Swiss consensus recommendations on urinary tract infections in children.

The kidneys and the urinary tract are a common source of infection in children of all ages, especially infants and young children. The main risk factors for sequelae after urinary tract infections (UTI) are congenital anomalies of the kidney and urinary tract (CAKUT) and bladder-bowel dysfunction. UTI should be considered in every child with fever without a source. The differentiation between upper and lower UTI is crucial for appropriate management. Method of urine collection should be based on age and risk factors. The diagnosis of UTI requires urine analysis and significant growth of a pathogen in culture. Treatment of UTI should be based on practical considerations regarding age and presentation with adjustment of the initial antimicrobial treatment according to antimicrobial sensitivity testing. All children, regardless of age, should have an ultrasound of the urinary tract performed after pyelonephritis. In general, antibiotic prophylaxis is not recommended.Conclusion: Based on recent data and in line with international guidelines, multidisciplinary Swiss consensus recommendations were developed by members of Swiss pediatric infectious diseases, nephrology, and urology societies giving the clinician clear recommendations in regard to diagnosis, type and duration of therapy, antimicrobial treatment options, indication for imaging, and antibiotic prophylaxis. What is Known: • Urinary tract infections (UTI) are a common and important clinical problem in childhood. Although children with pyelonephritis tend to present with fever, it can be difficult on clinical grounds to distinguish cystitis from pyelonephritis, particularly in young children less than 2 years of age. • Method of urine collection is based on age and risk factors. The diagnosis of UTI requires urine analysis and significant growth of a pathogen in culture. What is New: • Vesicoureteric reflux (VUR) remains a risk factor for UTI but per se is neither necessary nor sufficient for the development of renal scars. Congenital anomalies of the kidney and urinary tract (CAKUT) and bladder-bowel dysfunction play a more important role as causes of long-term sequelae. In general, antibiotic prophylaxis is not recommended. • A switch to oral antibiotics should be considered already in young infants. Indications for invasive imaging are more restrictive and reserved for patients with abnormal renal ultrasound, complicated UTI, and infections with pathogens other than E. coli.

Urological Outcome after Fetal Spina Bifida Repair: Data from the Zurich Cohort.

INTRODUCTION: Neurogenic lower urinary tract dysfunction (NLUTD) represents a severe burden for patients with open spina bifida (OSB). The effect of fetal OSB repair on the urological outcome remains unclear, as controversial data exist. The aim of this study was to further increment existing outcome data and to demonstrate that our earlier published positive preliminary results are not erratic. METHODS: Data from standardized urological follow-up appointments of patients with fetal OSB repair operated at our center were analyzed. Data were obtained from urodynamic studies (UDSs) and radiologic exams performed in the newborn (gestational age 37-39 weeks), at ages of 6, 12, 18, and 24 months, and then at yearly intervals. RESULTS: Of 82 patients (mean age 2.6 years, range 6 months to 7 years), 26 (32%) had a normal bladder function as demonstrated by UDSs. Of the 56 (68%) patients with NLUTD, 29 (51%) patients showed initially a normal UDS, but developed NLUTD in the follow-up, 19 (66%) of them spontaneously and another 10 (34%) in association with growth and development, or surgery of inclusion cysts. Radiologic abnormalities (upper tract dilatation and vesico-uretero-renal reflux) were seen in 15%, mainly patients with NLUTD. CONCLUSION: Our results add an important set of information to the existing body of evidence. The data reconfirm our earlier published favorable preliminary results and support other studies that show a possible benefit of prenatal OSB repair on the urological outcome, but they also demonstrate that the positive effect remains limited.

Increased autophagy contributes to impaired smooth muscle function in neurogenic lower urinary tract dysfunction.

