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SUPPLEMENT

Management of Neuromuscular Hip Dysplasia in Children


With Cerebral Palsy: Lessons and Challenges
Jon R. Davids, MD

muscle imbalance (ie, dominance of the hip flexors and


Abstract: Optimal clinical decision making and surgical manage- adductors over the hip extensors and abductors), occurring
ment of hip dysplasia in children with cerebral palsy (CP) requires in the setting of the growing hip, results in distinct pathoa-
an understanding of the underlying pathophysiology (patho- natomy of both the proximal femur and acetabulum.1–4 The
mechanics and pathoanatomy), incidence, and natural history. The incidence of CPHD is directly related to the degree of motor
incidence of hip dysplasia in children with CP is directly related to impairment in children with cerebral palsy (CP).5 A sub-
the degree of motor impairment. A subluxated or dislocated hip in luxated or dislocated hip in a child with CP can compromise
a child with CP can compromise the quality of life for both the the quality of life of both the child and their caregivers.6–8
child and their caregivers. The goal of this article is to highlight the The goal of this article is to highlight the events over
events over the last 25 years that have had the greatest impact on the last 25 years that have had the greatest impact on the
the management of hip dysplasia in children with CP. It is my clinical and surgical management of CPHD. Looking
opinion that the 2 most significant advances during this time have back, it is my opinion that the 2 most significant advances
been the development of a classification system based upon motor during this time have been the development of a classi-
impairment (the Gross Motor Function Classification System), and fication system based upon motor impairment (the Gross
the development of surveillance programs for hip dysplasia in Motor Function Classification System—GMFCS), and
children with CP. This article will contrast neuromuscular hip the development of hip surveillance programs for CPHD.
dysplasia with developmental dysplasia of the hip. It will be shown
how the development and utilization of the Gross Motor Function
Classification System has contributed to our understanding of the THE SAN DIEGO APPROACH
epidemiology and natural history of hip dysplasia in children with By the early 1990s, surgically oriented pediatric or-
CP, and to the assessment of outcomes following surgical man- thopaedic surgeons in North America generally believed
agement. The impact of hip surveillance programs on early soft that early soft tissue surgery (ie, hip adductor and flexor
tissue surgeries, skeletal hip reconstructions, and the incidence of muscle lengthening) was unpredictable and frequently in-
hip dislocations and salvage surgeries will be reviewed. Challenges effective in the management of CPHD, and that a single
in the implementation of hip surveillance programs in resource event, comprehensive surgical hip reconstruction (including
poor and decentralized health care delivery systems will be con- hip adductor and flexor muscle lengthening; proximal fem-
sidered, and innovative approaches identified. oral varus and rotation osteotomy—VRO; acetabular os-
teotomy as described by Dega; and open reduction with
Key Words: cerebral palsy, hip dysplasia, Gross Motor Function capsulorraphy), when done at the proper age (between 6 and
Classification System, hip surveillance 8 y of age) would be definitive (ie, no recurrence of sub-
(J Pediatr Orthop 2018;38:S21–S28) luxation or dislocation).9,10 This approach was developed at
a center with significant technical expertise in the surgical
management of developmental dysplasia of the hip (DDH),
and a commitment to caring for children with CP. It was
O ptimal clinical decision making and surgical man-
agement of hip dysplasia in children with cerebral
palsy (CPHD) requires an understanding of the underlying
this approach that guided my clinical decision making and
surgical management of CPHD in the early part of my
career (Fig. 1). The early results were generally successful,
pathophysiology (pathomechanics and pathoanatomy),
and seemed to be consistent with my training and validate
incidence, and natural history. The underlying dynamic
the paradigm. However, with time there were a few poor
outcomes, which appeared to be related to recurrence of
From the Shriners Hospitals for Children Northern California, Sacramento, CPHD with growth, particularly in younger children with
CA. more severe CP.11,12 Critical analysis of these cases, and
The author did not receive any outside funding or grants in support of advances in the understanding of CPHD from centers
their research for or in preparation of this work.
The author declares no conflicts of interest.
around the world taught me the following lessons.
Reprints: Jon R. Davids, MD, Shriners Hospitals for Children—NCA, 2425
Stockton Boulevard, Sacramento, CA 95817. E-mail: jdavids@shrinenet.
org.
LESSONS LEARNED: PATHOPHYSIOLOGY
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. It is now clear that the pathophysiology of CPHD is
DOI: 10.1097/BPO.0000000000001159 distinct from that associated with DDH. In CPHD there

