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Purpose: To develop a prediction model for postoperative complications after primary one-stage hypospadias correction to improve preoperative parental counseling., Materials and Methods: In this retrospective cohort study, data were collected from 356 patients with anterior or middle hypospadias who had a one-stage hypospadias correction from 2003 onwards. Potential treatment- and patient-related factors were selected and used to develop a prediction model for postoperative complications within one year (wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications). Multivariable logistic regression analysis with stepwise backward selection and a p-value of 0.20 was used to select the final model, which was internally validated using the bootstrap procedure., Results: Complications within one year postoperatively occurred in 66 patients (19%), of which 13% and 37% were seen in anterior and middle type of hypospadias, respectively. Hypospadias phenotype, surgical technique, chordectomy, and surgeon's experience were included in the final prediction model, whereas none of the patient-related factors were. The final model had a good discriminative ability (bias corrected C statistic 0.70) and calibration., Conclusion: Using easily obtainable information, this model showed good accuracy in predicting complications within one year after hypospadias surgery. It is a first step towards individualized risk prediction of postoperative complications for anterior and middle hypospadias and can assist in preoperative parental counseling., Type of Study: Prognostic study., Level of Evidence: Level II., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To analyze agreement on postoperative complications after hypospadias surgery according to medical records and parents' reports., Materials & Methods: In this retrospective cohort study, data were collected from 409 children who received an initial one-stage hypospadias correction in the Radboudumc, The Netherlands. Postoperative complications according to medical records were compared with parent-reported complications in an online questionnaire. Main complications studied were wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications. Agreement was determined by Cohen's kappa coefficient., Results: Slightly less complications were mentioned in medical records (37%) compared to parents' reports (42%). Overall agreement was moderate (κ = 0.50, 95% confidence interval (CI):0.41-0.59), but poor for some specific complications. Agreement was higher for complications that needed reoperation compared to when no reoperation was performed (κ = 0.53, 95% CI: 0.43-0.62 and κ = 0.18, 95% CI: 0.06-0.31) and for patients with recent surgery (<5 years before questionnaire completion) compared to less recent surgeries (κ = 0.69, 95% CI: 0.55-0.84 and κ = 0.43, 95% CI: 0.33-0.54)., Conclusions: Agreement on complications according to medical records and parents' reports was poor to moderate, but better after reoperation and more recent surgery. Some complications mentioned in medical records were missing from parents' reports and the other way around. Better agreement will give physicians and parents a more reliable view on postoperative outcome after hypospadias surgery., Type of Study: Diagnostic test., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Research regarding the etiology of birth defects and childhood cancer is essential to develop preventive measures, but often requires large study populations. Therefore, we established the AGORA data- and biobank in the Netherlands. In this study, we describe its rationale, design, and ongoing data collection., Methods: Children diagnosed with and/or treated for a structural birth defect or childhood cancer and their parents are invited to participate in the AGORA data- and biobank. Controls are recruited through random sampling from municipal registries. The parents receive questionnaires about demographics, family and pregnancy history, health status, prescribed medication, lifestyle, and occupational exposures before and during the index pregnancy. In addition, blood or saliva is collected from children and parents, while medical records are reviewed for diagnostic information., Results: So far, we have collected data from over 6,860 families (3,747 birth defects, 905 childhood cancers, and 2,208 controls). The types of birth defects vary widely and comprise malformations of the digestive, respiratory, and urogenital tracts as well as facial, cardiovascular, kidney, skeletal, and central nervous system anomalies. The most frequently occurring childhood cancer types are acute lymphatic leukemia, Hodgkin and non-Hodgkin lymphoma, Wilms' tumor, and brain and spinal cord tumors. Our genetic and/or epidemiologic studies have been focused on hypospadias, anorectal malformations, congenital anomalies of the kidney and urinary tract (CAKUT), and orofacial clefts., Conclusion: The large AGORA data- and biobank offers great opportunities for investigating genetic and nongenetic risk factors for disorders in children and is open to collaborative initiatives. Birth Defects Research (Part A) 106:675-684, 2016. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Introduction: Cloacal exstrophy is a complex and rare congenital malformation. Because of improvements in medicine, operative techniques, and perioperative management the survival rates are now approaching 100%. Currently, treatment is focused on improving quality of life. Since 1974, we encountered 20 patients with cloacal exstrophy in our hospital. The aim of this study is to evaluate our clinical experience and outcome during the last 39 years., Patients and Methods: A retrospective study of the medical records was performed. We evaluated anatomical status, phenotype and genotype at birth, subsequent surgical treatment and current gastrointestinal, urinary, spinal, genital, and gender status and outcome., Results: The records of all 20 patients who were treated in our center were included in this study. Of the 20 patients, 18 were primary patients, 2 referred. Six patients died within the first year of life. Median age of the surviving 14 patients was 25 years (range, 4-39 years) at time of evaluation. At the last follow-up, 11 patients had an endileostomy or endcolostomy. Three patients had a pull-through; two of them were continent for feces. Two patients were continent for urine; one with a sinus urogenitalis and one after urethra reconstruction. Eleven patients are incontinent; six of them had an incontinent urinary deviation (Bricker deviation). Of the other five incontinent patients, two had a urethral reconstruction, one vesicovaginal anastomosis, one perineotomy, and one epispadias bladder. Urinary continence was unknown in one patient. Of the surviving patients, nine have 46 XY karyotype and five 46 XX karyotype. Initially, six XY patients underwent external genital reconstruction with reassignment to female gender; however, one died postoperatively. One declared to feel like a man at age of 15 years. Remaining four XY patients underwent male external genital reconstruction. All XX patients underwent vagina or vulva reconstruction, except for one who still has aplasia vaginae. All patients were born with varying types of spinal dysraphism., Discussion: Despite medical efforts and development in treatment and support, satisfaction in outcome of cloacal exstrophy remains a challenge. Although reconstruction may be very difficult, male genital reconstruction in 46 XY patients has been given preference for more than a decade. A specialized multidisciplinary team should provide lifelong colorectal, urological, sexual, and psychosocial support to these patients, taking into account their age and phase of life., (Georg Thieme Verlag KG Stuttgart · New York.)