AIMS: To explore whether autophagy plays a role in the remodeling of bladder smooth muscle cells (SMCs) in children with neurogenic lower urinary tract dysfunction (NLUTD), we investigated the effect of autophagy in NLUTD in the paediatric population. METHODS: Bladder biopsies were taken from children with NLUTD and healthy donors as controls. Samples were labeled with the SMC markers calponin, smoothelin, and the autophagy proteins LC3, ATG5, and Beclin1. The contractile ability of bladder derived SMCs was investigated. RESULTS: ATG5 gene and protein was upregulated in NLUTD muscle tissue compared to normal bladder. NLUTD muscle exhibited a punctated immunostaining pattern for LC3 in a subset of the SMCs, confirming the accumulation of autophagosomes. Pronounced elevation of ATG5 in the SMC in NLUTD tissue was associated with a downregulation of the key contractile proteins smoothelin and calponin. Pharmacological blocking of autophagy completely stopped the cells growth in normal bladder SMCs. Inhibition of autophagy in the NLUTD SMCs, with already elevated levels of ATG5, resulted in a reduction of ATG5 protein expression to the basal level found in normal controls. CONCLUSIONS: Our study suggests that autophagy is an important factor affecting the remodeling of SMCs and the alteration of functionality in bladder smooth muscle tissue in the NLUTD. Since autophagy can be influenced by oral medication, this finding might lead to novel strategies preventing the deterioration of NLUTD muscle.

Prenatal Megacystis - Is Prediction of Outcome and Renal Function Possible?

OBJECTIVE: To assess prenatal prognostic criteria for fetuses with megacystis in order to counsel parents. METHODS: In a retrospective observational study at a single tertiary referral center, we assessed the clinical course of 53 fetuses with megacystis cared for at the Department of Obstetrics of the University Hospital Zurich between 1995 and 2008 and followed them up for 2 to 12 years. We determined fetal karyotype, amniotic fluid volume and fetal urinary biochemistry as prenatal prognostic factors. The renal function of survivors was grouped according to age-related creatinine values. Using logistic regression analysis, gestational age-dependent discrimination curves and corresponding ROC curves for fetal urine, beta-2 microglobulin, osmolarity and chloride were calculated. RESULTS: 43 out of 53 fetuses underwent vesicocentesis, and spontaneous remission occurred in 3 fetuses. 15 fetuses survived, termination of pregnancy was requested in 23 cases, and 12 neonatal and 3 intrauterine deaths were observed. Reduced amniotic fluid volume showed a significant (p = 0.0027) increase of impaired renal function or perinatal death. Discrimination between survivors and non-survivors was complete for fetal urine beta-2 microglobulin with an area under the curve (AUC) of 1.0. For fetal urine osmolarity and fetal urinary chloride, the AUC was 0.81 and 0.76, respectively. CONCLUSION: The assessment of prognosis for fetal megacystis should include fetal k aryotyping, determination of amniotic fluid and assessment of fetal urine biochemistry. Gestational age-dependent regression lines disclose clinically relevant discrimination and can be used as selection criteria for fetal interventions and parental counselling.

Prenatal myelomeningocele repair: Do bladders better?

AIMS: Prenatal myelomeningocele (MMC) repair has been proven to significantly reduce the need for hydrocephalus shunting and improve lower-extremity motor outcomes. The aim of this study was to evaluate the effect of prenatal MMC repair on the urological outcome. METHODS: All patients who underwent fetal MMC repair at our institution are followed prospectively. Assessments include medical history, renal and bladder ultrasound, voiding cystourethrogram and urodynamic study, need for clean intermittent catheterization (CIC) and anticholinergics, and the occurrence of urinary tract infections (UTI). RESULTS: Of the 30 patients who underwent prenatal MMC closure from December 2010 to December 2015, eight patients with a postnatal follow-up of at least 2 years were included in this study and compared with eight patients after postnatal MMC repair. The level of the bony spinal defect was similar in both groups. Urological evaluation at 2 years revealed normal bladder function in 50% after prenatal repair. Neurogenic bladder dysfunction requiring CIC and anticholinergic therapy was seen in 50% in the prenatal and in 100% in the postnatal group. Significant bladder wall thickening was found in 37.5% and 87.5%, respectively. Febrile UTIs occurred in 37.5% in the prenatal and 62.5% in the postnatal group during the observation period. CONCLUSIONS: Our data suggest a positive effect of prenatal MMC closure on lower urinary tract function. The long-term significance of these results remains unclear. Therefore, continued close monitoring of renal and bladder function are mandatory.

Outcome after prenatal diagnosis of congenital anomalies of the kidney and urinary tract.

