the incidence of
scoliosis depends not only on the severity of the neurolo-
gical decit, but also on the physiological category into
which the patient falls. Spinal deformity is more common
in spastic CP, with almost 70% of Madigan and Wallaces1
patients with spastic CP developing a scoliosis, compared
with only 40% of their dyskinetic group, and 50% of the
ataxic group.Thus, the severely aected child with spas-
tic CP is at the highest risk of developing a scoliosis.
Natural history
The natural history of scoliosis in CP diers from that of
adolescent idiopathic scoliosis in a number of important
Correspondence to: JBW.Tel: +44 (0) 161727 2170; Fax: +44 (0) 161727
2547; E-mail: bradwilliamson@ntlworld.com
cially in those with a severe neurological decit. There-
fore, there is a longer period of growth in which rapid
progression is possible. Thoracolumbar curves tend to
progress more than thoracic or lumbar curves, and the
larger a curve at presentation, the more likely it is to
get worse. Saito et al.2 found that patients whose curves
were 401 or more by the age of15 eventually progressed
to be more than 601. Patients with whole-body CP were
more likely to deteriorate than those with a lesser de-
gree of neurological involvement. The risk of curve pro-
gression in adult life is greater in CP scoliosis than in the
idiopathic form. Majd et al.3 examined the propensity of
the scoliosis to progress in the adult residents of a care
home. They found that many relatively small (o501)
curves progressed after skeletal maturity, but that the
risk was greater in patients with severe spastic CP. Inter-
estingly however, they were not able to relate the likeli-
hood of curve progression to initial functional status, but
did note that increasing curve severity led to a decline in
function. A number of patients in their study went from
being assisted sitters to being bed-bound as their scolio-
sis progressed.
Classication of spinal deformity in CP
It is much more dicult to recognise discrete patterns of
deformity in CP than it is in idiopathic scoliosis. However,
it is possible to dene two principal types of curvesFin
essence those which involve the sacropelvis and those
which do not. Lonstein and Akbarnia4 called predomi-
nantly thoracic or thoracolumbar curves and no pelvic
obliquity Type 1 curves, and those with long C-shaped
118
Figure 1 Classication of cerebral palsy scoliosis (taken from
Lonstein).
curves going into the sacropelvisType 2 (Fig. 1). They ad-
ditionally classied the patient with a collapsing type of
kyphosis and no coronal plane deformity as having aType
3 deformity.This is a classication which is clinically rele-
vant as dierent instrumentation strategies are appro-
priate for each.
The eects of scoliosis in CP
The eects of scoliosis on the patient with CP dier con-
siderably from those with the adolescent idiopathic de-
formity. The sentient patient with CP may share the
body image concerns of her sister with idiopathic scolio-
sis, but in addition to this, functional problems are com-
mon in CP scoliosis and not in idiopathic scoliosis.
Most of the functional problems seen relate to the loss
of truncal balance (Fig. 2). If the ambulatory patient with
CP loses truncal balance, this may deprive them of their
precarious ability to walk. For the non-ambulatory pa-
tient, whose deformity tends to be worse, the problems
caused by truncal decompensation relate to loss of sit-
ting balance. Loss of sitting balance may deprive them of
the ability to be upright.With the loss of sitting goes the
loss of the ability to observe the world, and to socialise.
The upper limbs become a prop, to arrest declining
sitting balance, and the patient is deprived of their
useFthey become afunctional quadriplegic. As scoliosis
and pelvic obliquity worsen, seating becomes impossible,
and the patient is forced into recumbency.
There is a complex relationship between the increas-
ing pelvic obliquity that is seen inType 2 curves, and hip
subluxation and dislocation.There has been debate about
the part played by pelvic obliquity in the genesis of hip
dislocation. The two commonly go together and the de-
bate has been of the chicken and egg variety. However,
the only conclusion which one can draw with any cer-
tainty from this discussion is that the two commonly co-
exist. The relationship between pelvic obliquity and hip
problems is an imprecise one, but the evidence is that
CURRENT ORTHOPAEDICS
Figure 2 Severe scoliosis showing loss of truncal balance.
the development of hip problems in the child with CP is
more related to asymmetry of action of the muscles
about the hip than any suprapelvic or pelvic obliquity.5
Patients with severe pelvic obliquity have an increased
likelihood of the development of pressure sores. These
may occur over the dependent ischial tuberosity, greater
trochanter, or sacrum. When they occur they are di-
cult to treat without addressing the underlying causeF
which itself is rendered more dicult by the presence of
an ulcer.
