Complications in adulthood for patients with paediatric genitourinary reconstruction.

BACKGROUND: Caring for adults with prior paediatric genitourinary reconstruction remains a challenge for adult providers. Reconstructions typically have occurred decades before; surgical records are not always available and patients and families may be unable to convey procedures performed. Spina bifida (SB) patients are vulnerable to cognitive decline which may compound these challenges. Changes in patient body habitus and loss of function may contribute to problems with previous reconstructions. METHODS: This is a non-systematic review of the literature and represents expert opinion where data are non-existent. This review focuses on the evaluation and management of complications arising from genitourinary reconstruction in congenital neurogenic bladder patients. RESULTS: Common complications experienced by congenital neurogenic bladder patients include recurrent urinary tract infection, incontinence of catheterizable channel and urinary reservoir as well as malignancy as this population ages. Preservation of renal function and prevention of urinary tract infection while optimizing continence are essential guiding principles in the care of these patients. Many of the recommendations, however, are gleaned from available data in the adult spinal cord patient (a more commonly studied population) or the paediatric urologic literature due to limited studies in adult management of such patients. CONCLUSION: Close follow-up and vigilance is warranted to monitor for infectious, mechanical and malignant complications while optimizing preservation of the upper urinary tracts and patient quality of life.

Malignancy after Augmentation Enterocystoplasty: A Nationwide Study of Natural History, Prognosis and Oncogene Panel Analysis.

PURPOSE: We report the natural history and prognosis of tumors after augmentation enterocystoplasty, with a molecular analysis using an oncogene panel to search for potential targeted therapies. MATERIALS AND METHODS: This multicenter, nationwide, retrospective study included 16 patients. A panel of 21 clinically relevant oncogenes was tested on archival tumor specimens using next-generation sequencing. Survival rate was the main clinical outcome and sequences were compared to the reference genome for the genetic outcome. RESULTS: Augmentation enterocystoplasties were performed mainly for congenital neurogenic bladder and bladder exstrophy at a median patient age of 17 years (range 4 months to 45 years). Most of the malignancies were diagnosed because of clinical manifestations, with a median latency period of 20 years. Adenocarcinomas were mainly found after gastrocystoplasty, whereas urothelial cell carcinomas were typically found after colocystoplasty. Of the 16 patients 13 were diagnosed at an advanced stage of the disease (positive lymph nodes in 7, distant metastases in 6). The overall 1-year survival rate was 56%. Only 3 patients remained disease-free at a median followup of 70 months. Of the 9 tumors with analyzable DNA 4 were wild-type and 5 harbored missense mutations (KIT-p.Pro573Ser, PDGFRA-p.Glu587Lys, KRAS-p.Gly12Asp, ERBB4p.Arg484Lys, CTNNB1-p.Ser37Phe and p.Ser47Asn). CONCLUSIONS: Malignancy after augmentation enterocystoplasty is diagnosed late with frequent metastases and a very low 1-year survival rate. More than half the tested samples harbored missense mutations in oncogenes accessible to targeted therapies. An international collaboration to enlarge the genetic panel analysis of these tumors may offer new therapeutic hope to patients.

Female Pelvic Medicine & Reconstructive Surgery (FPMRS) challenges on behalf of the collaborative research in pelvic surgery consortium (CoRPS): managing complicated cases series 2: management of urinary incontinence in a neurogenic patient.

Discussion and management of incontinence in a patient with spina bifida by four international experts followed by a literature review.

Clinical outcomes of non-surgical management of detrusor leak point pressures above 40 cm water in adults with congenital neurogenic bladder.