UNLABELLED: Congenital anomalies of the kidney and urinary tract are common findings on fetal ultrasound. The aim of this prospective observational study was to describe outcome and risk factors in 115 patients born 1995-2001. All prenatally diagnosed children were stratified into low- and high-risk group and followed postnatally clinically and by imaging at defined endpoints. Risk factors were evaluated using odds ratios. Neonatal diagnosis included pelvi-ureteric junction obstruction (n = 33), vesicoureteral reflux (n = 27), solitary mild pelvic dilatation (postnatal anteroposterior diameter 5-10 mm; n = 25), and further diagnosis as primary obstructive megaureter, unilateral multicystic dysplastic kidney, renal dysplasia and posterior urethral valves. In 38 children with prenatal isolated hydronephrosis, ultrasound normalized at median age of 1.2 years (range 0.1-9). Surgery was performed in 34 children at median age of 0.4 years (0.1-10.8). Persistent renal anomalies without surgery were present in 43 children and followed in 36 for median time of 16 years (12.2-18). Oligohydramnios and postnatal bilateral anomalies were significantly associated with surgery and impaired renal function. CONCLUSION: The majority of children had a favourable postnatal outcome, in particular children with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis. Oligohydramnios and postnatal bilateral anomalies were risk factors for non-favourable outcome. WHAT IS KNOWN: • In congenital anomalies of the kidney and urinary tract significantly poorer outcome is known in patients with bilateral renal hypoplasia or solitary kidney associated with posterior urethral valves. • Other factors as proteinuria and vesicoureteral reflux were associated with a higher risk of progression to chronic renal failure in these patients. What is New: • Unlike other studies giving us above-mentioned information, we included all patients with any kind of prenatally diagnosed congenital anomalies of the kidney and urinary tract. Our study shows long-term follow up (median 16 years, range 12.2-18 years), especially in patients not needing surgery, but with persistent anomalies. • During postnatal long-term follow up (median 2.2 years, range 0.1-18 years) one third each showed normalization, need of surgery or persistence of anomalies without need of surgery. Our study revealed a good prognosis in the majority of these children, in particular with prenatally low risk, i.e. isolated uni- or bilateral hydronephrosis, and revealed oligohydramnios and postnatal bilateral anomalies as risk factors for a non-favourable outcome, defined as need of surgery, persistent anomalies with impaired renal function, end stage renal failure or death.

What is a Good Looking Penis? How Women Rate the Penile Appearance of Men with Surgically Corrected Hypospadias.

INTRODUCTION: Some men with corrected hypospadias perceive their penile appearance to be abnormal, although health professionals consider these results satisfactory. AIM: The aim of this study was to investigate how relevant women consider single aspects of penile appearance to be. Moreover, we studied whether women perceive hypospadias-affected surgically repaired genitals (HASRGs) to be as normal-looking as circumcised genitals and identified the most relevant predictors that influence whether a penis is perceived as normal. METHODS: In this cross-sectional study, 105 women in different age groups (age range: 16-20, 25-30, and 40-45 years) completed a standardized questionnaire. MAIN OUTCOME MEASURES: Participants had to rate the importance of eight penile aspects and to indicate how normal they found the appearance of standardized photos of 10 HASRGs and of 10 circumcised genitals. Furthermore, they were asked about demographic characteristics and their sexuality. RESULTS: Results showed that women considered the position and shape of the meatus as the least important penile aspect. Furthermore, results showed that HASRGs with distal hypospadias were perceived to be as normal-looking as circumcised genitals, whereas genitals with more proximal hypospadias were perceived as significantly less normal. However, the difference need not be considered clinically relevant, as the effect size was small. Observer-related predictors of a more positive perception of HASRGs were higher age, higher sexual interest, and perceiving the general cosmetic penile appearance as more important and penile length as less important. CONCLUSIONS: Overall, women were found to consider the "position and shape of the meatus" as the least important penile aspect. These findings may stimulate reflections regarding the relevance of surgical correction of the meatus in minor forms of hypospadias. In addition, this study indicates that women perceived genitals of men with distal operated hypospadias (which represents the majority of hypospadias) to be as normal as nonaffected, circumcised genitals.

The Penile Perception Score: an instrument enabling evaluation by surgeons and patient self-assessment after hypospadias repair.

