Effect of selective dorsal rhizotomy on daily care and comfort in non-walking children and adolescents with severe spasticity.

BACKGROUND: In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort. AIM: To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions. METHODS: Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m-4y 3m). All eligible patients (n = 24, years 2009-2014) were included. RESULTS: Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m-19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1-9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes. INTERPRETATION: SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR.

Comparison of psychomotor outcome in patients with perinatal asphyxia with versus without therapeutic hypothermia at 4 years using the Ages and Stages Questionnaire screening tool.

INTRODUCTION: Therapeutic hypothermia improves outcome after perinatal asphyxia. The Ages and Stages Questionnaire is a screening tool to detect neurodevelopmental delay. In this study we examined the outcome of patients with perinatal asphyxia (defined as Apgar score <5 at 10 min, or continued need for resuscitation, or pH < 7.00 in umbilical cord or within one hour after birth) with and without therapeutic hypothermia treatment at the age of four years. METHODS: Cohort study of patients with perinatal asphyxia admitted to the Neonatal Intensive Care Units of the VU University Medical Center, Amsterdam and the Wilhelmina Children's Hospital, Utrecht in the year 2008. Parents were asked to fill out the 48 months Ages and Stages Questionnaire (ASQ). In Wilhelmina Children's Hospital treatment with therapeutic hypothermia was implemented in 2008, in the VU University Medical Center in 2009, providing a historical cohort. RESULTS/DISCUSSION: Twenty-three questionnaires were evaluated. Response rate of questionnaires for the VU Medical Center was 63% (n = 10) and Wilhelmina's Childrens Hospital 93% (n = 13). No significant differences were found in the mean scores between both groups. However, the untreated group scored more frequently under the -2 SD threshold. In the fine motor skills domain the difference was statistically significant (p = 0.031). In the treated group no patients developed cerebral palsy and in the untreated group two patients developed cerebral palsy. CONCLUSION: In this study patients treated with hypothermia tend to have a better neurodevelopmental outcome. No significant differences were found between the two groups, apart from the fine motor skills.

General movements in infants born from mothers with early-onset hypertensive disorders of pregnancy in relation to one year's neurodevelopmental outcome.

BACKGROUND: Assessment of general movements (GMs) at three months is considered useful for prediction of adverse neurological outcome in high risk infants. AIMS: To study the prevalence of abnormal GMs in infants born from women with early-onset hypertensive disorders of pregnancy and the association of GMs with neurodevelopmental outcome at one year. STUDY DESIGN: Prospective study, part of a randomised controlled trial of pre-birth management strategies. SUBJECTS: Infants born from women with early-onset hypertensive disorders of pregnancy. OUTCOME MEASURES: GMs observation and neurological examination at term and three months corrected age; at one year neurological examination and Bayley Scales of Infant Development. RESULTS: From 216 women included, 175 of 178 surviving infants (mean gestational age 31.6 weeks [SD 2.3], mean birth weight 1346 grams [SD 458]), were examined at three months. At term age normal, mildly abnormal and definitely abnormal GMs were observed in 54%, 36% and 10% respectively; and at three months in 47%, 40% and 13%. Mildly or definitely abnormal GMs at three months were not associated with abnormal neurological examination at one year, however, they were associated with delayed psychomotor development at one year (p = 0.01). CONCLUSIONS: In this prospective study, including small for gestational age, preterm infants about half of them did not have normal GMs at term and three months. There was no association of GMs at term nor three months with neurological outcome at one year, but there was a significant association of GMs at three months with one year psychomotor development.

Motor outcome at the age of one after perinatal hypoxic-ischemic encephalopathy.