Objective: To determine the reliability and internal validity of the Hypospadias Objective Penile Evaluation (HOPE)-score, a newly developed scoring system assessing the cosmetic outcome in hypospadias., Patients and Methods: The HOPE scoring system incorporates all surgically-correctable items: position of meatus, shape of meatus, shape of glans, shape of penile skin and penile axis. Objectivity was established with standardized photographs, anonymously coded patients, independent assessment by a panel, standards for a "normal" penile appearance, reference pictures and assessment of the degree of abnormality. A panel of 13 pediatric urologists completed 2 questionnaires, each consisting of 45 series of photographs, at an interval of at least 1 week. The inter-observer reliability, intra-observer reliability and internal validity were analyzed., Results: The correlation coefficients for the HOPE-score were as follows: intra-observer reliability 0.817, inter-observer reliability 0.790, "non-parametric" internal validity 0.849 and "parametric" internal validity 0.842. These values reflect good reproducibility, sufficient agreement among observers and a valid measurement of differences and similarities in cosmetic appearance., Conclusions: The HOPE-score is the first scoring system that fulfills the criteria of a valid measurement tool: objectivity, reliability and validity. These favorable properties support its use as an objective outcome measure of the cosmetic result after hypospadias surgery., (Copyright © 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Transfers to adult care can be problematic, resulting in postponement due to the protective nature of pediatric care and patient dependency. It is unknown whether these findings apply specifically to urology patients. Our department is taking part in a national general transition project. In this light, our aim was to investigate the specific needs of adolescent urologic patients, regarding their independence and transition., Patients and Methods: 80 patients, born in 1975-1998, with a chronic bladder condition received a questionnaire. They were divided into pre- and post-transfer groups. Parents (n = 7) of post-transfer patients formed a third group. Questionnaires were based on those used in the national transition study, supplemented with urological questions. Pre-transfer patients were asked about their level of independence, what subjects were discussed during consultations, and their expectations and wishes regarding transfer. Post-transfer patients and parents were asked for their opinions on the transfer process., Results: 73% (n = 58) responded (55 pre-transfer and 3 post-transfer patients plus parents). It appeared that the confidence built-up with the pediatric urologist impeded the transfer. An adequate level of disease-related knowledge was reported. Relationships, sexuality and fertility were hardly talked about (respectively n = 17, 16 and 18). Parents played an important role, which patients appreciated, confirming their dependency. Despite the 49% (n = 27) who stated they can arrange their urological care themselves, 44% (n = 24) felt ill-prepared for transfer., Conclusion: Although overall self-perceived knowledge is sufficient, the trust in and personal relationship with the pediatric urologist formed the greatest obstruction to successful transition. These findings have been used to improve support during transition by creating a transition protocol., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

The American Academy of Pediatrics recently released its new Technical Report and Policy Statement on male circumcision, concluding that current evidence indicates that the health benefits of newborn male circumcision outweigh the risks. The technical report is based on the scrutiny of a large number of complex scientific articles. Therefore, while striving for objectivity, the conclusions drawn by the 8 task force members reflect what these individual physicians perceived as trustworthy evidence. Seen from the outside, cultural bias reflecting the normality of nontherapeutic male circumcision in the United States seems obvious, and the report's conclusions are different from those reached by physicians in other parts of the Western world, including Europe, Canada, and Australia. In this commentary, a different view is presented by non-US-based physicians and representatives of general medical associations and societies for pediatrics, pediatric surgery, and pediatric urology in Northern Europe. To these authors, only 1 of the arguments put forward by the American Academy of Pediatrics has some theoretical relevance in relation to infant male circumcision; namely, the possible protection against urinary tract infections in infant boys, which can easily be treated with antibiotics without tissue loss. The other claimed health benefits, including protection against HIV/AIDS, genital herpes, genital warts, and penile cancer, are questionable, weak, and likely to have little public health relevance in a Western context, and they do not represent compelling reasons for surgery before boys are old enough to decide for themselves.

