You are on page 1of 8

Pediatric Radiology (2019) 49:1587–1594

https://doi.org/10.1007/s00247-019-04519-w

MUSCULOSKELETAL IMAGING

Cerebral palsy — beyond hip deformities


Jeffrey P. Otjen 1 & Ted C. Sousa 2 & Jennifer M. Bauer 2 & Mahesh Thapa 1

Received: 4 July 2019 / Revised: 29 July 2019 / Accepted: 28 August 2019 / Published online: 24 October 2019
# Springer-Verlag GmbH Germany, part of Springer Nature 2019

Abstract
Cerebral palsy is a neurologic condition with myriad musculoskeletal and articular manifestations. While every patient is unique
with innumerable variations in presentation, symptoms and treatments, there are broad themes and recognizable patterns of
development. Many of these findings spill over to other neurodevelopmental disorders, and lessons learned from children with
cerebral palsy translate well to multiple neurologic conditions. This review focuses on the more common manifestations involv-
ing the spine, knee, foot and ankle, with an emphasis on collecting and describing imaging features, along with clinical and
radiologic pearls and pitfalls.

Keywords Cerebral palsy . Children . Feet . Lower extremity . Musculoskeletal . Radiography . Spine

Introduction significant foot deformities. Spasticity is the most common


tone abnormality, often develops over time, and most com-
Cerebral palsy is a central nervous system abnormality that monly affects the paraspinal muscles, hip flexors and adduc-
affects 1–5 per 1,000 live births [1, 2]. The most common risk tors, hamstrings and calf musculature [2], though upper ex-
factors are prematurity and low birth weight, but any prenatal tremities are also frequently affected.
or perinatal insult can have this affect [2], including traumatic, Radiographic evaluation and monitoring are important parts
hypoxic, infectious and genetic. Although the underlying en- of the management of these children. This review covers com-
cephalopathy is static, the downstream muscular abnormality mon skeletal abnormalities of the spine, knees and feet associ-
can cause a variety of progressive musculoskeletal and gait ated with cerebral palsy, excluding the large topic of hip abnor-
disturbances [3–5]. There is heterogeneous presentation with a malities. Because the manifestations of cerebral palsy are fre-
variable degree of muscle weakness, spasticity, contractures quently shared across other neuromuscular disorders such as
and impaired sensation. Cerebral palsy is anatomically cate- myelomeningocele, spinal muscular atrophy, Charcot–Marie–
gorized with respect to the affected area, for example diplegia Tooth and the muscular dystrophies [7], an understanding of the
(affecting two limbs, usually legs, symmetrically), quadriple- pathology, imaging and treatment is widely applicable.
gia (synonymous with tetraplegia — affecting all four limbs)
and hemiplegia (affecting one side). Individuals can also be
grouped using the Gross Motor Functional Classification
System (GMFCS), which focuses on functional impairment Spine
[6]. GMFCS can be more prognostic with regard to develop-
ment of deformities such as scoliosis, hip dysplasia and Ideally spine radiographs should be in a standing or upright
position and acquired in similar position to prior examinations
to optimize comparability.
* Mahesh Thapa In contrast to the S-shape of idiopathic scoliosis, cerebral-
thapamd@uw.edu palsy-related neuromuscular scoliosis tends to have a broad C-
1
shape and is more frequently left convex [8] (Fig. 1). Scoliosis
Department of Radiology, Seattle Children’s Hospital,
University of Washington, Mail Stop MA.7.220,
is more common in males with cerebral palsy (in contrast to
4800 Sand Point Way NE, Seattle, WA 98105, USA idiopathic scoliosis), with an overall prevalence of 15–61%,
2
Department of Orthopedic Surgery,
and the degree of curvature varies highly [2] but can be severe.
Seattle Children’s Hospital, University of Washington, Curve progression can be increased by the onset of pubertal
Seattle, WA, USA growth spurt, deteriorating neurologic function or decreased
1588 Pediatr Radiol (2019) 49:1587–1594

