Natural History and Surveillance

of Hip Dysplasia in Cerebral Palsy

Freeman Miller

Contents
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Adolescence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
Adult . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Diagnostic Evaluations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Hip Radiograph . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Computed Tomography Scans . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Bone Scan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Arthrography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Surveillance Algorithm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
Surveillance Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12

Abstract highest risk between 3 and 5 years old. The

Children with cerebral palsy (CP) very com- subluxation is measured with anterior-
monly develop subluxation or dislocation of posterior supine pelvic radiographs, and the
the hip joint. This dislocation leads to difficulty amount of subluxation is approximately one
sitting, problems with custodial care, and hip degree a month which means radiographs are
pain. The hip in the child with CP is normal at required annually from age 2 to 8 years old and
birth, and because of the abnormal muscle pull then every 2 years till completion of growth.
and growth, it slowly becomes displaced mov- The high-risk group of children are those who
ing lateral and posterior superior. This process cannot walk or need to use assistive devices
typically begins around age 2 and has the like walkers or crutches (GMFCS III–V). For
children who can walk (GMFCS I–II), there
needs to be only one radiograph in early child-
F. Miller (*)
AI DuPont Hospital for Children, Wilmington, DE, USA
hood 2–4 years old; if it is normal (<25% MP),
e-mail: freeman.miller@gmail.com no further radiographs are needed. When the

# Springer International Publishing AG 2017 1

F. Miller et al. (eds.), Cerebral Palsy,
https://doi.org/10.1007/978-3-319-50592-3_126-1
2 F. Miller
MP reaches 25%, there should be an increase
in attention with increased radiograph every
6 months, and consideration of surgical inven-
tion occurs with subluxation between 25 and
40%. For MP greater than 40 percent, surgical
invention should be considered. Based on the
experience from Sweden, almost all children
with CP can reach adulthood with located hip
joints.
Keywords
Cerebral Palsy
Natural history
Surveillance

Hip subluxation
Hip dislocation
Migration
percent
Head-shaft angle
GMFCS

Spasticity
Hypotonia
Hemiplegia
Introduction
Children with cerebral palsy have a high risk of
developing hip dislocation, which over time can
become painful because they develop arthritis.
The hips also develop contractures which can
impair function such as sitting, standing, and
lying. Because severe adduction contractures
may develop, it also has a negative impact on
daily personal care such as toileting, bathing,
and dressing. The dislocated hip in the child
with CP however is very different from the
dislocated hip in a normal baby or child where
developmental hip dislocation (DDH) occurs at
birth. It is also important to always be aware that
the hip stability problems are very disease spe-
cific; therefore understanding the natural history
and the response to treatment has to be very dis-
ease specific. Some examples include the
dislocated hip in the child with spinal cord dys-
function, most typically in the child with
myelomeningocele who may also develop dislo-
cations in early childhood; however, these hips
tend to be flaccid and insensate. As a conse-
quence, these hips seldom become painful; they
remain mobile and almost never cause a signifi-
cant functional impact. Also attempts at recon-
struction have high failure rates because of the
lack of muscle strength and the muscle imbalance
which is difficult to correct surgically. Another
typical example of severe weakness is the child
with spinal muscular atrophy who may also
develop a dislocated hip; however, because the
child has sensation, the hip may become painful
and cause significant impairment. These hips are
very difficult to maintain in the hip joint because
of the severe weakness and may require resection
at the end stage. Children with arthrogryposis may
be born with dislocated hips; usually these are
very stiff and do not respond to early infant treat-
ment. Attempting to increase range of motion with
therapy helps to improve the child’s function as
they grow; then the treatment is directed by the
functional impairment present. Children with
cerebral palsy may be spastic, have a movement
disorder, or have a mixed pattern. There is a small
group who are also hypotonic. Each of these has
different impacts on the hip joints, as does the
child’s age and gross function as defined by the
Gross Motor Function Classification Scale
(GMFCS). This chapter will address the expected
natural history and with the understanding of the
natural history will outline a monitoring program
for early identification of hip displacement.
Natural History
The natural history of spastic hip disease follows a
very clear pattern with a defining feature being
that the hip at birth in these children is completely
normal (Fig. 1). If the hips are not completely
normal at birth, then these children have develop-
mental hip dysplasia (DDH) and not spastic hip
disease, and their treatment needs to be quite
different.
Childhood
The childhood stage of spastic hip disease is when
almost all the spastic hip pathology begins. This
stage is defined as the period from ages 1 to
8 years; however, the highest risk period is from
ages 2 to 6 years. Most children with CP start
developing spasticity in their second year of life,
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy 3

