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J Pediatr Orthop Volume 38, Number 6 Supplement 1, July 2018 www.pedorthopaedics.com | S21
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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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.
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. www.pedorthopaedics.com | S23
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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%.
Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved. www.pedorthopaedics.com | S25
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
S26 | www.pedorthopaedics.com Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
impairment, has improved our understanding of the 21. Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition
epidemiology and natural history of CPHD; and improved and classification of cerebral palsy April 2006. Dev Med Child Neurol
Suppl. 2007;109:8–14.
the assessment of technical domain and HRQOL outcomes 22. Hagglund G, Lauge-Pedersen H, Persson M. Radiographic threshold
following surgery. Development and implementation of hip values for hip screening in cerebral palsy. J Child Orthop. 2007;1:43–47.
surveillance programs for CPHD leads to earlier diagnosis 23. Shore B, Spence D, Graham H. The role for hip surveillance in
and treatment, resulting in better outcomes. Implementation children with cerebral palsy. Curr Rev Musculoskelet Med. 2012;5:
of hip surveillance programs in resource poor and decen- 126–134.
24. Willoughby KL, Graham HK. Early radiographic surveillance is
tralized health care delivery systems is challenging and will needed to prevent sequelae of neglected hip displacement in cerebral
require innovating thinking and processes. palsy. BMJ. 2012;345:e6675. Author reply e9. [Epub 11 October, 2012].
25. Wynter M, Gibson N, Willoughby KL, et al. Australian hip
REFERENCES surveillance guidelines for children with cerebral palsy: 5-year
1. Brunner R, Picard C, Robb J. Morphology of the acetabulum in hip review. Dev Med Child Neurol. 2015;57:808–820.
dislocations caused by cerebral palsy. J Pediatr Orthop B. 1997;6: 26. Givon U. Management of the spastic hip in cerebral palsy. Curr Opin
207–211. Pediatr. 2017;29:65–69.
2. Cornell MS, Boyd R, Baird G, et al. Imaging the acetabulum in 27. Hagglund G, Alriksson-Schmidt A, Lauge-Pedersen H, et al.
children with cerebral palsy. J Bone Joint Surg Br. 1994;76:982–983. Prevention of dislocation of the hip in children with cerebral palsy:
3. Davids JR, Gibson TW, Pugh LI, et al. Proximal femoral geometry 20-year results of a population-based prevention programme. Bone
before and after varus rotational osteotomy in children with cerebral palsy Joint J. 2014;96-B:1546–1552.
and neuromuscular hip dysplasia. J Pediatr Orthop. 2013;33:182–189. 28. Hagglund G, Andersson S, Duppe H, et al. Prevention of dislocation
4. Abel MF, Wenger DR, Mubarak SJ, et al. Quantitative analysis of of the hip in children with cerebral palsy. The first ten years of a
hip dysplasia in cerebral palsy: a study of radiographs and 3-D population-based prevention programme. J Bone Joint Surg Br. 2005;87:
reformatted images. J Pediatr Orthop. 1994;14:283–289. 95–101.
5. Soo B, Howard JJ, Boyd RN, et al. Hip displacement in cerebral 29. Abousamra O, Er MS, Rogers KJ, et al. Hip reconstruction in
palsy. J Bone Joint Surg Am. 2006;88:121–129. children with unilateral cerebral palsy and hip dysplasia. J Pediatr
6. Alriksson-Schmidt A, Hagglund G. Pain in children and adolescents Orthop. 2016;36:834–840.
with cerebral palsy: a population-based registry study. Acta Paediatr. 30. Hanna SE, Rosenbaum PL, Bartlett DJ, et al. Stability and decline in
2016;105:665–670. gross motor function among children and youth with cerebral palsy
7. DiFazio R, Shore B, Vessey JA, et al. Effect of hip reconstructive aged 2 to 21 years. Dev Med Child Neurol. 2009;51:295–302.
surgery on health-related quality of life of non-ambulatory children 31. Shore BJ, Yu X, Desai S, et al. Adductor surgery to prevent hip
with cerebral palsy. J Bone Joint Surg Am. 2016;98:1190–1198. displacement in children with cerebral palsy: the predictive role of the
8. Difazio RL, Vessey JA, Zurakowski D, et al. Differences in health- Gross Motor Function Classification System. J Bone Joint Surg Am.
related quality of life and caregiver burden after hip and spine 2012;94:326–334.
surgery in non-ambulatory children with severe cerebral palsy. Dev 32. Mubarak SJ, Valencia FG, Wenger DR. One-stage correction of the
Med Child Neurol. 2016;58:298–305. spastic dislocated hip. Use of pericapsular acetabuloplasty to
9. McNerney NP, Mubarak SJ, Wenger DR. One-stage correction of improve coverage. J Bone Joint Surg Am. 1992;74:1347–1357.
the dysplastic hip in cerebral palsy with the San Diego acetabulo- 33. Zhang S, Wilson NC, Mackey AH, et al. Radiological outcome of
plasty: results and complications in 104 hips. J Pediatr Orthop. reconstructive hip surgery in children with gross motor function
2000;20:93–103. classification system IV and V cerebral palsy. J Pediatr Orthop B.