AIMS: Neurogenic bladders (NGB) with detrusor leak point pressures >40 cm H2 O (dLPP > 40) have been associated with deterioration of renal function in children with myelomeningocele. For these children, careful pressure management preserves renal function. However, similar evidence is lacking in adult congenital urology (ACU) patients with NGB. We describe renal functional outcomes of non-surgical management of adults with dLPP > 40 or premicturition detrusor pressure (PMDP) >40 cm H2 O, consisting of close follow-up with urodynamic studies (UDS) and renal ultrasound (RUS), paired with adjustments to clean intermittent catheterization (CIC) frequency, anticholinergics, and addition of onabotulinumtoxinA toxin (BTX) injection. METHODS: We retrospectively reviewed the UDS of all patients at an ACU clinic from 2011 to 2016. Patients with dLPP/PMDP > 40 cm who elected for non-surgical management were included. We describe their management and renal functional outcomes. RESULTS: A total of 33/42 patients with dLPP/PMDP > 40 elected for non-surgical management. 28/33 (85%) were successfully managed without bladder augmentation or urinary diversion at follow-up of nearly 3 years. The median index dLPP/PMDP was 49 cm H2 O (IQR 44, 63) and final dLPP/PMDP was 28 (IQR 18, 43). There was a significant decrease in dLPP/PMDP and increase in bladder compliance between index and final UDS (P < 0.001). No patients advanced their CKD stage and 6/10 with baseline hydronephrosis had improvement or resolution of hydronephrosis with non-surgical management. CONCLUSIONS: A non-surgical protocol for ACU patients with NGB and dLPP/PMDP > 40, utilizing CIC, anticholinergics, and BTX is safe and effective when coupled with coordinated care and close follow-up.

Vejiga neurógena no neurogénica congénita (VNNN C): estudio evolutivo / Congenital non-neurogenic neurogenic bladder: evolutionary study

Introducción. Dentro de los trastornos funcionales vesicales de vaciado o disfunciones miccionales, uno de los más conocidos es el síndrome de Allen-Hinman, considerado como un proceso adquirido. Recientemente, se han descrito varios casos de aparición congénita, por lo que es una patología muy poco conocida, originando graves errores diagnósticos y terapéuticos. Caso clínico. Niña de 15 años de edad, controlada desde el nacimiento por presentar una gran distensión abdominal, evacuando 400 ml de orina; siendo los estudios realizados compatibles con vejiga neurógena no neurogénica congénita mediante el tratamiento médico y sondajes vesicales intermitentes, se ha conseguido que no exista afectación renal. Conclusiones. Consideramos que la VNNN congénita es excepcional. Gracias al tratamiento seguido, se ha logrado que no exista afectación renal, y ninguna complicación. Desde hace unos 5 años, a veces, presenta deseos de micción, que no se acompaña de la misma, por lo que debemos seguir realizando un control evolutivo preciso (AU)

Introduction. The Allen-Hinman syndrome, considered being a adquired process, is one of the best known within functional disorders of emptying, or voiding dysfunctions. Recently, several cases describing a congenital appearance has been seen. It is a very little-known pathology, resulting in serious diagnostical and therapeutical errors. Clinical case. A 15 year old girl, known since birth to present a great abdominal distension, removing 400 ml of urine; realizing through studies a compatibility with congenital non-neurogenic neurogenic bladder. Medical treatment and intermittent catheterization has come to show no renal involvement or any complications with the treatment. Conclusions. We consider that congenital VNNN is exceptional. Thanks to continued treatment, We have come to conclude there being no renal involvement as well as any complications with the treatment. During the last five years she has occasionally presented the desire to urinate, both by day time and night time, without urination following. Not knowing if it can be a future-related bladder, we have to secure a evolutionary control (AU)

Neuropathic bladder in the neonate.

The management of infants born with myelomeningocele depends on understanding how their bladder stores and empties urine. Storage at low pressure with effective emptying periodically throughout the day is the goal. Intervention is designed to impact on one or both of these processes so that infants can remain infection-free and at the same time allow for appropriate renal growth over time. Urodynamic evaluation plays an important role, so that neonates can be stratified according to their risk. Most patients require intermittent catheterization and pharmacotherapy to achieve these goals at some point in their lives.

Cystatin C as a marker of early renal insufficiency in children with congenital neuropathic bladder.