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Davids J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018

FIGURE 1. Pelvis radiographs of a 6-year-old boy with cerebral palsy, Gross Motor Function Classification System IV, and hip
dysplasia in children with cerebral palsy, who underwent comprehensive, single stage surgical hip reconstructions. A, Preoperative
radiograph showing bilateral hip subluxation. B, Immediate postoperative radiograph after bilateral proximal femoral varus and
rotation osteotomies and Dega acetabulopasties. Open reduction was not required. C, Pelvis radiograph 1 year following surgery,
before proximal femoral hardware removal and drill hemiepiphyseodesis. D, Pelvis radiograph 4 years following surgery. Skeletal
hypercontainment of both hips has been maintained.

are rarely soft tissue obstacles to reduction (which are seen that occurs by performing both femoral and pelvic osteotomies,
more commonly in DDH). Even in cases of extreme obviating the need for capsulorraphy.
subluxation (ie, migration percentage—MP, up to 100%), The other significant pathophysiological difference
as long as a portion of the femoral head is in contact with between CPHD and DDH relates to the role of growth
the margin of the acetabulum, there will not be soft tissue following surgical reconstruction of the hip. In DDH,
obstacles to reduction of the femoral head within the subsequent growth, particularly acetabular modeling, is a
acetabulum following combined femoral and acetabular necessary element for achieving a good long-term outcome.13,14
osteotomies. Open reduction to remove soft tissue ob- Subsequent growth is the surgeon’s friend in the management
stacles is only necessary in cases with complete dislocation of DDH. However, in CPHD, the underlying muscle im-
where the femoral head has migrated proximally and has balance described above usually persists, even after soft tissue
no contact with the acetabulum (ie, MP > 100%). In such or skeletal surgeries, and subsequent growth, particularly of the
cases the interposed iliopsoas tendon, and less frequently a proximal femur, has been shown to lead to recurrent deformity,
contracted capsule, are the soft tissue structures that need which may contribute to recurrent subluxation.15,16 The po-
to be addressed to facilitate reduction of the femoral head tential for acetabular modeling in CPHD is less well under-
within the acetabulum. The ligamentum teres may be de- stood, and will be discussed below. In general, subsequent
tached from the femoral head and atrophied, significant growth is not the surgeon’s friend in the management of
pulvinar is infrequent, and tightness of the transverse CPHD. Recent animal and clinical studies suggest that there
acetabular ligament is rare. Stability following open re- may be a role for guided growth of the proximal femoral physis
duction is best achieved by the skeletal hypercontainment to either prevent deformity early in the disease process or

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J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018 Management of Neuromuscular Hip Dysplasia