Skin excoriation in the angle between the ribs and the
pelvis is common and dicult to treat in a satisfactory
way.
Pain is a frequent concern of those involved in caring
for a child with CP. It is dicult to know the likelihood of
a curve being painful. Many children with scoliosis are not
able to articulate their concerns and the views of the
care-givers necessarily involve some speculation. How-
ever, it is my experience that a minority of severely af-
fected patients suer pain associated with their
deformity. Those who do are usually able to make their
care-givers aware of their suering by one means or an-
other.
TREATMENT
The objective of the management of spinal deformity in
CP is no dierent from the orthopaedic management of
appendicular deformityFthe maximisation of function.
As in the appendicular locomotor system, cosmetic con-
siderations exist, but these are of secondary importance
MANAGEMENTOF THE SPINE IN CEREBRAL PALSY
compared to functional considerations. In order to max-
imise function it is essential that truncal imbalance and
pelvic obliquity are corrected.
Non-operative treatment
These objectives may be achieved in a number of ways.
The easiest way to improve sitting balance is by the use
of a thoracolumbosacral orthosis (TLSO). This is usually
eective for children with moderate, exible curves and
has a surprising degree of acceptance amongst both pa-
tients and care-givers. TheTLSO must be custom-made,
from a rigid material. In our experience, braces made from
non-rigid materials such as Plastazote or canvas very sel-
dom have any benecial eect, but may be uncomfortable
for the patient. Any benet from bracing which accrues
to the patient is immediate and functional. The carers
and patient are therefore in a good position to assess the
benet from the brace compared to the inconvenience of
wearing. Those who experience a signicant functional
benet from brace wearing will continue to complyF
those who do not, will not. There is no evidence that
wearing a brace alters the propensity of the curve to pro-
gress in any way, nor does it alter curve shape.
Some patients are not helped by bracing, or are unable
to tolerate the brace. This group are often managed by
seating adaptations. Most seats are based on an o the
peg chassis. In the infant, this can be simply a standard
car seat.These can be modied in a variety of ways from
judiciously placed pads through a whole variety of modi-
ed chairs to modular seats such as matrix chairs and
even specially moulded seats. The vast majority of pa-
tients can be seated in a satisfactory fashion by a skilled
orthotist.
Surgical treatment
Surgical treatment has the ultimate aim of restoring a bal-
anced trunk over a level pelvis. A great improvement can
usually be expected following surgery, but such surgery is
a major undertaking, and should not be contemplated
without the back-up of a team of individuals, from anaes-
thetists and intensivists to family-care ocers who are
used to dealing with children with neuromuscular diseases.
The place of surgical treatment in the child with CP sco-
liosis remains controversial. The dilemma is that the pa-
tients most likely to have a surgical indication are also the
most profoundly handicapped, and often the least able to
withstand the physiological insult of major spinal surgery.
Patient selection for surgery is therefore dicult and
causes more heart searching than almost any other
group of patients. Spinal surgery in CP is never absolutely
indicated, rather the indications are always relative.This
situation is one which spinal or orthopaedic surgeons are
well used to. In other circumstances, the situation is
usually resolved by an informed discussion with the pa-
119
tient. In this manner, the nature of the surgery, the prob-
able outcome, and the potential for complications can be
shared with the patient. An informed discussion then al-
most invariably leads to a mutually agreeable decision
whether to proceed with operative treatment or not.
In the patient with CP and normal intelligence, this same
approach can be followed, but this is seldom the case in
my practice. One is therefore left in the unsatisfactory
situation of having to make a dicult decision without
full appreciation of the patients problem, and without
the ability to involve the person most aected by the de-
cision in the making of that decision. Many surgeons are a
little uncomfortable with this situation, and prefer to
have a blanket policy of either oering surgery on the
basis of radiological measurements, or of not operating
on CP scoliosis.Our policy is to make the best assessment
we can of the functional problems caused by a patients
scoliosis, and then to discuss the issues with the patient
and care-givers. It is generally found that the care-givers
have a long and intimate knowledge of the patient and
their problems, and how they have changed over time.
In this manner, we nd it possible to dene surgical ob-
jectives as best we are able, and reach a considered deci-
sion about maximising the potential of each patient.
