Gunnar Hägglund

Characteristics of children with hip displacement in cerebral palsy

BackgroundHip dislocation in children with cerebral palsy (CP) is a common and severe problem. The dislocation can be avoided, by screening and preventive treatment of children with hips at risk. The aim of this study was to analyse the characteristics of children with CP who develop hip displacement, in order to optimise a hip surveillance programme.MethodsIn a total population of children with CP a standardised clinical and radiological follow-up of the hips was carried out as a part of a hip prevention programme. The present study is based on 212 children followed until 9–16 years of age.ResultsOf the 212 children, 38 (18%) developed displacement with Migration Percentage (MP) >40% and further 19 (9%) MP between 33 and 39%. Mean age at first registration of hip displacement was 4 years, but some hips showed MP > 40% already at two years of age. The passive range of hip motion at the time of first registration of hip displacement did not differ significantly from the findings in hips without displacement.The risk of hip displacement varied according to CP-subtype, from 0% in children with pure ataxia to 79% in children with spastic tetraplegia. The risk of displacement (MP > 40%) was directly related to the level of gross motor function, classified according to the gross motor function classification system, GMFCS, from 0% in children in GMFCS level I to 64% in GMFCS level V.ConclusionHip displacement in CP often occurs already at 2–3 years of age. Range of motion is a poor indicator of hips at risk. Thus early identification and early radiographic examination of children at risk is of great importance. The risk of hip displacement varies according to both CP-subtype and GMFCS. It is sometimes not possible to determine subtype before 4 years of age, and at present several definitions and classification systems are used. GMFCS is valid and reliable from 2 years of age, and it is internationally accepted.We recommend a hip surveillance programme for children with CP with radiographic examinations based on the childs age and GMFCS level.

Prevention of dislocation of the hip in children with cerebral palsy. The first ten years of a population-based prevention programme.

In 1994, a register for cerebral palsy and a health-care programme were started in southern Sweden with the aim of preventing dislocation of the hip in children with cerebral palsy. It involved all children with cerebral palsy born in 1992 or later. None of the 206 affected children born between 1992 and 1997 has developed a dislocation following the introduction of the prevention programme. Another 48 children moved into the area and none developed any further dislocation. Of the 251 children with cerebral palsy, aged between five and 11 years, living in the area on January 1, 2003, only two had a dislocated hip. One boy had moved into the area at age of nine with a dislocation and a girl whose parents chose not to participate in the programme developed bilateral dislocation. One boy, whose condition was considered to be too poor for preventative surgery, developed a painful dislocation of the hip at the age of five years and died three years later. Eight of 103 children in a control group, consisting of all children with cerebral palsy living in the area between 1994 and 2002, and born between 1990 and 1991, developed a dislocation of the hip before the age of six years. The decreased incidence of dislocation after the introduction of the prevention programme was significant (p < 0.001). Dislocation of the hip in cerebral palsy remains a serious problem, and prevention is important. Our screening programme and early intervention when lateral displacement of the femoral head was detected appear to be successful.

Cerebral palsy in a total population of 4–11 year olds in southern Sweden. Prevalence and distribution according to different CP classification systems

