Dan Soudant

Long-term follow-up on continuous intrathecal Baclofen therapy in non-ambulant children with intractable spastic Cerebral Palsy

BACKGROUND Little is known about the long-term effects of Continuous intrathecal Baclofen (CITB) therapy in non-ambulant children with intractable spastic Cerebral Palsy (CP). AIM To determine whether short-term beneficial effects of CITB therapy are present at the long-term, and whether caregivers would choose CITB therapy for their child again considering the advantages and disadvantages encountered over the years. METHODS Long-term follow-up data were obtained of the children whom had previously participated in a RCT on CITB by the Dutch Study Group on Spasticity. Quality of life (QoL) was assessed by the Child Health Questionnaire (CHQ), current satisfaction with CITB was measured by use of a Visual Analogue Scale regarding previously set treatment goals, functioning in daily living was determined by a questionnaire concerning functioning of the child, and possible detrimental effects of CITB therapy encountered over the years were noted. All data were acquired via interview of the caregivers. RESULTS All 17 children of the former trial participated in this study. Previously identified significant positive effects on pain (CHQ 46.8 vs. 74.38, p = 0.002; VAS 2.4 vs. 8.01, p = 0.02), ease of care (VAS 2.0 vs. 7.26, p = 0.00), and mental health (CHQ 67.2 vs. 75.94, p = 0.010) were still present at the end of the trial. Novel significant positive effects were noted at six to nine years follow-up, i.e. significantly improved scores on the Parent Impact - Emotional subscale (CHQ 66.0 vs. 78.2, p = 0.008), Parent Impact - Time subscale (CHQ 68.9 vs. 91.72, p = 0.002), and the Physical Summary (CHQ 17.6 vs. 27.4, p = 0.019) compared to baseline. Ninety-four percent of the caregivers would choose CITB treatment again for their child again. CONCLUSION The beneficial effects of CITB are present at the long term and caregiver satisfaction is high.

The effect of intrathecal baclofen treatment on activities of daily life in children and young adults with cerebral palsy and progressive neurological disorders

INTRODUCTION Intrathecal baclofen (ITB) treatment is applied in patients with spastic cerebral palsy (SCP), dystonic cerebral palsy (DCP) and progressive neurological disease (PND). Our aim was to investigate whether ITB treatment has a different effect on activities of daily life (ADL) in these groups. METHOD A retrospective and cross-sectional survey was conducted using a questionnaire to assess the qualitative effect of ITB (Likert scale) on different domains of functioning (mobility, personal care, communication, comfort) and satisfaction with the results. Groups were compared using non-parametric statistics. RESULTS Questionnaires were completed for 68 patients (39 SCP, 13 DCP, 16 PND). Satisfaction scores were relatively high in all groups (7-8) and the positive effect on personal care and communication was similar in all groups. The PND group had the shortest follow-up and scored significantly less favourably for the effect on mobility and comfort. DISCUSSION This is the first study to show that ITB treatment has similar effects on personal care and communication in stable and progressive neurological disease. The decrease in mobility in the PND group is likely due to the progressive nature of the disease. The different effect on comfort between groups is mainly due to the smaller effect on startles in the PND group.

Percutaneous radiofrequency lesions adjacent to the dorsal root ganglion alleviate spasticity and pain in children with cerebral palsy: pilot study in 17 patients