Here, we report the first isolation of Wautersiella falsenii from the urine of an infant with a complicated urinary tract infection. W. falsenii was correctly identified by matrix-assisted laser desorption ionisation time of flight mass spectrometry. The identification was confirmed by 16S polymerase chain reaction. Susceptibility test results of this isolate are reported. Ciprofloxacin treatment resulted in clinical and microbiological improvement., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To contribute to the understanding of the etiology of undescended testis (UDT), by exploring a wide range of potential risk factors in a case-referent study., Patients and Methods: Cases and referents were recruited at five hospitals and included 200 boys with surgically corrected UDT and 629 boys with persistent middle ear effusion. Risk factor data were obtained by postal questionnaires to both parents. Clinical data were collected from medical files. Adjusted odds ratios (OR) with 95% confidence intervals (CI) were estimated using logistic regression., Results: The main findings include associations between UDT and familial occurrence of the disorder: OR 3.1 (95%CI 1.9-4.9), low birth weight: 2.2 (1.1-4.3), twinning: 2.2 (0.9-5.4), gestational preeclampsia: 1.9 (0.8-4.4), use of oral contraceptives after conception: 3.6 (1.0-12.5), in vitro fertilization/intracytoplasmic sperm injection treatment: 2.2 (0.8-6.0), paternal subfertility: 1.8 (0.8-4.1), and maternal occupational exposure to cosmetics: 3.0 (0.9-10.0). Subgroup analyses indicated differences in ORs for several factors between cases with (n = 92) and without (n = 103) inguinal hernia or hydrocele., Conclusion: The findings point towards a role for genetic predisposition, placental insufficiency, and possibly exposure to specific endocrine disrupting substances in the etiology of UDT. Further research should take into account potential etiologic differences between subgroups of cases with UDT., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Urological anomalies are frequently seen in patients with anorectal malformations (ARM) and can result in upper urinary tract deterioration. Whether the current method of screening is valid, adequate and needed for all patients is not clear. We, therefore, evaluated the urological screening methods in our ARM patients for changes in urological treatment, outcome and follow-up., Methods: The medical records of 331 children born with an ARM in the period 1983-2003 were retrospectively studied. Documentation of diagnosis, screening method, urological anomalies, treatment, complications, follow-up and outcome were measured., Results: The overall incidence of urological anomalies was 52%. The incidence of urological anomalies and urological follow-up time decreased with diminishing complexity of the ARM. Hydronephrosis, vesico-urethral reflux, lower urinary tract dysfunction and urinary incontinence were encountered most. Treatment invasiveness increased with the increase of complexity of an ARM. Lower urinary tract dysfunction needing urological care occurred in 43% in combination with lumbosacral or spinal cord anomalies and in 8% with no abnormalities in the lumbosacral-/spinal region., Conclusions: Urological anomalies in patients with complex ARM are more severe than in patients with less complex ARM. Ultrasonography of the urinary tract should be performed in all patients. Voiding cysto-urethrography can be reserved for patients with dilated upper urinary tracts, urinary tract infections or lumbosacral and spinal abnormalities. All patients with complex ARM need urodynamic investigations. When using these indications, the screening for urological anomalies in ARM patients can be optimized with long-term follow-up in selected patients.

Objective: To assess the effectiveness of urotherapy in children with lower urinary tract dysfunction, according to the new definitions of the International Children's Continence Society., Material and Methods: We performed a retrospective review of 122 children (aged 8.8 ± 2.0 years) treated in an outpatient program for lower urinary tract dysfunction. Exclusion criteria included all neurologic abnormalities. In 98 children (80%) daytime urinary incontinence was a predominant symptom. Therapy consisted of an individually adapted drinking and voiding schedule, pelvic floor relaxation, instructions on toilet behavior, biofeedback uroflowmetry and if necessary recommendations for regulation of defecation. Before and at the end of training, patients were evaluated for number and severity of daytime wet accidents per week, using a scoring system to grade the severity of incontinence. Secondary measurements of accompanying voiding symptoms were performed., Results: Of the 90 children with daytime urinary incontinence for whom sufficient objective data were collected, 42% became completely dry during the daytime and 36% showed a 50% or greater level of response. Secondary measurements showed a significant reduction in daily voiding frequency (mean 7.0 ± .3, P<0.0001) and mean post-void residual (P<0.003), and an improvement in flow pattern (P<0.05)., Conclusions: Urotherapy is successful for the treatment of daytime urinary incontinence in children. Additional benefit was evident in improvement of accompanying voiding symptoms. A combination of the definitions of the International Children's Continence Society and a scoring system to grade severity improved the evaluation method. Further research into long-term efficacy will be performed., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

A male newborn is described, in whom a large extra-abdominal prevesical pseudo-cyst as well as prune-belly features were present, both of which were supposedly secondary to posterior urethral valves. It is postulated that the subvesical obstruction caused pressure build-up in the urinary tract, followed by fetal bladder rupture. The resultant urinoma migrated extra-abdominally. Distension of the urinary tract, bilateral cryptorchidism and abdominal wall laxity contributed to a prune-belly phenotype.

