Elisabet Rodby-Bousquet

Prevention of dislocation of the hip in children with cerebral palsy 20-year results of a population-based prevention programme

In 1994 a cerebral palsy (CP) register and healthcare programme was established in southern Sweden with the primary aim of preventing dislocation of the hip in these children. The results from the first ten years were published in 2005 and showed a decrease in the incidence of dislocation of the hip, from 8% in a historical control group of 103 children born between 1990 and 1991 to 0.5% in a group of 258 children born between 1992 and 1997. These two cohorts have now been re-evaluated and an additional group of 431 children born between 1998 and 2007 has been added. By 1 January 2014, nine children in the control group, two in the first study group and none in the second study group had developed a dislocated hip (p < 0.001). The two children in the first study group who developed a dislocated hip were too unwell to undergo preventive surgery. Every child with a dislocated hip reported severe pain, at least periodically, and four underwent salvage surgery. Of the 689 children in the study groups, 91 (13%) underwent preventive surgery. A population-based hip surveillance programme enables the early identification and preventive treatment, which can result in a significantly lower incidence of dislocation of the hip in children with CP.

Use of manual and powered wheelchair in children with cerebral palsy: a cross-sectional study

BackgroundMobility is important for the cognitive and psychosocial development of children. Almost one third of children with cerebral palsy (CP) are non-ambulant. Wheelchairs can provide independent mobility, allowing them to explore their environment. Independent mobility is vital for activity and participation and reduces the dependence on caregivers. The purpose of this study was to describe the use of manual and powered wheelchair indoors and outdoors in relation to the degree of independent wheelchair mobility or need for assistance in a total population of children with CP.MethodsA cross-sectional study was performed including all children aged 3-18 years with CP living in southern Sweden during 2008. Data was extracted from a register and health care programme for children with CP (CPUP). There were a total of 562 children (326 boys, 236 girls) in the register. Information on the childs use of manual and powered wheelchair indoors and outdoors and the performance in self-propelling or need for assistance were analysed related to age, CP subtype and gross motor function.ResultsWheelchairs for mobility indoors were used by 165 (29%) of the 562 children; 61 used wheelchair for independent mobility (32 using manual only, 12 powered only, 17 both) and 104 were pushed by an adult. For outdoor mobility wheelchairs were used by 228 children (41%); 66 used a wheelchair for independent mobility (18 using manual only, 36 powered only, 12 both) and 162 were pushed. The use of wheelchair increased with age and was most frequent in the spastic bilateral and dyskinetic subtypes. Most powered wheelchairs were operated by children at GMFCS level IV.ConclusionIn this total population of children with CP, aged 3-18 years, 29% used a wheelchair indoors and 41% outdoors. A majority using manual wheelchairs needed adult assistance (86%) while powered wheelchairs provided independent mobility in most cases (86%). To achieve a high level of independent mobility, both manual and powered wheelchairs should be considered at an early age for children with impaired walking ability.

Sitting and standing performance in a total population of children with cerebral palsy: a cross-sectional study

BackgroundKnowledge of sitting and standing performance in a total population of children with cerebral palsy (CP) is of interest for health care planning and for prediction of future ability in the individual child. In 1994, a register and a health care programme for children with CP in southern Sweden was initiated. In the programme information on how the child usually sits, stands, stands up and sits down, together with use of support or assistive devices, is recorded annually.MethodsA cross-sectional study was performed, analysing the most recent report of all children with CP born 1990-2005 and living in southern Sweden during 2008. All 562 children (326 boys, 236 girls) aged 3-18 years were included in the study. The degree of independence, use of support or assistive devices to sit, stand, stand up and sit down was analysed in relation to the Gross Motor Function Classification System (GMFCS), CP subtype and age.ResultA majority of the children used standard chairs (57%), could stand independently (62%) and could stand up (62%) and sit down (63%) without external support. Adaptive seating was used by 42%, external support to stand was used by 31%, to stand up by 19%, and to sit down by 18%. The use of adaptive seating and assistive devices increased with GMFCS levels (p < 0.001) and there was a difference between CP subtypes (p < 0.001). The use of support was more frequent in preschool children aged 3-6 (p < 0.001).ConclusionAbout 60% of children with CP, aged 3-18, use standard chairs, stand, stand up, and sit down without external support. Adding those using adaptive seating and external support, 99% of the children could sit, 96% could stand and 81% could stand up from a sitting position and 81% could sit down from a standing position. The GMFCS classification system is a good predictor of sitting and standing performance.

