Tubularized Bladder Flap as a Continent Catheterizable Channel in Adults.

OBJECTIVE: To describe a modified, less invasive, surgical technique to create a continent catheterizable channel (CCC) in adults: the tubularized bladder flap (TBF). MATERIALS AND METHODS: We retrospectively reviewed records of patients in whom a TBF CCC was constructed at adult age between 2019 and 2023. We reported on demographics, operative outcomes, and 30-day and post-30-day complications. RESULTS: A total of 11 patients (10 female) were described. The median operative time was 96 (range 90-115) minutes in patients with only TBF creation. Estimated blood loss was <100 cc in all patients. Within 30 days postoperatively, 6/11 (55%) patients developed a complication, all grade 1 Clavien Dindo. No bowel complications occurred (paralytic ileus, mechanical obstruction, or leakage/perforation). Median follow-up was 25 (range 6-56) months. In 2/11 (18%) patients surgical revision for stenosis was done; 3/11 (27%) patients underwent surgical revision for stomal leakage. CONCLUSION: TBF as a means to create a CCC avoids intraperitoneal surgery, and bowel closure (appendicovesicostomy) or anastomosis (retubularized ileum). Postoperative bowel complications were not seen in any of our patients. Surgical revision rates for a TBF CCC appear to be comparable to other CCCs. Therefore, TBF could be considered in patients with sufficient bladder capacity as TBF is less invasive than other CCC techniques and avoids potential bowel complications.

"What should be next in lifelong posterior hypospadias: Conclusions from the 2023 ERN eUROGEN and EJP-RD networking meeting".

BACKGROUND: A congenital disease is for life. Posterior hypospadias, the severe form of hypospadias with a penoscrotal, scrotal, or perineal meatus, is a challenging condition with a major impact on lifelong quality of life. AIM: Our network meeting is aimed to identify what is currently missing in the lifelong treatment of posterior hypospadias, to improve care, quality of life, and awareness for these patients. METHODS: The network meeting "Lifelong Posterior Hypospadias" in Utrecht, The Netherlands was granted by the European Joint Programme on Rare Diseases-Networking Support Scheme. There was a combination of interactive sessions (hackathons) and lectures. This paper can be regarded as the last phase of the hackathon. RESULTS: Surgery for hypospadias remains challenging and complications may occur until adulthood. Posterior hypospadias affects sexual function, fertility, and hormonal status. Transitional care from childhood into adulthood is currently insufficiently established. Patients should be more involved in defining desired treatment approach and outcome measures. For optimal outcome evaluation standardization of data collection and registration at European level is necessary. Tissue engineering may provide a solution to the shortage of healthy tissue in posterior hypospadias. For optimal results, cooperation between basic researchers from different centers, as well as involving clinicians and patients is necessary. CONCLUSIONS: To improve outcomes for patients with posterior hypospadias, patient voices should be included and lifelong care by dedicated healthcare professionals guaranteed. Other requirements are joining forces at European level in uniform registration of outcome data and cooperation in basic research.

Continent catheterizable urinary channels: Lessons for lifelong urological care from a comparative analysis of very long-term complications and revision-free survival of three different types.

INTRODUCTION: Continent catheterizable channels (CCC) provide an alternative route to the bladder in both pediatric and adult patients. This study compared very long-term complications and revision-free survival of three different CCC techniques: appendicovesicostomy (AVS), ileum (Monti), and tubularized bladder flap (TBF). MATERIALS AND METHODS: A retrospective cohort study was performed. Data from adult patients with CCC under surveillance at our academic tertiary referral urological center in 2020 and 2021 were collected. Both patients who acquired the CCC as a child and as an adult were included. The primary outcome was revision-free survival of the three CCC types. The secondary outcome was the prevalence of complications requiring surgical revision. Revisions were categorized as major (open subfascial or complete revisions) and minor (open suprafascial or endoscopic). RESULTS: We included 173 CCCs (AVS 90, Monti 51, TBF 32). Median follow-up was 12.4 years (4.8-18). Mean revision-free survival was 162 ± 13 months, with no significant difference between the three types. Ninety-two individual CCCs (53%) required surgical revision and a total of 157 surgical revisions were performed. Seventy CCCs (40%) required major surgical revision: AVS (27/90%-30%), Monti (31/51%-61%), TBF (12/32%-38%). CONCLUSION: Complications of CCCs are common; in this study with very long-term follow-up, more than half of CCCs required surgical revision. Revisions were more common in Monti channels compared with AVS and TBF. The mean revision-free survival of >13 years illustrates the sustained long-term durability of CCCs which is important in the lifelong urological care of this population with high life expectancy.