Objective: To obtain more insight into the origin of hypospadias by exploring a wide range of potential risk factors in a case-referent study in which a distinction was made between different phenotypes., Patients and Methods: Cases and referents were 305 boys with hypospadias and 629 boys with middle ear effusion whose parents completed postal questionnaires. Hypospadias phenotype was classified as distal (195 boys), middle (67), and proximal (43). Adjusted odds ratios (OR) with 95% confidence intervals (CI) were estimated using logistic regression., Results: Low birth weight, being a twin or triplet, mother being a diethylstilbestrol-daughter, fertility treatments, paternal subfertility, obesity, prescriptive drug use, and familial occurrence of hypospadias or testicular cancer were associated with hypospadias in general. For familial occurrence of hypospadias, there were high risk estimates for the distal and middle phenotypes with an OR (95%CI) of 10.4 (4.5-24.1) and 9.0 (3.1-26.0), but not for the proximal type at 1.8 (0.2-14.9). By contrast, the association with low birth weight (a proxy for placental dysfunction) seemed much stronger for proximal hypospadias with an OR (95%CI) of 9.1 (3.4-24.2) compared with distal and middle hypospadias at 2.6 (1.4-5.0) and 2.3 (0.8-6.5). There were similar estimates for pre-eclampsia., Conclusion: These findings indicate aetiological heterogeneity of hypospadias and provide indications for the possible mechanisms through which specific risk factors may interfere with penile development.

Recently, in The Nederlands Tijdschrift voor Geneeskunde (Dutch Journal of Medicine), three papers expressing with conflicting opinions on the management of primary and acquired non-scrotal testes (NST) in boys were published. In this paper, the Paediatric Urology Workgroup of the Dutch Urological Association summarizes its current recommendations on treatment strategies for this condition: Primary NST is best treated with early orchiopexia at age > 6 months. Therefore, boys with NST should be referred as early as the age > 6 months. Boys with non-palpable NST should always be referred for analysis regardless of their age. The best treatment for acquired NST is less clear than that for primary NST. However, the fact that recent studies show a diminished risk for testicular cancer if orchiopexia is performed before puberty, is a strong argument to not postpone orchiopexia until puberty or later, even if a significant proportion of these testes will descend around puberty. In addition, it has not been proven that the non-scrotal position, of an acquired NST for a number of years, has no deleterious effects on the testis and its later fertility potential. The above-mentioned recommendations are in agreement with the recently published 'Nordic consensus on treatment of undescended testes'.

--Guidelines for the treatment ofundescended testis (UDT) are sparse. Often an operation in the second year of life is advised. --Recent data indicate that the normal maturation process, which will ultimately lead to a normal quantity and quality of germ cells, is impaired as early as in the first half year of a newborn's life. None of the guidelines take this into account. Spontaneous descent after the fourth month following birth, of testes that have previously not descended, hardly ever occurs. --No differences have been shown in complication numbers between surgery before and after the first birthday. Orchidopexy prior to the 13th birthday reduces the risk of testicular cancer. --Therefore, based on these data, it is advised to perform orchidopexy in the second half of the first year of a newborn's life. In older boys a UDT must be treated before the 13th birthday. --In the Netherlands a lot more orchidopexies are done despite what may be expected based on prevalence numbers of UDT: testes retaining a normal volume that would most probably have descended spontaneously come puberty. --It remains important to carry out a careful physical examination and document the position of the testes soon after birth, and later on if UDT is suspected, to avoid unnecessary operations on retractile testes and acquired UDT.

Bladder augmentation is an important tool in the management of children requiring reconstructions for urinary incontinence or preserving of the upper urinary tract in congenital malformations. We reviewed the literature and evaluated the long-term results of enterocystoplasty in the pediatric age group and summarized techniques, experimental options and future perspectives for the treatment of these patients. For this purpose, a directed Medline literature review for the assessment of enterocystoplasty was performed. Information gained from these data was reviewed and new perspectives were summarized. The ideal gastrointestinal (GI) segment for enterocystoplasty remains controversial. The use of GI segments for enterocystoplasty is associated with different short and long-term complications. The results of different centers reported in the literature concerning urological complications after enterocystoplasty are difficult to compare because of the non-comparable aspects and different items included by different authors. On the other hand, there are more and more case reports about cancer arising from bowel segments used for bladder augmentation in recent publications.Although bladder reconstruction with GI segments can be associated with multiple complications, such as metabolic disorders, calculus formation, mucus production, enteric fistulas and potential for malignancy, enterocystoplasty is unfortunately still the gold standard. However, there is an urgent need for the development of alternative tissues for bladder augmentation.

