Nonneurogenic Neurogenic Bladder Research Articles

In Patients with Neurogenic Detrusor Overactivity and Hinman's Syndrome: Would Intravesical Botox Injections Decrease the Incidence of Symptomatic Urinary Tract Infections.

PurposeTo study the effect of intravesical Botox injection on the incidence of recurrent symptomatic UTI in neurogenic bladder patients with detrusor overactivity.Patients and MethodsThis was a prospective cohort study for patients who received Botox intravesical injection. We included patients with neurogenic bladder with detrusor overactivity and symptomatic recurrent UTI. We compared the number of symptomatic UTIs at six months pre- and post-intravesical Botox injection. Patient files were reviewed for diagnosis, vesico-uretric reflux, hydronephrosis, urodynamic findings pre- and post-injection, and dose of Botox used. Patients were followed for the number of symptomatic UTIs post-Botox injection.ResultsThere were 93 patients diagnosed with neurogenic detrusor overactivity and symptomatic recurrent UTI. Patients were categorized into three categories: Group 1 – adults, Group 2 – pediatrics, Group 3 – non-neurogenic neurogenic bladder (Hinman’s syndrome). Spina bifida was diagnosed in 22 adults (Group 1) and 32 pediatric patients (Group 2). After receiving Botox injection, 75% of all patients (70) had a significant decrease in number of symptomatic UTIs. Urodynamic tests post-injection showed an improvement in bladder capacity, compliance, and detrusor pressure versus baseline in all three groups. Correlation analysis showed significant correlation between decreased symptomatic UTI post-Botox injection and detrusor pressure post-injection as well as bladder compliance; p-value=0.01 and p=0.021, respectively.ConclusionIntravesical Botox injection may decrease incidence of symptomatic UTI in neurogenic detrusor overactivity. This effect seemed to be related to better bladder management.

External Sphincter Injection of Onabotulinum-A Toxin in Treatment of Children with Bladder- Sphincteric Dyssynergia

Abstract Background: The first application of Onabotulinum-A Toxin in urology was its injection into the urinary sphincter to treat neurogenic detrusor-sphincter dyssynergia in quadri-plegic men. Since that first report by Dyskatra et al., in 1988, the results of focal Onabotulinum-A Toxininjections into the sphincter, the bladder wall and lately into the prostate have raised the interest of the urology community in this promising new therapeutic modality. This is an evidence-based review of the current indications, techniques and outcomes of Ona-botulinum-A Toxin injections into the urethral sphincter. Aim of Study: We evaluated the effect of detrusor injection of onabotulinum-A toxin into the urethral urinary sphincter in children with bladder-external sphincter dyssynergia to decrease urethral resistance and improve voiding. Material and Methods: Prospective treatment was per-formed in 25 children 4 to 15 years old (mean age 7) with bladder-neck dyssynergia using onabotulinum-A toxin. Pre-operatively all children were evaluated by ultrasound, voiding cystourethrography, excretory urography, magnetic resonance imaging and urodynamic studies, including pressure flow, electromyography and uroflowmetry. Using a rigid pediatric endoscope and a 4Fr injection needle 50 to 100 IU onabotu-linum-A toxin was injected into the external sphincter at the 5 and 7 o'clock positions. Follow-up was 6 to 15 months. Repeat injections every 6 months were given according to the response with a maximum of 3 injections. Results: Immediately after onabotulinum-A toxin injection all except 1 patient were able to void without catheterization. No acute complications occurred. Postoperatively post-void residual urine decreased by 30%, detrusor leak point pressure decreased significantly by a mean ± SD of 66±18 vs 37±4cm H2O and uroflowmetry showed a marked increase in maximum urine flow of 7±2 vs 17.3±8ml per second. Conclusions: Urethral sphincter onabotulinum-A toxin injection could be considered a reliable treatment modality in children with nonneurogenic neurogenic bladder after the failure of conservative therapy.