PURPOSE: Studies of the outcome of hypospadias repair must document quality, including assessment of complications and appraisal of appearance. To our knowledge the Pediatric Penile Perception Score is the first validated instrument for the outcome assessment of hypospadias repair in prepubertal males by surgeons and patients. We validated the instrument for adult genitalia. MATERIALS AND METHODS: Standardized photographic documentation was prepared for 19 men after hypospadias repair and 3 with normal genitalia after circumcision. This was sent to 21 urologists, who rated the outcome with a questionnaire comprising items on the penile meatus, glans, shaft skin and general appearance. Each item was rated with a 4-point Likert scale. The Penile Perception Score is a sum score of all items. Patients were asked to provide a self-assessment with the same instrument. RESULTS: When calculated with the ICC and the rank correlation using Kendall W, concordance among urologist scores was fair and good (0.46 and 0.64, respectively, p <0.001). Instrument stability was 0.78, indicating good reproducibility. Using the Spearman rank correlation coefficient general appearance correlated well with single items, including the meatus (r = 0.93, p = 0.000), glans (r = 0.92, p = 0.000) and shaft skin (r = 0.89, p = 0.000). No significant differences were found between patient and urologist Penile Perception Scores. CONCLUSIONS: The Penile Perception Score is a reliable instrument for urologist assessment and self-assessment of postpubertal genitalia after hypospadias repair. The instrument can be recommended for all age groups because it was previously validated for the pediatric population.

Multisystem neuroprosthetic training improves bladder function after severe spinal cord injury.

UNLABELLED: Severe spinal cord injury leads to neurogenic bladder dysfunction. We recently developed a multisystem neuroprosthetic training program that promotes plastic changes capable of restoring refined locomotion in rats with severe spinal cord injury. We investigated whether multisystem neuroprosthetic training would influence the development of posttraumatic bladder dysfunction. MATERIALS AND METHODS: Eight and 4 adult rats were randomly assigned to a spinal cord injury and an intact control group, respectively. Spinal cord injury consisted of 2 opposite lateral hemisections (T7 and T11), thus, interrupting all direct supraspinal input. After spinal cord injury 4 rats were subjected to a multisystem neuroprosthetic training program and 4 were not trained. At 8 weeks we performed urodynamics and evaluated kidney function using creatinine and cystatin C. Bladder investigation included morphological, histological and immunohistochemical evaluations. RESULTS: Bladder capacity increased threefold in trained and sevenfold in nontrained rats compared to intact rats. During filling we found a mean ± SEM of 2.7 ± 1.1 vs 12.6 ± 5.2 nonvoiding contractions in trained vs nontrained rats. Bladder morphology was similar in trained and intact rats. Nontrained rats showed detrusor hypertrophy, characterized by increased detrusor thickness and a decreased connective tissue-to-smooth muscle ratio. As labeled with protein gene product 9.5, general nerve density was significantly increased in trained and significantly decreased in nontrained rats. The relative proportion of neurofilament 200 positive afferent nerves was significantly lower in trained than in intact and nontrained rats. Neuropeptide Y positive fibers showed significantly lower density in nontrained rats. CONCLUSIONS: Multisystem neuroprosthetic training effectively counteracts the formation of neurogenic bladder dysfunction after severe spinal cord injury and might contribute to preserving bladder function and preventing long-term complications in patients with severe spinal cord injury.

Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe.

OBJECTIVE: To identify genetic and nongenetic risk factors that contribute to the severity of the bladder exstrophy-epispadias complex (BEEC). STUDY DESIGN: Patients with BEEC from North America (n = 167) and Europe (n = 274) were included. The following data were collected: associated anomalies, parental age at conception, mode of conception, periconceptional folic acid supplementation, maternal risk factors during pregnancy, and environmental risk factors. The patients were divided into 3 subgroups according to phenotype severity: (i) mild, epispadias (n = 43); (ii) intermediate, classic bladder exstrophy (n = 366); and (iii) severe, cloacal exstrophy (n = 31). These subgroups then were compared with identify factors that contribute to phenotype severity. RESULTS: Males were overrepresented in all subgroups. A relatively high prevalence of cleft lip, with or without cleft palate, was observed. Maternal smoking and medical radiation during the first trimester were associated with the severe cloacal exstrophy phenotype. Compliance with periconceptional folic acid supplementation was associated with the mildest phenotype (epispadias). CONCLUSIONS: Periconceptional folic acid supplementation appears to prevent the development of the severe phenotype of BEEC.

