Bladder dysfunction in Duchenne muscular dystrophy

Although bladder function is thought to be unaffected in Duchenne muscular dystrophy, 46/88 boys interviewed had urinary problems. Nine underwent video urodynamics, showing in eight a small capacity, hyperreflexic bladder, and in the ninth (post spinal surgery) hyperreflexia and detrusor sphincter dyssynergia. Urinary dysfunction is a treatable feature of DMD.     

Bladder dysfunction in Duchenne muscular dystrophy.

Although bladder function is thought to be unaffected in Duchenne muscular dystrophy, 46/88 boys interviewed had urinary problems. Nine underwent video urodynamics, showing in eight a small capacity, hyperreflexic bladder, and in the ninth (post spinal surgery) hyperreflexia and detrusor sphincter dyssynergia. Urinary dysfunction is a treatable feature of DMD.     

Urologic monitoring of children with meningomyelocele

In addition to neuropediatrical, neurosurgical and orthopedical care a continuous urological surveillance is indicated in children with myelodysplasia. Urodynamic assessment, voiding cystourethrography, kidney- and residual urine, ultrasound, excretory urography and radioisotope studies at birth and periodically until puberty are performed to evaluate the urinary tract. Early and specific treatment of bladder dysfunction is necessary to prevent urinary infection and preserve renal parenchyma. Urodynamic testing showed evidence of neurogenic bladder dysfunction in 13 of 14 evaluated children (including 8 newborns): detrusor-sphinkter-dyssynergia in 57 percent, detrusor hyperreflexia in 21 percent and detrusor areflexia in 14 percent. 4 of 5 children with detrusor-sphkincter-dyssynergia were treated effectively with anticholinergic agents and clean intermittend catheterization.     

血浆和尿中内皮素、一氧化氮在神经源性膀胱中的检测及意义

目的探讨内皮素和一氧化氮在神经源性膀胱病人血浆和尿中含量的变化及其临床意义。方法将2003年10月至2004年8月明确诊断的22例患儿作为实验组，均为反射亢进型膀胱源性膀胱。留取空腹血和晨尿，采用酶联免疫方法检测血浆和尿中内皮素（ET—1），一氧化氮（NO）含量，选取同期入院年龄匹配的儿童的血尿标本（腹股沟斜和鞘膜积液30例）设为对照组。结果实验组22例，血浆 ET—1含量（0．487±0．039）fmol／mL，NO 含量（69．57±36．68）μmol／L，尿中 ET —1含量为（0.453±0．029）fmol／mL，NO 含量（392．36±121．1）μmol／L。对照组血浆 ET —1含量（0．494±0.117） fmol／mL，NO 含量（74．56±11．39）μmol／L，尿中 ET—1含量（0．548±0.073）fmol／mL，NO 含量（268．92±88．38）μmol／L。实验组中血浆 ET—1含量高于尿中 ET—1含量（P ＜0．01），尿中 ET—1含量低于对照组（P ＜0．05），尿中 NO 含量高于对照组（P ＜0．05）；血浆中 ET —1、NO 含量较对照组无明显差异（P ＞0.05）。结论反射亢进型神经源性膀胱病人血浆中 ET 和 NO 的含量处于正常水平；而尿中 ET—1和NO 比例失衡。     

Neurogenic bladder in children: urodynamic studies and results with medical conservative treatment

We present the urodynamic findings and therapeutic results in 25 children with neurogenic bladder, ranging in age from 2 months to 14 years. We observed a significant reduction of urinary infections and vesicoureteral refluxes following medical treatment based on the urodynamic findings. Furthermore, we observed a worse evolution in patients with pre-voiding bladder pressure in excess of 50 cm H2O, incontinence being more difficult to treat in children with urethral closure pressure profile below 47 cm H2O. In our series, anticholinergic treatment improved hypertonia and hyperreflexia which disappeared in 66.6% of cases. Total continence was achieved in 37.5% and improved in 29.1% of children.     