Diagnosis of Urination Disorders in Young Patients with Lower Urinary Tract Symptoms of Uncertain Origin

Lower urinary tract symptoms (LUTS) are not uncommon in young men. The etiology of LUTS in men aged up to 50 years is diverse. According to various authors, it is recommended to use a complex urodynamics tests (CUT) to assess LUTS in young men. However, even in modern urological practice, many such patients continue to be treated empirically, and the use of urodynamic studies is still not widely practiced for this group. The objective: assess the clinical significance of CUT in men with LUTS under the age of 50 years. Materials and methods. The results of CUM in patients with LUTS under the age of 50 were evaluated. 87 patients aged 18 to 43 years with LUTS of uncertain origin were examined. Results. The leading diagnoses, with which patients were referred, were chronic prostatitis and neurogenic bladder, which were found in 28 (32.2%) and 22 (25.3%) patients, respectively. 21 (24.1%) patients admitted to the clinic had no primary diagnosis. As a result of the examination, infravesical obstruction (IVO) was found in 32 (36.8%) men, hyperactive bladder in 24 (27.6%), hypoactive bladder in 9 (10.3%) patients, non-neurogenic neurogenic bladder – in 7 (8.0%) and 15 (17.2%) men urological pathology was not detected. Conclusions. We have shown the effectiveness of CUT to clarify the diagnosis in young men under the age of 50 with LUTS. Diagnosed with a significant proportion of patients, urinary disorders have characteristic differences, reflecting the abnormalities found when using CUT. In most patients, the clinical diagnosis was changed after performing CUT. Thus, the use of CUT in young men with LUTS is crucial for a more accurate diagnosis and, therefore, for adequate therapy.

A rare case of female hypospadias associated with congenital heart disease

Female hypospadias is a rare lower urogenital female anomaly. Hypospadias is primarily a congenital defect of males in which the urethra opens on the underside (ventral aspect) of the penis and is one of the most common congenital anomalies in the USA, occurring in about one of every 125 live male births. The corresponding defect in females is opening of the urethra into the vagina, and is quite rare and often associated with other genitourinary anomalies such as Cloacal malformation, female pseudo hermaphrodism, nonneurogenic neurogenic bladder and urethral duplication. Early diagnosis requires a high index of suspicion. Early treatment plays an important role in reducing the psychological and psychosocial impacts, decreases indolent kidney damages and prevents urinary incontinence. Although, interestingly, sometimes, with conservative management and close follow-up (like in our case), symptoms and signs may get better spontaneously over time. The authors report on a 4-year-old girl with hypospadias presented by hydronephrosis and recurrent urinary tract infection in infancy associated with congenital heart disease who was closely observed until spontaneous resolution of her hypospadias.

P1815LONG-TERM OUTCOME OF BLADDER DYSFUNCTION IN CHILDREN

Abstract Background and Aims Bladder dysfunction is an important cause of chronic kidney disease (CKD) due to neurogenic causes such as a spinal lesion (SL), Hinman's syndrome, or non-neurogenic neurogenic bladder (NNNB), and valve bladder syndrome (VBS). In this study, we evaluated the outcomes of neurogenic bladder caused by different etiologies. Method Single-center retrospective study of children with neurogenic bladder (NB) over 13 years (2005-2017). Results Totally, 199 children (134 boys, 67.3%) were included and divided into three groups: Group 1 (75, NNNB), Group 2 (64, SL), and Group 3 (60, VBS). Urodynamic examination revealed impaired bladder capacity in 49 children (57.6%), atonic bladder in 1 child (1.1%), and normal bladder compliance in 35 children (41.1%). Additionally, 113 children (56.8%) experienced clean intermittent catheterization (CIC). A significant improvement in the mean estimated glomerular filtration rate and CKD stages was observed among the three studied groups, but the NNNB group showed the least improvement. Children diagnosed and treated during their first year of life had better outcomes than those who presented at a later age. CIC without the need for surgical intervention was associated with improved renal function. Pyelonephritis and hydronephrosis had significant negative impacts on kidney function Conclusion Early diagnosis and management of NB in infancy are associated with better outcome and preservation of renal function. CIC is associated with better preservation of kidney function, while vesicostomy is associated with a worse outcome. Children with NB caused by SL experienced the best kidney function preservation, while those with VBS experienced the worst.