Despite being one of the most common congenital defects in boys, the etiology of hypospadias remains largely unknown. In this case-referent study, we evaluated a wide spectrum of potential risk factors for hypospadias. Cases were identified from the hospital information system, and referents were recruited through the parents of the cases. Both parents of cases and referents completed written questionnaires that they received through the mail. Logistic regression analyses were used to assess the independent contribution of different factors to the risk of hypospadias. The final database included 583 cases and 251 referents. Hypospadias more often occurred in children whose father had hypospadias (OR=9.7; 95%CI: 1.3-74.0) and in children with a low birth weight (OR=2.3; 95%CI: 1.2-4.2). Indications for elevated risks were found when mothers were DES-daughters (OR=3.5; 95%CI: 0.8-15.6), fathers were subfertile (OR=1.8; 95%CI: 0.7-4.5), the parents had undergone fertility treatment (OR=2.3; 95%CI: 0.9-5.8), and in twin or triplet pregnancies (OR=2.0; 95%CI: 0.8-5.1). Maternal use of iron supplements (OR=2.2; 95%CI: 0.8-6.0), maternal smoking (OR=1.5; 95%CI: 1.0-2.4), paternal prescriptive drug use (OR=2.6; 95%CI: 1.1-6.6), and paternal exposure to pesticides (OR=2.1; 95%CI: 0.6-7.1) during the 3 months immediately prior to conception or in the first trimester of pregnancy also appeared to increase the risk of hypospadias. The associations found in this study support the hypothesis that genetic predisposition, placental insufficiency, and substances that interfere with natural hormones play a role in the etiology of hypospadias.

Purpose: We evaluated the long-term results of autoaugmentation in the pediatric age group and summarized technical adaptations, experimental options and future perspectives for treating these patients., Materials and Methods: A directed MEDLINE literature review was performed to assess different techniques and alternative options in autoaugmentation procedures. Of 150 studies 49 in the subgroup with the longest duration of followup to show the long-term outcome of the autoaugmentation procedures were chosen for this review. Information gained from these data was reviewed and new perspectives were summarized., Results: Enterocystoplasy is an effective mode of therapy with acceptable morbidity and satisfactory clinical results, although it is major intraperitoneal surgery with various complications and patients need prolonged convalescence to adapt to these surgical procedures. On the other hand, patient selection seems to be the most crucial step for the success of autoaugmentation procedures because the clinical outcome does not appear to be durable., Conclusions: Achievement of better compliance after autoaugmentation procedures seems to be less pronounced and of shorter duration than that of conventional enterocystoplasty. On the other hand, the low morbidity and lack of side effects of bowel integration into the urinary tract are the definite advantages of this technique. It is the responsibility of the physician to determine the balance between the limited efficacy of the procedures vs the definite advantages. Although functionally improved parameters are obtained in tissue engineered autologous bladders, there is an absolute need for additional studies before this challenging technique could be applied widely.

Background: In 2002, an increased risk of hypospadias was reported for sons of women exposed to diethylstilbestrol (DES) in utero, suggesting transgenerational effects of DES. The aim of this study was to further assess the association between parental DES exposure and hypospadias in a case-referent study., Methods: Cases with hypospadias were retrieved from the hospital information system. Referents were recruited via the parents of cases. Both parents completed postal questionnaires. Associations were estimated by odds ratios (OR) with 95% confidence intervals (CI). Additionally, conditional logistic regression analyses were performed for a matched subset of parents., Results: The final database included 583 cases and 251 referents. In the initial analyses, an indication was found for an increased risk of hypospadias when mothers were exposed to DES in utero: OR=2.3 (95% CI 0.7-7.9). Conditional logistic regression resulted in a stronger risk estimate: OR=4.9 (95% CI 1.1-22.3). Paternal exposure to DES did not increase the risk., Conclusions: The results confirm an increased risk of hypospadias when mothers were exposed to DES in utero. However, the excess risk appears to be of much smaller magnitude than in the 2002 study. Further research on the potential health risks for the third generation is of great importance.

Purpose: We investigated the prevalence and nature of LUTS after renal Tx in children. The focus of the study was the presence of LUTS in children without a history of urological symptoms. We also studied the relationship between the characteristics of these patients and the occurrence of LUTS., Materials and Methods: Data were gathered using a written questionnaire, frequency volume chart, free uroflowmetry, transabdominal ultrasonography and medical records. The study group (30 patients) consisted of 9 children (30%) undergoing renal transplantation with an underlying urological disease and 21 (70%) with an underlying nephrological disease., Results: In the nephrological group the incidence of high capacity bladder was 75%, residual urine 50%, UTI 43%, hesitancy 38%, intermittent flow 33%, bladder pain 33%, nighttime incontinence 29%, nocturia 24%, feeling of incomplete emptying 15%, daytime incontinence 14%, straining 14%, urgency 10%, burning sensation 10% and intermittency 5%. No substantial difference in the occurrence of LUTS, UTI or high bladder capacity after Tx was found between children with an underlying urological disease and those with an underlying nephrological disease. On average, patients in both groups suffered from 3 different LUTS., Conclusions: After renal Tx children with a nephrological disease demonstrated a high incidence of LUTS. The occurrence of LUTS combined with UTI and increased bladder capacity indicates that these children are at risk for development of myogenic failure. This finding emphasizes the importance of close urological followup after Tx in children with urological and nephrological disease.