Very long-term follow-up of Indiana Pouches proves durability.

INTRODUCTION: An Indiana Pouch (IP) is a heterotopic, continent, urinary diversion from an ileocolonic segment. Numerous studies have investigated its long-term outcomes, albeit none extending beyond a 5-year follow-up period. IPs can be used as urinary diversion for benign indications and as such are constructed in typically young patients. As a consequence of their extended lifespan, there is a need for very long-term (>5 years) IP outcome data and comprehensive complication analysis. MATERIALS AND METHODS: In this retrospective cohort study, the data of all patients attending our academic functional urology tertiary referral center for surveillance between 2015 and 2022 after an earlier IP procedure without uro-oncological indication were analyzed. The primary objective was to identify the prevalence of complications associated with IP, including stomal stenosis, ureter-pouch stenosis, pouch calculi, stomal leakage, pouch perforation, and parastomal herniation, and to determine the time span between creation of the IP and occurrence of complications. RESULTS: A cohort of 33 patients (23 female) was analyzed. Median age at IP creation was 38 (range 5-62) years. Median follow-up was 258 (range 24-452) months. During follow-up, 22 (67%) patients underwent at least one surgical revision. In total, 45 revision procedures were performed. The estimated mean revision-free survival was 198 (95%-CI 144-242) months. CONCLUSION: Two-thirds of our IP patients required surgical revision during very long-term follow-up. However, the mean revision-free survival was 198 months. This establishes the IP as a durable and resilient option for urinary diversion, yet underlines the need for lifelong follow-up as some of these complications and indication were subclinical. These results contribute significantly to patient counseling when discussing different options for urinary diversion, especially at a younger age.

Extracellular matrix analysis of fibrosis: A step towards tissue engineering for urethral stricture disease.

The urogenital tract is a target for many congenital and acquired diseases, both benign and oncogenic. In males, the urethra that transports urine and semen can be obstructed by a fibrotic disease called urethral stricture disease (USD). In severe USD, the whole organ including the vascular embedding, the corpus spongiosum (CS), is affected. Recurrent or severe USD is treated by reconstructive surgery. Tissue engineering may improve the outcome of urethral reconstruction in patients with complicated USD. Currently in urethral reconstruction only the epithelial layer is replaced, no substitution for the CS is provided, while the CS is important for mechanical support and vascularization. To develop a tissue engineering strategy for the CS, it is necessary to know the protein composition of the CS. As the extracellular matrix (ECM) plays an important role in the formation of fibrosis, we analyzed the distribution and localization of ECM components in human healthy and fibrotic CS tissue using immunohistology. The morphology of components of the elastic network were affected in USD. After decellularization a clear enrichment of proteins belonging to the ECM was found. In the proteomic analysis collagens COL15A1 and COL4A2 as well as inter-alpha-trypsin inhibitor ITIH4 were upregulated in fibrotic samples. The glycoproteins Periostin (POSTN), Microfibrillar-associated protein 5 (MFAP5) and EMILIN2 are downregulated in fibrotic tissue. To our knowledge this is the first proteomic study of ECM proteins of the CS in healthy and in USD. With these results a regenerating approach for tissue engineered CS can be developed, including relevant ECM proteins that reduce fibrosis and promote healthy healing in urethral reconstructive surgery.