Ten years’ experience of augmentation cystoplasty for varied indications and its outcome

Background: The aim of the study was to evaluate long term efficacy and complications of augmentation cystoplasty in patients with bladder dysfunction. Methods: Our series comprises of 30 patients undergoing enterocystoplasty from March 2009 till December 2019. Clinical findings and investigations result along with surgical techniques used were noted for these patients. Postoperative complications along with urinary continence and renal outcome were evaluated.Results: Mean age of patients was 7 years and their mean follow up was for 4 years. Major complications occurred in 5 patients which were successfully managed and minor complication in 8 patients. Of these 16 patients were with neurogenic bladder and 14 with non-neurogenic bladder. The primary etiology of non-neurogenic bladder was extrophy epispadias complex (10 patients), posterior uretheral valves (2 patients), anterior uretheral valve (1 patient), and bilateral ectopic ureter (1 patient). The primary etiology of neurogenic bladder was meningomyocele (4 patients), anorectal malformation with vertebral anomalies (7 patients), partial sacral ageneis (4), nonneurogenic neurogenic bladder (1 patient). Relative continence was achieved in 97%. The preoperative serum creatinine and blood urea nitrogen (BUN) at the time of bladder augmentation (termed creatinine-1 and BUN-1)and the serum creatinine and BUN at the last follow up after bladder augmentation (termed creatinine-2 and BUN-2)were sought and compared using chi square test showed statistically significant improvement (p<0.01).Conclusions: Augmentation cystoplasty is a necessary and safe procedure to increase the functional capacity of small contracted and poorly compliant bladder and allows patients to achieve relative continence and preserves renal function.

SUN-104 Long term outcome of childhood bladder dysfunction

Bladder dysfunction is an important cause of chronic kidney disease (CKD). It could be due to neurogenic causes mostly spinal lesion (SL), Hinman syndrome or non-neurogenic neurogenic bladder (NNNB) and following posterior urethral valve ablation named valve bladder syndrome (VBS). A retrospective study of all children with neurogenic bladder (NB) over 12 years duration from single center. 199 children (134, boy, 67.3%), were included and divided into three groups; group1 (75 NNNB), group2 (64 SL) and group 3 (60 VBS). Their mean (SD) age at presentation was 43.96(49.66) month. The mean (SD) duration of follow up 71.79(45.46) month. Urodynamic study was done in 85 children before any surgical intervention, it revealed impaired bladder capacity in 49 children (57.6%), atonic bladder in one child (1.1%) while normal bladder compliance in 35 children (41.1%). 113 children (56.8%) were performing clean intermittent catheterization (CIC). Surgical interventions were required in 86 (43.2%). A significant improvement in the mean eGFR and the CKD stages was observed among the three studied groups but the NNNB group showed the least improvement. Children diagnosed and started management during their first year of life had better outcome. Performing CIC without the need for surgical intervention were associated with more improvement of renal function. Pyelonephritis and hydronephrosis have significant negative impact on kidney function. Renal scars was positive in 62%, but did not have negative effect on eGFR at the last follow up. Forty children (20%) progressed to end stage kidney disease (ESKD), mainly from the VBS group. Renal replacement therapy was performed in 23 children (11.5%); 11 hemodialysis, 11 peritoneal dialysis and one child received renal transplantation. Mortality was 7.5% (15 children), caused y ESKD complications in 10 children, while the remaining 5 children died from non-renal causes. Diagnosis and management of NB in early infancy is associated with better outcome. Successful CIC is more beneficial than vesicostomy and NB caused by spinal lesion is the best regarding preservation of kidney function, while VBS group is the worst.

Management of vesicoureteral reflux in neurogenic bladder.