Purpose: We evaluated the outcome of postpubertal patients who underwent 1-stage hypospadias repair before onset of puberty with at least 10 years of followup., Materials and Methods: A total of 2,053 patients underwent surgical repair of hypospadias between January 1981 and May 2004 at our hospital. We evaluated patients who are now at least 18 years old and who underwent 1-stage hypospadias repair. Data were collected in regard to familial incidence, age at operation, operative technique, complications and followup of at least 10 years. Evaluation was subdivided based on operative procedure., Results: A total of 126 patients were included in the study. The familial incidence was 7.9% of cases and an associated undescended testis was seen in 9.5%. There were 62 cases with no complications. At least 1 complication was seen in 64 cases, including infection in 3, tissue necrosis in the operative area in 3, wound dehiscence in 6, fistula in 29, meatal stenosis in 15, urethral stenosis in 10, cosmetic dissatisfaction in 16 and other in 2., Conclusions: Even in the hands of the experienced pediatric urologists the complication rate after hypospadias repair remains high, with an average of 54% at long-term followup. Our results clearly showed that additional complications can present after long-term followup (a first complication can present after more than 5 years with the longest interval being 14 years). Therefore, final outcome of hypospadias surgery can only be evaluated once the patient has reached adulthood. The results seem to depend on the severity of the abnormality, reflected in the complication distribution rather than operative technique.

A rabbit model was used for the evaluation of a collagen-based biomatrix of small intestinal submucosa (SIS, COOK) in comparison to a biochemically reconstructed biomatrix for bladder tissue regeneration. Rabbits underwent partial cystectomy and cystoplasty with SIS patch graft or with a biochemically defined collagen biomatrix. The grafts of the regenerated bladder wall were harvested at different intervals and tissue regeneration was evaluated. The results of the SIS and biochemically defined biomatrix grafts were comparable. At harvesting, we found five bladder stones and encrustation of the biomatrix in 21/56 animals. No stone formation was observed in the control group. The results of the molecularly defined biomatrix are thus far comparable to SIS. Both matrices show good epithelialization and ingrowth of smooth muscle cells. Both biomatrices show considerable encrustation, which appears to disappear in time. The rabbit model is suitable for bladder tissue engineering studies as it is an easy model to use. In this model, besides tissue regeneration, also some of the clinical problems are seen such as encrustation of foreign body material in the bladder. These aspects are subject for further pre-clinical studies in this animal model.

Objective: To study the pathophysiological changes of renal tubular drug transport mechanisms in congenital renal obstruction, by developing a model for perfusing the isolated kidney (IPK) after neonatal surgical induction of partial ureteric obstruction in Hanover Wistar rats., Material and Methods: Moderately severe obstruction of the right kidney of male rats was created by burying a segment of the right ureter under the psoas fascia at 5-7 days after birth. Different fluorescent substrates for renal organic anion and cation drug transport systems were added to the IPK, and the concentration of these substances with time analysed in perfusate and urine., Results: The reproducibility in all groups of the glomerular filtration rate (GFR) and drug excretion was remarkably good. GFR was significantly lower in obstructed kidneys than in unobstructed kidneys. 123Rhodamine, a marker for organic cation and P-glycoprotein transport, had a significantly lower maximum excretion rate in the obstructed than in unobstructed kidneys. Renal fractional clearance (123rhodamine clearance corrected for diminished GFR) was also significantly lower in obstructed kidneys. There was no significant difference in maximum excretion (absolute and corrected GFR) for Lucifer Yellow, a marker for sodium-dependent organic anion transport. The maximum excretion rate of calcein, a marker for sodium-independent organic anion transport, was significantly lower in the obstructed than in the unobstructed kidneys, but significantly higher after correcting for reduced GFR., Conclusion: The IPK is a good model for studying the effect of neonatal renal obstruction on tubular drug transport. These results show that organic anion and cation transport mechanisms are affected differently by obstruction.

