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Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort.Patients who underwent at least 4 IDI-TBA between 2005 and 2017 for neurogenic bladder related issues were included (detrusor overactivity and low compliance). Clinical and cystometric data were collected before and after the first injection and after the last injection. The primary endpoint was the proportion of patients with non-abnormal cystometry (no detrusor overactivity and normal compliance). Secondary outcomes were the evolution of the observed bladder capacity/expected ratio, surgical complications and acquired kidney impairment.From the 832 patients referred to our institution for neurogenic bladder, 48 underwent IDI-TBA, and 17 at least 4 injections. Among them, a total of 95 procedures were performed (median per patient 5 [4-8]). While the first injection had a significant effect for 82.3% patients, the last injection improved the medical condition for only 53.0% cases. The bladder capacity ratio, initially 36.1%, increased to 80.3% after the first injection but decreased to a level of 57.1% at last. After a median follow-up of 57 [34-102] months, no severe complications were reported but 11.8% of patients presented with repeated pyelonephritis. A bladder augmentation surgery was finally indicated for 35.3% cases.Despite a low complication rate and impressive cystometric results after the first injection, IDI-TBA efficacy decreased with time and repetition. These findings prone a long-term follow-up and a "à-la-carte" management of this specific population depending on the long-term response to IDI-TBA.

IntroductionOur objective was to provide guidelines covering all aspects of intermittent catheterisation (intermittent self-catheterisation and third-party intermittent catheterisation).Materials and methodsA systematic review of the literature based on Pubmed, Embase, Google scholar was initiated in December 2014 and updated in April 2019. Given the lack of robust data and the numerous unresolved controversial issues, guidelines were established based on the formal consensus of experts from steering, scoring and review panels.ResultsThis allowed the formulation of 78 guidelines, extending from guidelines on indications for intermittent catheterisation, modalities for training and implementation, choice of equipment, management of bacteriuria and urinary tract infections, to the implementation of intermittent catheterisation in paediatric, geriatric populations, benign prostatic hyperplasia patients and continent urinary diversion patients with a cutaneous reservoir as well as other complications. These guidelines are pertinent to both intermittent self-catheterisation and third-party intermittent catheterisation.ConclusionThese are the first comprehensive guidelines specifically aimed at intermittent catheterisation and extend to all aspects of intermittent catheterisation. They assist in the clinical decision-making process, specifically in relation to indications and modalities of intermittent catheterisation options. These guidelines are intended for urologists, gynaecologists, geriatricians, paediatricians, neurologists, physical and rehabilitation physicians, general practitioners and other health professionals including nurses, carers…; IntroductionL’objectif était de réaliser des recommandations sur le cathétérisme intermittent dans toutes ses composantes.Matériel et méthodesUne revue systématique de la littérature à partir de Pubmed, Embase, Google scholar a été menée en décembre 2014 avec mise à jour en avril 2019. Devant l’absence de données suffisamment robustes et compte tenu des controverses concernant cette thématique, la méthode de recommandations par consensus formalisé d’experts a été appliquée. Des comités de pilotage, cotation et de lecture ont été constitués.RésultatsAu final, 78 recommandations ont pu être élaborées. Elles portent sur les indications, les modalités d’apprentissage et de réalisation, le choix du matériel, la gestion de la colonisation et de l’infection urinaire, sa réalisation dans les populations pédiatriques, gériatriques, chez les hommes ayant une hyperplasie bénigne de la prostate et chez les patients ayant une dérivation urinaire cutanée continente et la gestion des complications. Elles portent à la fois sur l’autosondage et l’hétérosondage aussi bien en structure de soins qu’en ville.ConclusionIl s’agit des premières recommandations portant spécifiquement sur le cathétérisme intermittent et incluant toutes ses composantes. Elles vont permettre d’aider les utilisateurs dans leur décision clinique relative aux indications et aux modalités du cathétérisme intermittent. Ces recommandations s’adressent aux cliniciens urologues, gynécologues, gériatres, pédiatres, neurologues, médecins de médecine physique et de réadaptation, médecins généralistes ainsi qu’autres professionnels de santé notamment les infirmières, les aidants…