Fig. 1 The natural history of spastic hip dislocation is
consistent and well understood. The following series of a
well-documented patient with no treatment shows the typical
changes over time. In infancy, the hip is normal, as shown in
this radiograph at age 18 months with a 10% migration
percentage (a). 1 year later at age 2.5 years, the femoral
neck is in valgus, and there has been a noticeable increase
in lateral migration to a 25% migration percentage (b).
By age 5 years, the femoral head continues to migrate
laterally, now with 40% migration percentage. Also, there
is thickening of the medial wall of the pelvis, the lateral rim
of the acetabulum is showing signs of deformity from high
pressure, and the femoral head already has some lateral
osteoporosis with a very high femoral neck-shaft angle (c).
By age 8 years, the femoral head has become oblong, and the
acetabular dysplasia is much worse, with damage at the
4 F. Miller
and as they grow bigger and the brain is develop-
ing, the spasticity gets worse. Also, the muscles
are growing stronger so that they can generate
more force with the spasticity. This higher force
then causes the pathomechanics discussed previ-
ously (chapter ▶ “Etiology of Hip Displacement
in Children with Cerebral Palsy”). In younger
children with spasticity, because most of their
acetabulum is cartilage, the hips are most suscep-
tible to developing instability and anatomical
deformity. In these young children, the initial
sign of the hip at risk for developing subluxation
be limited hip abduction with the hip and knee
extended. This is a screening aspect of the phys-
ical examination that should be consistently
performed on all spastic children and is especially
important for treatment considerations. As the
force continues to get worse, radiographs start
demonstrating lateral migration of the femur.
The early stages of subluxation are usually silent
with no evidence of pain. As the subluxation
becomes more severe, some children will have
periods of pain as their hips develop some syno-
vitis response. This pain occurs when they have
very high force from spasticity, and the hip starts
to migrate laterally, developing severe subluxa-
tion that reaches 60% to 80%. Once the hip starts
developing subluxation as demonstrated by the
migration percentage (MP) measured radiograph-
ically in childhood, it is usually progressive. Sub-
luxation tends to increase at a rate of
approximately 2% per month (Miller and Bagg
1995) if the migration index is less than 50% or
60%. Once the migration index reaches 50% to
60%, the hip may go to full dislocation in child-
hood very quickly, sometimes going from 60% to
100% within several months.
The hips in the child with a mixed movement
disorder maybe somewhat less predictable than
Fig. 1 (continued) lateral acetabular corner (d). By age
10 years, there is severe acetabular dysplasia, a very shal-
low acetabulum as the medial wall has become very thick,
the femoral head is overgrown laterally with severe osteo-
porosis, and there is a very high neck-shaft angle (e). By
age 13 years, the femur has completely dislocated and
appears somewhat irregular. The acetabulum is now
extremely dysplastic (f). As the process continues to age
the child with only spasticity. The child with
only a movement disorder especially with
athetosis has in my experience a lower risk of
hip dislocation; however, monitoring is still
required, since dislocation can occur in rare
instances. Children with hypotonia, some of
whom are ambulatory, may develop hip dysplasia,
which may develop slowly all through middle
childhood. Because they have wide hip abduction,
there may be reduced diligence in monitoring, and
the subluxation may be severe when it presents.
Also these hips do have a different profile in that
the acetabulum is usually shallow but not opened
by force the same way as the hip of the child with
spasticity. For this reason, the treatment and mon-
itoring considerations are somewhat different
(Fig. 2). The only group of children with CP
who have not been reported to develop hip sub-
luxation is the child with pure ataxia (Hagglund
et al. 2007).
Adolescence
The adolescent period, from the ages of 8 to
18 years, is a time when the skeleton is much
more mature with less cartilage in the hip joint
and much more bone. During this time, the risk for
the development of spastic hip disease in a hip that
is otherwise normal goes from a relatively low
risk at age 8 years to no risk by skeletal maturity.
For children who come to preadolescence with
some hip subluxation in the range of 30% to
60%, the subluxation may continue to progress;
however, the progression is usually quite slow,
less than 1% per month (Miller and Bagg 1995).
During the period of rapid adolescent growth, the
development of pelvic obliquity and scoliosis may
impact the hips. For hips with mild to moderate
15 years, the severely osteoporotic lateral aspect of the
femoral head collapses, and severe arthritic changes
occur at the small contact area of the medial femoral head
and the lateral acetabulum (g). This is the stage when most
children develop severe pain with motion, and sometimes
severe pain at rest develops
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy
subluxation, and if the hip is on the high side of
the pelvic obliquity, it has an increased risk of
developing further subluxation. However, if the
Fig. 2 The hip radiograph shows the typical features of
the hypotonic hip with severe coxa valga, eccentric epiph-
ysis, and a shallow acetabulum. This hip may remain stable
through growth as one follows the MP. However, it is also
possible that suddenly the hip is developing Barlow
positive-type hip dislocation or becoming painful if the
child is growing fast and is physically active
Fig. 3 This boy is GMFCS I level function with a hemi-