Objective: The evaluation of collagen-based biomatrix (SIS COOK((R))) in comparison to a biochemically reconstructed biomatrix for replacement of the urethra in a rabbit model as a preclinical model., Material and Methods: Rabbits underwent partial urethra replacement (resection of 0.5 to 1.0 cm segment of the urethra), which was replaced with 1 or 4 layers Small Intestinal Submucosa (SIS COOK) patch grafts or with a biochemically defined collagen biomatrix, partly sutured with unresolvable sutures for future reference. Six animals underwent a sham control operation. The grafts of regenerated urethras were harvested at 1, 3 and 9 months after implantation. Urethrography was performed pre-operatively and before sacrificing. The animals were evaluated macroscopically and by routine histology and immunohistochemistry., Results: At 1 month after implantation, the biomatrices (1 layer, 4 layers and our biochemically defined biomatrix) were well distinguishable from the normal surrounding tissues and showed blood vessels at the periphery. Macroscopically, the unresolvable reference sutures were easy to find at all time points. At 3 months the graft was still distinguishable in the 4 layers SIS group. In the 1 layer and the defined biomatrix group a good regeneration of the urethra within the graft was seen with some central fibrosis. Histological and immunohistochemical evaluation showed urothelium regeneration on the 1 layer and on biochemically defined biomatrix with decreasing number of inflammatory cells from 1 month on. In the group treated with 4 layers SIS the urothelium was completely regenerated at 3 months. Histologically, the regeneration of muscle cells in the three biomatrices was comparable. The smooth muscle cells regenerated very slowly as 1 month after implantation no muscle cells were detectable within the grafts. At 3 months a few muscle cells were present in the graft, but cell density did not increase in the following 6 months. Strictures were not observed on control urethrography pre-operatively in the animals. In one case slight narrowing of the urethra on urethrography was seen, but apparently without causing voiding problems. One rabbit developed a fistula near the operation site., Conclusion: The biomatrices investigated are feasible scaffolds to repair urethral lesions. The results with our biochemically defined biomatrix are comparable to one layer Small Intestinal Submucosa. Almost no smooth muscle cells population was observed after nine months for the three biomatrices. We conclude that an improved molecularly defined biomatrix focussed on stimulation of smooth muscle cell growth may be necessary to obtain optimal cellular grafting results.

A foreign body in the bladder is a well-recognized, although rare, cause of urinary tract infections. We describe a 15-year-old girl who presented with abdominal pain and recurrent urinary tract infections. On analysis, a forceps was found, with the two legs of the instrument separately perforating both the back wall of the bladder and the trigone, with the top of the forceps lying in the vagina, covered with a large calculus. The forceps must have been left behind during laparotomy for bowel invagination in her first year of life.

Aims: Ruptures of the uropoietic system resulting in either urinary ascites or urinoma are rare complications in the neonate. Although ruptures without clear predisposing factors are described, in most cases they are associated with obstructive uropathy. The diagnosis is often delayed and the prognosis is related to the degree of renal damage. There is discussion about possible protective mechanisms of the rupture for renal function in patients with obstructive uropathy., Methods: We retrospectively analyzed the clinical presentation, predisposing factors and the renal function before and after treatment of 10 neonates with a rupture of the pyelum or urinary bladder in our hospital., Results: The group consisted of 9 boys and 1 girl. The average birth weight was 3,880 g. The patients presented with distended abdomen (n = 10), abdominal mass (n = 2), ascites (n = 5), oligohydramnion (n = 2), hypertension (n = 1) and anuria (n = 1). Underlying diagnosis included obstruction of the ureteropelvic junction (UPJ obstruction) in 3 children and posterior urethral valves in 7 children. Five children presented with urinoma, 3 children had a urinoma in combination with ascites and 2 children had isolated ascites. All children had reduced renal function at the time of diagnosis. In all 10 cases, the serum creatinine decreased after treatment. Scintigraphic investigation with mercapto-acetyltriglycerine (MAG III) demonstrated diminished function and perfusion of all 3 kidneys with UPJ obstruction and isolated urinoma even after treatment. Children with posterior urethral valves and urinoma revealed better function of the ruptured kidney and diminished function of the kidney which was not ruptured. One child with a rupture of the urinary bladder and urinary ascites showed good function and perfusion of both kidneys., Conclusion: Perinatal ruptures of the uropoietic system are rare. The clinical presentation is aspecific. One should consider a rupture of the urinary bladder or pyelum in a neonate with a distended abdomen, hydronephrosis and ascites. The long-term prognosis depends on the underlying diagnosis and the location of the rupture. Probably, a UPJ obstruction with an isolated urinoma is associated with irreversible renal damage of the ruptured kidney. A rupture resulting in urinary ascites apparently provides better decompression with better function of the ruptured kidney. Scintigraphic investigation is necessary for a separate evaluation of the single kidney function.

