[Evaluation of adherence and satisfaction with intermittent catheterization in children: Feasibility and first steps of validation of InCaSaQ and ICAS in the pediatric population]. / Évaluation de l'adhérence et de la satisfaction aux sondages intermittents chez l'enfant : étude de faisabilité et premières étapes de validation d'InCaSaQ et ICAS dans la population pédiatrique.

INTRODUCTION: In pediatric neurourology, clean intermittent catheterization (CIC) setting, and then self catheterization learning are important steps for children with neurogenic bladder. There is no adherence and satisfaction evaluation scale for children who are using self or hetero CIC. The aim of this article is to study the feasibility of using InCaSaQ (Intermittent Catheterization Satisfaction Questionnaire) and ICAS (Intermittent Catheterization Adherence Scale) in children, and to validate the first steps. PATIENTS AND METHODS: Scale validation monocentric study. Inclusion criterias were patients with neurogenic bladder, under the age of 18, using CIC (auto or hetero). The questionnaires ICAS and InCaSaQ were sent twice between 2017 March and April, and then filled by the child or his parents depending on who was doing the CIC. The internal concistency (Cronbach's alpha) measured the construct validity. The reproductibility was measured by the intraclass correlation cœfficient (ICC) and the Wilcoxon and McNemar tests. Filling facility was evaluated for each score (evaluation with a 0 to 10 scale from the person who filled the questionnaire). RESULTS: Twenty two patients were included, and 50 questionnaires filled (25 ICAS and 25 InCaSaQ), twice each. Internal consistency was good for InCaSaQ (Cronbach's alpha>0,7) and so was ICAS and InCaSaQ reproductibility (ICC>0,7 for most of the questions). Patients under hetero-CIC had a worse adherence than parents of children under hetero-CIC (ICAS 3,25 versus 0,7 for children under hetero-CIC). The lowest InCaSaQ item was the way of throwing away their catheter. CONCLUSIONS: ICAS and InCaSaQ are interesting tools which can be used for children under auto and hetero-CIC. Studies with more patients will be necessary for finalizing the validation of these scales in the pediatric population. LEVEL OF PROOF: 4.

[Preliminary study: Lower urinary tract dysfunction and anorectal disorders in children with cerebral palsy]. / Étude préliminaire des troubles vésico-sphinctériens et anorectaux chez les enfants paralysés cérébraux.

GOAL: Describe lower urinary tract dysfunction and anorectal disorders to children with cerebral palsy (CP), indicating their impact on quality of life. MATERIALS: This was a prospective single-center study. A data collection was: type of PC, Gross Motor Function-Classification System (GMF-CS), mainstream education or not, Functional Independence Measure in children (MIF-kid), standardized vesicosphincteric symptoms and quality of life questionnaires (specific issue of impact sphincter dysfunction and generic scale Kidscreen-52). RESULTS: Between January and March 2013, 19 children aged 5-17 years were included, including 16 into mainstream schooling. Of the 19, 16 had bladder and sphincter disorders: 14 urinary incontinence, 3 nycturies, 6 dysuria, 12 urgenturies, no urinary infection. Of the 14 urinary incontinence, 13 were in school. Of the 16 children enrolled, 5 were daytime fecal incontinence and 2 nocturnal fecal incontinence. Functional scores (GMF-CS and MIF-kid) children urinary incontinence were lower than those of children urinary continents (P=0.04 and 0.0007). Ten children had an impact of these disorders on quality of life. All were enrolled, eight (80%) in the mainstream. CONCLUSION: The bladder and sphincter disorders were common in children with CP. They led to an impact on quality of life in more than half of the children studied, mostly educated in mainstream schools.

A survey of medical and paramedical involvement in children with cerebral palsy in Britanny: preliminary results.

AIM: To describe the amount of medical and paramedical involvement in a sample of Breton children with cerebral palsy as a function of the Gross Motor Function Classification System (GMFCS). MATERIALS AND METHODS: This is a transversal descriptive study. All children with cerebral palsy in Brittany were eligible. Parents who accepted to participate were asked to fill in a questionnaire regarding medical and paramedical involvement with their child. RESULTS: One hundred and thirty-three parents participated. 40.6% of the children were level I on the GMFCS, 20.3% II, 12.03% III, 13.53% IV and 13.53% were level V. Thirty-nine percent of the children took at least one medication (of which 43% were antiepileptic drugs). 33.1% of the children had received at least one injection of botulinum toxin within the year. Forty-four percent used a mobility aid. Eighty-five percent of the children had at least one orthotic device, most often a night ankle-foot orthosis. The median number of rehabilitation sessions per week was 3.85 [0.5-11.5]. The frequency and type of sessions were mostly related to the GMFCS level. CONCLUSION: This study reports high levels of medical and paramedical involvement. Studies must attempt to define optimal practice.

Pain assessment in Charcot-Marie-Tooth (CMT) disease.