plegic IV pattern because he had moderate internal rotation
during gait causing lot of tripping. This was corrected with
a femoral derotation osteotomy at age 6 at which time he
had very mild acetabular dysplasia, but due to his high
5
hip is on the down side of the pelvic obliquity, a
subluxated hip may actually reduce and end up
having a normal radiographic appearance
(Frischhut and Krismer 1990). The other higher-
risk group for progression is the children with type
IV hemiplegia, meaning that they have some spas-
ticity of the hip adductors and internal hip rotation
gait. Although these tend to be very highly func-
tional GMFCS I and II level ambulation, they may
develop severe hip subluxation during the adoles-
cent growth period (Abousamra et al. 2015; Rutz
et al. 2012) (Fig. 3).
Adult
The natural history of spastic hips in adults is not
as well defined. If the hip is normal, defined as an
MP of less than 25% or 30%, the risk of develop-
ing hip subluxation in adulthood is virtually non-
existent. If the hip has mild to moderate
subluxation, defined as 30% to 60%, there may
rarely be some progression in adulthood. Most
hips seem to remain stable. However, individuals
who reach adulthood with hip subluxation of
greater than 60% will, slowly over time, go to
full dislocation in almost all cases (McHale et al.
1990).
functional level was not felt to have significant risk of
further subluxation (a). His next presentation was with a
complaint of severe hip pain made worse with athletic
activity. He now presents with severe acetabular dysplasia
and lateral subluxation (b)
6 F. Miller
The major disability caused by spastic hips
with subluxation or dislocation as the children
age is limitation of motion, sometimes severely
interfering with custodial care. As an example, it
is very difficult to provide adequate perineal care
during menstrual cycles for a young adult woman
with severe hip adduction contractures from fixed
hip dislocation. As outlined above, the subluxated
and dislocated hips become arthritic and, like
many arthritic joints, become painful. There is a
myth in the medical community that the hips do
not ever become painful in individuals who are
noncommunicative. The fact that these individ-
uals develop painful hips from neglected disloca-
tions is absolutely clear to physicians who
routinely care for these individuals; however, it
is often difficult to determine how much pain
individuals are experiencing. Just as with elderly
individuals who have degenerative joints, some-
times individuals with severe changes on radio-
graphs have only mild pain, and others with mild
radiographic changes have severe pain. This same
discrepancy is seen in people with spasticity and
hip dysplasia. Although the published literature
varies widely, probably 50% to 75% of individ-
uals with spastic hip dislocation experience
enough pain that it is recognized by the caretakers
or medical personnel (Hodgkinson et al. 2000,
2001; Terjesen et al. 2004). Although not entirely
conclusively defined, we found that subluxated
hips had less pain than dislocated hips (Miller
and Bagg 1995). There are no good data on how
near-normal spastic hips have to be to remove the
risk of becoming painful with aging.
Another element of the natural history of the
neglected dislocated hip is the effect of chronic
pain in the individual leading to decreased seating
tolerance and more difficult custodial care
decreasing general medical condition. There
have been improvements in assessing monitoring
this impact using structured caretaker question-
naires. The best one for the child or adult with
severe CP who is noncommunicative is the CP
CHILD (Jung et al. 2014). With the goal of
improving the quality of life for the child and the
caregivers, this type of instrument is important.
There are however still no good ways to assess the
impact of pain on the child’s health which may be
mild or can be severe leading to the child’s death.
How often the dislocated hip leads to a child’s
death is unknown and usually undocumented;
however, it clearly does occur as demonstrated
by this case (Fig. 4).
Diagnostic Evaluations
The most important work in evaluating the diag-
nostic monitoring of children with the typical
posterosuperior spastic hip disease was done by
Reimers (1980) who defined the radiographic
measurement of the standard AP pelvis radio-
graph. The physical examination evaluation of
spastic hips at risk was defined and popularized
by Rang et al. (Silver et al. 1985). This examina-
tion monitors the degree of hip abduction with the
hips and knees fully extended (Fig. 5). Dr. Rang
promoted the screening concept that all spastic
children should have this measure of hip abduc-
tion monitored every 6 months during childhood
at least to age 8 years. This monitoring could be
performed by a trained physical therapist. When
better population screening was started in Sweden
by Hagglund, they found that the PE was not an
accurate method to detect early hip subluxation
(Hagglund et al. 2007). Therefore the focus for all
screening programs has shifted to a supine AP
pelvic radiograph. The use of ultrasound screen-
ing could potentially reduce radiation exposure
and has been reported as successful in children
with CP to age 8 (Smigovec et al. 2014); however,
it is not in widespread use and has the main
drawback of requiring specialized expertise to do
the scan correctly. Additional imaging such as CT
scans is useful for planning surgical treatment but
has too much radiation and is too expensive for
routine monitoring.
Hip Radiograph
The primary method for screening the child with
CP for hip subluxation is the pelvic radiograph.
The standard anterosuperior supine radiograph of
the pelvis should have the legs in neutral or rela-
tively neutral position with the child lying
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy 7