Indications for surgical treatment
We believe that consideration should be give to surgical
treatment when the scoliosis is responsible for a decline
in function.This may be categorised as follows:
K Patients with pain associated with their scoliosis
K Those whose sitting balance has deteriorated to such
an extent that it is impossible to seat them adequately
by the use of orthoses or seating modications
K Those whose pelvic obliquity is such that the skin over
the dependent buttock is at risk of breakdown, or that
costo-iliac impingement means that excoriation is not
amenable to conservative treatment.
K Ambulatory patients whose truncal balance is
deteriorating so much that it interferes with the
eciency of gait
K Patients of normal or near-normal intellect pose much
less of a dilemma for the treating surgeon. In this
group, the indications for surgery are similar to those
with adolescent idiopathic scoliosis, bearing in mind
the greater propensity of curves associated with CP
to progress.
I believe that there is seldom an indication for surgical
treatment in the child who is not aware of his surroundings.
Preoperative assessment
A preoperative assessment of the patient with CP must
always begin with a detailed assessment of their func-
tional level, and a reappraisal of the indications for surgi-
cal treatment. It is easy to assess the gross functional
120
levelFare they bed or wheelchair bound, can they
stand, can they walk inside or walk outside? However,
assessment of ner functional level is useful in surgical
decision making. For example a child who can feed him-
self or is fed by his carers (feeding is often the childs
greatest social interaction) has more to gain by maintain-
ing sitting balance than a child who is fed by a gastro-
stomy. Once the surgeon has convinced himself of the
indications for surgery, the remainder of the preopera-
tive assessment can be seen to subserve two functionsF
determination of the operative strategy and determina-
tion of the patients tness for surgery.
Fitness for surgery
Respiratory tness
Children with CP often have disordered upper gastro-
intestinal mobility and poorly coordinated swallowing.
Because of these factors, aspiration with accompanying
respiratory infection is common. This may be addressed
by fundoplication, or simply by gastrostomy feeding.
These issues should be treated before consideration of
spinal surgery. It is seldom possible to perform pulmon-
ary function tests in CP patients.Those with the intellect
to co-operate with the testing process seldom have the
degree of neuromuscular coordination needed to per-
form the tests. An adequate assessment can usually be
made by the performance of arterial blood gases.
Nutrition
Many children with CP have a very low body mass index,
and some are frankly malnourished. Spinal surgery is a
major physiological insult and patients are catabolic for
a considerable period. If the nutritional strain of spinal
surgery is superimposed on a patients already marginal
nutritional status, then the situation is exacerbated and
poor wound healing is likely to result. A simple assess-
ment of nutritional status may be made by examination
of the serum albumin (a value of less than 35 g/l indicating
cause for concern) and peripheral lymphocyte count (a
value of less than1.5 10/l indicating malnutrition). It has
been suggested that the weight should be plotted on a
centile chart, and those less than the 5th centile indicates
a higher risk for postoperative complications. Almost all
of our CP patients would fall below this cuto, but not all
are malnourished.
Patients giving rise to nutritional concern should have
a period of preoperative hyperalimentation, either
through their gastrostomy, or overnight through a ne
bore feeding tube.
Skin
The presence of acne is a contraindication to surgery as
the sub-acute wound infection rate may be higher in this
group.
CURRENT ORTHOPAEDICS
Urinary tract
Urinary tract sepsis is a contraindication to surgical
treatment.
Haematology
Scoliosis surgery is often a cause of major blood loss. So-
dium valproate causes a von Willebrands like coagulo-
pathy. With this condition, the usual clotting tests are
normal, and only measurement of the extrinsic pathway
by bleeding time alerts the clinician to the problem. The
paediatric neurologist should give consideration to chan-
ging the antiepileptic medication for the perioperative
period.
Relationship between spinal surgery and
treatment of hip subluxation
Pelvic obliquity and hip subluxation commonly coexist.
Fixed deformities of the hips are important to the
spinal surgeon for a number of reasons. Firstly, in the
surgical candidate such contractures may make position-
ing on the operating table dicult. We have had to
perform a preliminary soft-tissue release of the hips
purely to facilitate operative positioning. Secondly,
the question of timing of hip and spinal surgery often
arises. Our approach has been that if there is an indica-
tion for spinal surgery then this should be performed
rst. The hip surgeon is then dealing with a stable situa-
tion, and this allows a more accurate evaluation of the
situation.