Fig. 1 Extreme scoliosis. Anteroposterior supine spine radiograph in a


10-year-old girl with cerebral palsy shows extreme scoliosis. Note the C-
shape and the rotatory component, with the lumbar spine in near lateral
projection. Hip dysplasia is also present

ambulation. More severely affected cerebral palsy patients, as


measured by the GMFCS, tend to suffer from more spinal
deformity progression than those less affected because of the
degree of abnormal motor coordination [9]. Once skeletally
mature, larger curves progress more rapidly than smaller ones,
and curves tend to be more severe in non-ambulatory children. Fig. 2 Pelvic obliquity. Anteroposterior upright spine radiograph in a 17-
Although C-shape spinal curves are more frequently seen in year-old boy with cerebral palsy shows pelvic obliquity and shortening of
the distance between ribs and the relatively elevated right iliac crest
cerebral palsy, a child with enough head control might posi-
tion his or her head over her pelvis and develop a compensa-
deformity and restricted lung function and drive a decision
tory spinal curve, leading to a more S-shape curvature (Fig. 2).
for surgery. These larger curves, particularly with pelvic obliq-
Lateral spinal radiographs should be evaluated for excessive
uity, can also cause discomfort while sitting in a chair for long
thoracic kyphosis and lumbar lordosis.
periods or inability to remain in an upright position to interact
Radiographs can also measure the angle of pelvic obliquity,
with the environment, and these problems are other important
a contributor to these processes, as the angle a line across the
iliac crests makes with respect to horizontal on an upright film
(Fig. 2). Severe pelvic obliquity can be a cause of difficulty
sitting and soft-tissue pressure injury. A short distance be-
tween the lower ribs and the iliac crests or frank impaction
between these structures also denotes more severe deformity
and a potential source of pain. Bending or traction films can
help to determine the current flexibility as well as the amount
of surgical correction likely to be needed (Fig. 3), and both
curve reduction and any change in rotational component or
“unwinding” of the spine should be noted.
Young patients prior to triradiate cartilage closure and those
with surgical contraindications can be treated with soft bracing
and seating support, which can slow but not stop curve pro-
gression [10] and is less effective than the bracing commonly
used in idiopathic scoliosis. A decision for spinal fusion is
complex and multifactorial. The magnitude of the curve is
Fig. 3 Traction films. Supine neutral (a) and traction (b) anteroposterior
an important aspect because curves over 50° are likely to
spine radiographs in a 5-year-old girl (the same patient as in Fig. 1). Note
continue to progress no matter the patient’s age. Larger curves the lessening of the curve with traction (b). A mild rotatory component
might cause respiratory compromise from chest wall does not significantly change
Pediatr Radiol (2019) 49:1587–1594 1589

surgical indications. The median surgical curve magnitude


range for neuromuscular scoliosis is 70–80° [11].
For skeletally mature patients, definitive fusion prevents
further curve progression. Fusion hardware frequently extends
to the high thoracic region in its cranial extent. The caudal
extent might include pelvic fixation because this is the best
way to control pelvic obliquity, but a surgical decision might
be made to stop in the lumbar spine instead, based on curve
characteristic and clinical status [1, 8, 10, 12].
Severe scoliosis or rapidly progressive scoliosis before ap-
proaching the age of triradiate cartilage closure can require
surgical intervention and growing spinal rods might be used
[10] (Fig. 4). As with growing constructs placed in other pa-
tient populations, complications such as infection, hardware
fracture or loosening are seen, and more commonly so in this
population [10] (Fig. 5).
Fig. 5 Hardware complications. Lateral spine radiograph in a 12-year-old
Low back pain can inhibit ambulation and degrade quality boy shows a complication of hardware placement, with a fractured and
of life, and is common in children with cerebral palsy [13]. displace inferior rod
Children with cerebral palsy are also at risk for increased
lumbar lordosis (Fig. 6), leading to increased shear stress on Knee
the posterior elements of the lower lumbar spine and potential
pars defects and spondylolisthesis [2, 13]. Hamstring spasticity is associated with flexion contrac-
As a final note, non-orthopedic issues such as constipation tures, the most common knee abnormality seen in cerebral
could be present and cause pain. While radiographs cannot palsy. This leads to a characteristic “crouch knee” defor-
diagnose constipation and radiographic stool burden is not mity while standing (Fig. 7) and can cause difficulties
associated with constipation [14, 15], trends in degree of stool with sitting if fixed greater than 90°. Chronic knee flexion
burden in a child clinically diagnosed with constipation might causes over-stretching of the quadriceps muscles and knee
be a helpful guide to manage therapy. When considering the extensor mechanism, which leads to patella alta. This is
cause of pain and discomfort in a non-verbal child with cere- seen in 58–72% of children with spastic cerebral palsy
bral palsy, constipation should be ruled out prior to ascribing
the pain to scoliosis.

Fig. 6 Lumbar lordosis. Lateral standing lumbar spine radiograph in a


Fig. 4 Expandable rods. Anteroposterior upright spine radiograph in a 6- 20-year-old woman with cerebral palsy and low back pain shows
year-old girl after placement of posterior expandable growing rods exaggerated lordosis, but no current spondylolisthesis
1590 Pediatr Radiol (2019) 49:1587–1594

Fig. 7 Crouch knee deformity.