recurrence of deformity following VRO.17–19 Further study is children with CP, occurring in 69% and 89% of children at
required before this procedure can be recommended with the GMFCS levels IV and V, respectively. Outcomes
confidence. following surgical management of CPHD are improved
when performed earlier in the course of the disease process,
supporting efforts at early diagnosis in those groups at the
LESSON LEARNED: THE GMFCS greatest risk for developing CPHD.23,24,26–28 Interestingly,
Development and utilization of the GMFCS, a CPHD also occurs in 15% of children functioning at the
classification scheme based upon motor impairment level, high motor levels (GMFCS I and II).5 Recent work has
has contributed greatly to our understanding of the identified a subset of children with hemiplegic type CP, with
epidemiology and natural history of CPHD, and to the gait kinematic deviations about the hip who are at risk for
assessment of outcomes following surgical management CPHD.6,13,29 Appropriately timed assessment for CPHD in
(Fig. 2).20,21 In children with CP, hips with MP > 30% are this group of children is therefore recommended.
at risk for the development of CPHD.22–25 Population- Understanding of the natural history of CPHD has
based studies have shown that the incidence of CPHD previously been focused upon the presence or absence of
(defined as MP > 30%) in children with CP is directly pain associated with severe subluxated or dislocated hips in
correlated with the degree of motor function impairment adults with CP.6,13 This literature is inconsistent, due in part
as measured by the GMFCS.5 CPHD is very common in to a lack of tools to accurately measure relevant clinical

FIGURE 2. Descriptors and illustrations of the levels of the Gross Motor Function Classification System (GMFCS). Reproduced with
permission from H. Kerr Graham. Copyright [H. Kerr Graham], [Royal Children's Hospital, Melbourne, AU]. All permission requests
for this image should be made to the copyright holder.

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Davids J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018

unfortunately those that need it the most (ie, children at


GMFCS IV and V) benefit the least from early soft tissue
surgery for CPHD. Despite these poor results, I still offer
early soft tissue surgery to children at the GMFCS IV and
V levels as the improved hip range of motion achieved by
the surgery facilitates diapering and perineal hygiene. In
addition, this relatively simple surgery provides an op-
portunity for the family and child to experience anesthesia,
surgery, and recovery, which can help in deciding whether
to undergo the more rigorous skeletal hip reconstruction
surgery in the future.
Outcomes following skeletal hip reconstruction sur-
gery for CPHD are best understood through the filter of
the GMFCS. Early reports, that did not classify patients
FIGURE 3. Graph of change in motor function [as measured by motor functional level, described 95% success rates (ie,
by the Gross Motor Function Measure (GMFM-66)], by age, in maintenance of reduction, no further surgery required)
children with cerebral palsy segregated by Gross Motor Func- following skeletal hip reconstruction at 7.5 years follow-
tion Classification System (GMFCS) level. The plots (solid lines) up.9,32 A subsequent study, which classified patients by
show that all children with cerebral palsy, regardless of GMFCS GMFCS level, noted a 75% success rate in GMFCS IV
level, show improvement in gross motor up to 7 years of age. and V patients at 5 years of follow-up.33 Several recent
For children at the higher functional GMFCS levels, gross reports evaluating acetabular response following isolated
motor function level is maintained into young adult life (solid VRO for CPHD have shown that measurable remodeling
lines within solid circle). For children at the lower functional does occur, more commonly in GMFCS II and III pa-
GMFCS levels, gross motor function level deteriorates
throughout the second decade of life (solid and dashed lines
tients, but rarely in GMFCS IV and V patients.34–36
within dashed circle). Modified with permission from Hanna Combining this information with previous studies doc-
et al.30 Copyright [Wiley and Sons], [San Francisco, CA]. All umenting recurrent femoral deformity with growth fol-
permission requests for this image should be made to the lowing VRO for CPHD supports the surgical paradigm of
copyright holder. *Indicates GMFCS levels in which motor skeletal hypercontainment with combined femoral and
function deteriorates during the second decade of life. pelvic osteotomies for CPHD in GMFCS IV and V pa-
tients, and suggests that a second skeletal reconstruction
status and outcomes. The most reasonable interpretation of may be necessary when hip reconstructive surgery is re-
these studies is that the longer a subject survives, the greater quired in younger children (ie, between 4 and 6 y of age)
the likelihood of pain associated with a subluxated or dis- with severe CP.
located hip. However, the impact of CPHD on motor Assessment of health-related quality of life (HRQOL)
function (which is a significant element of quality of life) in in children with severe CP (GMFCS IV and V) has been
subjects with CP can be seen when Gross Motor Function improved by the development of tools such as the Caregiver
Measure scores relative to age are evaluated with the sub- Priorities and Child Health Index of Life with Disabilities
jects segregated by GMFCS level (Fig. 3).30 Motor function (CPCHILD).37 A recent study of children severe CP under-
improves across all GMFCS levels up to 7 years of age. For going hip reconstruction surgery found a negative correlation
those children functioning at the highest motor levels (ie, between MP and CPCHILD scores both preoperatively and
GMFCS I and II), motor function level remains stable into postoperatively, with improved CPCHILD scores following
young adulthood. For those at the GMFCS III, IV, and V surgery.7 These finding suggest that CPHD negatively im-
levels there is deterioration of motor function level pacts HRQOL in children with CP, and that HRQOL is
beginning in the teenage years and progressing into young improved following hip reconstructive surgery.
adulthood. The distribution of these changes across The GMFCS can provide clinical decision making
GMFCS levels closely mirrors the incidence of CPHD guidelines for the management of CPHD based upon the
between GMFCS levels, suggesting that CPHD may be a subject’s motor impairment level. CPHD is rare at
contributing factor to the deterioration of motor function in GMFCS I and II levels, but when identified early should
teenagers and young adults with CP. be managed by soft tissue surgery. Cases identified later
The GMFCS has also contributed to improved un- should be managed by skeletal reconstruction with VRO;
derstanding of technical domain outcomes following the acetabular osteoteomy is indicated when significant dys-
surgical management of CPHD. The effectiveness of early plasia is present. Excellent outcomes should be antici-
soft tissue surgery (lengthening of the hip adductor and pated. CPHD in children at the GMFCS III and IV levels
flexor muscles) has been shown to be related to the degree should be managed by early soft tissue surgery, with the
of motor impairment present at the time of surgery, with family counseled that subsequent skeletal reconstruction
the successful outcomes (ie, maintenance of MP <30%) surgery will be likely. In cases where hip reconstruction
seen in 94% of children at GMFCS level II, 49% at is required at a relatively young age, a second skeletal
GMFCS level III, 27% at GMFCS level IV, and only 14% reconstruction may be required in the future due to re-
at GMFCS level V.31 As noted by the investigators, currence of femoral deformity with growth. Management