脊髓损伤致神经源性膀胱尿动力检查及上尿路早期形态功能改变

目的 建造高位脊髓损伤所致神经源性膀胱的大鼠模型,研究神经源性膀胱排尿功能的变化及肾脏功能形态学的早期变化.方法 60只雌性Wistar大鼠随机分为模型组和对照组.模型组于T10处横断脊髓.对照组只在相同平面暴露脊髓.不做横断.分别在术后6、10、14周对2组大鼠行尿动力学检测;取尿液检测尿酶NAG水平及细菌培养、鉴定;取血测尿素氮;取.肾脏标本观察形态学变化.结果 尿动力学检杳模型组大鼠在膀胱充盈期表现为逼尿肌反射亢进、逼尿肌漏尿点压力增高、顺应性初期增高后随着造模时间延长逐渐下降.模型组大鼠均存在泌尿系感染,尿酶NAG增高明显.大体标本未见明显输尿管增粗、肾积水.光镜下可见肾盂上皮有炎性浸润.肾小管出现损害,间质可见灶状分布的纤维化.肾小球未见明显异常.电镜下见肾小管上皮细胞肿胀.细胞器减少,肾小管刷状缘、线粒体嵴排列紊乱,基底膜基本完整.结论脊髓损伤后神经源性膀胱排尿功能发生明显异常,肾小管间质病变在评价肾功能损害上比肾小球病理改变更有意义.     

脊髓发育不良性膀胱逼尿肌形态学改变及临床意义

目的　探讨脊髓发育不良性膀胱的病理变化与尿流动力学分型之间的关系以及临床意义。方法　采用免疫组化和Masson三色法观察 38例脊髓发育不良性膀胱 (MB)及 11例正常膀胱组织标本中平滑肌与结缔组织之间的结构变化。结果　①MB组和正常组对α 平滑肌肌动蛋白均表达 ,而对α 横纹肌肌动蛋白均不表达 ;②MB组的结缔组织含量逼尿肌反射低下组 (0 .2 2 3± 0 .0 97) ,逼尿肌反射亢进组 (0 .5 4± 0 .11)均明显高于正常组 (0 .13± 0 .0 8) (P <0 .0 1) ,而逼尿肌反射亢进组与反射低下 /无反射组之间差异亦有显著性意义 (P <0 .0 5 ) ;③逼尿肌反射亢进组中有 17例(6 8.0 % )可见平滑肌细胞核排列紊乱 ,而反射低下 /无反射组肌束明显细小。结论　MB的病理改变与尿流动力学结果密切相关 ,临床上应采用相应的手术方法进行处理     

神经源性膀胱扩大术远期疗效观察

目的 评价保留膀胱黏膜的双层肠管浆肌层膀胱扩大术的远期疗效.方法 病例选择条件:术前有明确支配膀胱的神经性损害,膀胱容量明显小于同龄儿童的正常值,同时伴有Ⅳ度以上膀胱输尿管反流,经过一段时间的口服抗胆碱能药物、清洁间歇导尿等保守治疗后,膀胱内压仍然较高,输尿管反流无明显改善,我们对符合上述条件的75例神经源性膀胱患儿行切除部分逼尿肌保留膀胱黏膜的双层肠浆肌层膀胱扩大术,同时根据患儿的具体情况选择性联合应用膀胱输尿管移植抗反流、膀胱颈紧缩、膀胱颈悬吊等手术方式,术后对患儿进行长期随访,对手术前后临床症状、肾脏功能、尿流动力学(膀胱容量、残余尿、逼尿肌压和顺应性)等方面进行评价.结果 75例手术患儿中68例获得随访,随访时间平均4.3年,术后无一例发生肾脏功能衰竭,45例获得一定的临床治疗改善.23例术后在尿流动力学、临床症状方面无明显改善.结论 术后膀胱容量不能有效扩大是导致术后疗效不理想的重要原因,保留膀胱黏膜的双层肠浆肌层膀胱扩大术是治疗神经源性膀胱的一种方法,但该方法有待改进.     