Objective: In this study, we evaluated the urological complications and their consequences after renal transplantation in children., Materials and Methods: A retrospective study was performed concerning urological complications after kidney transplantation in children at the University Medical Center St Radboud Nijmegen from August 1977 until July 1999. The mean age of the cadaver kidney donors was 23.2 (range 1-74.3) years., Results: In this period 146 children received 183 renal allografts. Twenty-four urological complications (13.1%) were noted in 23 patients. Urinary leakage was seen in 8 cases (4.4%) and a ureteral stenosis was present in 7 cases (3.8%), 5 patients developed stones (2.7%) and 4 severe lymph leakage needing surgical treatment (2.2%). In 3 patients the urological complication induced graft loss. In cadaver kidneys from donors younger than 5 years there was no significantly increased complication rate in comparison to older cadaver kidneys [2/32 (6.3%) versus 16/125 (12.2%)]. In a subgroup of 24 patients treated for severe posterior urethral valves the complication rate was not significantly increased in comparison to the others (8.3% in the group with posterior urethral valves against 13.7% in the group without)., Conclusions: Urological complications are a small threat for graft function and the results are comparable with earlier studies. Routine ultrasound can be important for early detection of problems in the postoperative period. A close observation in the postoperative care and good collaboration between pediatric nephrologist and urologist is mandatory. Sometimes, early intervention can be undertaken with the possibility of saving graft function and reducing morbidity.

Benign and malignant testicular tumors are rare in infancy. Moreover, only a few cases of bilateral testicular tumors in children have been reported to date. To our knowledge, we report the first case of an asynchronous bilateral simple testicular cyst and testicular teratoma in an infant. This case demonstrates that although both lesions are benign in the prepubertal child, treatment decisions should be made carefully.

Purpose: We evaluated new intervention techniques and surgical instruments in a fetal monkey model to determine improvements that would be useful for early intrauterine intervention. Our findings may be helpful in the future for treating select cases of severe prenatal obstructive uropathology., Materials and Methods: In a series of experiments on 18 pregnant rhesus monkeys (Macaca mulatta) at mid trimester we assessed various endoscopic intra-amniotic access techniques as well as morbidity, mortality and possibilities for fetoscopy., Results: In all 18 fetuses adequate fetoscopy was possible with no maternal mortality. Of the 18 pregnancies 14 went to term with no early or late postoperative complications. Technical improvements changed the intrauterine access technique from open placement of trocars to the use of the Seldinger technique, gun introduction of needles with small caliber sheets and small caliber introduction trocars, resulting in minimal amniotic membrane separation. Various rigid and flexible endoscopes were evaluated for fetoscopy and up to 3 cannulas were placed. No change in the fetal growth pattern was observed on postoperative ultrasound. Subsequent pregnancies occurred during this study period, and there were no acceptance problems of the newborns by the mothers., Conclusions: New techniques have led to improved intrauterine fetal access. Morbidity mainly depends on the disruption of amniotic membranes, which has an important preterm role. Adapted endoscopes and other instruments offer new possibilities for fetal diagnosis and therapy in the future. Our primate model seems to be suitable for evaluating these new techniques before they are used in a clinical setting.

We present a rare complication causing severe infravesical obstruction after hypospadias repair after an initially successful double-faced island flap repair. The obstruction was caused by partial dissection of the epithelium of the urethral wall, comparable to intima dissection in an aortic aneurysm. To our knowledge, this type of urethral obstruction after a hypospadias repair has not been reported before.

In 207 men with symptomatic benign prostatic hyperplasia the international prostate symptom score and score on quality of life were collected. Cystometric tests with pressure-flow studies were performed in these patients with special attention to the assessment of obstruction grade. Of the men 24% appeared not to have obstruction. A significant correlation was found between size of the prostate and grade of obstruction. No correlation was found between obstruction grade, and any symptom score or quality of life score, while symptom score and quality of life correlated well. In 41 patients the outcome of laser prostatectomy was evaluated 6 months after the procedure. Mean symptom score decreased from 19.1 to 5.4 and mean quality of life score decreased from 3.7 to 1.3 in the 31 obstruction patients, compared to decreases of 19.6 to 10.1 and 3.6 to 2.6, respectively, in 10 without obstruction. The improvement in the obstruction patients was significantly better but nonobstruction patients will benefit from prostatectomy as well.

Objective: Assessment of the results of laser prostatectomy, as a treatment for benign prostatic hyperplasia (BPH)., Design: Prospective case control study., Setting: University Hospital Utrecht, the Netherlands., Method: Between February 1992 and May 1993, 54 men with their micturition complaints due to BPH were treated with laser prostatectomy (TULIP system). Results were assessed using the international prostatic symptom score (IPSS), the maximal flow and urodynamic tests. The results were compared retrospectively with results of transurethral resection of the prostate (TURP; n = 40): both groups were urodynamically identical., Results: Of the 54 patients, 10 could not be evaluated 6 months after treatment (5 of them underwent TURP or a second laser prostatectomy). In 40 patients complete evaluation including urodynamics before and six months after treatment was possible. A significant decrease in the symptom score from 19.3 (SD: 7.6) to 6.3 (SD: 5.4) and increase of the maximal flow during pressure-flow studies from 9.6 to 15.8 ml per second were observed. The decrease of the voiding pressure at 6 months after TURP in comparison with laser prostatectomy was close to significance (p = 0.05); the other improvements after urodynamics were comparable., Conclusion: Laser prostatectomy is a promising new therapy for BPH.