Fig. 4 This is a 12-year-old girl who was a GMFCS III

ambulatory with a posterior walker until she started to
develop severe hip pain and was noted to have severe
subluxation with degenerative arthritis. A proximal resec-
tion with valgus (McHale procedure) was performed, and
she never became pain-free. Over 2 years she finally could
no longer even get out of bed because of the severe pain in
her hip. We planned to do a total hip replacement; however,
1 month before the planned surgery, the chronic pain clinic
physician significantly increased her narcotic dose, and she
died in her sleep most likely due to narcotic overdose to
control the hip pain
Fig. 5 The primary screening and physical examination
method is to record the hip abduction with the hips and
comfortably. If needed, the child may have a small knees extended. This is the most sensitive physical exam-
pillow under his knees for comfort, but the hips ination measure to determine at risk and subluxated spastic
should not be flexed more than 20 degrees. The hips. It is important to make sure that asymmetric abduc-
tion is noted by observing motion of the pelvis as the hip is
frequency of obtaining a radiograph is based on
abducted
the GMFCS level of the child and the child’s age.
Because of the unpredictable nature of the
GMFCS in young children 2–5 years old, we appropriate to only look at the radiograph,
simplify this to say if the child is walking without because it is impossible to tell the difference
assistance, he should have one hip x-ray that is between an MP of 20% and one of 35% without
normal around 2 years of age. If the child is using measuring. It is no more appropriate to only look
an assistive device or not walking, an x-ray is at an anteroposterior pelvis radiograph of a spastic
required every year till age 8 and then if it is child than it is to monitor idiopathic scoliosis by
normal every 2 years till skeletal maturity. If the obtaining a scoliosis radiograph and only looking
MP is over 30% and there is no intervention, x-ray at it without measuring the curve. The measure-
is required every 6 months till age 8 and then ment of the parameter that is most predictive of
every year. The MP of this radiograph must be outcome is clearly the MP, as demonstrated by
measured and recorded (Fig. 6). It is not Reimers (1980). Another measure of the lateral
8 F. Miller
Fig. 6 The most important measure to monitor on the
radiograph for monitoring spastic hips is the Reimers
migration percentage (MP). This should be an ante-
roposterior supine radiograph with the child’s hips in the
extended and relatively normal position. The hip should
not be forced into abduction or external rotation if the child
resists. The first line on the radiograph should be the
transverse Hilgenreiner’s line (h), which goes through the
center of the triradiate cartilages. If the triradiates are fused
or not apparent, the inferior border of the acetabulum or
ischium may also be used. Next, a perpendicular Perkins’
line is drawn from the lateral corner of the acetabulum ( p).
The medial and lateral borders of the femoral epiphysis are
next defined. Next, a measure of the distance from the
Perkins’ line to the lateral border of the acetabulum is
measured (A); then the whole width of the femoral epiph-
ysis is measured (B)
migration that is used in the monitoring of DDH is
the center-edge angle. The center-edge angle is a
poor measure for spastic hip disease and has a
correlation coefficient of only 0.7 when compared
with the MP. The reason the center-edge angle is
not a good measure is that it requires defining a
center rather than a line in the femoral head and
also requires defining a point in two-dimensional
space of the lateral acetabulum. Both these points
are often quite diffuse in these young and growing
children, making the measurement of the center-
edge angle quite inaccurate. The center-edge
angle is also a measure that is not linear but
follows the sine curve. Therefore, changes in the
area of interest, between 20% and 40%, tend to
fluctuate wildly based on these inaccuracies and
make monitoring for treatment methods
extremely poor. In summary, the center-edge
angle has no role in the ongoing monitoring of
spastic hip disease. Additional radiographs, such
as frog-leg lateral and weightbearing radiographs
of the hips, also add no information to the moni-
toring and treatment decision making for children
with spastic hip disease. Because these children
often require many radiographs over their life-
time, it is important to limit radiographs to only
those that directly add to the clinical decision
making, thereby limiting the radiation exposure
of these children as much as possible.
Another measure that has been recently added
to the AP hip x-ray is the head-shaft angle
(Hermanson et al. 2015a, b). This measures the
head angle as measured by a line through the
growth plate relative to the femoral shaft. This
measurement is similar to the neck-shaft angle
but is likely less impacted by the hip rotation.
This was reported to be able to help separate
those hips with 30–40% migration into those
who are likely to progress and those less likely
to progress. This measure may also be a better
measure of the current summated hip joint reac-