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J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018 Management of Neuromuscular Hip Dysplasia

of CPHD in children with the most severe level of motor implementation of hip surveillance results in a switch from
impairment (GMFCS V) requires preliminary moral and a reactive to proactive treatment paradigm, which is
ethical discussion of proactive versus reactive treatment beneficial from a population perspective and presumably
strategies with the family, due to significant medical co- cost effective from a system perspective.
morbidities that increase the risk of perisurgical compli- Australia and Sweden both have comprehensive,
cations and shorten anticipated lifespan. When a proactive centralized health care delivery systems, which facilitate
strategy is selected, management of CPHD is similar to the implementation and utilization of hip surveillance for
that described above for GMFCS III and IV levels. When CPHD in children with CP. The program may be deliv-
a reactive strategy is selective, soft tissue release for range ered by trained professionals working at tertiary referral
of motion and palliative skeletal salvage surgeries are centers where children with CP are concentrated to receive
utilized.38 specialized care and services; or mandated centrally and
implemented in regional centers designated to provide
health care services to children with CP. Implementation
LESSONS LEARNED: HIP SURVEILLANCE in countries with limited resources, or with decentralized,
Systematic hip surveillance programs designed to market driven health care delivery systems is challenging.
promote early detection and treatment of CPHD in chil- In centralized systems, change is general initiated from
dren with CP have been developed in Australia and above, mandating the implementation below. In decen-
Sweden.24,25,27,28 These programs have established that tralized systems, change is frequently initiated from below.
hips with MP > 30% are at risk for progressive sub- In this circumstance, any advocate for the child (eg, pri-
luxation, that the incidence of CPHD is related to the mary care provider, physical therapist, or parent) may
degree of motor impairment as described by the GMFCS, promote hip surveillance.
that the skeletal pathoanatomy of CPHD is related to the Several smartphone apps have been developed to
degree of motor impairment as described by the GMFCS, educate and empower such advocates. The CPUP Hip
and that botulinum toxin injection into the hip adductor Score, developed in Sweden, predicts the risk of develop-
and flexor muscle groups is not effective in preventing ing progressive CPHD, defined as an MP > 40%, within
progression of CPHD.5,22,39,40 Implementation of com- 5 years.41–43 The CPUP Hip Score is calculated using 4
prehensive hip surveillance results in an increase in early variables: the subject’s GMFCS level, age, and 2 x-ray
soft tissue surgeries and skeletal hip reconstructions, and measures of hip dysplasia (MP; and head shaft angle)
decreases the incidence of hip dislocations and salvage (Fig. 4). The hipscreen app, developed in the United States,
surgeries after 10 to 20 years of screening.27,28 The provides educational materials about hip surveillance,