OBJECTIVE: The aim of this report is to describe the motor outcome in one year-old children who were born at full-term with perinatal hypoxic-ischemic encephalopathy (HIE). Relationships between motor ability tests and neurological examination at one year, and between these tests and neonatal brain magnetic resonance imaging (MRI) were investigated. PARTICIPANTS AND METHODS: 32 surviving children, born full-term with perinatal HIE, are included in this report. All children had a neonatal MRI. At one year, motor ability was assessed with the Alberta Infant Motor Scale and the Bayley Scales of Infant Development (2nd version). Neurological examinations included the neurological optimality score (NOS). RESULTS: At one year, 14 children (44%) had normal motor ability, nine (28%) had mildly delayed, and nine had significantly delayed motor ability. The NOS ranged from 14.6-27 points. All children with normal motor ability had (near) optimal NOS, however, not all children with high NOS had normal motor ability. Eleven children (34%) had normal neonatal MRI; at one year, six of them had normal, and five had mildly delayed motor ability. Eight children with normal motor ability showed abnormalities on neonatal MRI. CONCLUSION: Neonatal brain MRI does not predict motor outcome at one year. Motor ability tests and neurological examinations should be used in a complementary manner to describe outcome after HIE.

One-year stability of the Measure of Processes of Care.

BACKGROUND: The Measure of Processes of Care (MPOC) is a 56-item self-administered measure designed to examine what parents of a child with a chronic health problem think of the services they and their child receive, and to measure the extent to which these services are family-centred. Reliability and validity of the MPOC were established in prior studies. The aim of the present study was to assess the 1-year stability of the MPOC to justify its use as an evaluative tool. METHODS: Nine paediatric rehabilitation centres in the Netherlands participated in this short longitudinal survey study. Subjects were 205 parents (response rate 74.8%) of children aged 1-18 years who received care in one of the participating paediatric rehabilitation centres. All subjects filled out two MPOCs with a 1-year interval. RESULTS: All correlations between the scale scores of the MPOC at the first and second administration were relatively high and significant (range: 0.443-0.609, all P < 0.001), demonstrating high inter-individual stability. However, all mean scale scores, except for Providing General Information, significantly reduced after 1 year. CONCLUSIONS: The MPOC has a moderate 1-year stability. However, because of its tendency to score lower when repeated after 1 year, its use as an evaluative follow-up instrument to assess the effectiveness of a programme intervention is restricted.

Transparency and tuning of rehabilitation care for children with cerebral palsy: a multiple case study in five children with complex needs.

PURPOSE: Generally, there is a lack of knowledge whether and how parent-identified problems, treatment goals and treatment activities are tuned in the treatment of children with cerebral palsy. This study aimed to observe whether parent-identified problems and treatment goals were documented ('transparency' of the rehabilitation process), and to examine the relationship ('tuning') of parent-identified problems to treatment goals and to treatment activities of children with cerebral palsy in pediatric rehabilitation in the Netherlands. METHOD: Five school-aged subjects with cerebral palsy were observed in detail during physical, occupational, and speech therapy, and their written reports were studied. Parent-identified problems, treatment goals, and treatment activities were linked to the ICF components, domains and categories and the amount of agreement was determined by code comparison. RESULTS: Only two children with a total of six parent-identified problems could be documented. Forty-five treatment goals were identified and 72 treatment activities were observed. The analyses indicated some tuning of parent-identified problems, goals and activities in all subjects, but only four of the treatment activities were perfectly tuned to treatment goals as well as to parent-identified problems. CONCLUSIONS: We conclude that transparency and tuning of the pediatric treatment process described in the present study could be improved substantially. Several suggestions for improving the transparency and tuning of the treatment process are discussed.

Parent participation in paediatric rehabilitation treatment centres in the Netherlands: a parents' viewpoint.