Right side (a) and posterior (b)
photographs of a 9-year-old girl
with tetraplegic cerebral palsy
with asymmetrical limb
involvement. Note the crouch
stance caused by flexion at the
hips and knees. The feet show
planovalgus, with right-greater-
than-left hindfoot and ankle
equinus with a heel lift, or toe
walk

[2]. Although there are many variations on technique to Variable combinations of spasticity, weakness and muscle
measure patella alta, the most common is the Insall– control can lead to other alignment problems, such as genu
Salvati ratio: the craniocaudal length of the patellar tendon recurvatum from abnormal quadriceps and gastric-soleus
to that of the patella, with normal less than 1.2–1.5 [2, 16]. complex muscles [2, 17]. Genu varus or valgus might also
Radiographs might additionally show fragmentation at the be seen, frequently caused by abnormal femoral version or
lower patella or tibial tuberosity resembling Sinding– instability at the ankle or subtalar joints.
Larsen–Johansson or Osgood–Schlatter disease, respec-
tively (Fig. 8).
Foot and ankle

As many as 93% of children with cerebral palsy have foot or


ankle abnormalities [18]. Numerous radiographic measure-
ments have been developed to describe foot alignment and
deformities, the most widely used based on a three-segment
(hindfoot, midfoot and forefoot) and two-column (medial and
lateral) biomechanical model, and these have been applied to
children with cerebral palsy [19, 20].
Chronic relative overactivity and spasticity of the
gastrocnemius-soleus frequently lead to hindfoot equinus
deformity (plantar flexion of the calcaneus) (Fig. 9).
Clinically this often manifests as toe walking, or heel lift
in children with spastic calf muscles (Fig. 7). Equinus can
be seen in conjunction with valgus or varus hindfoot, de-
pending on the relation of the anterior calcaneus to the
talus [2]. If the calcaneal head is positioned under the talus,
varus hindfoot results, but more commonly it is pulled
Fig. 8 Lower patella fragmentation. Lateral knee radiograph in a 15-year-
old boy with cerebral palsy shows an elongated, high patella (patella alta)
from under the talus, and valgus hindfoot is seen.
with fragmentation at its inferior pole. There is mild thickening of the Equinovalgus is more common in children with diplegia
patellar tendon and quadriplegia, whereas children with hemiplegia more
Pediatr Radiol (2019) 49:1587–1594 1591

contribute to overall foot deformity, as does great toe


malalignment (e.g., hallux valgus).
Inability to dorsiflex an ankle equinus to neutral position
despite bracing and medical management are indications for
surgery [21]. Once foot or ankle realignment surgery has been
deemed necessary, a combination of tendon lengthening/trans-
ferring, osteotomies, bone grafts, and arthrodeses might be
employed [3]. Prior to orthopedic surgery, medical manage-
ment of tone with botulinum toxin and baclofen or neurosur-
gery with selective dorsal rhizotomies (or baclofen pumps) are
frequent therapies [20].
Fig. 9 Hindfoot equinus deformity. Lateral standing foot radiograph in a
15-year-old girl with cerebral palsy shows hindfoot equinus with a
Congenital vertical talus or oblique talus can be seen in
plantar-flexed anterior calcaneus and heel lift. Also note the severe the setting of cerebral palsy, arthrogryposis and other neu-
forefoot inversion as evidenced by the ladder-like arrangement of the rologic abnormalities. This is an equinus position of the
metatarsals talus with standing or simulated standing, resulting in ab-
normal talonavicular alignment, the navicular dorsally
commonly have equinovarus deformity [21]. Midfoot and subluxed with respect to the talar head [22]. With maximal
forefoot cavus and planus deformities are also associated plantar flexion this relationship is irreducible (vertical ta-
with these alignment problems, usually equinoplanovalgus lus; Fig. 10) or reducible (oblique talus; Fig. 11). In young
and equinocavovarus, though other alignment combina- children, the navicular might not be ossified and its posi-
tions can occur if the segments that are normally coupled tion must be inferred from the position of the lateral cune-
are decoupled [20]. Abnormal midfoot abduction/ iform and 2nd metatarsal base because these midfoot bones
adduction and pronation/supination alignment also would be in plane with the navicular [23].