tion force since the growing child will relatively
quickly align the growth plate perpendicular to
the principle joint reaction force. This combina-
tion of the child’s age, GMFCS, MP, and head-
shaft angle has been combined into an APP to
calculate risk of progression as an aid in deter-
mining treatment options. As more data is col-
lected in screening programs, these calculation
applications should become more useful in
guiding care.
Computed Tomography Scans
The use of computed tomography (CT) scan to
evaluate hips with spastic hip disease has been
extensively reviewed in the literature. It is impor-
tant to note that not all hips have a typical post-
erosuperior subluxation of the femoral head, and
by far the best mechanism for evaluating the
direction of the hip dysplasia is the CT scan
(Brunner and Robb 1996; Buckley et al. 1991;
Kim and Wenger 1997; Zimmermann and Sturm
1992). The CT scan is especially useful to clearly
define the position of the femoral head. Some-
times direct anterior subluxation or dislocation
can have an almost normal radiographic appear-
ance or a very minimal abnormality. At other
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy
times, the femoral head can be situated laterally so
that it is very difficult to tell whether this is a
lateral anterior subluxation or dislocation or a
posterolateral subluxation or dislocation. The CT
scan is extremely accurate in defining this posi-
tion. Using the CT scan to evaluate the exact area
of the deformity of the acetabulum and to define
the response of the acetabular shape to reconstruc-
tion is also useful (Chung et al. 2008). In the
typical standard posterosuperior subluxation in
which the leg contracture and position are pre-
dominantly situated in adduction flexion and
internal rotation, it does not add much clinical
information. In summary, not all children who
are anticipated to have hip reconstruction need to
have a CT scan; however, if there is any concern
about understanding the exact direction of the
subluxation as part of the preoperative planning,
a CT scan should be obtained. Computed tomog-
raphy scan has also been demonstrated to be an
excellent mechanism for measuring femoral neck
anteversion, especially if a normal femoral neck-
shaft angle is present (Horstmann and Mahboubi
1987; Mahboubi and Horstmann 1986; Pons et al.
2013). The use of the CT scan to measure ante-
version is most useful after the femoral neck-shaft
angle has been corrected and abnormal effects of
the femoral osteotomy are present.
The migration percentage (MP) is equal to A
divided by B (MP = A/B). Acetabular index can
also be monitored; however, accurate measure-
ments are often difficult.
Ultrasound
Ultrasound of the hip has been used extensively in
the evaluation of infants with DDH; and there are
reported attempts to use it as a screening evalua-
tion of subluxated spastic hips (Smigovec et al.
2014). Ultrasound is a noninvasive and inexpen-
sive mechanism but does require experienced spe-
cialized expertise. It has also been used to measure
femoral anteversion. It is especially useful in mea-
suring femoral anteversion in spastic children
who have not had previous hip surgery and have
high femoral neck-shaft angles (Haspl and Bilic
1996; Miller et al. 1997).
9
Bone Scan
Technetium bone scans may be used to evaluate
the source of unknown pain in children who are
noncommunicators. These scans are especially
useful when evaluating children who have a stable
subluxated hip or a dislocated hip that has not
been painful previously but suddenly develops
discomfort without a readily apparent source.
The use of the bone scan in this situation allows
defining whether there is any reaction in the hip
joint that may be the source of the pain. Techne-
tium bone scan also allows localizing occult frac-
tures, such as fractures in the femoral neck. If
there is a question of heterotopic ossification
developing when the radiograph is still normal,
the bone scan will be clearly positive even if the
radiograph is still normal.
Arthrography
Arthrography of the hip has been used extensively
in evaluating and deciding treatment processes for
DDH and Perthes disease of the hip. There have
been reports of using hip arthrography in spastic
hips (Huh et al. 2011; Heinrich et al. 1991); how-
ever, this test only confirms what is well known
and adds little useful clinical information that can
help in diagnostic decision making. There is no
routine role for hip arthrography in spastic hip
disease.
Treatment
Surveillance Algorithm
Based on the data of the natural history and the
strong evidence from population surveillance pro-
grams in northern Europe and Australia, the risk
of hip displacement is directly related to the
GMFCS functional level of the child (Gordon
and Simkiss 2006; Connelly et al. 2009; Elkamil
et al. 2011; Hermanson et al. 2015a; Kentish et al.
2011; Wynter et al. 2011; Wynter et al. 2015).
Based on the data collected and reported, we
tried to collate the data from multiple studies
10 F. Miller

Fig. 7 This graph is a Risk of developing MP>30% or MP>33%

summary of the risk of
developing hip subluxation 0.8
based on the GMFCS level
compiled data of 1082
reported patients from four 0.6
separate surveillance
studies