BackgroundThe aim of this study was to investigate the prevalence of cerebral palsy (CP) as well as to characterize the CP population, its participation in a secondary prevention programme (CPUP) and to validate the CPUP database.MethodsThe study population was born 1990–1997 and resident in Skåne/Blekinge on Jan 1st 2002. Multiple sources were used. Irrespective of earlier diagnoses, neuropaediatrician and other professional medical records were evaluated for all children at the child habilitation units. The CPUP database and diagnosis registers at hospital departments were searched for children with CP or psychomotor retardation, whose records were then evaluated. To enhance early prevention, CP/probable CP was searched for also in children below four years of age born 1998–2001.ResultsThe prevalence of CP was 2.4/1,000 (95% CI 2.1–2.6) in children 4–11 years of age born in Sweden, excluding post-neonatally acquired CP. Children born abroad had a higher prevalence of CP with more severe functional limitations. In the total population, the prevalence of CP was 2.7/1,000 (95% CI 2.4–3.0) and 48% were GMFCS-level I (the mildest limitation of gross motor function).One third of the children with CP, who were born or had moved into the area after a previous study in 1998, were not in the CPUP database. The subtype classification in the CPUP database was adjusted in the case of every fifth child aged 4–7 years not previously reviewed.ConclusionThe prevalence of CP and the subtype distribution did not differ from that reported in other studies, although the proportion of mild CP tended to be higher.The availability of a second opinion about the classification of CP/CP subtypes is necessary in order to keep a CP register valid, as well as an active search for undiagnosed CP among children with other impairments.

Prevention of severe contractures might replace multilevel surgery in cerebral palsy: results of a population-based health care programme and new techniques to reduce spasticity.

During the 1990s three new techniques to reduce spasticity and dystonia in children with cerebral palsy (CP) were introduced in southern Sweden: selective dorsal rhizotomy, continuous intrathecal baclofen infusion and botulinum toxin treatment. In 1994 a CP register and a health care programme, aimed to prevent hip dislocation and severe contractures, were initiated in the area. The total population of children with CP born 1990–1991, 1992–1993 and 1994–1995 was evaluated and compared at 8 years of age. In non-ambulant children the passive range of motion in hip, knee and ankle improved significantly from the first to the later age groups. Ambulant children had similar range of motion in the three age groups, with almost no severe contractures. The proportion of children treated with orthopaedic surgery for contracture or skeletal torsion deformity decreased from 40 to 15% (P=0.0019). One-fifth of the children with spastic diplegia had been treated with selective dorsal rhizotomy. One-third of the children born 1994–1995 had been treated with botulinum toxin before 8 years of age. With early treatment of spasticity, early non-operative treatment of contracture and prevention of hip dislocation, the need for orthopaedic surgery for contracture or torsion deformity is reduced, and the need for multilevel procedures seems to be eliminated.

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.

Association between gross motor function (GMFCS) and manual ability (MACS) in children with cerebral palsy. A population-based study of 359 children

BackgroundThe Gross Motor Function Classification System (GMFCS) has become an important tool to describe motor function in children with Cerebral Palsy (CP). The Manual Ability Classification System (MACS) was developed recently as a corresponding classification of manual ability. The aim of this study was to describe the association between gross motor function and manual ability in a total population of children with cerebral palsy.Methods365 children, born 1992 to 2001, who were registered in a population-based health care programme (CPUP) for children with CP living in the south of Sweden were included in the study. GMFCS was evaluated by the childs physiotherapist and MACS by the occupational therapist. CP diagnosis and subtype were determined by the neuropaediatrician at or after the age of four.ResultsGMFCS levels were available in all 365 children, MACS levels in 359 (98%). There was a poor overall correlation between gross motor function and manual ability. However, different associations between gross motor function and manual ability were found in the different diagnostic subtypes. Children with spastic hemiplegia generally had a lower level of manual ability than gross motor function (p < 0.001). The reverse association was generally found in children with spastic diplegia (p < 0.001). Children with dyskinetic CP had large limitations in both gross motor function and manual ability, with no significant discrepancy between GMFCS and MACS levels.ConclusionGross motor function and manual ability are often discrepant in children with CP, and the patterns seem to vary across the different subgroups based on the predominant neurological findings. To give a complete clinical picture when evaluating these children, both aspects have to be described. The GMFCS and the MACS seem to work well in this context and seem very useful in population-based studies, in health care registers for children with CP, and in clinical practice.