Vesicoureteral reflux (VUR) is a significant risk factor for pyelonephritis and renal scarring. VUR can occur through a defective ureterovesical junction (UVJ) or an overwhelmed normal UVJ mechanism such as in bladder dysfunction of congenital, acquired, or behavioral etiology. There are numerous causes for the development of a neurogenic bladder from spinal dysraphisms to spinal cord trauma and even centrally based abnormalities in children with apparently normal motor function (inappropriately termed nonneurogenic neurogenic bladder). The foundation of managing reflux in these neurogenic bladders is to maintain low bladder pressures which will commonly mean that compliance will be normal as well. There have been several publications that have shown that if bladder pressures are lowered simply with clean intermittent catheterization and medications that the reflux can resolve spontaneously. Alternatively, the patients that are in need of bladder augmentation can have spontaneous resolution of their reflux with the resulting increase in capacity. Surgical intervention is called for when bladder capacity is adequate and the reflux persists or if it is part of a larger operation to increase capacity and to manage outlet resistance. In some instances, reimplantation is necessary because the ureters interfere with the bladder neck procedure. Aside from open and robotic surgical intervention the use of endoscopic injectable agents is beginning to become more popular especially when combined with intravesical botulinum toxin A injections. Great strides are being made in the management of patients with neurogenic bladders and we are seeing more choices for the urologist to be able to manage these patients.

HPSE2 Mutations in Urofacial Syndrome, Non-Neurogenic Neurogenic Bladder and Lower Urinary Tract Dysfunction

Background: Urofacial syndrome (UFS) is characterised by congenital bladder dysfunction accompanied by a characteristic abnormal grimace upon smiling and crying. In recent years, biallelic mutations of HPSE2 and LRIG2 have been reported in UFS patients. Non-neurogenic neurogenic bladder (NNNB) has a bladder identical to UFS without typical facial features. The aim of this study was to analyse HPSE2 mutations in patients with UFS and NNNB or severe lower urinary tract dysfunction (LUTD) without abnormal facial expression. Methods: Patients with UFS, NNNB and severe LUTD were enrolled in the study. We examined a total of 35 patients from 33 families. There were seven UFS patients from five different families, 21 patients with NNNB and seven with LUTD. HPSE2 gene mutation analysis was performed using the polymerase chain reaction protocol followed by Sanger sequencing in these patients. Results: A twin pair with UFS was found to be homozygous for c.457C>T (p.Arg153*) mutation. No other pathogenetic variant was detected. Conclusion:HPSE2 mutations were found in one UFS family but not detected in patients with NNNB and severe LUTD. Considering the increasingly recognised cases of NNNB that were diagnosed in early childhood period, genetic factors appear to be responsible. Thus, further genetic studies are needed to discover novel associated gene variants in these bladder anomalies.

Urinary tract effects of HPSE2 mutations.

Urofacial syndrome (UFS) is an autosomal recessive congenital disease featuring grimacing and incomplete bladder emptying. Mutations of HPSE2, encoding heparanase 2, a heparanase 1 inhibitor, occur in UFS, but knowledge about the HPSE2 mutation spectrum is limited. Here, seven UFS kindreds with HPSE2 mutations are presented, including one with deleted asparagine 254, suggesting a role for this amino acid, which is conserved in vertebrate orthologs. HPSE2 mutations were absent in 23 non-neurogenic neurogenic bladder probands and, of 439 families with nonsyndromic vesicoureteric reflux, only one carried a putative pathogenic HPSE2 variant. Homozygous Hpse2 mutant mouse bladders contained urine more often than did wild-type organs, phenocopying human UFS. Pelvic ganglia neural cell bodies contained heparanase 1, heparanase 2, and leucine-rich repeats and immunoglobulin-like domains-2 (LRIG2), which is mutated in certain UFS families. In conclusion, heparanase 2 is an autonomic neural protein implicated in bladder emptying, but HPSE2 variants are uncommon in urinary diseases resembling UFS.