PURPOSE: Due to decreased muscle mass in children with congenital neuropathic bladder there may be significant inaccuracy when using the creatinine based estimated glomerular filtration rate. Cystatin C is highly sensitive and specific for measuring changes in the glomerular filtration rate in children and in patients with muscle wasting conditions. We hypothesized that a cystatin C calculated glomerular filtration rate would be more sensitive than the standard creatinine based modified Schwartz equation to detect renal insufficiency in children with congenital neuropathic bladder. MATERIALS AND METHODS: We prospectively identified children with congenital neuropathic bladder at a multidisciplinary spina bifida clinic who underwent serum creatinine and serum cystatin C testing. Clinical history and anthropomorphic variables at the time of laboratory testing were catalogued. The creatinine based glomerular filtration rate was estimated using the modified (bedside) Schwartz formula and the cystatin C based rate was calculated using the Zappitelli cystatin C formula. RESULTS: Dual estimated glomerular filtration rate calculation was done in 69 children at a total of 74 patient encounters. Absolute creatinine was within age range normal limits in each patient, including 1 with chronic kidney disease stage 3A. The median creatinine based estimated glomerular filtration rate was 123 ml per minute/1.73 m(2) (range 58 to 229). The median cystatin C based estimated rate was 103 ml per minute/1.73 m(2) (range 47 to 144) for an absolute median rate reduction of 15.4%. Using cystatin C estimates chronic kidney disease stage was upgraded from stage 1 to 2 in 13 patients (18.8%). CONCLUSIONS: In children with neuropathic bladder the cystatin C estimated glomerular filtration rate is a better screening test for early renal insufficiency that is not detected by creatinine based rate calculations. To our knowledge it remains to be determined whether the cystatin C estimated glomerular filtration rate can ultimately improve the clinical outcome in this population.

Single-center experience with botulinum toxin endoscopic detrusor injection for the treatment of congenital neuropathic bladder in children: effect of dose adjustment, multiple injections, and avoidance of reconstructive procedures.

OBJECTIVE: Since 2007, intra-detrusor OnabotulinumtoxinA (OnabotA) injections have been selectively offered at our institution for cases in which maximal anticholinergic therapy failed or was not tolerated. Herein we present our experience with this approach. MATERIALS AND METHODS: We prospectively obtained data on 17 patients who underwent OnabotA injections over a 4-year period. Demographic information, number of injections, and dose delivered were captured. Children were monitored with baseline and post-injection renal ultrasound, urodynamics, and assessed for side effects, satisfaction, and symptom improvement. RESULTS: Forty-three sessions were performed with injections given every ∼ 6 months. Mean patient age was 10.7 years (range, 3-17). Compared with baseline, after the first injection, mean bladder capacity adjusted for age and compliance improved by 27% (p = 0.039) and 45.2% (p = 0.041), respectively. After subsequent injections, these values increased to 35.7% (p = 0.043) and 55.1% (p = 0.091), respectively. Out of 13 symptomatic patients, ≥ 50% improvement was reported in ten (76.9%) and complete resolution in seven (53.8%). However, all three patients in whom the maximum dose of OnabotA was reduced from 300 to 200 units complained of recurrent symptoms. Fourteen children avoided surgical reconstruction as a second line of treatment. Overall patient/parental reported satisfaction rate was 70.6% (12/17). CONCLUSIONS: Intra-detrusor OnabotA injection is a promising intervention for management of neuropathic bladder in selected patients. Our data demonstrate improvement in symptoms and urodynamic parameters. Although an optimal dose has not been determined for children, we found optimal response with a maximum administration of OnabotA up to 300 units.

Development of upper tract stones in patients with congenital neurogenic bladder.

OBJECTIVE: Patients with neurogenic bladder are at increased risk of developing upper tract stones. We hypothesized that patients with lower urinary tract stone disease are at greater risk of developing upper tract stones. METHODS: We performed a 10-year retrospective case-control study of patients with neurogenic bladder to determine the association between bladder and upper tract stones. Independent risk factors for upper tract stones were assessed. Cases and controls were matched 1:1. Univariable analysis was performed by Fisher's exact test and the Mann-Whitney U test. Multivariable logistic regression was performed. RESULTS: 52 cases and controls were identified. Cases were significantly more likely to be non-ambulatory, have bowel-urinary tract interposition, thoracic level dysraphism, and history of bladder stones. On multivariable analysis, independent predictors of stone formation were male sex (OR 2.82; p = 0.02), dysraphism involving the thoracic spine (OR 3.37; p = 0.014) bowel-urinary tract interposition (OR 2.611; p = 0.038), and a history of bladder stones (OR 3.57; p = 0.015). CONCLUSION: Patients with neurogenic bladder are at increased risk for upper tract stones. The presence of bladder stones may herald the development of upper tract stones. The predictors of stone disease identified should guide prospective studies to better understand the natural history of upper tract stone development in this population.