0.4

0.2

0
GMFCS I GMFCS II GMFCS III GMFCS IV GMFCS V

Fig. 8 The percent of the % of total subluxation (MP>30% or MP>33%) occurred at certain age
total population studied % of total dislocation occurred at certain age
who had dislocated hips at 30
the specific age (green line)
and the percent of the total
population who had
subluxated hip at a defined 22.5
age (blue line). This shows
that the peak dislocation by
age risk occurs 2–3 years 15
after the peak subluxation
risk. This includes
182 subluxated hips and
38 dislocated hips in which 7.5
the data can define when it
occurred

0
1 2&3 4 5 5&6 8 9 11&12 15&16

(Pruszczynski et al. 2015). Based on this data we
documented the linear relationship with GMFCS
level and risked of hip subluxation (Fig. 7). There
is also a more complex relationship between age
and risk for hip displacement (Fig. 8). There is a
peak of subluxation risk between 2–5 years old,
and then the risk of dislocation peaks later in the
5- to 8-year-old period. Through adolescents there
continues to be a risk for both subluxation and
dislocation. The only clinical group that seems to
have no risk of hip displacement is the ambulatory
child with only ataxia. Another relatively high-
risk group are children with Winter classification
type IV hemiplegia. Almost all these children are
high-functioning GMFCS I or II but have hips that
are adducted and internally rotated with equinus
during gait. This places a high force on the poste-
rior superior edge of the acetabulum leading to hip
dysplasia and subluxation. The age of occurrence
seems to be late childhood and during the adoles-
cent growth period; therefore the subgroup is
unique because of good ambulatory function in
the late onset (Abousamra et al. 2015; Graham
et al. 2005; Rutz et al. 2012).
The initial hip surveillance program was
started by Mercer Rang in the early 1980s to
encourage early treatment of hip subluxation (Sil-
ver et al. 1985). This screening program was
based on the supine physical examination, which
was then used to indicate the need for radiograph.
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy 11

Hip Monitoring Protocol for Cerebral Palsy

Goal: To monitor all children withCP to prevent hip dislocaon.
Monitoring schedule:
GMFCS I and II (full ambulation without assistive device) –
One x-ray after 2 years of age – no other needed if normal
Exception: Hemiplegia type 4 (with hip involvement, Rotation or flexion)
X-ray every two years from age 8 to skeletal maturity

GMFCS III, IV, & V (non-ambulatory to walking with assistive device)

Until age 8 – x-ray every year, MP
X-ray every 6 month - For children with severe spasticity or MP >25
Age 8 to Skeletal maturity – if previous x-ray normal – every two
years
If previous x-ray MP > then 25% - every year x-ray
Treatment Recommendaons:
Up to age 8 Years:
MP 30 to 60% and hip abduction less then 30º with hip and knee extended – STR
MP 30 to 60% and hip abduction over then 30º with hip and knee extended – Observe
MP > 60% and healthy child – Reconstruction
MP > 60% and hip abduction < 30º and child with multiple medical problems – STR Migration Percent (MP)- A single
AP supine pelvis x-ray with the child
Over age 8 years: lying in a relaxed, comfortable neutral
MP > 40% - Abduction less then 45º - Reconstruction position with about (10°) abduction, may
MP > 50% - Abduction over 45º - Reconstruction have a small blanket roll under the knees
Dislocated hip – Painful but not severe degenerative changes – Reconstruction to slightly flex the hips and knees if this
makes the child more comfortable. Hip
Dislocated hip – Painful with severe degenerative changes – Palliative Procedure rotation should be approximately neutral
but do not force into any position.

Fig. 9 This is the poster we have in each clinic to remind and explain the CP hip surveillance program