Quantifying bladder outflow obstruction in men: A comparison of four approximation methods exploiting large data samples.

INTRODUCTION: A pressure flow study (PFS), part of the International Continence Society standard urodynamic test, is regarded gold standard for the classification and quantification of the urethral resistance (UR), expressed in the bladder outflow obstruction (BOO). For men with benign prostatic hyperplasia, the minimum urethral opening pressure (pmuo ), found at the end of the passive urethral resistance relation is considered the relevant parameter describing BOO. However, in clinical practice, direct measurements of pmuo are easily confounded by terminal dribbling. For that reason, alternative methods were developed to derive pmuo , and thereby assess BOO using the maximum urine flow rate (Qmax ) and the corresponding pressure (pdetQmax ) instead. These methods were never directly compared against a large data set. With the increasing variety of treatments becoming available more precise grading of UR may become of relevance. The current study compares four well-known methods to approximate pmuo and examines the relation between pmuo and pdetQmax . METHODS: In total, 1717 high-quality PFS of men referred with lower urinary tract symptoms between 2003 and 2020 without earlier lower urinary tract surgery were included. From these recordings, pmuo was calculated according to three one-parameter methods. In addition, a three-parameter method (3PM) was used, based on a fit through the lowest pressure flank of the pressure-flow plot. The estimated pmuo 's were compared with a precisely assessed pmuo . A difference of <10 cmH2 O between an estimate and the actual pmuo was considered accurate. A comparison between the four approximation methods and the actual pmuo was visualized using a Bland-Altman plot. The differences between the actual and the estimated slope were assessed and dependency on pmuo was analyzed. RESULTS: A total of 1717 studies were analyzed. In 55 (3.2%) PFS, 3PM analysis was impossible because all pressures after Qmax were higher than pdetQmax . The 3PM model was superior in predicting pmuo , with 75.9% of the approximations within a range of +10 or -10 cmH2 O of the actual pmuo . Moreover, pmuo according to urethral resistance A (URA) and linearized passive urethral resistance relation (linPURR) appear equally reliable. Bladder outflow obstruction index (BOOI) was significantly less accurate when compared to all others. Bland-Altman analysis showed a tendency of BOOI to overestimate pmuo in men with higher grades of UR, while URA tended to underestimate pmuo in those cases. The slope between pmuo and pdetQmax -Qmax increased with larger pmuo , as opposed to the constant relation proposed within BOOI. Although significant differences were found, the clinical relevance of those differences is not known. CONCLUSION: Of the four methods to estimate pmuo and quantify BOO, 3PM was found the most accurate and BOOI the least accurate. As 3PM is not generally available and performance in lower quality PFS is unknown, linPURR is (for now) the most physiologically accurate.

Dysfunctional Voiding: Exploring Disease Transition From Childhood to Adulthood.

OBJECTIVE: To improve our transitional care, we explored how childhood dysfunctional voiding (DV) develops into adulthood. DV is a common condition in both children and adults. However, the long-term course of childhood DV into adulthood is unknown and treatment over the ages differs. METHODS: A cross-sectional follow-up was performed in a cohort of 123 females treated from 2000 to 2003 for childhood DV with urinary tract infections (UTIs) and/or daytime urinary incontinence (DUI). The main outcome was a staccato or intermittent urinary flow pattern, possibly indicating persistent or recurred DV according to the International Continence Society criteria. Flow patterns of healthy women were used to compare results. RESULTS: Twenty-five patients participated in this study, with a mean duration of 20.8 years after urotherapy. In 10/25 (40%) cases, a staccato or interrupted urinary flow pattern was found on the current measurement, compared to 5/47 (10.6%) in the control group. Around 50% (5/10) of the patients with a dysfunctional flow pattern reported UTIs and 50% (5/10) experienced DUI. In the group with a normal flow pattern, 2/15 (13%) reported UTIs and 9/15 (60%) DUI. The impact of DUI on quality of life was moderate to high in both groups. CONCLUSION: Our results show that 40% of females who had extensive urotherapy for DV in childhood, still have DV according to International Continence Society criteria as an adult, 56% still experience DUI, and 28% UTIs. These data should be taken into account in the counseling of patients and for guiding the process of transition into adulthood.