International Children's Continence Society's recommendations for initial diagnostic evaluation and follow-up in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: The objective of this ICCS standardization document is to report the initial diagnostic evaluation and subsequent work-up of children with neuropathic bladder dysfunction. MATERIALS AND METHODS: Due to a paucity of level I or level II, "levels of evidence" publications, these recommendations are actually a compilation of best practices because they seem to be effective and reliable, although not with any control. RESULTS: Throughout the document, the emphasis is on promoting early, comprehensive evaluation of lower urinary tract function that is thorough but with a minimum of unnecessary testing. This includes what tests to order, when to order them and what to do with the results. Some of the recommendations may not be practical in various worldwide locations but the suggested testing should be considered the ideal approach to completely diagnosing and then promulgating treatments based on the full knowledge of the condition and its effect on urinary tract function. Once the findings are delineated, those lower urinary tract patterns of dysfunction that put the kidneys at risk for deterioration, that are barriers to attaining eventual continence, and that have long-term consequence to the lower urinary track can be obviated by specific management recommendations. The indications and timing of investigations to achieve these objectives are clearly defined in each diagnostic category and during follow-up. RECOMMENDATIONS: This document should be used as a basis for appropriate evaluation and timely surveillance of the various neuro-urologic conditions that affect children.

International Children's Continence Society's recommendations for therapeutic intervention in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: We present a consensus view of members of the International Children's Continence Society on the therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. MATERIAL AND METHODS: Discussions were held by a group of pediatric urologists and gastroenterologists appointed by the board. The following draft review document was open to all the ICCS members via the ICCS web site. Feedback was considered by the core authors and by agreement, amendments were made as necessary. The final document is not a systematic literature review. It includes relevant research when available as well as expert opinion on the current understanding of therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. RESULTS: Guidelines on pharmalogical and surgical intervention are presented. First the multiple modalities for intervention that do not involve surgical reconstruction are summarized concerning pharmacological agents, medical devices, and neuromodulation. The non-surgical intervention is promoted before undertaking major surgery. Indicators for non-surgical treatments depend on issues related to intravesical pressure, upper urinary tract status, prevalence of urinary tract infections, and the degree of incontinence. The optimal age for treatment of incontinence is also addressed. This is followed by a survey of specific treatments such as anticholinergics, botulinum-A toxin, antibiotics, and catheters. Neuromodulation of the bladder via intravesical electrical stimulation, sacral nerve stimulation, transcutaneous stimulation, and biofeedback is scrutinized. Then follows surgical intervention, which should be tailored to each individual, based on careful consideration of urodynamic findings, medical history, age, and presence of other disability. Treatments mentioned are: urethral dilation, vesicostomy, bladder, augmentation, fascial sling, artificial urinary sphincters, and bladder neck reconstruction and are summarized with regards to success rates and complications. Finally, the treatment on neuropathic bowel dysfunction with rectal suppositories irrigation and transrectal stimulation are scrutinized.

Renal function in children with congenital neurogenic bladder.

AIMS: Preservation of renal function in children with congenital neurogenic bladder is an important goal of treatment for the disease. This study analyzed the evolution of renal function in patients with congenital neurogenic bladder. METHODS: We reviewed the records of 58 pediatric patients with respect to the following attributes: gender, age, etiology of neurogenic bladder, reason for referral, medical/surgical management, episodes of treated urinary tract infections, urodynamics, DMSA scintigraphy, weight, height, blood pressure, glomerular filtration rate, microalbuminuria and metabolic acidosis. Statistical analysis was performed, adopting the 5% significance level. RESULTS: The mean age at presentation was 4.2 ± 3.5 years. Myelomeningocele was the most frequent etiology (71.4%). Recurrent urinary tract infection was the reason for referral in 82.8% of the patients. Recurrent urinary tract infections were diagnosed in 84.5% of the patients initially; 83.7% of those patients experienced improvement during follow-up. The initial mean glomerular filtration rate was 146.7 ± 70.1 mL/1.73 m²/min, and the final mean was 193.6 ± 93.6 mL/1.73 m²/min, p = 0.0004. Microalbuminuria was diagnosed in 54.1% of the patients initially and in 69% in the final evaluation. Metabolic acidosis was present in 19% of the patients initially and in 32.8% in the final assessment. CONCLUSIONS: Patient referral to a pediatric nephrologist was late. A reduction in the number of urinary tract infections was observed with adequate treatment, but microalbuminuria and metabolic acidosis occurred frequently despite adequate management.