Fig. 10 Vertical talus. Lateral foot


radiographs while standing (a)
and in maximum plantar flexion
(b) in a 5-year-old boy with rigid
flat feet. Radiographs show a
talus in equinus position, with
anterior talus plantar-flexed, and
abnormal dorsal displacement of
the navicular, which does not
reduce with maximum
plantarflexion, consistent with a
vertical talus

Fig. 11 Oblique talus. Lateral foot radiographs with simulated standing the midfoot, which remains in line with the navicular. In contrast to the
(a) and in maximum plantar flexion (b) in a 20-month-old boy with flat boy in Fig. 10, with maximum plantarflexion the (unossified) navicular
feet. The position of the navicular must be inferred from the position of reduces to a normal position at the talar head
1592 Pediatr Radiol (2019) 49:1587–1594

Fig. 12 Coleman block test.


Dorsoplantar foot radiographs in
two 9-year-old girls, each with
cerebral palsy and undergoing the
modified Coleman block test. a, b
The first girl shows hindfoot
varus with little change in
alignment between standing on
flat ground (a) and standing with
lateral foot on the block (b). c, d
The second girl shows milder
hindfoot varus with flat standing,
demonstrating superimposed
talus and calcaneus (c), which
does reduce on the Coleman
block (d). In the latter case, a less
extensive surgical correction
could be considered

Radiographic evaluation of foot alignment in the cavovarus obtained in the child’s typical standing position, followed by
foot can often be augmented by use of the modified Coleman standing with the lateral foot on a 2- to 4-cm-thick block,
block test. In this test, dorsoplantar and lateral images are allowing for the first ray and medial foot to hang off the block
Pediatr Radiol (2019) 49:1587–1594 1593

Fig. 13 Varying lateral views. Lateral radiographs illustrate different therefore the ankle must be in valgus. b Dedicated ankle radiograph
views obtained at the ankle. a Radiograph shows cavovarus hindfoot, shows normalization of this relationship and allows for better scrutiny
but also the physes of the distal tibia and fibula are at the same level, of the talar dome
which should not happen when viewing a normally aligned ankle, and

(Fig. 12). While the predictive utility of this test is debated [24, and dedicated ankle radiographs should be recommended
25], if the hindfoot alignment corrects, then surgical correction to evaluate for ankle valgus that might require treatment,
of the forefoot alone can be considered (with cuneiform and to better profile the talar dome. The best method to
dorsiflexion osteotomy). If the hindfoot is rigid, then surgery evaluate for ankle valgus, however, is on anteroposterior
must include both forefoot and hindfoot (with calcaneal slide view of the ankle. The angle between a vertically oriented
osteotomy to take hindfoot out of varus position). line along the distal tibial shaft and a horizontally oriented
Obtaining standardized weight-bearing radiographic line along the talar dome should be close to 90°; 10° or
views of the foot and ankle can be challenging because less of ankle valgus is often well tolerated (Fig. 14). In
of altered anatomy and physical limitations in these chil- addition, the lateral distal tibial epiphysis is asymmetrical-
dren. For example, cavovarus foot alignment is frequently ly smaller and the distal fibula appears shorter (high fib-
seen with valgus ankle alignment, possibly as a compen- ular station) [26].
satory mechanism to bring the extremity into a more neu-
tral overall alignment. Weight-bearing lateral foot radio-
graphs might show the physis of the fibula at or above the Conclusion
level of the tibial physis. Outside of infancy (where a high
fibular physis might be normal) this relationship indicates Cerebral palsy is a common condition that manifests in
the image was not obtained lateral with respect to the n u m e r o u s w a y s o n t h e m u s c ul o s k el e t a l s y s t e m .
ankle but rather lateral with respect to the foot (Fig. 13), Recognizable patterns of malformations develop, and

Fig. 14 Evaluating for ankle


valgus. a Anteroposterior
radiography of a 10-year-old boy
with cerebral palsy demonstrates
ankle valgus. b On the same
image, the increased tibio-talar
angle is shown by lines drawn
across the top of the talar dome
and along the long axis of the
tibia, smaller lateral distal tibial
epiphysis and high fibular station
1594 Pediatr Radiol (2019) 49:1587–1594