Hazard of botulinum toxin type A injection into the detrusor in patients with an artificial urinary sphincter

We performed a retrospective chart review of two patients with a previously implanted artificial urinary sphincter (AUS) who became incontinent after the injection of botulinum toxin type A into the detrusor. Two prepubertal males with a myelomeningocele, neuropathic bladder and urinary incontinence developed bladder hypertonicity. Treatment by botulinum toxin type A injection into the detrusor resulted in a loss of fluid from the balloon and recurrent incontinence. This method of treatment in patients with a previously implanted AUS should be avoided, as not only does it appear to be of only temporary benefit, but also as demonstrated by these cases it can damage the AUS system.     

Bladder augmentation and/or continent urinary diversion: 10-year experience.

OBJECTIVE: We evaluated the success and the long-term complications associated with augmentation cystoplasty and/or continent urinary diversion in children with urinary incontinence due to neurogenic or malformed bladder. MATERIALS AND METHODS: The records of 23 patients (12 females, 11 males) who underwent such procedures between 1994 and 2004 were reviewed retrospectively. The most common type of augmentation cystoplasty was ileocystoplasty. The most common type of conduit for the urinary continent diversion was appendicovesicostomy. Combined bladder neck closure was not performed systematically. Neocystoureterostomy was done in 14 refluxing ureters. RESULTS: Of the 21 patients who underwent augmentation cystoplasty, only one was incontinent after the procedure and required reconstruction of the bladder neck using the Young-Dees procedure. The most common complications were stomal stenosis and bladder stone formation. CONCLUSION: Augmentation cystoplasty and continent urinary diversion procedures can increase the functional capacity of the small bladder and allow the majority of patients to achieve continence while preserving renal function. Combined bladder neck closure is not necessary to obtain urinary continence; on the contrary, it eliminates a useful pop-off mechanism. Neocystouretrostomy is not required for every refluxing ureter unless it can be performed on the original bladder. Bladder stones and stomal stenosis are the most significant long-term complications in these patients.     

Neurofibromatosis involving the lower urinary tract in children A report of three cases and a review of the literature

Three children with neurofibromatosis involving the lower urinary tract are reported and their clinical, radiological and pathological findings are described. Lower urinary tract involvement in neurofibromatosis has previously been reported in 17 children, 12 of whom had other stigmata of von Recklinghausens disease. Lower urinary tract involvement may be asymptomatic and can be found incidentally. Every case with neurofibromatosis presenting with what may appear to be even only insignificant urinary symptoms should be thoroughly investigated urologically. Symptoms are usually related to urinary tract obstruction or neurogenic bladder dysfunction due to the involvement of the nerves supplying the bladder. Urinary diversion is the treatment of choice as surgical removal of the extensive tumour is seldom feasible. Following treatment the prognosis for survival appears to be good.     

Replacement of the urinary bladder with rectum

The rectum can be used as a substitute for the urinary bladder such that the normal outlet for feces at the anus is left undisturbed and continent. Perfect and complete continence is achieved by creating a narrow neck of the rectal bladder with a sphincter around it and a narrow urinary outlet, a neourethra, also with a sphincter around it. Three different types of anomaly necessitating reconstruction of the urinary bladder were operated upon by this procedure. Three patients are doing well in respect to micturition and defacation, having achieved total continence for both.     

Continent urinary diversion in the epispadias-exstrophy complex

Despite improvements in modern surgical reconstructive techniques, many patients with epispadias-exstrophy continue to experience urinary incontinence. Continent diversion is commonly performed to achieve urinary continence and improve quality of life. In this work we describe the population that can be considered for continent urinary diversion, consider the benefits and implications of concurrent augmentation and bladder neck closure, and review recent literature regarding continence outcomes and common complications. Even in this complex patient population, urinary continence can be reliably achieved by bladder augmentation and the use of intermittent catheterization via a catheterizable cutaneous stoma with or without closure of the bladder neck.     

