Repeated detrusor injection of botulinum toxin A for neurogenic bladder in children: A long term option?

AIMS: Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Intermittent catheterization: Clinical practice guidelines from Association Française d'Urologie (AFU), Groupe de Neuro-urologie de Langue Française (GENULF), Société Française de Médecine Physique et de Réadaptation (SOFMER) and Société Interdisciplinaire Francophone d'UroDynamique et de Pelvi-Périnéologie (SIFUD-PP).

INTRODUCTION: Our objective was to provide guidelines covering all aspects of intermittent catheterisation (intermittent self-catheterisation and third-party intermittent catheterisation). MATERIALS AND METHODS: A 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. RESULTS: This 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. CONCLUSION: These 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….

Multicentric evaluation of the adherence to Peristeen® transanal irrigation system in children.

BACKGROUND: Since 2009 in France, the Peristeen® transanal irrigation (TAI) device has represented an alternative treatment of faecal incontinence (FI). OBJECTIVE: The primary objective of this study was to assess the mid-term adherence to TAI in paediatric patients. The secondary objective was to identify factors determining TAI continuation. METHODS: This observational study conducted in 5 French paediatric centres prospectively reviewed from March to May 2012 all children educated in TAI for at least 9months. RESULTS: We included 149 children (mean [SD] age 10.6 [4.1] years) educated in TAI. Children mainly had neurogenic disorders (52.3%) or congenital malformations (30.9%). The main symptoms motivating TAI initiation were recurring faecaloma (59.7%) and daily FI (65.1%). At last follow-up (mean 14 [7.4] months), 129 (86.6%) children continued the TAI procedure, independent of pathology or age. The main motivation was resolution of FI and/or constipation (77.3%). In total, 107 (82.9%) children fulfilled the initial therapeutic contract established with their healthcare professional before TAI initiation was met. Twenty children had stopped the TAI when they answered the questionnaire, at a mean duration of 16 (8.4) months. The reasons were mainly "lack of motivation" (45%), "poor tolerance" (35%), "difficulties" performing the procedure (35%) and "inefficacy" (30%). Factors related to continuation were performing at least one TAI procedure under a nurse's supervision during the initial training and prescribing TAI at a daily frequency (P=0.014 and P=0.04). Continuing constipation treatment after the training session was a factor in discontinuation (P=0.024). CONCLUSION: This study reports a very high mid-term adherence to TAI in a paediatric cohort, provided that the training is pragmatic, personalized and repeated.

[Preliminary results from the French study on prenatal repair for fetal myelomeningoceles (the PRIUM study)]. / Résultats préliminaires de l'étude PRIUM : programme de réparation in utero des myéloméningocèles.

OBJECTIVES: To present the preliminary results of the PRIUM study, a French pilot study of prenatal repair for myelomeningoceles (MMC). MATERIAL AND METHODS: The fetal surgery for MMC is offered in cases of patients that did not opt for termination of pregnancy. RESULTS: 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. CONCLUSION: 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.

[Definition of botulinum toxin failure in neurogenic detrusor overactivity: Preliminary results of the DETOX survey]. / Définition et prise en charge de l'échec d'une première injection de toxine botulique Botox ® 200 U pour hyperactivité détrusorienne neurogène: résultats de l'enquête DETOX.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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 pressure>40 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%). CONCLUSION: 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.

[Dermal sinus: a neurosurgical emergency]. / Sinus dermique : une urgence neurochirurgicale.

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.

[Endoscopic management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children]. / Prise en charge endoscopique de l'incontinence urinaire des vessies neurologiques d'origine médullaire de l'enfant.

INTRODUCTION: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Bladder and bowel function in children with congenital spinal lipomatus malformations. A retrospective study of 114 cases]. / Devenir vésicosphinctérien et anorectal des enfants ayant un lipome du cône. Analyse rétrospective sur 114 cas.

OBJECTIVE: To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Botulinum toxin type A for neurogenic detrusor overactivity due to spinal cord lesions in children: a retrospective study of seven cases.

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.

[Botulinum toxin type A in children: evaluation of indications with a review of the literature]. / Toxine botulinique type A chez l'enfant: évaluation des indications à partir d'une revue de la littérature.

INTRODUCTION: The aim of this study is to review the literature to indications of botulinum toxin type A in children. METHOD: We review the international literature from 1990 to 2002 by querying the Pubmed database with the keywords "children" and "botulinum toxin". RESULTS: 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. DISCUSSION: 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. CONCLUSION: 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.

Function of the upper limb after surgery for obstetric brachial plexus palsy.

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.

[Pickrell intervention in children for anal incontinence secondary to anorectal malformation]. / Intervention de Pickrell chez l'enfant pour incontinence anale secondaire à une malformation anorectale.

STUDY AIM: The aim of this study was to report the results of unilateral Pickrell procedure in children with fecal incontinence secondary to anal atresia. PATIENTS AND METHOD: 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. RESULTS: 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. CONCLUSION: 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.

[Acute urine retention: a rare mode of revelation of cervico-dorsal syringomyelia caused by cyproheptadine]. / Rétention aiguë d'urines: un mode de révélation rare d'une syringomyélie cervicodorsale à l'occasion de la prise de cyproheptadine.

BACKGROUND: 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. CASE REPORT: 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. CONCLUSION: Cyproheptadine may have revealed latent neurogenic bladder in this case, although urodynamic studies, performed 3 months later, detected no bladder dysfunction.

Lumbosacral agenesis. Three cases of reconstruction using Cotrel-Dubousset or L-rod instrumentation.

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.

[Diagnosis of spinal cord tumor in pediatric orthopedics]. / Diagnostic d'une tumeur de la moelle épinière en orthopédie pédiatrique.

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.