Resume Le terme spina bifida regroupe plusieurs types de malformations congenitales, dont les dysraphismes spinaux fermes. Parmi ceux-ci, 10 % sont des sinus dermiques : communication ouverte au travers d’un pertuis entre la peau et l’espace intradural dont la principale complication est une infection du systeme nerveux central (SNC). Nous rapportons l’observation d’un enfant porteur d’une depression cutanee de la region lombaire, initialement consideree comme une fossette sacro-coccygienne. Aucune indication neurochirurgicale n’avait ete posee. A l’âge de cinq mois, puis de un an, l’enfant a presente une infection intra-medullaire. L’imagerie par resonnance magnetique (IRM) a alors mis en evidence des empyemes cerebraux, un œdeme et des abces intra-medullaires. Une intervention neurochirurgicale a ete realisee en urgence au decours de la deuxieme myelite, qui a confirme l’existence du sinus dermique avec une moelle attachee basse et permis de decouvrir un kyste dermoide accole au pertuis. Deux ans apres l’intervention l’enfant presentait une paraplegie des deux membres inferieurs associee a une vessie et un colon neurologiques. Cette observation a pour interet de rappeler la difference entre une fossette sacro-coccygienne et un sinus dermique, associe a un kyste dermoide dans 50 % cas. Les consequences neurologiques des complications infectieuses dues aux sinus dermiques negliges rendent le traitement prophylactique neurochirurgical urgent. L’autre interet de cette observation est de presenter la prise en charge tres specialisee des consequences de la paraplegie du petit enfant.

Resume Introduction La prise en charge urologique des vessies neurologiques d’origine medullaire de l’enfant a pour objectif d’obtenir une continence sociale tout en preservant le haut appareil, en combinant le catheterisme vesical intermittent aux anticholinergiques. L’objectif de cette etude etait de rapporter les resultats sur la continence de la prise en charge endoscopique vesico-sphincterienne des enfants avec intolerance ou echec du traitement de premiere intention. Patients et methodes Sur les 364 enfants suivis pour vessie neurologique dans notre institution, 22 ont necessite une prise en charge endoscopique entre 2000 et 2012. L’incontinence urinaire etait liee a une hyperactivite detrusorienne pour 16 et a une insuffisance sphincterienne pour 13, necessitant une ou plusieurs injections de toxine botulique A (TBA) intradetrusorienne et/ou de dextranomere-acide hyaluronique (Dx/Ha) dans le col vesical. La continence etait reevaluee entre six et huit semaines par le score de Schulte-Baukloh. Resultats Au terme d’un suivi moyen de quatre ans, 16 enfants avaient recu 54 injections de TBA et 13 enfants avaient recu 24 injections de Dx/Ha. La continence sociale, definie par un score 0 ou 1, etait acquise rapidement apres injection de TBA et necessitait de repeter les injections tous les 8,7 mois (6–12) avec une tres faible morbidite. Concernant le Dx/Ha, 69 % des enfants ont ete ameliores de maniere significative apres la premiere injection et avec de meilleurs resultats chez les filles, passant d’un score 1 a un score 0 ou d’un score 2–3 a un score 1. Conclusion Une prise en charge endoscopique adaptee a permis une amelioration de la continence chez deux tiers des enfants en echec apres un traitement de premiere intention de leur vessie neurologique, permettant de differer ou d’eviter une intervention chirurgicale lourde.

Les dysraphismes vertebromedullaires sont la premiere cause d’atteinte medullaire chez l’enfant. Les dysraphismes fermes, maladie uniquement locoregionale, entrent souvent dans un contexte polymalformatif avec une deficience neuro-orthopedique et des troubles sphincteriens. Les dysraphismes ouverts, anomalie generale du systeme nerveux central, comportent en plus des troubles cognitifs une malhabilete manuelle. Le passage de l’adolescent en milieu adulte obeit a des regles. Le suivi en milieu adulte est peu different des atteintes medullaires acquises.