Expression of virulence factors among Escherichia coli isolated from the periurethra and urine of children with neurogenic bladder on intermittent catheterization

BACKGROUND: Patients with neurogenic bladder caused by spinal cord injury or myelomeningocele empty their bladder several times a day by intermittent catheterization. Bacteriuria without symptoms of infection is frequently present in these patients. Occasionally a clone of Escherichia coli that has been carried for weeks without symptoms causes a symptomatic urinary tract infection. Virulence factors are commonly expressed among E. coli causing infection in patients with normal urinary tracts. However, it is unknown whether expression of virulence factors by an E. coli clone colonizing the neurogenic bladder increases the risk of subsequent infection. In this study we examined the prevalence of virulence factor expression among E. coli isolated from the periurethra and urine of patients with neurogenic bladder. METHODS: The prevalence of virulence factors was examined among E. coli isolated from the periurethra and urine in patients with neurogenic bladder who received intermittent catheterization and were followed for 6 months. Representative isolates from the 37 clonal types of E. coli detected in the periurethra and urine of children with neurogenic bladder were assessed for O antigen, hemolysin, aerobactin, serum resistance and type I and P-adhesin. RESULTS: All clones were serum-resistant and expressed type I adhesin, none expressed aerobactin and two expressed hemolysin. The presence of P-adhesin was not unique to clones associated with symptomatic infection. The presence of P-adhesin carried for weeks in a clone did not predict subsequent infection in the neurogenic bladder. CONCLUSION: Bacterial virulence factors did not predict infection of the neurogenic bladder.     

Vesicoureteral reflux in the primate. IV. Infection as cause of prolonged high-grade reflux

High-grade reflux commonly lasts longer than moderate reflux, which disappears with maturation of the ureterovesical junction. It is known that ureteral function is affected by urinary tract infection from studies in experimental animals, as well as through clinical findings in patients with upper tract infection. Whether infection might affect the ability of the ureter to prolong high-grade reflux was questioned. This observation might explain why high-grade reflux does not disappear as rapidly as moderate reflux in children with recurrent urinary tract infections. Vesicoureteral reflux was produced surgically in combination with bladder neck obstruction using infant monkeys. The reflux thus produced was high grade with ureteral dilation and caliectasis. In the group of animals in which the bladder neck obstruction was relieved surgically, the reflux rapidly disappeared. In the other group, a bladder infection was produced with Escherichia coli at the time of release of the bladder neck obstruction. The reflux lasted significantly longer, an average of 18 months. Therefore, it appears that treatment of urinary tract infection rather than vesicoureteral reflux is the most important therapy.     

Surgical management of the neuropathic bladder

In a simplified view, the "normal" bladder, through a multifaceted neuromuscular event, allows the basic functions of urinary storage and emptying. More specifically, the urinary bladder accommodates increasing urinary volume with little to no increase in vesicular pressure while maintaining continence. The normal act of emptying integrates the relaxation of the urinary sphincters (external and internal) with the subsequent bladder contraction to void to completion when full. There are a multitude of conditions, both congenital and acquired, that can affect the bladder's ability to perform these functions in a smooth and coordinated fashion. The most common causes of pediatric bladder dysfunction necessitating surgical intervention are those associated with spina bifida/myelodysplasia, posterior urethral valves, and bladder exstrophy. Over the last 2 decades, the evolution of complex reconstruction for lower urinary tract dysfunction has resulted in an improved quality of life for children afflicted with upper urinary tract changes or incontinence despite maximum utilization of nonoperative therapies. Because each patient represents a unique therapeutic entity, an individualized approach to each child is recommended.     

