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Upper-Limb Contracture Development in Children With Cerebral Palsy: A Population-Based Study
Upper-Limb Contracture Development in Children With Cerebral Palsy: A Population-Based Study
1 Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm; 2 Centre for Clinical Research S€ormland, Uppsala University, Eskilstuna; 3
Department of Clinical Science and Education, Karolinska Institutet, Stockholm; 4 Department of Hand Surgery, S€odersjukhuset, Stockholm, Sweden.
Correspondence to Jenny Hedberg-Graff, Neuropediatric Unit, Q2:07, Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden. E-mail: Jenny.hedberg@ki.se
PUBLICATION DATA AIM The aim of this study was to investigate the longitudinal development of passive range
Accepted for publication 4th July 2018. of motion (ROM) in the upper limbs in a population-based sample of children with cerebral
Published online palsy (CP), and to investigate which children are more likely to develop contractures related
to functional level, CP subtype, and age.
ABBREVIATIONS METHOD Registry data of annual passive ROM measurements of the upper limbs from 771
CPUP Cerebral Palsy Follow-up children with CP (417 males, 354 females; mean age 11y 8mo, [SD 5mo] range 1–18y) were
Programme analysed. Mixed models were used to investigate at what age decreased passive ROM
MACS Manual Ability Classification occurs. Odds ratios were calculated to compare risks and logistic regression analysis was
System used to predict contracture development.
ROM Range of motion RESULTS Thirty-four per cent of the children had developed contractures. Among these
children, decreased passive ROM was significant at a mean age of 4 years for wrist extension
and 7 years for shoulder flexion, elbow extension, and supination. Children at Manual Ability
Classification System (MACS) level V had a 17-times greater risk of contractures than children
at MACS level I.
INTERPRETATION One-third of the children in the total population developed upper-limb
contractures while passive ROM decreased with age. MACS level was the strongest predictor
of contracture development.
The underlying neurological pathology in cerebral palsy year. From the age of 16 to 18 years, children can con-
(CP) is by definition not progressive, but motor symptoms tinue in the adult follow-up programme. Ninety-five per
often change over time. Most young children with CP cent of all children with CP in Sweden are registered in
exhibit full passive range of motion (ROM), but joint stiff- the CPUP. The assessments include, among many other
ness develops gradually and is sometimes not recognized variables, passive ROM measurements.7 The CPUP has
until it is pronounced and disabling. Many adolescents and been shown to be effective in preventing hip dislocations
adults with CP exhibit joint deformities with severe, fixed, and contracture development in the lower limbs.8
painful contractures.1,2 The occurrence and timing of con- It is possible that early detection might assist in prevent-
tracture development in the upper limbs in CP is largely ing the development of a progressive decrease in passive
unknown.3,4 movements by identifying which children are at greater
Knowledge is lacking about which children with CP are risk and, therefore, need to be monitored more closely
more prone to develop upper-limb contractures, which concerning joint motion. The aim of this study was to
joints are most commonly affected, and at what age con- investigate the longitudinal development of passive ROM
tractures typically occur. Evidence for the effects of treat- in the upper limbs in children with CP, as well as to inves-
ment of contractures is also lacking.5 tigate which children are more likely to develop contrac-
In Sweden, a national register and follow-up programme tures related to functional level, CP subtype, and at what
for children and adults with CP was started in 1994, age contractures begin to be significant.
referred to as the CP Follow-up Programme (CPUP).6
Children are invited to the programme as soon as they METHOD
have a diagnosis of suspected CP. Within the CPUP, all This longitudinal population-based study used CPUP reg-
children are assessed by their physical and occupational istry data from the southern part of Sweden. This region
therapists at their local paediatric rehabilitation centre has a CP prevalence of 2.3 per 1000 individuals and was
twice a year until the age of 6 years, and after that once a chosen because it was the first to introduce a registry and,
Table I: Distribution of cerebral palsy (CP) subtypes, Manual Ability Classification System (MACS) levels, Gross Motor Function Classification System
(GMFCS) levels, and measurement occasions (n=771)
CP subtype, n
MACS and GMFCS levels Spastic unilateral Spastic bilateral Ataxic Dyskinetic Non-classifiable Missing n (%)
150 0
Degrees
Degrees
100
–50
50
–100
0
I II III IV V I II III IV V
MACS MACS
100
100
50 50
Degrees
Degrees
0 0
–50 –50
–100 –100
I II III IV V I II III IV V
MACS MACS
100
Restricted passive ROM - yellow
50
Severly restricted passive ROM - red
Degrees
–50
–100
I II III IV V
MACS
Figure 1: Passive range of motion (ROM) measurements in degrees for upper-limb movements at the last measurement occasion distributed across the
five Manual Ability Classification System (MACS) levels (n=771). The median is marked inside the box. The length of the whiskers is determined by mul-
tiplying the interquartile range by a factor of 1.5. Observations outside of the whiskers are so-called outliers and are marked with a circle (o). Outliers
outside a range of 3 times the interquartile range are extreme outliers and are depicted with an asterisk (*).