imaging is valuable for monitoring, treatment planning 11. Cognetti D, Keeny HM, Samdani AF et al (2017) Neuromuscular
scoliosis complication rates from 2004 to 2015: a report from the
and follow-up. Having familiarity with and the ability to
Scoliosis Research Society morbidity and mortality database.
describe these radiologic manifestations allows for better Neurosurg Focus 43:E10
communication with care teams and helps to make radi- 12. McCall RE, Hayes B (2005) Long-term outcome in neuromuscular
ologists valuable resources for clinical providers. scoliosis fused only to lumbar 5. Spine 30:2056–2060
13. Harada T, Ebara S, Anwar MM et al (1993) The lumbar spine in
spastic diplegia. A radiographic study. J Bone Joint Surg Br 75:
Acknowledgments We would like to thank the musculoskeletal radiolo-
534–537
gists and orthopedic surgery department at Seattle Children’s Hospital.
14. Benninga MA, Tabbers MM, van Rijn RR (2016) How to use
a plain abdominal radiograph in children with functional def-
Compliance with ethical standards ecation disorders. Arch Dis Child Educ Pract Ed 101:187–193
15. Berger MY, Tabbers MM, Kurver MJ et al (2012) Value of abdom-
Conflicts of interest None inal radiography, colonic transit time, and rectal ultrasound scan-
ning in the diagnosis of idiopathic constipation in children: a sys-
tematic review. J Pediatr 161:44–50.e2
16. Shabshin N, Schweitzer M, Morrison W, Parker L (2004) MRI
References criteria for patella Alta and Baja. Skelet Radiol 33:445–450
17. Bauer J, Patrick Do K, Feng J et al (2017) Knee recurvatum in
1. DeLuca PA (1996) The musculoskeletal management of children children with spastic diplegic cerebral palsy. J Pediatr Orthop.
with cerebral palsy. Pediatr Clin N Am 43:1135–1150 https://doi.org/10.1097/BPO.0000000000000985
2. Morrell DS, Pearson JM, Sauser DD (2002) Progressive bone and 18. O’Connell PA, D’Souza L, Dudeney S, Stephens M (1998) Foot
joint abnormalities of the spine and lower extremities in cerebral deformities in children with cerebral palsy. J Pediatr Orthop 18:
palsy. Radiographics 22:257–268 743–747
3. Kedem P, Scher DM (2015) Foot deformities in children with ce- 19. Westberry DE, Davids JR, Roush TF, Pugh LI (2008)
rebral palsy. Curr Opin Pediatr 27:67–74 Qualitative versus quantitative radiographic analysis of foot
4. Rodda J, Graham HK (2001) Classification of gait patterns in spas- deformities in children with hemiplegic cerebral palsy. J
tic hemiplegia and spastic diplegia: a basis for a management algo- Pediatr Orthop 28:359–365
rithm. Eur J Neurol 8:98–108 20. Davids JR (2010) The foot and ankle in cerebral palsy. Orthop Clin
5. Davids JR, Bagley AM (2014) Identification of common gait dis- N Am 41:579–593
ruption patterns in children with cerebral palsy. J Am Acad Orthop 21. Karamitopoulos MS, Nirenstein L (2015) Neuromuscular foot.
Surg 22:782–790 Foot Ankle Clin 20:657–668
6. Palisano R, Rosenbaum P, Walter S et al (2008) Development and 22. Mckie J, Radomisli T (2010) Congenital vertical talus: a review.
reliability of a system to classify gross motor function in children Clin Podiatr Med Surg 27:145–156
with cerebral palsy. Dev Med Child Neurol 39:214–223 23. Stein-Wexler R, Wootton-Gorges SL, Ozonoff MB (2015) Pediatric
7. Driscoll SW, Skinner J (2008) Musculoskeletal complications of orthopedic imaging. Springer-Verlag, Heidelberg, pp 499–500
neuromuscular disease in children. Phys Med Rehabil Clin N Am 24. Myerson MS, Myerson CL (2019) Cavus foot. Foot Ankle Clin 24:
19:163–194 347–360
8. McCarthy JJ, D′Andrea LP, Betz RR, Clements DH (2006) 25. Krähenbühl N, Weinberg MW (2019) Anatomy and biomechanics
Scoliosis in the child with cerebral palsy. J Am Acad Orthop Surg of cavovarus deformity. Foot Ankle Clin 24:173–181
14:367–375 26. Aurégan JC, Finidori G, Cadilhac C et al (2011) Children ankle
9. Lee SY, Chung CY, Lee KM et al (2016) Annual changes in radio- valgus deformity treatment using a transphyseal medial malleolar
graphic indices of the spine in cerebral palsy patients. Eur Spine J screw. Orthop Traumatol Surg Res 97:406–409
25:679–686
10. McElroy MJ, Sponseller PD, Dattilo JR et al (2012) Growing rods
for the treatment of scoliosis in children with cerebral palsy: a crit- Publisher’s note Springer Nature remains neutral with regard to
ical assessment. Spine 37:E1504–E1510 jurisdictional claims in published maps and institutional affiliations.

You might also like