There is currently no guideline regarding the management of neurogenic detrusor overactivity (NDO) refractory to intra-detrusor botulinum toxin injections. The primary objective of the present study was to find a consensus definition of failure of botulinum toxin intra-detrusor injections for NDO. The secondary objective was to report current trends in the managment of NDO refractory to botulinum toxin.A survey was created, based on data drawn from current literature, and sent via e-mail to all the experts form the Group for research in neurourology in french language (GENULF) and from the comittee of neurourology of the French urological association (AFU). The experts who did not answer to the first e-mail were contacted again twice. Main results from the survey are presented and expressed as numbers and proportions.Out of the 42 experts contacted, 21 responded to the survey. Nineteen participants considered that the definition of failure should be a combination of clinical and urodynamics criteria. Among the urodynamics criteria, the persistence of a maximum detrusor pressure40 cm H2O was the most supported by the experts (18/21, 85%). According to the vast majority of participants (19/21, 90.5%), the impact of injections on urinary incontinence should be included in the definition of failure. Regarding the management, most experts considered that the first line treatment in case of failure of a first intra-detrusor injection of Botox(®) 200 U should be a repeat injection of Botox(®) at a higher dosage (300 U) (15/20, 75%), regardless of the presence or not of urodynamics risk factors of upper tract damage (16/20, 80%).This work has provided a first overview of the definition of failure of intra-detrusor injections of botulinum toxin in the management of NDO. For 90.5% of the experts involved, the definition of failure should be clinical and urodynamic and most participants (75%) considered that, in case of failure of a first injection of Botox(®) 200 U, repeat injection of Botox(®) 300 U should be the first line treatment.

To present the preliminary results of the PRIUM study, a French pilot study of prenatal repair for myelomeningoceles (MMC).The fetal surgery for MMC is offered in cases of patients that did not opt for termination of pregnancy.Thirty-six patients were referred in an 18-month period. Eight patients were not eligible for prenatal repair. Another type of dysraphism was made in 6 cases (one spina-lipoma, 5 cases of limited dorsal myeloschisis). Twenty-two patients were eligible to fetal surgery. A prenatal repair was performed in three cases (14%). Four patients opted for a conventional postnatal treatment. Fifteen patients opted for termination of the pregnancy.The establishment of a prenatal repair of MMC protocol in France was justified. The experience of the first 18months of this study however suggests that only a limited number of couples will choose this procedure after specialized counseling in a reference center.

Resume Introduction. – L’objectif de ce travail est de proceder a une revue de la litterature pour determiner les indications des injections de toxine botulique type A chez l’enfant. Methode. – Nous avons procede a une analyse de la litterature internationale portant sur les annees 1990–2002 en interrogeant la banque de donnees Pubmed avec pour mots cles children, botulinum toxin. Resultats. – Deux cent quarante-huit articles ont ete selectionnes et 64 retenus. L’analyse de ces articles revele plusieurs interrogations : quelle est la dose optimale, a quelle periodicite pratiquer les injections, a quel âge traiter pour obtenir une efficacite prolongee. Le critere principal de jugement est l’amelioration fonctionnelle. Les effets secondaires notables sont inexistants. L’utilisation principale de la toxine est rapportee dans le traitement de la spasticite du membre inferieur des infirmites motrices cerebrales. Discussion. – Cette revue de la litterature permet de preciser les interets et les limites de l’utilisation de la toxine botulique chez l’enfant. Son efficacite au membre superieur n’est pas prouvee. Les autres indications restent a etudier de maniere plus precise. Conclusion. – Ce travail rapporte l’existence de nombreuses etudes concernant les indications de la toxine botulinique. L’indication essentielle rapportee concerne le membre inferieur de l’enfant infirme moteur cerebral sans pour autant exclure les autres possibilites therapeutiques. Des etudes prospectives, en double aveugle avec des cohortes suffisamment importantes restent a mener.