10. Valencia FG. Management of hip deformities in cerebral palsy. 2014;23:430–434.
Orthop Clin North Am. 2010;41:549–559. 34. Chang FM, Ma J, Pan Z, et al. Acetabular remodeling after a varus
11. Stasikelis PJ, Davids JR, Johnson BH, et al. Rehabilitation after femoral derotational osteotomy in children with cerebral palsy. J Pediatr
osteotomy in cerebral palsy. J Pediatr Orthop B. 2003;12:311–314. Orthop. 2016;36:198–204.
12. Stasikelis PJ, Lee DD, Sullivan CM. Complications of osteotomies in 35. Chang FM, May A, Faulk LW, et al. Outcomes of isolated varus
severe cerebral palsy. J Pediatr Orthop. 1999;19:207–210. derotational osteotomy in children with cerebral palsy hip dysplasia and
13. Albinana J, Dolan LA, Spratt KF, et al. Acetabular dysplasia after predictors of resubluxation. J Pediatr Orthop. 2016. [Epub ahead of print].
treatment for developmental dysplasia of the hip. Implications for 36. Huh K, Rethlefsen SA, Wren TA, et al. Surgical management of hip
secondary procedures. J Bone Joint Surg Br. 2004;86:876–886. subluxation and dislocation in children with cerebral palsy: isolated
14. Malvitz TA, Weinstein SL. Closed reduction for congenital dysplasia VDRO or combined surgery? J Pediatr Orthop. 2011;31:858–863.
of the hip. Functional and radiographic results after an average of 37. Narayanan UG, Fehlings D, Weir S, et al. Initial development and
thirty years. J Bone Joint Surg Am. 1994;76:1777–1792. validation of the Caregiver Priorities and Child Health Index of Life with
15. Brunner R, Baumann JU. Long-term effects of intertrochanteric Disabilities (CPCHILD). Dev Med Child Neurol. 2006;48:804–812.
varus-derotation osteotomy on femur and acetabulum in spastic 38. Root L. Surgical treatment for hip pain in the adult cerebral palsy
cerebral palsy: an 11- to 18-year follow-up study. J Pediatr Orthop. patient. Dev Med Child Neurol. 2009;51(suppl 4):84–91.
1997;17:585–591. 39. Graham HK, Boyd R, Carlin JB, et al. Does botulinum toxin a
16. Rutz E, Vavken P, Camathias C, et al. Long-term results and combined with bracing prevent hip displacement in children with
outcome predictors in one-stage hip reconstruction in children with cerebral palsy and “hips at risk”? A randomized, controlled trial.
cerebral palsy. J Bone Joint Surg Am. 2015;97:500–506. J Bone Joint Surg Am. 2008;90:23–33.
17. Lee WC, Kao HK, Yang WE, et al. Guided growth of the proximal 40. Robin J, Graham HK, Baker R, et al. A classification system for hip
femur for hip displacement in children with cerebral palsy. J Pediatr disease in cerebral palsy. Dev Med Child Neurol. 2009;51:183–192.
Orthop. 2016;36:511–515. 41. Apple. CPUP. 2016. Available at: https://itunes.apple.com/us/app/
18. McCarthy JJ, Noonan KJ, Nemke B, et al. Guided growth of the cpup-hip-score. Accessed June 6, 2017.
proximal femur: a pilot study in the lamb model. J Pediatr Orthop. 42. Hermanson M, Hagglund G, Riad J, et al. Prediction of hip
2010;30:690–694. displacement in children with cerebral palsy: development of the
19. Torode IP, Young JL. Caput valgum associated with developmental CPUP hip score. Bone Joint J. 2015;97-B:1441–1444.
dysplasia of the hip: management by transphyseal screw fixation. 43. Hagglund G. CPUP. 2016. Available at: https://play.google.com/store/
J Child Orthop. 2015;9:371–379. apps/details?id=com.appinmed.clinic.cpup&hl=en. Accessed September
20. Palisano RJ, Rosenbaum P, Bartlett D, et al. Content validity of the 6, 2017.
expanded and revised Gross Motor Function Classification System. 44. Kulkarni VA. Hipscreen. 2016. Available at: www.hipscreen.org/.
Dev Med Child Neurol. 2008;50:744–750. Accessed September 6, 2017.
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