Clomiphene citrate for male infertility: A systematic review and meta-analysis.

BACKGROUND: Male infertility is a prevalent and worldwide problem with various difficulties in treatment. Clomiphene citrate is a selective estrogen receptor modulator and may improve semen quality by stimulating hormone synthesis and spermatogenesis. There is lack of evidence on the efficacy of clomiphene citrate as therapy for male infertility. OBJECTIVES: Therefore, a systematic review and meta-analysis was performed to assess the efficacy of clomiphene citrate on sperm quality in infertile men. METHODS: A search was conducted in the PubMed, EMBASE and Cochrane databases for effectiveness in infertile males treated with clomiphene citrate. Both intervention and observational studies were included. Primary outcome measures were semen parameters (concentration, motility and morphology). Secondary outcomes included hormonal evaluation, pregnancy rate and side effects. Studies were included for meta-analysis if they provided absolute numbers for outcomes before and during treatment with appropriate SD or SE. RESULTS: Total 1799 studies were identified during the search, 18 studies remained for qualitative analysis (n = 731) and 15 studies for meta-analysis (n = 566). Study populations ranged between 11 and 140 participants. Sperm concentration was higher during treatment, with a mean difference 8.38 × 106 /ml (95% confidence interval: 5.17-11.59; p < 0.00001; I2  = 87%). Total sperm motility was higher during treatment, with a mean difference of 8.14% (95% confidence interval: 3.83-12.45; p < 0.00001; I2  = 76%). There was no difference in sperm morphology before and during treatment. Total testosterone, follicle-stimulating hormone, luteinizing hormone and estradiol were higher during clomiphene citrate treatment. During follow-up, no serious adverse effects occurred. In 10 studies, pregnancy rate was reported and yielded a mean of 17% during clomiphene citrate treatment (range: 0%-40%). CONCLUSIONS: Clomiphene citrate increased sperm concentration and motility and could be considered as a safe therapy for improving sperm parameters in infertile males.

Familial Male-limited Precocious Puberty (FMPP) and Testicular Germ Cell Tumors.

OBJECTIVE: The purpose of this study is to report development of a malignant testicular germ cell tumor (GCT) in 2 young adult males with familial male-limited precocious puberty (FMPP) because of LHCGR pathogenic variants in 2 families. Secondarily, to study the possible relation between FMPP and testicular tumors and to investigate whether FMPP might predispose to development of malignant testicular tumors in adulthood a literature review is conducted. METHODS: Data on 6 cases in 2 families are obtained from the available medical records. In addition, a database search is performed in Cochrane, PubMed, and Embase for studies that report on a possible link between FMPP and testicular tumors. RESULTS: The characteristics of 6 males with FMPP based on activating LH receptor (LHCGR) germline pathogenic variants are described, as are details of the testicular GCTs. Furthermore, a literature review identified 4 more patients with signs of FMPP and a (precursor of) testicular GCT in adolescence or adulthood (age 15-35 years). Additionally, 12 patients with signs of precocious puberty and, simultaneously, occurrence of a Leydig cell adenoma or Leydig cell hyperplasia are reported. CONCLUSION: There is a strong suggestion that FMPP might increase the risk of development of testicular GCTs in early adulthood compared with the risk in the general population. Therefore, prolonged patient monitoring from mid-pubertal age onward including instruction for self-examination and periodic testicular ultrasound investigation in patients with a germline LHCGR pathogenic variant might contribute to early detection and thus early treatment of testicular GCT.