Resume L’osteogenese imparfaite est une maladie genetique rare caracterisee par une osteopenie, une perte de mobilite et des douleurs osseuses chroniques. Nous avons etudie l’evolution de deux marqueurs osseux, la phosphatase alcaline osseuse et les C-telopeptides chez 46 enfants, âges de 1 mois a 17 ans, traites par le pamidronate. Le biphosphonate est utilise a la dose de 0,5 a 1 mg/kg/jour tous les 2 a 4 mois suivant l’âge des patients. Les parametres biochimiques du remodelage osseux sont doses regulierement durant le traitement. Sous pamidronate le marqueur de resorption diminue des la 1re cure et plus precocement que le marqueur de formation ; cette diminution s’explique par l’action inhibitrice de ce biphosphonate sur l’activite osteoclastique. Cette normalisation se maintient au moins durant 24 mois. L’utilisation de ces dosages permet d’evaluer precocement les variations des marqueurs osseux et d’anticiper les gains ulterieurs de masse osseuse.

OBJECTIF : Revue systematique de la litterature de langue anglaise et francaise sur l'appareillage du tronc dans les maladies neuromusculaires non acquises au sein des techniques de reeducation. METHODES : Recherche documentaire par interrogation des banques de donnees medicales et specialisees et consultation des sites Internet de societes savantes. Mots cles: liste des maladies neuromusculaires retenues par l'Association francaise contre la myopathie associee a orthosis ou orthotic devices ou equipement design. RESULTATS: Trente-sept references sont selectionnees. Sept sont d'ordre general, vingt-et-une concernent la dystrophie muculaire de Duchenne, sept l'amyotrophie spinale infantile, une la dystrophie facio-scapulo-humerale et deux les neuropathies peripheriques. CONCLUSION: Dans la dystrophie musculaire de Duchenne, un consensus existe sur l'inefficacite des ortheses de tronc a « tenir » une scoliose. L'indication du corset est exceptionnelle. Dans l'amyotrophie spinale, le corset type garchois permet d'atteindre l'âge de l'arthrodese vertebrale sans aggravation. Dans les autres pathologies, aucune etude ne permet de repondre aux questions posees.

OBJECTIF: Revue systematique de la litterature de langue anglaise et francaise sur l'appareillage du membre inferieur et la verticalisation dans les maladies neuromusculaires non acquises au sein des techniques de reeducation. METHODES: Recherche documentaire par interrogation des banques de donnees medicales et specialisees et consultation des sites Internet de societes savantes. Mots cles: liste des maladies neuromusculaires retenues par l'Association francaise contre la myopathie associee a orthosis ou orthotic devices ou equipement design. RESULTATS: Quarante-neuf references sont selectionnees dans Embase, Medline, Cochrane, Myodoc, Reedoc. Trente-cinq references concernent la dystrophie musculaire de Duchenne, deux concernent la neuropathie de Charcot-Marie-Tooth, une la myopathie facio-scapulo-humerale, une la dystrophie myotonique, une l'amyotrophie spinale infantile. CONCLUSION: II semble que l'utilisation des ortheses genoux-cheville-pied prolonge une marche aidee et la verticalisation, mais pas la marche fonctionnelle. Les attelles anti-equins portees la nuit retardent la deformation en equin. Dans les autres pathologies, aucune etude ne permet de repondre aux questions posees.

OBJECTIF : Revue systematique de la litterature de langue anglaise et francaise sur les troubles de la continence urinaire et anorectale dans les maladies neuromusculaires non acquises. METHODES : Recherche documentaire par interrogation des banques de donnees medicales et specialisees et consultation des sites Internet de societes savantes. Mots cles: liste des maladies neuromusculaires retenues par l'Association francaise contre la myopathie associee a bladder dysfunction ou urinary dysfunction ou urine incontinence ou feces incontinence ou Incontinence ou fecal incontinence ou urinary incontinence ou bladder disorder, neurogenic. RESULTATS: Trente-deux references bibliographiques sont selectionnees, dix-huit references sont retenues. Douze references se rapportent a la dystrophie myotonique de Steinert, deux a la myasthenie, deux a la dystrophie musculaire de Duchenne, une a la myopathie des ceintures, une a la dermatomyosite. CONCLUSION: La litterature est tres pauvre sur le sujet et consiste essentiellement en des cas rapportes. Ce travail ne permet pas de repondre a la question de l'evaluation et encore moins du traitement des troubles de la continence urinaire et anorectale dans les maladies neuromusculaires.