FIGURE 4. CPUP Hip Score. A, Traditional measurement of the migration percentage (MP) on an anteroposterior radiograph of the
pelvis. The horizontal black dotted line is drawn through the triradiate cartilage of both hips. The vertical white line is drawn at the
lateral margin of the femoral head. The vertical black line is drawn at the medial margin of the femoral head. The vertical black
dashed line is drawn at the lateral margin of the acetabulum. The MP equals A/B×100. The MP of the right hip is 47%. The MP of
the left hip is 23%. B, Anteroposterior radiograph of the left hip in a 6-year-old boy with cerebral palsy Gross Motor Function
Classification System level IV, showing the measurement of the head shaft angle (angle indicated by dashed line), which is one of
the predictor variables for the CPUP calculator. C, Example of use of CPUP to predict the risk of progressive hip dysplasia in a child
with cerebral palsy. In this example, the child's Gross Motor Function Classification System level is IV, the head shaft angle is 170
degrees, MP is 34%, and the age is 6 years. The risk of developing progressive hip dysplasia within 5 years is 20% to 30%.

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Davids J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018

FIGURE 5. The hipscreen app. A, The smartphone camera is used to take a photograph of the anteroposterior pelvis radiograph.
The rotate function is used to align the image. B, The pan and zoom functions are used to align the hip with the calculator grid. C,
When the lateral margin of the femoral head is aligned with the white line (solid white arrow), and the medial margin of the
femoral head is aligned with the black line (solid black arrow), the head is divided into 10% increments (vertical gray lines). The
location of lateral margin of the acetabulum is assessed (gray arrow), and a numerical value, corresponding to the MP, is
determined. If the lateral margin falls within the black box (as seen in this example), the hip is at risk or in need of treatment and
referral to the appropriate physician or center is indicated.

proper techniques for obtaining and measuring x-rays of the manipulated using the pan and zoom functions. The ruler
hips, definitions of GMFCS levels, and summaries of all function contains guidelines for continued surveillance or
published hip surveillance programs for CPHD.44 The app referral to a treatment center based upon the calculated MP.
provides a specific, detailed hip surveillance schedule based
upon age and GMFCS level, to guide providers and SUMMARY
advocates that can be applied to individual subjects. The Management of the hip in children with CP over the
final and most innovative element of the hipscreen app is the last 25 years has been enhanced by improved understand of
Migration Percentage Ruler, which facilitates calculation of the pathophysiology and pathoanatomy of CPHD. Devel-
the MP from a pelvis x-ray (Fig. 5). The smartphone camera opment and utilization of the GMFCS, a classification
is used to photograph the x-ray, which is then aligned and system for children with CP, based upon degree of motor

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J Pediatr Orthop  Volume 38, Number 6 Supplement 1, July 2018 Management of Neuromuscular Hip Dysplasia

impairment, has improved our understanding of the 21. Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition
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25. Wynter M, Gibson N, Willoughby KL, et al. Australian hip
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