Renal function in children with congenital neurogenic bladder

AIMS: Preservation of renal function in children with congenital neurogenic bladder is an important goal of treatment for the disease. This study analyzed the evolution of renal function in patients with congenital neurogenic bladder. METHODS: We reviewed the records of 58 pediatric patients with respect to the following attributes: gender, age, etiology of neurogenic bladder, reason for referral, medical/surgical management, episodes of treated urinary tract infections, urodynamics, DMSA scintigraphy, weight, height, blood pressure, glomerular filtration rate, microalbuminuria and metabolic acidosis. Statistical analysis was performed, adopting the 5 percent significance level. RESULTS: The mean age at presentation was 4.2 ± 3.5 years. Myelomeningocele was the most frequent etiology (71.4 percent). Recurrent urinary tract infection was the reason for referral in 82.8 percent of the patients. Recurrent urinary tract infections were diagnosed in 84.5 percent of the patients initially; 83.7 percent of those patients experienced improvement during follow-up. The initial mean glomerular filtration rate was 146.7 ± 70.1 mL/1.73 m²/min, and the final mean was 193.6 ± 93.6 mL/1.73 m²/min, p = 0.0004. Microalbuminuria was diagnosed in 54.1 percent of the patients initially and in 69 percent in the final evaluation. Metabolic acidosis was present in 19 percent of the patients initially and in 32.8 percent in the final assessment. CONCLUSIONS: Patient referral to a pediatric nephrologist was late. A reduction in the number of urinary tract infections was observed with adequate treatment, but microalbuminuria and metabolic acidosis occurred frequently despite adequate management.

The effect of epigenetic therapy on congenital neurogenic bladders--a pilot study.

OBJECTIVES: To demonstrate that human smooth muscle cells derived from neurogenic bladders produce more collagen in vitro than smooth muscle cells derived from normal bladders, and that epigenetic therapy may normalize this increased collagen production. METHODS: Human smooth muscle cells from normal (n = 3) and neurogenic bladders (n = 3) were cultured in normal culture media and at different concentrations of the histone deacetylase inhibitors trichostatin A, valproic acid, and the DNA methylation inhibitor 5-azacytidine (5-aza). Collagen type I and III gene expression was measured using real-time quantitative reverse transcription-polymerase chain reaction after varying doses of drug exposure. Cell viability was measured using trypan blue. RESULTS: The smooth muscle cells from neurogenic bladders released significantly more collagen than the normal bladder cells (mean 4.1 vs 1.8 microg/mL in control media) when grown in normal conditions. Treatment with trichostatin A at 50 ng/mL decreased the collagen level in cells from neurogenic bladders to almost normal levels (2.1 microg/mL). In addition, valproic acid treatment decreased collagen types I and III gene expression relative to controls, with maximal effect at 300 mg/mL. These treatments had little effect on cell viability. CONCLUSIONS: Histone deacetylase inhibitors decreased collagen production of smooth muscle cells from neurogenic bladders in vitro. These agents may be a means of effectively preventing bladder fibrosis in patients with this condition.

Masa escrotal como presentación de divertículo uretral gigante / Scrotal mass presenting as giant urethral diverticulum

El divertículo uretral en el varón es una entidad rara que puede ser congénita o adquirida. Son comunes en pacientes parapléjicos que son propensos a desarrollar este problema de forma adquirida debido a cateterizaciones prolongadas. Las enfermedades mas frecuentes en pacientes lesionados medulares son estenosis, fístulas y divertículos. Pacientes con divertículos típicamente se presentan con síntomas de incontinencia urinaria, disuria, dolor perineal o una masa genital o en periné. El tratamiento de elección es siempre quirúrgico y debería realizarse una extirpación completa del mismo (AU)

Urethral diverticulum in the male is a rare entity that may be congenital or acquired. They are common in paraplegic patients, who are prone to developing this disorder on an acquired basis because of prolonged catheterization. The most common diseases in patients with spinal cord injury are stricture, fistula and diverticula. Patients with diverticula typically present with symptoms of urinary incontinence, dysuria, perineal pain, or a mass on the ventral aspect of the genitalia or perineum. Treatment of choice is always surgical and a complete extirpation should be performed (AU)

Artificial urinary sphincter: 11-year experience in adolescents with congenital neuropathic bladder.