BackgroundCerebral palsy (CP) may cause severe spasticity, requiring neurosurgical procedures. The most common neurosurgical procedures are continuous infusion of intrathecal baclofen and selective dorsal rhizotomy. Both are invasive and complex procedures. We hypothesized that a percutaneous radiofrequency lesion of the dorsal root ganglion (RF-DRG) could be a simple and safe alternative treatment. We undertook a pilot study to test this hypothesis.MethodsWe performed an RF-DRG procedure in 17 consecutive CP patients with severe hip flexor/adductor spasms accompanied by pain or care-giving difficulties. Six children were systematically evaluated at baseline, and 1 month and 6 months after treatment by means of the Modified Ashworth Scale (MAS), Gross Motor Function Measure (GMFM) and a self-made caregivers questionnaire. Eleven subsequent children were evaluated using a Visual Analogue Scale (VAS) for spasticity, pain and ease of care.ResultsA total of 19 RF-DRG treatments were performed in 17 patients. We found a small improvement in muscle tone measured by MAS, but no effect on the GMFM scale. Despite this, the caregivers of these six treated children unanimously stated that the quality of life of their children had indeed improved after the RF-DRG. In the subsequent 11 children we found improvements in all VAS scores, in a range comparable to the conventional treatment options.ConclusionRF-DRG is a promising new treatment option for severe spasticity in CP patients, and its definitive effectiveness remains to be defined in a randomised controlled trial.

Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome

OBJECTIVE Tight filum syndrome (TFS) is caused by a thick (abnormal T1 MRI), shortened (low-lying conus), or non-elastic filum (strictly normal MRI). We carefully analyzed children treated for suspect TFS with or without radiological abnormalities. METHODS Twenty-five children, operated between 2002 and 2009, were retrospectively identified. All children had been evaluated by a multidisciplinary team preoperatively. Symptoms, signs and diagnostic test results were categorized (neurologic, urologic, orthopedic, dermatologic) and compared pre- and one year postoperatively. Normal MR was defined as conus medullaris (CM) at or above mid-body L2 and filum diameter less than 2 mm. Occult TFS (OTFS) was defined as TFS with normal MR. RESULTS DEMOGRAPHICS 17 girls, 8 boys, age 2-18 years, including 11 syndromal children. CLINICAL PRESENTATION all children had problems in the neurologic category and at least one other category: urologic (n = 17), orthopedic (n = 21), and dermatologic (n = 11). MR findings: low-lying CM (n = 14) including 2 with thick filum, normal CM but fatty filum (n = 2), strictly normal (n = 9). Clinical outcome one year postoperatively: neurologic 20 improved, 5 stabilized; urologic 13 improved, 3 stabilized, 1 worsened; orthopedic (8 children presenting with scoliosis) 3 improved, 4 stabilized, 1 worsened. All children with OTFS (n = 9) improved in at least one and 8 improved in all affected categories. CONCLUSIONS Children with strong clinical suspicion for TFS (≥ 2 affected categories) with or without abnormal MR findings will likely benefit from surgery. In such cases we suggest a detailed full spine MR, a multidisciplinary diagnostic work-up, and eventual untethering through an interlaminar microsurgical approach.

Diagnostic radiation exposure in children with spinal dysraphism: an estimation of the cumulative effective dose in a cohort of 135 children from The Netherlands

Objective Based on the assumption that children with spinal dysraphism are exposed to a large amount of ionising radiation for diagnostic purposes, our objective was to estimate this exposure, expressed in cumulative effective dose. Design Retrospective cohort study. Settings The Netherlands. Patients 135 patients with spinal dysraphism and under 18 years of age treated at our institution between 1991 and 2010. Results A total of 5874 radiological procedures were assessed of which 2916 (49.6%) involved ionising radiation. Mean cumulative effective dose of a child with spinal dysraphism during childhood was 23 mSv, while the individual cumulative effective dose ranged from 0.1 to 103 mSv. Although direct radiography accounted for 81.7% of examinations, the largest contributors to the cumulative effective dose were fluoroscopic examinations (40.4% of total cumulative effective dose). Conclusions Exposure to ionising radiation and associated cancer risk were lower than expected. Nevertheless, the use of ionising radiation should always be justified and the medical benefits should outweigh the risk of health detriment, especially in children.

Quality of Life of Children with Cerebral Palsy: A Cross-Sectional KIDSCREEN study in the Southern part of the Netherlands.