The frequency was based on the work of Reimers
who showed that hips progressed at a rate of
approximately one degree a month in the early
stages of subluxation (Reimers 1980). The hip
monitoring program at AI DuPont Hospital
started in 1988 based on the data of Rang and
Reimers but has evolved to include the current
information. It is now based on age and
GMFCS, with exception for the child with type
IV hemiplegia. The population-based data on the
child with hemiplegia is still very weak, since it is
small group to start with and only a small number
develops hip subluxation. Our surveillance pro-
gram is outlined in table below (Fig. 9).
Surveillance Results
A surveillance program for early detection of hip
subluxation in children with CP only makes sense
if there is early intervention that is successful in
preventing later hip dislocation. By careful mon-
itoring of the surveillance results and encouraging
early intervention, there has been a dramatic
reduction in the number of children who need
hip reconstruction based on the data from the
Swedish and Australian population screening
(Dobson et al. 2002; Elkamil et al. 2011;
Hagglund et al. 2014). Although there is no
agreed universal treatment protocol for the hip
with mild subluxation, there are several generally
accepted strategies. The need for some raised
level of concern is between 25 and 40% subluxa-
tion. The first-level response is increased fre-
quency of observation based on other factors
such as severe spasticity, nonambulatory, and
windblown posture. This is also the time to initiate
the conversation with the parents that some surgi-
cal intervention may be needed in the future which
helps them get psychologically prepared. Chil-
dren with spasticity who are GMFCS III–V and
whose migration is increasing toward 40% should
12
then be prepared for surgical intervention based
on the physical examination and tone pattern. For
the child with spasticity and hip abduction less
than 30 degrees, adductor lengthening is usually
considered. For children with hypotonia, further
observation to 50% subluxation would be reason-
able. The important factors in making a treatment
algorithm require knowing the age, the degree of
abduction of the hip, and the MP from a radio-
graph. The hip treatment is then divided into three
areas, as follows. The first area is prevention, in
which the treatment is addressed at the root cause
of the spastic hip disease. The second area is
reconstruction, in which the treatment is primarily
directed at reversing the secondary effects of spas-
tic hip disease. The third, or palliative, area is the
treatment directed at addressing the tertiary symp-
toms and deformities of spastic hip disease.
Conclusion
The natural history for hip displacement in chil-
dren with cerebral palsy is well documented in the
childhood age range and is heavily influenced by
GMFCS functional level of the children. The nat-
ural history in adolescence and in adulthood is less
clearly defined. Surveillance program based on
radiographic imaging of the hips in a scheduled
program has demonstrated the ability to maintain
the hips’ location and reduce the amount of sur-
gery. The best time and type of intervention based
on this early identification are still not clear.
References
Abousamra O, Er MS, Rogers KJ, Nishnianidze T, Dabney
KW, Miller F (2015) Hip reconstruction in children
with unilateral cerebral palsy and hip dysplasia.
J Pediatr Orthop 36:834
Brunner R, Robb JE (1996) Inaccuracy of the migration
percentage and center-edge angle in predicting femoral
head displacement in cerebral palsy. J Pediatr Orthop B
5:239–241
Buckley SL, Sponseller PD, Magid D (1991) The acetab-
ulum in congenital and neuromuscular hip instability.
J Pediatr Orthop 11:498–501
Chung CY, Choi IH, Cho TJ, Yoo WJ, Lee SH, Park MS
(2008) Morphometric changes in the acetabulum after
F. Miller
Dega osteotomy in patients with cerebral palsy. J Bone
Joint Surg Br 90:88–91
Connelly A, Flett P, Graham HK, Oates J (2009) Hip
surveillance in Tasmanian children with cerebral
palsy. J Paediatr Child Health 45:437–443
Dobson F, Boyd RN, Parrott J, Nattrass GR, Graham HK
(2002) Hip surveillance in children with cerebral palsy.
Impact on the surgical management of spastic hip dis-
ease. J Bone Joint Surg Br 84:720–726
Elkamil AI, Andersen GL, Hagglund G, Lamvik T,
Skranes J, Vik T (2011) Prevalence of hip dislocation
among children with cerebral palsy in regions with and
without a surveillance programme: a cross sectional
study in Sweden and Norway. BMC Musculoskelet
Disord 12:284
Frischhut B, Krismer M (1990) Pelvic tilt in neuromuscular
diseases. Der Orthopade 19:292–299
Gordon GS, Simkiss DE (2006) A systematic review of the
evidence for hip surveillance in children with cerebral
palsy. J Bone Joint Surg Br 88:1492–1496
Graham HK, Baker R, Dobson F, Morris ME (2005) Multi-
level orthopaedic surgery in group IV spastic hemiple-