Repeated Botulinum-A toxin injection in the treatment of neuropathic bladder dysfunction and poor bladder compliance in children with myelomeningocele.

AIMS: To evaluate the efficacy of intradetrusor injection of Botulinum-A toxin (BoNT/A) in children with neuropathic bladder dysfunction and poor bladder compliance, due to meningomyelocele. METHODS: We retrospectively reviewed the charts of 11 children (mean age 6.7 ± 5.3 years) with neuropathic bladder and poor bladder compliance (mean 7 ml/cmH(2) O) treated with intradetrusor injection of BoNT/A. After urodynamic evaluation a standardized injection was performed. The authors describe their results in 11 children after the first (5×) or repeated (6×) BoNT/A injections. Re-evaluation was performed 3 and 12 months after each injection. RESULTS: Three months after injection detrusor pressure decreased by 17% and bladder capacity increased by 33%. In all but 1 patient bladder compliance stayed poor. Twelve months after treatment patients still had an effect but the values were already reverting towards preoperative levels. With repeated injections we achieved a similar effect on capacity and detrusor pressure as after the first injection. Overall, bladder compliance normalized (>20 cmH(2) O) in only 1 patient. CONCLUSIONS: The results confirmed the positive temporary effect of BoNT/A on detrusor pressure and bladder capacity in children with myelomeningocele, even after repeated injections. Nevertheless, bladder compliance stayed severely pathological. Whether an earlier treatment may prevent poor bladder compliance is a yet unanswered question.

A rare case of symptomatic hydrometrocolpos in a 5y old female.

Late presentation of symptomatic hydrometrocolpos is uncommon. We present a 5 years old continent girl with prenatally diagnosed multicystic dysplastic left kidney and late-onset of lower abdominal pain. Investigations revealed a nonfunctioning left kidney with an ectopic ureter draining into the left hemivagina, and a vaginal duplication with an obstructed and urine-filled left hemivagina. Surgical therapy included resection of the vaginal septum and laparoscopic nephroureterectomy. Not only renal agenesis but also dysplasia or multicystic kidney may part of an OHVIRA syndrome. In girls with unilateral renal dysplasia, a duplication of the internal genitalia must always be considered.

Laparoscopic Ureteroureterostomy vs. Common Sheath Ureteral Reimplantation in Children With Duplex Kidney Anomalies.

Purpose: Laparoscopic ureteroureterostomy (LUU) has been proposed as an alternative to common sheath ureteral reimplantation (CSUR) in children with symptomatic duplex kidneys. However, data is limited for LUU in the pediatric population. The aim of this study was to analyze our experience with LUU and to compare the results with those after CSUR to assess whether a less invasive surgical approach could be a valid alternative. Patients and methods: The data of all children with duplex kidneys who underwent either LUU or CSUR at our center from 2006 to 2018 were reviewed retrospectively. After parental counseling, the option of LUU was provided as an alternative to CSUR for unilateral procedures and in the absence of vesicoureteral reflux to the receiving ureter. Baseline characteristics, indication for surgery, hospitalization and operative times, and intraoperative, post-operative, and late complications were analyzed. Preoperative and 1-year post-operative sonographies were reviewed by a pediatric radiologist. Increasing renal pelvic diameter (Δ >5 mm) was regarded as a sign of ureteral obstruction. Results: Forty children were included in this study, with 16 children receiving LUU and 24 children receiving CSUR. The children had a mean age of 2.7 years (7 months-9.8 years) and were followed up in our outpatient clinic for an average of 3.9 years (3 months-10.6 years) after surgery. The median hospital stay was 2 days shorter after LUU. Initially, a considerably longer time was needed for LUU, but after more experience was gained, similar operative times were observed for both procedures. Complications were encountered in both groups. After LUU, two patients developed anastomotic leakage: one was managed conservatively, and one required temporary nephrostomy. In the CSUR group, one patient developed vesicoureteral obstruction during follow-up and required reoperation with LUU. The occurrence of post-operative urinary tract infections was similar in both groups. No complications related to the ureteral stump after LUU arose. Conclusion: LUU is a safe and efficacious treatment option for children with duplex kidney anomalies and can be used as an alternative to CSUR. All children receiving LUU showed a non-obstructive, patent anastomosis and no signs for stenotic compromise of the receiving ureter.