OBJECTIVE To compare the quality of life (QoL) of 8-18 year old children with cerebral palsy (CP) in the Southern part of The Netherlands to a sample of European children from the general population and to investigate factors associated with possible differences. DESIGN A cross-sectional KIDSCREEN-52 (by-proxy version) study. SUBJECTS/PATIENTS The parents of 80 out of 81 children (mean age 13.4 years, SD 2.98; 49 boys, 31 girls; Gross Motor Function Classification System (GMFCS) level 1: 21, 2: 5, 3: 16, 4: 18, 5: 20) agreed to participate. METHODS Two-sample T-tests were used to compare domain scores between groups. Regression analysis was used to identify factors associated with deviant QoL scores. RESULTS Parents reported significantly higher QoL for the domains of parent relation & home life and school environment. On the other hand significantly lower QoL was reported for the domains of psychical well-being, social support & peers, and social acceptance. Factors associated with deviant QoL scores were lower cognitive levels, less communication skills, and higher GMFCS levels. CONCLUSION This study exposed several problem domains of QoL in children with CP living in the Southern part of the Netherlands. Several possible explanations for these findings are given. This information can be used to inform caregivers and service-providers.

The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism

PurposeOur goal was to validate the hypothesis that the lumbosacral angle (LSA) increases in children with spinal dysraphism who present with progressive symptoms and signs of tethered cord syndrome (TCS), and if so, to determine for which different types and/or levels the LSA would be a valid indicator of progressive TCS. Moreover, we studied the influence of surgical untethering and eventual retethering on the LSA.MethodsWe retrospectively analyzed the data of 33 children with spinal dysraphism and 33 controls with medulloblastoma. We measured the LSA at different moments during follow-up and correlated this with progression in symptomatology.ResultsLSA measurements had an acceptable intra- and interobserver variability, however, some children with severe deformity of the caudal part of the spinal column, and for obvious reasons those with caudal regression syndrome were excluded. LSA measurements in children with spinal dysraphism were significantly different from the control group (mean LSA change, 21.0° and 3.1° respectively). However, both groups were not age-matched, and when dividing both groups into comparable age categories, we no longer observed a significant difference. Moreover, we did not observe a significant difference between 26 children with progressive TCS as opposed to seven children with stable TCS (mean LSA change, 20.6° and 22.4° respectively).ConclusionsWe did not observe significant differences in LSA measurements for children with clinically progressive TCS as opposed to clinically stable TCS. Therefore, the LSA does not help the clinician to determine if there is significant spinal cord tethering, nor if surgical untethering is needed.

Functional electrical stimulation of the ankle dorsiflexors during walking in spastic cerebral palsy: a systematic review

To assess the effect of functional electrical stimulation (FES) of ankle dorsiflexors in children and adolescents with spastic cerebral palsy (CP) during walking.

Conscious sedation or general anaesthetic for intramuscular botulinum toxin injections in children - a two centre cross-sectional prospective audit.

Dear Sir, With great interest we have read the recently published letter by Forrester et al. commenting on the paper by Brochard et al. In his letter he highlighted the technical and clinical factors associated with pain for children in France undergoing botulinum toxin injections under nitrous oxide and anaesthetic cream. He stated there is no established standard of care for sedation of children or adolescents who receive botulinum toxin (type A) injections. One study by Bakheit collected data from 17 different European centres. Practice varied widely between the centres, from the use of no anaesthetic or sedation in some institutions, to the routine use of general anaesthetic. Forrester et al. performed a similar study in Australia. This was a prospective observational cross-sectional audit at two paediatric tertiary hospitals. The aim of the study was to compare caregiver satisfaction and perception of procedure related pain and distress for intramuscular Botulinum toxin injections administered to children and adolescents, using 2 methods of analgesia/sedation: conscious sedation using nitrous oxide and topical local anaesthetic versus general anaesthesia. This study showed carer and clinicians perception of patient distress upon entering the procedure room and applying the mask was similar with conscious sedation and general anaesthesia. The carers perceived the pain caused by the procedure under conscious sedation as significantly higher than under general anaesthesia. However there was no significant difference in overall satisfaction with the analgesia/sedation given in both procedures. These studies mainly focussed on pain perception and satisfaction. In our point of view in the procedure of intramuscular botulinum toxin injections there are several other aspects which should be considered.