Vejiga neurogénica no neurogénica (síndrome de Hinman). Dos tratamientos diferentes a un mismo problema

Nonneurogenic neurogenic bladder, also known as Hinman syndrome, is a rare entity characterized by nonneurogenic urinary bladder dysfunction. It is a diagnosis of exclusion and is assigned to those children that present with clinical, radiologic, and urodynamic characteristics of a neurogenic bladder with external sphincter dyssynergia, but with no evidence of any neurologic alteration. In 1973, Hinman and Baumann were the first to report a case series of 14 children with nonneurogenic neurogenic bladder and treated these patients with bladder reeducation and hypnosis. Bladder re-training and medical treatment have been recognized as effective management. However, when there is established damage to the upper urinary system, or risk thereof, surgery is preferred over conservative treatment. Due to the low incidence of the disease, there is still no consensus as to the most adequate treatment or management, making it essential to describe every case possible in order to gain the necessary knowledge for providing the best treatment for patients with this syndrome.We present the cases of two boys with Hinman syndrome, 10 and 12 years old, for whom conservative treatment was not sufficient given that they already presented with upper urinary system damage. They underwent successful surgical treatment that was individualized for each case, resulting in 2 different solutions for the same problem.

Outcomes of Incontinent Ileovesicostomy in the Pediatric Patient

Ileovesicostomy is a reconstructive option in complex urological cases but pediatric specific outcomes are lacking. We report our results with pediatric ileovesicostomy. We retrospectively evaluated patients younger than 18 years undergoing incontinent ileovesicostomy at Vanderbilt University. History, urinary tract management and operative course were reviewed in the electronic medical record. Particular attention was given to immediate and long-term postoperative complications. Nine patients underwent incontinent ileovesicostomy between 2000 and 2013 at a mean age of 10.3 years (range 1.4 to 15.5). Surgical indication was sequelae of neurogenic or nonneurogenic neurogenic bladder (such as infection or worsening hydronephrosis) in 5 patients, reversal of vesicostomy in 3 and closure of cloacal exstrophy in 1. All 9 patients were thought incapable of reliable clean intermittent catheterization due to family unwillingness, poor social support or patient refusal. Median followup was 11.5 months (mean 48.2, range 1.3 to 144.8). Immediate postoperative complications included ileus requiring total parenteral nutrition and a wound infection in 1 patient. Long-term complications included urinary tract infection in 2 patients (febrile in 1 and positive culture for foul smelling urine in 1), stomal issues in 2 and temporary urethral leakage in 1. Constipation affected 3 children in long-term followup (all with neurogenic bowel preoperatively). Postoperative creatinine was stable or improved in all patients. Ileovesicostomy is a viable approach in children left with few other options, particularly those who are noncompliant or physically/socially unable to handle catheterization. This operation can help keep such patients out of diapers.

Urofacial syndrome: a genetic and congenital disease of aberrant urinary bladder innervation

The urofacial, or Ochoa, syndrome is characterised by congenital urinary bladder dysfunction together with an abnormal grimace upon smiling, laughing and crying. It can present as fetal megacystis. Postnatal features include urinary incontinence and incomplete bladder emptying due to simultaneous detrusor muscle and bladder outlet contractions. Vesicoureteric reflux is often present, and the condition can be complicated by urosepsis and end-stage renal disease. The syndrome has long been postulated to have neural basis, and it can be familial when it is inherited in an autosomal recessive manner. Most individuals with urofacial syndrome genetically studied to date carry biallelic, postulated functionally null mutations of HPSE2 or, less commonly, of LRIG2. Little is known about the biology of the respective encoded proteins, heparanase 2 and leucine-rich repeats and immunoglobulin-like domains 2. Nevertheless, the observations that heparanase 2 can bind heparan sulphate proteolgycans and inhibit heparanase 1 enzymatic activity and that LRIG2 can modulate receptor tyrosine kinase growth factor signalling each point to biological roles relevant to tissue differentiation. Moreover, both heparanase 2 and LRIG2 proteins are detected in autonomic nerves growing into fetal bladders. The collective evidence is consistent with the hypothesis that urofacial syndrome genes code for proteins which work in a common pathway to facilitate neural growth into, and/or function within, the bladder. This molecular pathway may also have relevance to our understanding of the pathogenesis of other lower tract diseases, including Hinman-Allen syndrome, or non-neurogenic neurogenic bladder, and of the subset of individuals who have primary vesicoureteric reflux accompanied by bladder dysfunction.