[Living with bladder exstrophy - the patients' perspective]. / Trotz Blasenexstrophie ein gutes Leben haben: Untersuchung zum Leben und Umgang mit Blasenexstrophie.

Bladder exstrophy (BE) is a rare but severe urogenital malformation that requires complex clinical management. It can lead to physical restriction as well as to psychosocial and psychosexual malfunction. For the first time, this qualitative study describes the experiences of persons living with BE from childhood into adulthood. The primary goal was to explore the experiences of persons with BE in order to optimise treatment, care and counselling at the children hospital in Zurich. Semi-structured interviews were conducted with 4 women and 16 men (38 to 68 years). Using content analysis techniques, three major categories with two respectively four and five subcategories emerged from the data. The first category "having a good life despite BE" describes the perception of the affected persons to lead a life worth living despite BE. It was categorised by episodes and experiences that were repeated throughout the persons' life, such as "being in hospital", "living with symptoms", "being different", "showing oneself" and "gathering information". The second category "the affected ones" demonstrated that "parents" and "partners" were affected too and provided considerable support to the person with BE. The third category "the influencing factors" revealed that the perception of living with BE was influenced by individual and contextual factors. The results of this study illustrated that persons with BE can live a good life, despite repeated health-related challenges, if the support provided by their caregivers permits a positive attitude towards their illness. For this, patients with BE and their family members require continuous and highly competent treatment, care and education by an interdisciplinary team.

Detrusor bioengineering using a cell-enriched compressed collagen hydrogel.

OBJECTIVE: The gold standard for bladder regeneration in end-stage bladder disease is the use of intestinal tissue, which is however associated with significant long-term complications. Our study aims to bioengineer functional detrusor muscle combining bladder smooth muscle cells (SMC) and SMC-like adipose-derived stem cells (pADSC) in compressed collagen (CC) hydrogels and to investigate biocompatibility and tissue regeneration of such detrusor-equivalents in a rat detrusorectomy model. METHODS: Compressed collagen hydrogels seeded with 1 × 106 or 4 × 106 SMC alone or in combination with pADSC in a 1:1 ratio were investigated. Morphology, phenotype, and viability as well as proteomic secretome analysis were assessed in the 1:1 co-cultures and the respective monocultures. The hydrogels were implanted into rat bladders after partial detrusorectomy. Bladders were harvested 8 weeks after transplantation, and assessed for tissue morphology, detrusor regeneration, neo-vascularization and -innervation. RESULTS: Co-cultured cells exhibited native SMC morphology, high viability and proliferated to form microtissues in vitro. The pro-angiogenic factors angiogenin, vascular endothelial growth factor (VEGF)-A and -D were increased in the secretome of the pADSC samples. After 8 weeks of in vivo, the regenerated bladder wall showed a multilayered structure containing all bladder wall components. The overall performance of the bladder wall regeneration of CC seeded with 4 × 106 cells was significantly better than with 1 × 106 cells and the combination SMC:pADCS performed slightly better than SMC alone. CONCLUSION: Compressed collagen possesses an adequate regenerative potential to promote regeneration of bladder wall tissue in vivo. Seeded with a combination of pADSC and SMC this may well be the first step towards a functional bladder reconstruction especially in patients suffering of end-stage bladder diseases.

Long-term followup (37 to 69 years) in patients with bladder exstrophy treated with ureterosigmoidostomy: psychosocial and psychosexual outcomes.

PURPOSE: We describe the long-term psychosocial and sexual outcomes of patients born with bladder exstrophy treated with ureterosigmoidostomy at our institution. MATERIALS AND METHODS: Of 42 patients born with bladder exstrophy between 1937 and 1968, 28 were located, of whom 25 agreed to participate in this study. Evaluation consisted of studying pediatric and adult medical records, medical examination, and reports of sexual and social history. RESULTS: Four female and 21 male patients with a mean age of 50 years (range 39 to 67) were evaluated. Of the patients 21 (84%) were married or lived in a stable relationship, 6 (24%) had several children after normal conception and an additional 2 (8%) became parents with assisted reproductive technology. Nine patients (36%) reported no sexual activity or were unable to engage in sexual intercourse. All patients except 2 are professionally and socially successful. CONCLUSIONS: The long-term followup of this study of 37 to 68 years provides valuable information on patients who live with bladder exstrophy during a long period. Most followed patients who were born with bladder exstrophy and treated with ureterosigmoidostomy were able to lead satisfactory lives socially and professionally. However, at least 36% of the patients are severely restricted in their sexual lives.