We reviewed a consecutive series of 33 infants who underwent surgery for obstetric brachial plexus palsy at a mean age of 4.7 months. Of these, 13 with an upper palsy and 20 with a total palsy were treated by nerve reconstruction. Ten were treated by muscle transfer to the shoulder or elbow, and 16 by tendon transfer to the hand. The mean postoperative follow-up was 4 years 8 months. Ten of the 13 children (70%) with an upper palsy regained useful shoulder function and 11 (75%) useful elbow function. Of the 20 children with a total palsy, four (20%) regained useful shoulder function and seven (35%) useful elbow function. Most patients with a total palsy had satisfactory sensation of the hand, but only those with some preoperative hand movement regained satisfactory grasp. The ability to incorporate the palsied arm and hand into a co-ordinated movement pattern correlated with the sensation and prehension of the hand, but not with shoulder and elbow function.

Resume But de l’etude : Evaluer les resultats chez l’enfant de l’anomyoplastie unilaterale de Pickrell pour incontinence anale secondaire a une malformation anorectale. Patients et methode : Entre 1975 et 1997, 23 enfants ont ete operes ; leur âge moyen lors de l’intervention etait de 8,5 ans. Les resultats fonctionnels ont ete evalues systematiquement chez 20 patients par le score clinique de Kelly, complete chez certains enfants par une manometrie anorectale et une electromyographie de l’appareil sphincterien. Resultats : Il n’y a pas eu de mortalite postoperatoire. Une colostomie a ete realisee au debut de l’experience pour un abces local recidivant. Le resultat fonctionnel avec un recul moyen de six ans a ete juge bon dans 25 % des cas, moyen dans 45 % des cas et mauvais dans 30 % des cas. Une amelioration clinique sur la continence a ainsi ete obtenue chez 70 % des patients. Conclusion : L’amelioration de la continence apres intervention de Pickrell depend essentiellement des criteres de selection des patients, de la reeducation pre et postoperatoire et de la realisation de lavements evacuateurs reguliers. En cas de resultat insuffisant, les auteurs proposent une bilateralisation de l’anomyoplastie qui a permis chez neuf enfants d’ameliorer huit fois le resultat.

Le torticolis, qui signifie litteralement cou tordu, n'est pas forcement douloureux; la cervicalgie peut y etre associee. Il est du a une contraction ou une retraction asymetrique des muscles du cou, souvent limitee au muscle sternocleidomastoidien. La tete est inclinee du cote de l'atteinte du muscle mais tournee du cote oppose. Parfois, il existe une translation de la tete du cote sain qui s'explique par l'anatomie musculaire. En effet, de bas en haut, le muscle sternocleidomastoiden (SCM) a un trajet oblique d'avant en arriere et de dedans en dehors ( figure 1 ).

Spinal dysraphisms include various types of congenital malformations. About 10% are dermal sinuses, i.e., a connection between the skin elements and intradural space through an open tract. The major complication of this malformation is a central nervous system infection that occurs most frequently before the age of 5 years. We report the case of a lumbosacral skin defect initially described as a coccygeal pit, which happened to be a dermal sinus with a tethered cord after ultrasound imaging in a newborn infant. No indication for surgery was recognized. Later, the infant experienced cerebral empyema and spinal cord edema when he was 5 months old, while a second infection, with a medullar abscess, occurred 7 months later. Both infections began with high fever and were complicated by neurologic deficits. An emergency surgery was performed during the second myelitis. The operative findings confirmed a dermal sinus and a tethered cord. Moreover, they found a dermoid cyst next to the tract. After 2 years of recovery, the child has neurologic deficits including lower limb paraplegia with neurological bladder and bowel. This observation recalls the importance of the differential diagnosis between a coccygeal pit and a dermal sinus, the latter being associated with a dermoid cyst in 50% of cases. Serious neurologic consequences due to the possible infectious complications can occur when a dermal sinus is neglected. A preventive operative excision of both the dermal sinus and a possible cyst is a surgical emergency for spinal dysraphism.