Vesicoureteric reflux in children

Aim:This study aimed to identify the differences between primary and secondary vesicoureteric reflux (VUR) and the effect of associated bladder abnormalities on kidney function.Patients and Methods:We retrospectively reviewed the medical records of children with VUR who were followed up at King Abdulaziz University Hospital from January 2005 to December 2010. The review included results of radiological investigations and kidney function tests. We used Chi-square test for statistical analysis and paired t-test to compare group means for initial and last creatinine levels.Results:Ninety-nine children were included in this study. Twenty (20.2%) had primary VUR, 11 had high-grade VUR, while 9 had low-grade reflux. All children with low-grade VUR had normal dimercaptosuccinic acid (DMSA). Renal scars were present in 72% of the children with high-grade VUR. The mean creatinine levels (initial and last) for both groups were normal. Seventy-nine (79.8%) children had secondary VUR, which was due to posterior urethral valves (PUV) (46.8%), neurogenic bladder caused by meningomyelocele (25.3%), non-neurogenic neurogenic bladder (NNB) (21.5%), or neurogenic bladder associated with prune belly syndrome (6.3%). Children with NNB, meningomyelocele and PUV had high creatinine at presentation with no considerable worsening of their kidney functions during the last visit. Renal scars were present in 49.4% of the children with secondary VUR.Conclusion:Children with primary VUR and normal bladder had good-functioning kidneys, while those with secondary VUR associated with abnormal bladder caused by NNB, spina bifida or PUV had abnormal kidney functions. DMSA scans were useful in predicting higher grades of VUR in children with primary reflux.

Pattern of Renal Diseases in Children Presented to King Abdulaziz University Hospital

The pattern of renal diseases in children presented to the tertiary pediatric nephrology center at King Abdulaziz University Hospital was reviewed respectively over the period of 8 years (20052012). A total of 850 children were seen. Male: female ratio is 2:1. Median (range) age at presentation was 9 (0.01-16) years. Fifty-five percent were Saudi and 45% were from various nationalities. Underlying etiology was congenital or inherited disease in 55.5% of the children. Glomerular diseases were the most common in acquired causes while obstructive uropathy was the most common in congenital causes. Non-neurogenic neurogenic bladder was the most common underlying diagnosis of children with urological abnormalities (28%), followed by posterior urethral valve (26%). Nephrotic syndrome was the most common of glomerular diseases with 31% with steroid resistant nephrotic syndrome. Renal impairment was present in 315 children with low glomerular filtration rate; chronic kidney disease stage 2 in 88, stage 3 in 84, stage 4 in 35 and stage 5 in 108. In conclusion, childhood congenital or inherited renal diseases were the majority. Non-neurogenic neurogenic bladder was the most common urological abnormality and nephrotic syndrome was the most common glomerular disease with high percentage of steroid resistant nephrotic syndrome. A considerable percentage presented with advanced chronic kidney disease.

Advanced Transitional Cell Carcinoma of the Bladder in a 16-Year-Old Girl With Hinman Syndrome

The present report describes a 16-year-old girl with a history of Hinman syndrome who was found to have invasive transitional cell carcinoma of the bladder. Bladder cancer is rare in children. Typically, the tumors are of low grade and stage. We present the first case of a teenage girl diagnosed with Hinman syndrome who developed an invasive bladder transitional cell carcinoma. The patient with a neurogenic bladder who has undergone bladder augmentation is at increased risk for bladder cancer. This risk may now have to be extended out to the non-neurogenic neurogenic bladder population if further cases are identified.

Reply

Non-neurogenic neurogenic bladder (NNNB) lies at the most severe end of the spectrum of the non-neurogenic elimination disorders in children. 1 Leclair M.D. Heloury Y. Non-neurogenic elimination disorders in children. J Pediatr Urol. 2010; 6: 338-345 Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar When Ochoa and Gorlin 2 Ochoa B. Gorlin R. Urofacial (Ochoa) syndrome. Am J Med Genet. 1987; 27: 661-667 Crossref PubMed Scopus (54) Google Scholar reported their results in 1987, 33% of the 66 patients had renal functional impairment, 26% had hypertension, and 24% had end-stage renal failure. Editorial CommentUrologyVol. 78Issue 4PreviewNon-neurogenic neurogenic bladder is a term used to describe a form of bladder-sphincteric dysfunction in children. It is typically characterized by a combination of urinary incontinence, impaired and inefficient emptying, and recurrent urinary tract infections. Bowel dysfunction, including constipation and encopresis, are also common. The clinical and urodynamic sequelae are often identical to those in children with neurogenic bladder, except that no neurologic pathologic features can be demonstrated on physical examination or radiographic imaging. Full-Text PDF