Planning the surgical strategy
It is important to establish the extent, severity and ex-
ibility of all spinal curves in all three planes, and to deter-
mine a strategy for their correction. Careful clinical
examination will do this to a considerable extent. An
adequate clinical assessment can usually be made by ex-
amination in the seated position, with the clinician on the
other side of the examination couch. The exibility of
the curve may be assessed by suspending the patient by
the axillae. Spinal balance, and the exibility of any pelvic
obliquity can be assessed in this position. Type 1 curves
can certainly be dierentiated from Type 2 curves using
this manoeuvre, and the need to instrument the pelvis
determined.
Radiographs are essential in the planning of spinal fu-
sion levels. PA and lateral erect radiographs are the main-
stay of radiological assessment.These are however a less
reliable tool than in the idiopathic population, as a con-
siderable number of children being considered for sur-
gery cannot sit unaided. They therefore need to be
supported by their parents during the examination, and
this support inuences the apparent severity of the
spinal deformity. Spinal exibility is commonly assessed
MANAGEMENTOF THE SPINE IN CEREBRAL PALSY
by the use of supine bending radiographs. However,
these are of limited value in patients with CP.The patient
is often unable to co-operate with side bending in which
case a suspension or traction lm gives a reliable esti-
mate of the curve exibility.
If the curve is sti, and does not correct on suspen-
sion, then consideration should be given to the per-
formance of a preliminary anterior release. This is
especially so if truncal balance is not restored on suspen-
sion. In this procedure, the intervertebral discs and end-
plates of the apical segments are excised. It is usually
possible to excise at least six discs through a single thor-
acotomy or thoracoabdominal approach.The curve ex-
ibility is increased greatly by this technique, and an
anterior fusion is promoted, lessening the pseudarthro-
sis rate.This procedure is usually accomplished under the
same anaesthetic as posterior spinal fusion, with less
morbidity than if staged surgery is performed. It is how-
ever a physically and mentally arduous undertaking, and
is probably best undertaken by a team of at least two
spinal surgeons.
The selection of fusion levels is done on the basis of
the radiographs. All vertebrae involved in the curve must
be instrumented. In determining the lower instrumented
vertebra it is useful to bear Duboussets concept of the
sacropelvic vertebra in mind. This regards the sacropel-
vic unit as an additional vertebra. If it is involved in the
curveFa type 2 curveFthen sacropelvic instrumenta-
tion should be performed. If the curve does not extend
to the sacropelvic unit then instrumentation is usually
performed to the stable or neutral vertebra, whichever
is most caudal. With respect to the proximal fusion level
the situation is much simpler. Instrumentation to below
the high thoracic spine is associated with a high rate of
junctional kyphosis, and our policy is to fuse toT2.
Anaesthetic considerations
Spinal deformity surgery is a major physiological insult,
and physiological parameters need to be monitored
carefully. An invasive measurement of blood pressure
and measurement of central venous pressure are manda-
tory. We also use transoesophageal Doppler to assess
cardiac output.Urinary output is monitored using a blad-
der catheter and Uri meter.
Blood loss in these procedures can be major. Children
with CP are not suitable for autologous blood predona-
tion, but it is our policy to perform acute normovolaemic
haemodilution in the anaesthetic room. In this way, 500 ^
1000 ml of blood can be removed from the circulating vo-
lume before the operative blood loss begins. Peropera-
tive blood loss can be minimised by careful preoperative
positioning and meticulous packing during dissection.We
have found that the use of an aprotonin infusion reduces
the blood loss dramatically. Intraoperative cell salvage
further reduces the need for homologous transfusion.
121
There is debate about the place of spinal cord monitor-
ing in neuromuscular scoliosis.This debate has centred on
the technical feasibility of monitoring, and also the ratio-
nale for monitoring in patients who are already pro-
foundly neurologically compromised. As far as the
former goes, we have found little diculty in performing
SSEP monitoring in CP6 and others have shown the e-
cacy of cortical SSEP monitoring.7 With respect to the
latter, we would contend that the neurologically compro-
mised patient with CP deserves every chance to maintain
their neurological status after spinal deformity surgery.
Postoperatively, most patients return to the high de-
pendency unit, but a small numberFusually those hav-
ing sequential anterior and posterior surgery go to the
intensive-care unit, where they are often ventilated
overnight. An epidural catheter, placed peroperatively
by the surgeon gives rst-rate postoperative analgesia.