gia. J Bone Joint Surg Br 87:548–555
Hagglund G, Lauge-Pedersen H, Wagner P (2007) Char-
acteristics of children with hip displacement in cerebral
palsy. BMC Musculoskelet Disord 8:101
Hagglund G, Alriksson-Schmidt A, Lauge-Pedersen H,
Rodby-Bousquet E, Wagner P, Westbom L (2014) Pre-
vention of dislocation of the hip in children with cere-
bral palsy: 20-year results of a population-based
prevention programme. Bone Joint J 96-B:1546–1552
Haspl M, Bilic R (1996) Assessment of femoral neck-shaft
and antetorsion angles. Int Orthop 20:363–366
Heinrich SD, MacEwen GD, Zembo MM (1991) Hip dys-
plasia, subluxation, and dislocation in cerebral palsy:
an arthrographic analysis. J Pediatr Orthop 11:488–493
Hermanson M, Hagglund G, Riad J, Rodby-Bousquet E,
Wagner P (2015a) Prediction of hip displacement in
children with cerebral palsy: development of the CPUP
hip score. Bone Joint J 97-B:1441–1444
Hermanson M, Hagglund G, Riad J, Wagner P (2015b)
Head-shaft angle is a risk factor for hip displacement in
children with cerebral palsy. Acta Orthop 86:229–232
Hodgkinson I, Vadot JP, Metton G, Berard C, Berard J
(2000) Prevalence and morbidity of hip excentration
in cerebral palsy: review of the literature. Revue de
chirurgie orthopedique et reparatrice de l'appareil
moteur 86:158–161
Hodgkinson I, Jindrich ML, Duhaut P, Vadot JP, Metton G,
Berard C (2001) Hip pain in 234 non-ambulatory ado-
lescents and young adults with cerebral palsy: a cross-
sectional multicentre study. Dev Med Child Neurol
43:806–808
Horstmann H, Mahboubi S (1987) The use of computed
tomography scan in unstable hip reconstruction.
J Comput Tomogr 11:364–369
Huh K, Rethlefsen SA, Wren TA, Kay RM (2011) Surgical
management of hip subluxation and dislocation in
Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy
children with cerebral palsy: isolated VDRO or com-
bined surgery? J Pediatr Orthop 31:858–863
Jung NH, Pereira B, Nehring I, Brix O, Bernius P,
Schroeder SA, Kluger GJ, Koehler T, Beyerlein A,
Weir S, von Kries R, Narayanan UG, Berweck S,
Mall V (2014) Does hip displacement influence
health-related quality of life in children with cerebral
palsy? Dev Neurorehabil 17:420–425
Kentish M, Wynter M, Snape N, Boyd R (2011) Five-year
outcome of state-wide hip surveillance of children and
adolescents with cerebral palsy. J Pediatr Rehabil Med
4:205–217
Kim HT, Wenger DR (1997) Location of acetabular defi-
ciency and associated hip dislocation in neuromuscular
hip dysplasia: three-dimensional computed tomo-
graphic analysis. J Pediatr Orthop 17:143–151
Mahboubi S, Horstmann H (1986) Femoral torsion: CT
measurement. Radiology 160:843–844
McHale KA, Bagg M, Nason SS (1990) Treatment of the
chronically dislocated hip in adolescents with cerebral
palsy with femoral head resection and subtrochanteric
valgus osteotomy. J Pediatr Orthop 10:504–509
Miller F, Bagg MR (1995) Age and migration percentage
as risk factors for progression in spastic hip disease.
Dev Med Child Neurol 37:449–455
Miller F, Liang Y, Merlo M, Harcke HT (1997) Measuring
anteversion and femoral neck-shaft angle in cerebral
palsy. Dev Med Child Neurol 39:113–118
Pons C, Remy-Neris O, Medee B, Brochard S (2013)
Validity and reliability of radiological methods to
assess proximal hip geometry in children with cerebral
palsy: a systematic review. Dev Med Child Neurol
55:1089–1102
13
Pruszczynski B, Sees J, Miller F (2015) Risk factors for hip
displacement in children with cerebral palsy: system-
atic review. J Pediatr Orthop 36:829
Reimers J (1980) The stability of the hip in children. A
radiological study of the results of muscle surgery in
cerebral palsy. Acta Orthop Scand Suppl 184:1–100
Rutz E, Passmore E, Baker R, Graham HK (2012) Multi-
level surgery improves gait in spastic hemiplegia but
does not resolve hip dysplasia. Clin Orthop Relat Res
470:1294–1302
Silver RL, Rang M, Chan J, de la Garza J (1985) Adductor
release in nonambulant children with cerebral palsy.
J Pediatr Orthop 5:672–677
Smigovec I, Ethapic T, Trkulja V (2014) Ultrasound
screening for decentered hips in children with severe
cerebral palsy: a preliminary evaluation. Pediatr Radiol
44(9):1101
Terjesen T, Lofterod B, Myklebust G (2004) Orthopaedic
problems in adults with cerebral palsy. Tidsskr Nor
Laegeforen 124:156–159
Wynter M, Gibson N, Kentish M, Love S, Thomason P,
Kerr Graham H (2011) The development of Australian
standards of care for hip surveillance in children with
cerebral palsy: how did we reach consensus? J Pediatr
Rehabil Med 4:171–182
Wynter M, Gibson N, Willoughby KL, Love S, Kentish M,
Thomason P, Graham HK, Group National Hip Sur-
veillance Working (2015) Australian hip surveillance
guidelines for children with cerebral palsy: 5-year
review. Dev Med Child Neurol 57:808–820
Zimmermann SE, Sturm PF (1992) Computed tomo-
graphic assessment of shelf acetabuloplasty. J Pediatr
Orthop 12(5):581