OBJECTIVE: We assess our experience over the last 11 years in the use of an artificial urinary sphincter (AUS) to treat urinary incontinence in children with neuropathic bladders. MATERIALS AND METHODS: Between 1994 and 2005 an AUS was implanted in 35 patients (mean age 14.4; range 11.5-18). Upper urinary tract (UUT) evaluations and urodynamic studies were performed in all patients pre- and post-AUS implantation. Thirteen patients underwent enterocystoplasty combined with AUS placement and 22 underwent AUS implantation alone. RESULTS: An AUS was implanted in 35 patients. Mean follow-up is 5.5 years (range 0.4-11 years). Nine mechanical malfunctions occurred in seven patients (20%). Of the 22 patients who underwent AUS implantation alone, seven (31.2%) eventually required an enterocystoplasty because of unexpected bladder behaviour changes, usually within three years of AUS implantation. In seven patients (20%), a continent catheterisable stoma was made (before or during the follow-up) because of problems with clean intermittent catheterisation (CIC) through the urethra. Three AUS (8.6%) were removed because of sphincter erosion at the bladder neck. All 32 patients (91.4%) with the AUS currently in place are dry, three void their bladders spontaneously, and 29 need CIC. CONCLUSIONS: AUS must be considered as an elective treatment in the surgical management of these patients because it produces better continence rates than other methods. However, these patients need long-term follow-up because their bladder behaviour may undergo unexpected clinically asymptomatic changes that could negatively affect their UUT and require bladder augmentation.

Bladder neck cinch for pediatric neurogenic outlet deficiency.

PURPOSE: The fascial bladder neck sling achieves continence in 50% to 90% of children with neurogenic outlet deficiency. Most slings apply only partial pressure around the bladder neck. We evaluated the effectiveness of a rectus fascia bladder neck cinch which applies circumferential pressure around the bladder neck and elevation as a means of increasing outlet resistance. MATERIALS AND METHODS: Fifteen children with spina bifida underwent a fascial bladder neck cinch procedure at the time of augmentation cystoplasty. A 1 to 1.5 cm width of variable length rectus fascia was harvested and a vertical slit was made in 1 end. The fascia was "cinched" tightly around the bladder neck and secured to the symphysis or rectus fascia. RESULTS: The 14 girls and 1 boy ranged in age range from 4 to 17 years. All children had neurogenic intrinsic sphincter deficiency and a poorly compliant and/or small capacity bladder. Followup ranged from 10 to 36 months (followup in 12 greater than 1 year). Postoperatively, all children perform clean intermittent catheterization. At the last followup 8 girls and the boy (60%) were dry (no leak and no pads at 4 hours from the last catheterization and dry throughout the night). CONCLUSIONS: Rectus fascia used as a bladder neck cinch is effective but no better than other bladder neck slings for decreasing urinary incontinence. The bladder neck cinch appears to be an acceptable addition to the technique of fascial slings. However, we have subsequently changed our technique because these results did not meet our expectations.

Alpha blocker therapy for children with dysfunctional voiding and urinary retention.