The urological management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children is intended to achieve social continence while preserving the upper urinary tract, combining clean intermittent catheterization with anticholinergic agents. The objective of this study was to report the results on continence of endoscopic management of bladder and/or sphincter of children with failure or intolerance to first intention therapy.Of the 364 children followed for neurologic bladder in our institution, 22 required endoscopic management between 2000 and 2012. Urinary incontinence was related to detrusor overactivity in 16 children and sphincter deficiency in 13 children, requiring one or several intradetrusor injections of botulinium toxin-A (BTA) and/or dextranomer/hyaluronic acid (Dx/Ha) injection in the bladder neck. Continence was reassessed between six and eight weeks after the last injection using the Schulte-Baukloh score.At the end of the mean follow-up of four years, 16 children received 54 injections of BTA and 13 children had 24 injections of Dx/Ha. Social continence, defined as a score between 0 and 1, was acquired quickly after injection of BTA and required to repeat the injections every 8.7 months (6-12) with a very low morbidity. After the first injection of Dx/Ha, 69% of the children improved significantly their incontinence score (from 1 to 0 or from 2 or 3 to 1) with better results for girls.An appropriate endoscopic management has enabled an improvement of the continence of two-thirds of children who fail first intention treatment for their neurogenic bladder. This is an alternative to delay or avoid major surgery.

To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery.Retrospective analysis of 114 children with a lipomas of the conus, followed in our pediatric neuro-urology department from 1993 to 2010. Several data were collected: bladder and bowel symptoms, bladder and anorectal continence, neurosurgical indication and age, clinical modification after neurosurgery, investigations carried out in pre- and post-surgery treatment, associated bladder and bowel treatment.Forty-nine of the 77 children (63.6%) operated on had never been seen before surgery in our neuro-urology department. Seventy-seven children (67.5%) underwent a neuro-surgery, 60% indicated due to a neurogenic bladder. Before neurosurgery, 66 children (85.7%) had spontaneous miction. Five children (6.5%) had bladder intermittent catheterization. Forty of these patients (56.3%) were continent. After neurosurgery and a specialized consultation in neuro-urology, 54 children (70.1%) were continent. Thirty-seven children (48%) had spontaneous miction. Thirty-seven children (48%) had bladder intermittent catheterization and drug of overactive detrusor. Fifty-two children (67.5%) were constipated after surgery. Seventy-seven percent of the treatments for bowel symptoms were effective in terms of continence.The existence of a neurogenic bladder was one of the main indications for neurosurgery. These results suggest that the complexity of care requires neurosurgical, urological surgeon and neuro-urology physician to achieve the explorations and urinary and digestive treatment in order to preserve renal function and both continences.

Three patients with lumbosacral agenesis underwent surgery to lock the lumbopelvic instability. All three patients had an unstable sitting position and a kyphotic bearing that impaired intestinal transit or hampered further colostomy or ureterostomy. Luque instrumentation with iliac fixation performed according to Galveston was used in one patient. Cotrel-Dubousset instrumentation was used for the two other patients. Autografts plus allografts provided sufficient bone for fusion without requiring lower leg amputations. Increased hip flexion was obtained after pelvic stabilization, but knee flexion contracture remained the same. All patients showed improved intestinal transit or decreased urinary infections, and two patients attained a stable sitting position without aid.

Objective To assess the effectiveness and safety of intradetrusor injections of botulinum toxin type A (BTA) used to treat neurogenic detrusor overactivity in children. Patients and methods We retrospectively reviewed the records of seven children treated at least once (7/7: one injection; 4/7: two and three injections; 2/7: four injections; 1/7: five injections) with intradetrusor BTA injections in 2005–2008, for neurogenic detrusor overactivity with incontinence despite timed bladder catheterizations and anticholinergic agents. Clinical, urodynamic, and imaging study data at baseline were collected. Clinical efficacy criteria were the urinary tract infection (UTI) rate, continence, and need for anticholinergic agents. The following urodynamic data were assessed: maximum catheterized volume without leakage, reflex volume, maximum detrusor pressure, and bladder compliance. Results Seven children received 18 injections. Social continence was achieved from the first injection. No further recurrent lower UTIs occurred. Maximum catheterized volume and reflex volume increased, and maximum detrusor pressure decreased. Detrusor compliance became interpretable and increased. Grade II right vesicoureteral reflux, present at baseline in one patient, resolved after BTA therapy. UTIs were the only adverse effects. Conclusion Injection with BTA proved effective and safe in the short term. This may be an alternative to surgery in children with neurogenic detrusor overactivity.