Hand Function in Cerebral Palsy. Report of 367 Children in a Population-Based Longitudinal Health Care Program

PURPOSE To describe aspects of hand function in a total population of children with cerebral palsy (CP). METHODS Upper extremity data were collected for 367 children who were born between 1992 and 2001 and were registered in a population-based health care program for children with CP. Hand function was classified according to the Manual Ability Classification System (MACS), the House functional classification, and the Zancolli classification. The type of spastic thumb-in-palm deformity was evaluated according to House. RESULTS In the total population of children with CP aged 4 to 14 years, 60% had more than minor problems with hand function (>MACS I). Independence in age-relevant, daily manual activities (MACS I-II) was noted in 87% of children with spastic unilateral CP and in 63% of children with spastic bilateral CP, but in only 20% of children with dyskinetic CP. According to the House functional classification, both hands were spontaneously and independently used in 55% of children (House 7-8), whereas 5% did not use either of their hands (House 0). Minor increase of flexor muscle tone (Zancolli level 1) was found in 69% of all children. Only 2% were in level 3 in both hands. Spastic thumb-in-palm deformity in 1 hand was found in 25% and in both hands in another 15%. CONCLUSIONS Limitations in hand function are common in all types of CP, but characteristics of the disability vary considerably between different CP subtypes. The MACS classification is useful to evaluate how well children can handle objects in daily activities. The House functional classification describes grip function in each hand separately; the Zancolli classification of finger and wrist extension and the classification of thumb-in-palm deformity according to House give an estimate of dynamic spasticity. All these classifications were shown to be useful in a population-based health care program, but further studies of the psychometric properties are required.

Comparison of the Gross Motor Function Measure and Paediatric Evaluation of Disability Inventory in assessing motor function in children undergoing selective dorsal rhizotomy

This study was designed to compare assessment with the functional outcome measures Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) over time, in children with cerebral palsy (CP) undergoing selective dorsal rhizotomy combined with individualised physiotherapeutic interventions. Using the Gross Motor Function Classification System (GMFCS), 18 children with spastic diplegia were divided into two groups according to age‐related severity of motor function impairment. Data were collected preoperatively, and at 6 and 12 months postoperatively. Both instruments were sensitive to changes in function over time in the series as a whole and in the group with milder impairment, although the PEDI detected significant changes earlier. In the group with more severe impairment, changes in function were detected only with the PEDI, not with the GMFM. Thus, the instruments are to be considered complementary tests, because they measure different aspects of function.

Cerebral palsy in southern Sweden. II. Gross motor function and disabilities

The gross motor function and disabilities in children with cerebral palsy in southern Sweden were investigated and related to clinical features. The study covered the birth year period 1990‐1993 and comprised 167 children, 145 of them born in Sweden and 22 born abroad. The clinical features and gross motor function were analysed at a mean age of 6.8 y. Clinical features were obtained from a continuing healthcare follow‐up programme. Gross motor function was classified according to the Gross Motor Function Classification System (GMFCS). Walking independently was possible for 86% of the hemiplegic, 63% of the pure ataxic, 61% of the diplegic and 21% of the dyskinetic children. None of the tetraplegic children was able to walk. The classification of gross motor function revealed that 59% of the children were categorized into levels I and II (mildly disabled), 14% into level III (moderately disabled) and 27% into levels IV and V (severely disabled). Children born abroad were more severely disabled.

Bilaterality in slipped upper femoral epiphysis

The frequency of slipping and osteoarthritis of the contralateral hip was recorded in 260 patients with slipped upper femoral epiphysis between 1910 and 1960. Twenty-three of these patients (9%) had primary bilateral slipping, 32 (12%) had a contralateral slip diagnosed later during adolescence and a further 104 (40%) had signs of contralateral slipping at follow-up 16 to 66 years later, giving a total of 159 cases (61%) with bilateral slips. Of the 104 slippings diagnosed at follow-up, 25% showed osteoarthritis. It is concluded that, with a slipped epiphysis, prophylactic contralateral pinning should be performed to avoid slipping and to reduce the risk of osteoarthritis.