PURPOSE: Alpha blocker therapy has been successfully used to decrease residual urine in children with complex neuropathic and nonneuropathic voiding dysfunction. We evaluated the safety and efficacy of using selective alpha blocker therapy for children with uncomplicated voiding dysfunction and underlying poor bladder emptying. MATERIALS AND METHODS: A total of 55 patients with a mean age of 7.9 years presented with symptoms of urinary incontinence, urgency and urinary tract infection. All patients had increased post-void residual (PVR) on bladder ultrasound, with a mean residual volume of 65 ml (22% of age expected capacity). All patients were treated with doxazosin, a selective alpha-1 adrenergic antagonist, at dosages of 0.5 mg to 2.0 mg daily. Of the patients 38 were treated at presentation with a regimen of anticholinergics, timed voiding and antibiotic prophylaxis before initiating alpha blocker therapy. Patients were reevaluated with post-void ultrasound of the bladder 6 weeks after initiating alpha blocker therapy. RESULTS: After starting doxazosin average PVR decreased to 8 ml (p <0.0001), representing an 88% reduction in residual urine (or reduction to only 2.7% of age expected bladder capacity). Medication was discontinued in 2 patients due to minor side effects. CONCLUSIONS: Selective alpha blocker therapy appears to be effective for improving bladder emptying in children with an overactive bladder, wetting, recurrent infection and increased PVR urine. This therapy may be used as either a replacement or in addition to biofeedback in patients with urinary retention. Further investigation, including a prospective randomized trial of alpha blocker therapy in children with urinary tract dysfunction, is warranted based on the findings of our study.

[Haematemesis and dysphagia in a 20-year-old woman with congenital spine malformation and situs inversus partialis]. / Hämatemesis und Dysphagie bei 20 Jahre alter Frau mit kongenitalem kaudalen Regressionssyndrom und Situs inversus partialis.

A 20-year-old woman with a four-week history of dysphagia, weight loss of four kilograms and unspecific abdominal pain was admitted because of sudden haematemesis. The physical examination showed a patient with a prominent kyphoskoliosis. The patient reported of having a situs inversus abdominalis and a tethered cord syndrome. Bladder function disorders were present since childhood. Upper endoscopy demonstrated a 4 cm large, exophytically growing necrotic tumour of the oesophagus. The CT scan showed a space occupying tumour of the oesophagus and metastases in a size of 1.5 cm in both lungs. Further imaging revealed a UICC-Stadium IVB (T2NxMIb ). Histology of the tumour biopsies showed a poor differentiated squamous cell carcinoma. Staging after the 6 th dose cisplatin (100 mg/m2/die) and 5-fluorouracil (5 x 1000 mg/m2/die) showed a mild reduction of the tumour and the metastases. The patient died ten months later of multiorgan failure after severe progress of tumour and metastatic growth. The manifestation of squamous cell carcinoma of the oesophagus is unusual in people at the age of twenty. Genetic and chromosomal analysis of the patient gave no evidence for a hereditary disorder. Drug history revealed that the patient had been treated with the alpha-receptor blocking drug phenoxybenzamine over at least 12 years for bladder dysfunction. Animal experiments of rats with exposition of phenoxybenzamine over 24 months produced gastrointestinal malignomas. By the German admission board phenoxybenzamine is only recommended for short term therapy. It seems to be likely that even in humans phenoxybenzamine acts as a mutagenic substance and should be carefully used in long-term treatment.

The use of small intestinal submucosa as an off-the-shelf urethral sling material for pediatric urinary incontinence.

PURPOSE: Use of autologous rectus fascia for urethral slings in the pediatric population has produced reliable and predictable results. However, the potential morbidity and complications associated with harvesting the autologous rectus fascia have driven efforts to find a reliable off-the-shelf material for urethral slings. Small intestinal submucosa is a collagen based material that has been shown to promote tissue specific regeneration in a variety of organs. We report the clinical experience at 4 institutions with small intestinal submucosa for urethral slings. MATERIALS AND METHODS: A total of 20 patients 3 to 18 years old (mean age 8.7) received urethral slings using the commercially available form of small intestinal submucosa (STRATASIS, Cook Urologic Spencer, Indiana) via a sling suspension procedure from a suprapubic approach. RESULTS: The material was consistently uniform to work with and user-friendly. All 20 patients tolerated the procedure well with no intraoperative complications. Postoperative followup has ranged from 9 to 26 months (mean 13), and 14 (70%) patients are completely dry (85% in females and 43% in males). Of the 14 dry patients 13 are on intermittent catheterization and 1 female with epispadias voids spontaneously. CONCLUSIONS: This report is the largest and longest followup series using small intestinal submucosa as an "off the shelf" urethral sling material in children. These continence rates are equal to autologous fascia without additional morbidity of graft harvest.