A combined surveillance program and quality register improves management of childhood disability

Abstract Purpose: To describe a concept for prevention of secondary conditions in individuals with chronic neuromuscular disabilities by using two Swedish developed follow-up-programmes for cerebral palsy (CP; CPUP) and myelomeningocele (MMC; MMCUP) respectively as examples. Method: This paper describes and outlines the rationale, development and implementation of CPUP and MMCUP. Results: Both programmes are multidisciplinary longitudinal follow-up programmes that simultaneously serve as national registries. The programmes are population-based and set in Swedish habilitation clinics. Most children (95%) born 2000 or later with CP are enrolled in CPUP and the recruitment of adults is underway. CPUP has also been implemented in Norway, Denmark, Iceland, Scotland and parts of Australia. In MMCUP, almost all children with MMC born 2007 or later participate and individuals of all ages are now invited. The registries provide epidemiological profiles associated with CP and MMC and platforms for population-based research and quality of care improvement. Conclusions: Through multidisciplinary follow-up and early detection of emerging complications individuals with CP or MMC can receive less complex and more effective interventions than if treatment is implemented at a later stage. Possibilities and challenges to design, implement and continuously run multidisciplinary secondary prevention follow-up programmes and quality registries for individuals with CP or MMC are described and discussed. Implications for rehabilitation Individuals with disabilities such as cerebral palsy or myelomeningocele are at risk of developing secondary conditions. Multidisciplinary population-based longitudinal follow-up programmes seem effective in preventing certain types of secondary conditions.

Postural asymmetries in young adults with cerebral palsy.

The purpose was to describe posture, ability to change position, and association between posture and contractures, hip dislocation, scoliosis, and pain in young adults with cerebral palsy (CP).

Windswept hip deformity in children with cerebral palsy: a population-based prospective follow-up

AbstractPurpose To analyze the development of windswept hip deformity (WS) in a total population of children with cerebral palsy (CP) up to 20 years of age, the association between WS and hip dislocation, and femoral varus osteotomy and scoliosis, and the impact of a hip surveillance program on the subsequent incidence of WS.MethodsThis is a prospective study on children with CP in southern Sweden included in the Swedish follow-up programme and registry for CP (CPUP). All children born between 1990 and 1995 with CP were included; those born between 1990 and 1991 did not partake in the hip surveillance program until they were older (3–5 years of age) and served as a historic control group. Children born between 1992 and 1995 were included in the hip surveillance program from about 2 years of age and constituted the study group.ResultsIn the control group, 12 of 68 children (18 %) developed WS. In the study group of 139 children, 13 (9 %) developed WS (p = 0.071). Of all 25 children with WS, 21 also developed scoliosis and 5 developed a hip dislocation. The number of children with WS starting in the lower extremities was significantly lower in the study group (p = 0.028). No difference between the two groups was seen regarding WS that started in combination with scoliosis.ConclusionWith early inclusion in a hip surveillance program and early treatment of contractures, it appears possible to reduce the frequency of WS starting in the lower extremities.