Editorial Comment

Non-neurogenic neurogenic bladder is a term used to describe a form of bladder-sphincteric dysfunction in children. It is typically characterized by a combination of urinary incontinence, impaired and inefficient emptying, and recurrent urinary tract infections. Bowel dysfunction, including constipation and encopresis, are also common. The clinical and urodynamic sequelae are often identical to those in children with neurogenic bladder, except that no neurologic pathologic features can be demonstrated on physical examination or radiographic imaging. The urodynamic evaluation will reveal detrusor-external sphincter dyssynergia, and the urinary tract decompensation can range from diurnal incontinence to elevated postvoid residual urine volumes to upper tract deterioration from vesicoureteral reflux, elevated filling pressures, and impaired bladder compliance. The treatment of this condition has been aimed at restoring low-pressure urinary storage and efficient, timely bladder emptying. Urofacial Syndrome: A Subset of Neurogenic Bladder Dysfunction Syndromes?UrologyVol. 78Issue 4PreviewThe urofacial syndrome (Ochoa syndrome) is considered to represent a subgroup of the non-neurogenic bladder dysfunction, characterized by non-neuropathic bladder-sphincter dysfunction, along with a characteristic inversion of the facial expression with laughing. Recent research suggests that it is probably a genetic inherited disease transmitted in an autosomal recessive fashion and might represent a distinct entity. We report a case of this syndrome in a 14-year-old boy who presented with left pyelonephritis, hydronephrosis, and bladder dilation. Full-Text PDF ReplyUrologyVol. 78Issue 4PreviewNon-neurogenic neurogenic bladder (NNNB) lies at the most severe end of the spectrum of the non-neurogenic elimination disorders in children.1 When Ochoa and Gorlin2 reported their results in 1987, 33% of the 66 patients had renal functional impairment, 26% had hypertension, and 24% had end-stage renal failure. Full-Text PDF

Non-neurogenic elimination disorders in children

Non-neurogenic elimination disorders cover a wide spectrum of lower urinary tract and bowel dysfunctions, observed in the absence of a neurological background or lower urinary tract malformation. We reviewed conditions responsible for incontinence during bladder filling with normal voiding patterns (such as overactive bladder, giggle incontinence, post-void dribbling), and dysfunctional voiding syndromes. Dysfunctional elimination syndrome usually includes detrusor-sphincter dyscoordination, small-capacity overactive bladder or decompensated large poorly contractile bladder, and large-volume post-micturition residuals, occasionally associated with bowel dysfunction. At the most severe end of the spectrum lies the non-neurogenic neurogenic bladder syndrome, characterized by the association of a severe impairment of the upper urinary tract with a dysfunctional elimination syndrome. It must be emphasized that if the term 'non-neurogenic' relates to the absence of a neurological lesion, it is however conceivable that these conditions actually do have an underlying neurological cause that remains to be identified.

Botulinum Toxin-A Therapy in Pediatric Urology: Indications for the Neurogenic and Non-Neurogenic Neurogenic Bladder

Although, the role of Botulinum Toxin-A in the treatment of the neurogenic and non-neurogenic neurogenic bladder is becoming more defined, this is the first review article to characterize the emerging role of Botulinum Toxin-A in the pediatric urologic population. Injection of Botulinum Toxin-A at the level of the bladder works by inhibiting uninhibited bladder contractions and, possibly, by blocking some of the sensory nerve fibers. In children with sphincter dyssynergy, injection at the level of the urethral sphincter works by inhibiting the involuntary guarding reflex and blocking dyssynergic voiding.