The aim of this study is to review the literature to indications of botulinum toxin type A in children.We review the international literature from 1990 to 2002 by querying the Pubmed database with the keywords "children" and "botulinum toxin".Two hundred and forty-eight articles are retrieved. We selected the most relevant 64 articles among them. Several questions remain pending: which optimal dose, which periodicity for injections, which optimal age to prolong efficiency? The main criteria to estimate efficiency is functional ability improvement. No significant side effects are noted. The main use of botulinum toxin is the management of the spastic lower limb in cerebral palsy.This review of the literature makes it possible to specify the interests and the operational limits of botulinum toxin in the child. Its effectiveness with the upper limb is not proven. The other indications remain to be studied in a more precise way.This review shows many studies give the indications of botulinum toxin in children. The main indication is the lower limb spaticity in cerebral palsy. New prospective and double-blind studies should be performed with larger samples.

Objectif faire le point en 2013 de l’organisation de la neuro-urologie pediatrique francaise Materiel/methode envoi par mail aux 37 membres de la Societe Francaise d’Urologie Pediatrique d’un questionnaire sur les modes d’exercice, de recrutement, de fonctionnement, de relai ado-adulte et sur le nombre annuel d’actes lies a la neuro-urologie. Resultats 24 reponses documentees de 23 CHU rapportent un recrutement hospitalier majoritaire, l’implication constante du chirurgien pediatre et d’une infirmiere plus rarement d’un medecin de medecine physique, une moyenne annuelle de 5,25 consultations ou reunions dediees a cette activite et l’absence de suivi de recommandations officielles/protocoles pour 20 equipes, un nombre moyen de 50 enfants suivis / an [10–250], des consultations relai13 fois a l’âge moyen de 18 ans, le jeune adulte recevant la synthese pediatrique dans 3 equipes. Conclusion la majorite des equipes francaises d’urologie pediatrique assure une activite de neurourologie dont l’organisation est tres heterogene avec peu de connaissance des recommandations pediatriques necessitant un travail a venir de structuration en terme de diagnostic, prise en charge et de relai vers le suivi adulte.

The aim of this study was to report the results of unilateral Pickrell procedure in children with fecal incontinence secondary to anal atresia.Twenty three children were operated between 1975 and 1997. The mean age at the time of the operation was 8.5 years. Functional results were systematically evaluated by Kelly's clinical score and by anal sphincter electromyography and anal manometry for some patients.There was no postoperative mortality. One colostomy was performed for recurrent local sepsis. The functional result with a mean follow-up of 6 years was estimated to be good in 25%, intermediate in 45% and poor in 30%. Clinical improvement of continence was obtained in 70% of cases.The improvement of continence after Pickrell procedure essentially depends on the patient selection criteria, pre- and postoperative rehabilitation and regular enemas. In the case of poor results, the authors propose bilateral Pickrell procedure which improved the results in eight out of nine children in this group.

Syringomyelia is rare in children aged less than 10 years, and bladder dysfunction is an unlikely first manifestation. This report describes a case of repeated episodes of acute urinary retention in a young girl revealing syringomyelia and Arnold-Chiari malformation.A 2.5 year-old girl was admitted because she was suffering from acute urinary retention. Her poor appetite had been treated with cyproheptadine, a histamine type I blocking drug. Clinical investigation revealed no local cause for this bladder dysfunction except moderate spasticity of the legs. Cystography showed no vesicoureteral reflux. Because the episodes of urinary retention recurred each day, magnetic resonance imaging (MRI) was performed; this showed the typical features of syringomyelia extending from C5 to T11 plus Arnold-Chiari malformation. The cyproheptadine was discontinued and the urinary retention disappeared.Cyproheptadine may have revealed latent neurogenic bladder in this case, although urodynamic studies, performed 3 months later, detected no bladder dysfunction.