OBJECTIVE: The objective of our study was to describe and evaluate the prevalence of chronic pain in persons with Charcot-Marie-Tooth (CMT) disease during a multidisciplinary consultation at the Center of Reference for Neuromuscular Diseases. METHODOLOGY: This prospective study was conducted between 2008 and 2010, it was a partnership between a Center of Reference for Neuromuscular Diseases (Centre de référence des maladies neuromusculaires [CRMD]) and a Department for the Assessment and Treatment of Pain (Département d'évaluation et de traitement de la douleur [DETD]). The evaluation consisted in a complete assessment of each patient during the first multidisciplinary consultation, with a previously established diagnosis validated by genetic testing, by various specialists: neurologist, PM&R physician, pain management specialist and physiotherapist. The evaluation tools used were Visual Analogical Scale (VAS), Hospital Anxiety and Depression Scale (HAD), DN4 scale, Neuropathic Pain Symptom Inventory (NPSI) (if DN4≥4), Pain Questionnaire of Saint Antoine (QDSA) (if DN4<4), body representation to define the painful areas, Overall Neuropathy Limitations Scale (ONLS), Medical Research Council scale (MRC), Short Questionnaire on Pain (QCD), VAS during transfers, self-care, getting dressed and physiotherapy sessions and quantified use of analgesics. RESULTS: A total of 50 patients were included (28 women, 22 men); two patients (one man and one woman) were discarded from the study because of missing pain assessment data. Mean age was 47years (R: 14-85), in average the symptoms had been present for the past 20years (R: 0.3-68), most patients had little impairment, the mean MRC was 53 (R: 36-60), with CMT1A being predominant (CMT1A: 76.9%, CMTX: 13.5%, CMT2: 5.8%, CMT4: 3.8%). It is noted that 65.4% of patients reported some pain with a mean duration of pain at 140months (R: 5-660). The mean VAS was 5.5 (R: 1-10), greater than 4 in 79.4% of cases, requiring the use of analgesics in 38.4% of cases (step 1: 60%, step 2: 40% on the WHO pain relief ladder). The predominant location of the pain was distal, peripheral and symmetric (64.7%); furthermore the feet were affected in 80% of cases. DN4 was positive in 40.6% of painful patients. In 62.5% of the cases, the pain did not have an underlying mechanical origin. The emotional impact remained quite modest (HAD: A=8; D=5). Patients with CMT1A seemed less affected by pain (P=0.03). CONCLUSION: This original study describes the pain in patients with CMT disease during a primary multidisciplinary consultation. We see that in 66% of cases, patients do report some pain, this pain is usually moderate, preferentially located in the extremities and is symmetric. In 62.5% of cases, the pain has a neuromuscular origin with a positive DN4 in 50% of cases in this etiology. In our study, patients with CMT1A report less pain than patients with other CMT types. This disease being quite rare (rare disease), the number of patients did not allow us to bring up statistically significant results. The pain must be thoroughly screened for because of its frequency in persons with CMT.

Lower extremity surgery in muscular dystrophy.

INTRODUCTION: The natural progression of neuromuscular diseases results in inevitable musculotendinous contractures, most often in spite of early treatment. Surgery corrects this. The aim of this study was to evaluate the results of tendon surgery in these cases. MATERIALS AND METHODS: Twenty children with muscular dystrophy underwent hip surgery (tensor fascia lata tenectomy, rectus femoris and sartorius tenotomy), knee surgery (gracilis, semitendinosus and semimembranosus tenotomies) and/or ankle surgery (lengthening or tenotomy of the Achilles tendon with or without posterior tibial tendon transfer). Articular range of motion was evaluated preoperatively, 6 months after surgery, at 1 year and at the final follow-up (7.4 years). RESULTS: Three children underwent surgery before they had lost their walking capacity, eight soon afterwards, and nine long afterwards. Surgery was bilateral in all cases: 38 hips, 12 knees and 36 ankles with 22 posterior tibial tendon transfers. Hip extension, which was limited by a 30° flexion contracture improved to 10° and stabilized at 14° at the final follow-up. Adduction which was -19° before surgery increased to 35° and had stabilized at 32° at the final follow-up. Knee flessum (38°) was only slightly improved (24°) and had regressed at the final follow-up. Equinus deformity (42°) was corrected to 9° of dorsal flexion with a slight loss (2°) at the final follow-up. Varus was improved from 11° to 6°, but this had regressed at 1 year (10°) and at the final follow-up (11°). Achilles tendon tenotomies resulted in 28° of dorsal flexion at 6 months compared to 20° with lengthening. Correction of varus was better with posterior tibial tendon transfer at 6 months, 1 year and at the final follow-up (11° versus 5°). In the three patients who underwent surgery before losing their walking ability, one continued walking for 1 year and two for 6 months. None of the eight patients who underwent surgery soon after losing ambulation were able to regain walking. All patients were able to continue device assisted upright positioning for a mean 3 years. DISCUSSION: Hip and ankle surgery can release contractures. In the knees, surgery should be restricted to cases with knee flessum greater than 30°. The best results are obtained with extensive m. tensor fascia lata tenectomy and posterior tibial tendon transfer. Achilles tendon tenotomy results in better recovery of dorsal flexion. Surgery is indicated when contractures are severe and the patient is no longer able to walk. The aim should not be to continue walking but to release contractures, so that the patient can continue assisted upright positioning as long as possible. Especially in cases of asymmetric contractures, surgery can help slow the progression of scoliosis.