Ultrasonographical aspects of urinary schistosomiasis: Assessment of morphological lesions in the upper and lower urinary tract

Ultrasonographic evaluation of 213 patients with urinary schistosomiasis in different age groups was performed in an endemic area of the Democratic Republic of Congo. The results were compared with 94 age matched controls without urinary schistosomiasis. In patients the bladder showed thickening of the wall, polypoid lesions of the mucosa and bladder wall, calcifications and urinary retention. Urinary tract obstruction, predominantly unilateral, was demonstrated. The lesions increased in severity with the intensity of infection, parallel to an increase in ova excretion. Children aged between 8 and 19 years were most severely affected. Pathological lesions of the upper urinary tract were rare in patients over 25 years of age. The combination of morphological abnormalities was interpreted as being specific for urinary schistosomiasis as they did not occur in the control group. It is suggested that real time ultrasonography may be used to identifiy morphological lesions in urinary schistosomiasis.     

保留膀胱黏膜肠浆肌层膀胱扩大术的尿流动力学评价

目的评价神经性膀胱行保留膀胱黏膜双层小肠浆肌层膀胱扩大术后的尿流动力学变化。方法45例逼尿肌反射亢进型神经性膀胱行保留膀胱黏膜的小肠浆肌层膀胱扩大术，男26例，女19例，年龄4～14岁。36例随访2年，手术前后行尿流动力学检查。结果手术后膀胱容量、膀胱顺应性、尿流率较术前增加，逼尿肌压降低，残余尿量／膀胱容量较术前减小，无抑制收缩减轻。逼尿肌括约肌不协调和尿道闭合压无改变。30例临床症状改善。结论保留膀胱黏膜的双层肠浆肌层膀胱扩大术可增加膀胱容量及顺应性，降低逼尿肌压，减轻逼尿肌反射亢进的程度，逼尿肌括约肌不协调以及尿道闭合压无明显变化。     

Renal transplantation and congenital anomaly of the kidney and urinary tract

Paediatric renal transplantation in children differs from adult series because of the high incidence of abnormalities of the lower urinary tract. We report our experience concerning five children with end-stage renal disease and associated bladder dysfunction due to posterior urethral valves, and lower urinary tract abnormalities (‘valve bladder’), who underwent renal transplantation without prior correction of the uropathy. Of 151 paediatric patients (aged 34 months to 23 years) who underwent renal transplantation, 58 had lower urinary tract abnormalities, and 42 underwent surgical correction of the uropathy prior to renal transplantation. In 15 patients, the uropathy was fibrotic bladder secondary to posterior urethral valves (‘valve bladder’). After clinical and urodynamic evaluation, all 15 patients were considered as candidates for bladder augmentation. In 10 patients, the bladder augmentation was performed prior to renal transplantation. The remaining five patients presented with oligoanuria at the time of the evaluation, and the decision to consider bladder augmentation was postponed until the post-transplant period. At the time of renal transplantation, 2 of the 5 patients underwent ureterostomy, and three had a ureteral reimplant associated with a suprapubic catheter for 2 months. Periodic assessment of renal function and bladder capacity/compliance was performed, as was renal ultrasound. After 4 months, the five patients were re-evaluated for the need for bladder augmentation. At 1, 2, 3 and 4 months follow-up, the five patients had normal renal function with improvement of bladder capacity and absence of hydronephrosis. In 3 of the 5 children, bladder augmentation was judged to be no longer necessary due to the complete restoration of clinical and urodynamic parameters. Therefore, renal transplantation can be safely performed without pre-emptive reconstruction of the lower urinary tract. When possible, ureteral reimplantation is recommended, even in a very small valve bladder, since the initial indication for bladder augmentation may be modified once normal diuresis has been restored.     

A method of determining bladder sphincter reflex in children

A new method of recording the bladder sphincter reflux is suggested. It lies in carrying out urethra profilography followed by stimulation investigations. The data of profilography and stimulation investigations are compared. Standard parameters of the bladder sphincter reflux were defined in patients without derangement of urinary function and were compared to the findings obtained in patients suffering from disorders fo urinary function (hypotonia, hyporeflexia, reflux). The method can be used for diagnosing disordered interrelations between the urinary bladder and sphincter apparatus (urethra) in patients with neurogenous urinary bladder and other pathology.