Three patients with lumbosacral agenesis underwent surgery to lock the lumbopelvic instability. All three patients had an unstable sitting position and a kyphotic bearing that impaired intestinal transit or hampered further colostomy or ureterostomy. Luque instrumentation with iliac fixation performed according to Galveston was used in one patient. Cotrel-Dubousset instrumentation was used for the two other patients. Autografts plus allografts provided sufficient bone for fusion without requiring lower leg amputations. Increased hip flexion was obtained after pelvic stabilization, but knee flexion contracture remained the same. All patients showed improved intestinal transit or decreased urinary infections, and two patients attained a stable sitting position without aid.

OBJECTIF : Revue systematique de la litterature de langue anglaise et francaise sur l'appareillage du membre superieur dans les maladies neuromusculaires non acquises au sein des techniques de reeducation. METHODES : Recherche documentaire par interrogation des banques de donnees medicales et specialisees et consultation des sites Internet de societes savantes. Mots cles: liste des maladies neuromusculaires retenues par l'Association francaise contre la myopathie associee a orthosis ou orthotic devices ou equipement design. RESULTATS: Trois references sont selectionnees dans Embase, quatre references dans Medline, quatre dans REEDOC. Une seule evoque l'appareillage dans les myosites a inclusion. CONCLUSION: L'analyse de la litterature ne permet pas de retrouver un travail concernant l'appareillage dans le cadre des techniques de reeducation et ne permet donc pas de repondre a la question posee.

Objectif Le syndrome de Currarino (SC) est une maladie genetique dominante autosomique, a penetrance et expression variables, defini par la triade clinique : malformation anorectale (MAR), anomalie du rachis terminal et tumeur presacree. En pratique clinique, il est difficile d’affirmer le diagnostic dans les formes incompletes. Ce travail a pour but de distinguer les formes incompletes des autres diagnostics. Materiel et methodes Il s’agit d’une etude retrospective de 31 enfants ayant au moins 2 criteres de la triade, inclus entre janvier 1978 et decembre 2006. Ils etaient âges de 1 jour a 10 ans (mediane : 2 j) a la date du diagnostic dont 7 avec anomalies echographiques antenatales et 3 avec antecedents familiaux. Selon les anomalies retrouvees, les enfants ont ete classes en 3 groupes : triade complete, 2 elements isoles et 2 elements avec anomalies associees. Les parametres etudies etaient la MAR, le sacrum, l’existence d’une masse presacree, les anomalies associees, les examens complementaires realises, la prise en charge et les resultats. Resultats Six enfants avec SC complet ont ete inclus dans le groupe 1 ; 17 ont ete classes comme SC incomplets, dans le groupe 2 et 8 comme etant un diagnostic differentiel dans le groupe 3. Sur 30 MAR, 9 etaient hautes, 7 intermediaires et 14 basses. Une anomalie sacree existait dans 28 cas, asymetrique dans 4/6 cas de syndrome complet. Sept tumeurs presacrees ont ete mises en evidence. Les anomalies associees etaient surtout medullaires et urogenitales. Une anomalie du gene HLXB9 a ete retrouvee 2 fois sur 3 recherches. Un enfant est decede par volvulus du mesentere avant toute chirurgie. Vingt-neuf enfants ont eu une intervention digestive. Sept enfants ont necessite une intervention neurochirurgicale et 7 une chirurgie urologique. Conclusion Le syndrome de Currarino doit etre evoque devant tout element de la triade. Le bilan doit etre digestif, medullaire et urologique, il doit preciser le type des anomalies du syndrome et les malformations associees. Le SC incomplet peut donc se definir par la presence de 2 elements de la triade isoles ou avec des antecedents familiaux. Le diagnostic doit etre ecarte en cas d’anomalies constitutionnelles associees.

The clinical findings leading to the diagnosis of a spinal cord tumor in pediatric orthopedics are reported with a discussion of the neuro-urological aspects.