180 0
170
–10
160
Degrees
Degrees
150
–20
140
130
–30
120
110 –40
1–3 4–6 7–9a 10–12 13–15 16–18 1–3 4–6 7–9a 10–12 13–15 16–18
Age, y (3y interval) Age, y (3y interval)
80 70
70
50
60 Degrees
Degrees
50
30
40
30
10
20
10 –10
1–3 4–6 7–9a 10–12 13–15 16–18 1–3 4–6a 7–9 10–12 13–15 16–18
Age, y (3y interval) Age, y (3y interval)
30
Degrees
–30
1–3 4–6a 7–9 10–12 13–15 16–18
Age, y (3y interval)
Figure 2: Estimated development in passive range of motion (ROM) measurements in degrees for upper-limb movements across age groups in children
who developed contractures. Results from a mixed model analysis (n=262). aAge when the passive ROM significantly declines from the reference group
(1–3y). CI, confidence interval.
development over time in children and adolescents with extension with extended fingers. In the children who devel-
CP. We found that 34% of the children had developed oped contractures, the decrease in passive ROM had
contractures. Contractures were most common in wrist started during the first years of life and had become
significant at the age of 4 years for wrist extension and at contractures was significantly reduced by early interven-
7 years for shoulder flexion, elbow extension, and supina- tions.6,15 The authors concluded that just being involved in
tion. The highest proportion of contractures was found at the systematic follow-up programme might have had an
the highest GMFCS levels, MACS levels, and in children important impact on identifying signs of decreased passive
with dyskinetic CP. MACS level was the most important ROM at an early age. This might also be the case for
variable predicting contracture development (OR=16.6, upper-limb contractures, but unfortunately we do not have
p=0.01) when adjusting for all included predicting factors data on the pre-CPUP status for the upper limbs.
in the analysis (adjusted OR=12.1, p=0.01) but also Most of the children who developed contractures had
GMFCS level V (adjusted OR=6.4, p=0.01) and spastic several joints involved, but the contracture development
unilateral CP (adjusted OR=8.2, p=0.01) were found to be often started with wrist extension with simultaneous finger
predictors of the development of contractures of the upper extension. Decreased passive ROM in the wrist and hand
limbs. has been described as the most common upper-limb move-
In this study, the proportion of individuals demonstrat- ment restriction in children with CP.12 A flexed posture of
ing upper-limb contractures was actually lower than the wrist can be a consequence of shortened wrist flexors
expected. From a clinical perspective, we had the impres- and/or shortened finger flexors and weakness in the exten-
sion that nearly all children with CP would have at least sor muscles.16 This imbalance and the flexed position of the
some passive ROM restriction, and it has been reported wrist weakens the grip and makes grasping and perfor-
that four out of five children with CP have contractures.13 mance of daily activities difficult. Thus, contractures in the
In this population-based study, however, only a third of wrist and fingers might have serious functional conse-
the children had developed upper-limb contractures. This quences and steps should be taken to try to prevent them.17
difference is probably due to the fact that previous studies This study showed that upper-limb passive ROM for the
have been based on patient cohorts rather than complete children who did develop contractures had already started
populations of children with CP, as used here. to deteriorate during the first years of life and continued
It could also be argued, however, that a prevalence of until our last measurement point at 18 years. Furthermore,
34% of contractures is high, considering that all children a refraction point with a greater rate of decrease was seen
in Sweden have access to free health care and that all of at the age of 12 to 13 years, which corresponds to the
the children in this study had regular contact with habilita- onset of puberty and is a common age for growth spurts.
tion teams.6 Many of the included children had probably Taken together, our results suggest that once contracture
received interventions aimed at contracture prevention or development starts, it is hard to stop it. The underlying
improvement such as stretching, orthotics, botulinum toxin mechanisms behind contractures are currently a topic for
injections, and hand surgery. Children with a sparse, self- discussion and research is ongoing.18 Little is known about
induced active movement repertoire are likely to have had whether and how well interventions actually affect contrac-
a daily passive ROM regimen performed by their care- tures in spastic muscles, but we do know that botulinum
givers; though the effects of such interventions have been toxin might be helpful in postponing a need for surgery
questioned.5 and, in combination with occupational therapy, might
A study of the lower limbs, with access to data from a improve function.5,19
control group, indicated that for children who were fol- A possible limitation of this study could be that, even
lowed in the CPUP, the development of severe though the children were expected to have yearly follow-
REFERENCES
1. Kerr Graham H, Selber P. Musculoskeletal aspects of 3. Gough M, Shortland AP. Could muscle 4. de Bruin M, Smeulders MJ, Kreulen M. Why is joint
cerebral palsy. J Bone Joint Surg Br 2003; 85: 157–66. deformity in children with spastic cerebral palsy be range of motion limited in patients with cerebral palsy?