Prediction of hip displacement in children with cerebral palsy: development of the CPUP hip score

Hip displacement, defined in this study as a migration percentage (MP) of more than 40%, is a common, debilitating complication of cerebral palsy (CP). In this prospective study we analysed the risk of developing hip displacement within five years of the first pelvic radiograph. All children with CP in southern and western Sweden are invited to register in the hip surveillance programme CPUP. Inclusion criteria for the two groups in this study were children from the CPUP database born between 1994 and 2009 with Gross Motor Function Classification System (GMFCS) III to V. Group 1 included children who developed hip displacement, group 2 included children who did not develop hip displacement over a minimum follow-up of five years. A total of 145 children were included with a mean age at their initial pelvic radiograph of 3.5 years (0.6 to 9.7). The odds ratio for hip displacement was calculated for GMFCS-level, age and initial MP and head-shaft angle. A risk score was constructed with these variables using multiple logistic regression analysis. The predictive ability of the risk score was evaluated using the area under the receiver operating characteristics curve (AUC). All variables had a significant effect on the risk of a MP > 40%. The discriminatory accuracy of the CPUP hip score is high (AUC = 0.87), indicating a high ability to differentiate between high- and low-risk individuals for hip displacement. The CPUP hip score may be useful in deciding on further follow-up and treatment in children with CP.

Demographic and modifiable factors associated with knee contracture in children with cerebral palsy

To identify the prevalence of knee contracture and its association with gross motor function, age, sex, spasticity, and muscle length in children with cerebral palsy (CP).

Inter- and intra-rater reliability of the head-shaft angle in children with cerebral palsy

Abstract Purpose Children with cerebral palsy (CP) are at increased risk for hip dislocation. This can be prevented in most cases using surveillance programmes that include radiographic examinations. Known risk factors for hip dislocation include young age, high Gross Motor Function Classification System (GMFCS) level and high migration percentage (MP). The head-shaft angle (HSA) has recently been described as an additional risk factor. The study aim was to determine inter- and intra-rater reliability of the HSA in a surveillance programme for children with CP. Methods We included hip radiographs from the CP surveillance programme CPUP in southern Sweden during the first half of 2016. Fifty radiographs were included from children at GMFCS levels II-V, with a mean age of 6.6 (SD 3.2) years. Three raters measured the HSA of one hip (left or right) at baseline and four weeks later; intraclass correlation coefficient (ICC) was used to estimate inter- and intra-rater reliability. Results Inter- and intra-rater reliability were excellent for the HSA, with ICC 0.92 (95% CI 0.87-0.96) and ICC 0.99 (95% CI 0.98-0.99), respectively. Conclusion The HSA showed excellent inter- and intra-rater reliability for children with CP, providing further evidence for use of the HSA as an additional factor for identifying risk for further hip displacement or dislocation.

The effect of asymmetrical limited hip flexion on seating posture, scoliosis and windswept hip distortion

BACKGROUND Postural asymmetries with seating problems are common in adults with cerebral palsy. AIMS To analyse the prevalence of asymmetrical limited hip flexion (<90°) in adults with CP, and to evaluate the association between asymmetrical limited hip flexion and postural asymmetries in the sitting position. METHODS AND PROCEDURES Cross-sectional data of 714 adults with CP, 16-73 years, GMFCS level I-V, reported to CPUP, the Swedish cerebral palsy national surveillance program and quality registry, from 2013 to 2015. Hip range of motion was analysed in relation to pelvic obliquity, trunk asymmetry, weight distribution, scoliosis and windswept hip distortion. OUTCOMES AND RESULTS The prevalence of asymmetrical limited hip flexion increased as GMFCS level decreased. Of adults at GMFCS level V, 22% had asymmetrical limited hip flexion (<90°). The odds of having an oblique pelvis (OR 2.6, 95% CI:1.6-2.1), an asymmetrical trunk (OR 2.1, 95% CI:1.1-4.2), scoliosis (OR 3.7, 95% CI:1.3-9.7), and windswept hip distortion (OR 2.6, 95% CI:1.2-5.4) were higher for adults with asymmetrical limited hip flexion compared with those with bilateral hip flexion>90°. CONCLUSIONS AND IMPLICATIONS Asymmetrical limited hip flexion affects the seating posture and is associated with scoliosis and windswept hip distortion.