We accept that under certain circumstances this may
cloud the neurological picture, but the quality of analge-
sia is so good that it hastens the patients recovery su-
ciently for us to accept the trade-o. It is seldom dicult
to dierentiate between epidural induced paraesthesias
and spinal cord injury on clinical grounds.
Because of the high blood loss, a degree of coagulopa-
thy is common after surgery. This usually corrects
spontaneously within 72 h, although occasionally it is
necessary to give fresh frozen plasma perioperatively.
We withhold non-steroidal anti-inammatories until the
coagulation prole has normalised.
Surgical techniques
In order to correct a spinal deformity we need to have
the ability to apply corrective forces to the spine in three
planes, and the spine needs to be rendered exible
enough to respond to these forces.
The correction manoeuvre is performed by the use of
instrumentation. Longitudinal members (rods or plates)
are attached to the spine, and correctional forces are
transmitted to the spine by anchors (screws, hooks and
wires). The rod is thus bent to the shape we wish the
spine to be (often a compromise between the anatomical
shape of the spine and the maximal correction which can
be achieved safely), and the spine is drawn towards it
(translated) by the anchors. Dierent anchors are used
for dierent purposes:
Pedicle screws are the best all-round anchors and can
be used to apply forces in any direction.They are techni-
cally dicult to use in deformity cases, especially in the
thoracic spine.
Sublaminar wires have the greatest length adjustability.
They can only apply a force in one direction.
Hooks can apply forces in multiple directions, but their
strength is not as great as other anchors.
Special strategies are required for instrumentation of
the pelvis. Screws may be introduced into the thick bone
122
Figure 3. (a) Before surgery; (b) after surgery.
of the ilium, or a bent rod may be driven into the same
area. Thus, the forces of exion, extension, and rotation
may be applied.
Complications
As with any major surgery there is an appreciable
incidence of complications with spinal corrective surgery
in CP. The occurrence of complications is related to
both the severity of the surgical intervention and the
preoperative state of health of the patients. Risk factors
for the development of postoperative complications in-
clude severity of neurological involvement, recent his-
tory of signicant medical problems, and severity of
scoliosis.8 Interestingly, those who had both anterior
and posterior surgery did not have a higher complication
rate than those having posterior surgery alone. Pulmon-
ary and urinary infections were the commonest compli-
cations, and there was a signicant incidence of wound
infection, in keeping with our own experience. More
than 40% of Liptons patients8 were intubated for longer
than 3 days and almost 70% were hospitalised for more
than a fortnight.
The incidence of wound infection after this surgery
is a cause for concern. These patients are immunocom-
promised by their malnutrition, and their immune sys-
tem is further down-modulated by exposure to large
amounts of homologous blood products. A large wound
is open for many hours, and bulky implants provide
a sizeable haven for contaminating bacteria. It is no
surprise therefore that a wound infection rate of
CURRENT ORTHOPAEDICS
around 10% is reported in this population.9,10 Our
experience is that acute postoperative wound infections
can always be managed by thorough debridement. Sec-
ondary closure can usually be achieved, but occasionally
it is necessary to allow closure by secondary intention,
aided by a suction dressing. The metalwork can always
be preserved. Subacute or late wound infections are
due to low virulence organisms such as propionobacter
acnes or coagulase negative staphylococci, and are
cured by metalwork removal after consolidation of the
fusion.
Our experience has been that it is possible to reduce
wound infection rates signicantly by adherence to old
fashioned theatre discipline. Double-wound prepara-
tion, frequent change of sucker tips, and drastic reduc-
tion in theatre trac pay dividends in the long term.
Results
The results of surgery in terms of Cobb angle correction,
reduction in pelvic obliquity and restoration of sitting
balance are generally favourable (Fig. 3). Our experience
is that care-givers are almost universally delighted
with the surgical results, but a common theme is
they consistently underestimate the severity of the
surgery to be undertaken. However, once over the
perioperative period, almost all care-givers feel that
surgery has been worthwhile, and would put their
charges through surgery again. There is of course no
patientorientated outcome measure applicable to this
patient group.
MANAGEMENTOF THE SPINE IN CEREBRAL PALSY
PRACTICE POINTS
K Scoliosis is common in patients with whole-body
spastic CP
K Treatment should be aimed at increasing the
functional level of the patient, rather than
improving the radiographs
K Surgery is a major undertaking, but generally gives
satisfying results
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