AIM: The importance of family-centred care and services has been increasingly emphasized in paediatric rehabilitation. One aspect of family-centred care is parent involvement in their child's treatment. The aims of this study were (1) to describe how, and to what extent parents are involved in the paediatric rehabilitation treatment process in the Netherlands; (2) to determine the level of parents' satisfaction about the services they and their child have received; and (3) to describe what ideas parents have to enhance their involvement in the treatment process. METHODS: A total of 679 parents of children aged 1-20 years who participated in our longitudinal study on family centred care in the Netherlands. The children had various diagnoses and were treated in nine out of 23 Dutch paediatric rehabilitation centres. A random sample of 75 parents was interviewed within 4 weeks after completion of the Measure of Processes of Care and the Client Satisfaction Questionnaire. A Quality of Care cycle with six stages was used to structure the evaluation. RESULTS: The data showed that parents are involved in all stages of their child's rehabilitation process in various ways. The average level of parent satisfaction about the services received was high. According to the interviewed parents, the communication between professionals and parents, parents' involvement in goal setting, and parents' involvement in treatment could be improved upon. CONCLUSION: Parents are to a large extent involved in all stages of the treatment process in Dutch paediatric rehabilitation settings. Although parents valued the services received, they suggested various ways to enhance parent participation.

The measure of processes of care (MPOC): validation of the Dutch translation.

AIM: The objective was to validate the Dutch translation of the Canadian measure of processes of care (MPOC) questionnaire for use in children's rehabilitation centres in the Netherlands. MPOC consists of 56 items (assessing five domains) and was designed to find out what parents of children with chronic health problems think of the services they and their child receive and to measure the extent to which these services are family-centred. METHODS: The Canadian validation procedures were followed, consisting of construct and concurrent validation and reliability analyses. Participants were parents of 427 children aged 1-18 years recruited through nine children's rehabilitation centres in the Netherlands. RESULTS: The construct validity of the Dutch version of MPOC (MPOC-NL) was examined with confirmative analyses of the scale structure. These analyses all supported the construct validity of MPOC-NL. MPOC-NL showed adequate internal consistency, with Cronbach's alpha ranging from 0.80 to 0.95. The intraclass correlation coefficients (ICCs) ranged from 0.79 to 0.94, which demonstrated good stability of MPOC-NL. The Spearman correlations between MPOC-NL scores and satisfaction questions ranged from 0.39 to 0.73, and thus supported the construct validity of MPOC-NL. Correlations between MPOC-NL scores and a question about parents' stress in relation to services received were moderately negative (r(s) = -0.28 to -0.39). CONCLUSION: The construct and concurrent validity of MPOC-NL was shown by confirmative analyses of the original Canadian scale structure, and by modest Spearman correlations between MPOC-NL scores and satisfaction and stress variables. MPOC-NL is internally consistent and reliable.

Diffusion-weighted MRI in severe neonatal hypoxic ischaemia: the white cerebrum.

Presently, prognosis of infants with perinatal hypoxia-ischaemia is estimated using the Sarnat scale, which combines clinical evaluation and electroencephalography, in combination with magnetic resonance imaging (MRI) and or evoked potentials. While the value of conventional MRI is limited during the first week of life, diffusion-weighted MRI demonstrates effects of acute cerebral ischaemia within hours of onset. However, the exact value of diffusion MRI in the prognosis of infants with hypoxia-ischaemia has to be established in larger follow-up studies. In this report we describe 5 term (post-conceptional age 40 1/7 to 41 2/7 week) neonates with severe hypoxia-ischaemia and a characteristic pattern of diffusion changes. T 1 -weighted images showed a hyperintense cortical signal in only one case and extensive hyperintensity in the basal nuclei in all 5 cases. T 2 -weighted images showed nearly complete loss of cortical delineation in three cases. Increased signal on diffusion-weighted images was seen throughout all cortical and subcortical areas while the cerebellum remained normal. This pattern, which we refer to as the "white cerebrum", is most readily apparent on coronal images. The apparent diffusion coefficient (ADC) was calculated and compared to that of four control infants. In the cortex ADC values were lowered (0.70 +/- 0.17 micro m 2/msec [mean +/- standard deviation (SD)]; controls [n = 4]: 1.18 +/- 0.02 micro m 2/msec) as compared to values of ADC in the cerebellum (1.31 +/- 0.06 micro m 2/msec [mean +/- SD]; controls [n = 4]: 1.25 +/- 0.06 micro m 2/msec). All infants died in the perinatal period. In summary, the "white cerebrum" on diffusion-weighted MRI indicates severe neonatal hypoxia-ischaemia and is the counterpart of the white cerebellum on CT.