Alternative management of bladder exstrophy.

PURPOSE OF REVIEW: Recent long-time outcome studies of patients with bladder exstrophy treated with primary urinary diversions or primary reconstruction force pediatric urologists to reassess the place of alternative management options in the armamentarium of bladder exstrophy treatment. RECENT FINDINGS: After classic ureterosigmoidostomy (USS) or Mainz II pouch, continence rates are very high even in the long-term follow-up with a mean of 50 years. In contrast, multiple procedures including bladder neck repair (BNR) were needed in the majority of patients after complete primary repair of bladder exstrophy (CPRE) to achieve normal voiding and to provide cosmetically acceptable and functional genitalia. After multiple staged repair of bladder exstrophy (MSRE), the requirements of multiple procedures including bladder augmentation to achieve urinary continence, not normal voiding, is well known. The augmentation rates published in the literature range form 0 to 82%. There is increasing concern that the risk for tumor formation might be the same for any type of inclusion of intestinal tissue in the urinary tract even without the direct exposure to the fecal stream. SUMMARY: The hope of providing normal voiding with a single operation (CPRE) might not be fulfilled even in the most skilled hands. This means that existing concerns after reconstructive techniques in bladder exstrophy regarding risk of tumor formation and BNR not standing the test of time remain important. Long-term psychosocial and sexual outcomes, including fertility of patients after reconstruction or primary diversion, will need to be taken into consideration.

Tissue Engineering in Pediatric Bladder Reconstruction-The Road to Success.

Several congenital disorders can cause end stage bladder disease and possibly renal damage in children. The current gold standard therapy is enterocystoplasty, a bladder augmentation using an intestinal segment. However, the use of bowel tissue is associated with numerous complications such as metabolic disturbance, stone formation, urine leakage, chronic infections, and malignancy. Urinary diversions using engineered bladder tissue would obviate the need for bowel for bladder reconstruction. Despite impressive progress in the field of bladder tissue engineering over the past decades, the successful transfer of the approach into clinical routine still represents a major challenge. In this review, we discuss major achievements and challenges in bladder tissue regeneration with a focus on different strategies to overcome the obstacles and to meet the need for living functional tissue replacements with a good growth potential and a long life span matching the pediatric population.

The Pediatric Penile Perception Score: an instrument for patient self-assessment and surgeon evaluation after hypospadias repair.

PURPOSE: The aim of this study was to develop and evaluate an instrument that allows assessment and comparison of penile perception of patients, parents and surgeons. MATERIALS AND METHODS: A total of 77 boys 6 to 17 years old who had undergone hypospadias repair were interviewed by a psychologist with a standardized questionnaire concerning penile self-perception with regard to meatus, glans, skin and general appearance. The Pediatric Penile Perception Score was derived from the sum of these 4 items. The results were compared with a control group of age matched boys following inguinal hernia repair. Parents were asked via questionnaire to report the penile appearance of their son using the Pediatric Penile Perception Score. A total of 56 patients accepted standardized photographic documentation, and their pictures were sent for evaluation with the Pediatric Penile Perception Score to 6 blinded urologists. RESULTS: The Pediatric Penile Perception Score allowed us to assess perception of the genitalia by patients, parents and urologists. Statistical analysis of the Pediatric Penile Perception Score assigned by the urologist revealed good interrater reliability (interclass correlation 0.75 to 0.88) and stability (r = 0.59 to 0.83). Intercorrelation of the items "meatus," "glans" and "skin" with "general appearance" was good among the boys, parents and urologists. Patients with hypospadias expressed high satisfaction with the penile appearance, which did not differ significantly from age matched controls. However, parents and urologists were less satisfied with the penile appearance than were the patients themselves. CONCLUSIONS: The Pediatric Penile Perception Score is a reliable instrument to assess penile self-perception in children after hypospadias repair, and for appraisal of the surgical result by parents and uninvolved urologists.