2. Smith LR, Chambers HG, Lieber RL. Reduced satellite related to an impairment of muscle growth and J Hand Surg Eur 2013; 13: 8–13.
cell population may lead to contractures in children with altered adaptation? Dev Med Child Neurol 2012; 54: 5. Novak I, McIntyre S, Morgan C, et al. A systematic
cerebral palsy. Dev Med Child Neurol 2013; 55: 264–70. 495–9. review of interventions for children with cerebral palsy:
RESUMEN
~
DESARROLLO DE CONTRACTURAS EN LOS MIEMBROS SUPERIORES EN NINOS
CON PARALISIS CEREBRAL: UN ESTUDIO DE
POBLACION
OBJETIVO El objetivo de este estudio fue investigar el desarrollo longitudinal del rango de movimiento pasivo (ROM) de las
extremidades superiores en una muestra poblacional de nin ~ os con paralisis cerebral (PC) e investigar que nin~ os tienen ma s
probabilidades de desarrollar contracturas relacionadas a. el nivel funcional, subtipo PC y edad.
METODO Se analizaron los registros anuales de los datos de las mediciones de ROM de los miembros superiores de 771 nin~ os
con PC (417 varones, 354 mujeres, edad media 11 an ~ os 8meses, [DS 5 meses] en un grupo etario de 1-18 an ~ os). Se usaron
modelos mixtos para investigar a que edad disminuyo la movilidad pasiva. Los Odds ratios se calcularon para comparar los
el ana
riesgos y se utilizo lisis de regresio n logıstica para predecir el desarrollo de la contractura.
RESULTADOS Treinta y cuatro por ciento de los nin~ os habıan desarrollado contracturas. Entre estos nin~ os, la movilidad pasiva
disminuyo significativamente, especıficamente; a la edad promedio de 4 an ~ os para la extensio n de la mun ~ eca y a los 7 an~ os para
n del hombro, la extensio
la flexio n del codo y la supinacio n de antebrazo. Los nin ~ os en el nivel V del Sistema de Clasificacio n de
Habilidad Manual (MACS, siglas en ingle s) tenıan un riesgo 17 veces mayor de contracturas que los nin ~ os en el nivel I de MACS.
INTERPRETACION Un tercio de los nin~ os de la poblacio n total desarrollaron contracturas en los miembros superiores, y el ROM
disminuyo con la edad. El nivel de MACS fue el predictor ma s fuerte del desarrollo de contracturas en los miembros superiores.
RESUMO
ß AS COM PARALISIA CEREBRAL: UM ESTUDO
DESENVOLVIMENTO DE CONTRATURA DE MEMBROS SUPERIORES EM CRIANC
POPULACIONAL
OBJETIVOS O objetivo deste estudo foi investigar o desenvolvimento longitudinal da amplitude de movimento passiva (ADM) em
membros superiores em uma amostra populacional de criancßas com paralisia cerebral (PC), e investigar quais criancßas sa ~o mais
vulnera veis ao desenvolvimento de contraturas relacionadas ao nıvel funcional, subtipos de PC e idade.
METODOS Foram analisados os registros anuais das medidas de amplitude de movimento passiva de membros superiores de 771
criancßas com PC (417 meninos, 354 meninas; idade me dia de 11 anos e 8 meses [ 5 meses] variando de 1 a 18 anos). Modelos
mistos foram aplicados para identificar a idade de ocorre ~ o da ADM passiva. Odds ratios foram calculados para
^ncia da diminuicßa
comparar riscos, e ana lise de regressa
~o logıstica foi usada para predizer o desenvolvimento de contraturas.
RESULTADOS 34 % das criancßas desenvolveram contraturas. Entre elas, houve diminuicßa~o significativa da ADM passiva em media
com 4 anos para extensa ~o de punho e 7 anos para flexa ~o de ombros, extensa ~o de cotovelos e supinadores. Criancßas com Sistema
de Classificacßa~o Manual de Habilidades (MACS) nıvel V apresentaram riscos de desenvolver contraturas 17 vezes maior que
criancßas MACS nıvel I.
INTERPRETAC ~ Um tercßo das criancßas da populacßa~o total desenvolveram contraturas de membros e a ADM passiva diminuiu
ß AO
com a idade. O nıvel do MACS foi o preditor mais forte para o desenvolvimento de contraturas.