Arterio-ureteral fistula: a nationwide cross-sectional questionnaire analysis.

PURPOSE: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but potentially lethal. Although the number of reports has increased over the past two decades, the true incidence and contemporary urologists' experience and approach in clinical practice remains unknown. This research is conducted to provide insight in the incidence of AUF in The Netherlands, and the applied diagnostic tests and therapeutic approaches in modern practice. METHODS: A nationwide cross-sectional questionnaire analysis was performed by sending a survey to all registered Dutch urologists. Data collection included information on experience with patients with AUF; and their medical history, diagnostics, treatment, and follow-up, and were captured in a standardized template by two independent reviewers. Descriptive statistics were used. RESULTS: Response rate was 62% and 56 AUFs in 53 patients were reported between 2003 and 2018. The estimated incidence of AUF in The Netherlands in this time period is 3.5 AUFs per year. Hematuria was observed in all patients; 9% intermittent microhematuria, and 91% presenting with, or building up to massive hematuria. For the final diagnosis, angiography was the most efficient modality, confirming diagnosis in 58%. Treatment comprised predominantly endovascular intervention. CONCLUSION: The diagnosis AUF should be considered in patients with persistent intermittent or massive hematuria.

Arterio-Ureteral Fistula: Systematic Review of 445 Patients.

PURPOSE: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but increasingly reported and potentially lethal. This systematic review comprehensively presents risk factors, pathophysiology, location and clinical presentation of AUF aiming to increase clinical awareness of this rare but life-threatening condition, and to put this entity into a contemporary perspective with modern diagnostic tools and treatment strategies. MATERIALS AND METHODS: This review was performed according to the PRISMA (Preferred Reporting Items for a Systematic Review and Meta-Analysis of Individual Participant Data) guidelines. A literature search in PubMed® and EMBASE™ was conducted. In addition, retrieved articles were cross-referenced. Data parameters included oncologic, vascular and urological history, diagnostics, treatment, and followup, and were collected using a standard template by 2 independent reviewers. RESULTS: A total of 245 articles with 445 patients and 470 AUFs were included. Most patients had chronic indwelling ureteral stents (80%) and history of pelvic oncology (70%). Hematuria was observed in 99% of the patients, of whom 76% presented with massive hematuria with or without previous episodes of (micro)hematuria. For diagnosis, angiography had a sensitivity of 62%. The most predominant location of AUF was at the common iliac artery ureteral crossing. AUF-specific mortality before 2000 vs after 2000 is 19% vs 7%, coinciding with increasing use of endovascular stents. CONCLUSIONS: AUF should be considered in patients with a medical history of vascular surgery, pelvic oncologic surgery, irradiation and/or chronic indwelling ureteral stents presenting with intermittent (micro)hematuria. A multidisciplinary consultation is necessary for diagnosis and treatment. The most sensitive test is angiography and the preferred initial treatment is endovascular.

Clomiphene citrate for men with hypogonadism: a systematic review and meta-analysis.

BACKGROUND: Male hypogonadism is a clinical and biochemical androgen insufficiency syndrome, becoming more prevalent with age. Exogenous testosterone is first-choice therapy, with several side effects, including negative feedback of the hypothalamic-pituitary-gonadal axis, resulting in suppression of intratesticular testosterone production and spermatogenesis. To preserve these testicular functions while treating male hypogonadism, clomiphene citrate is used as off-label therapy. This systematic review and meta-analysis aimed to evaluate the effectiveness and safety of clomiphene citrate therapy for men with hypogonadism. METHODS: The EMBASE, PubMed, Cochrane databases were searched in May 2021, for effectiveness studies of men with hypogonadism treated with clomiphene citrate. Both intervention and observational studies were included. The Effective Public Health Practice Project Quality Assessment Tool, a validated instrument, was used to assess methodological study quality. The primary outcome measure was the evaluation of serum hormone concentration. Secondary outcomes were symptoms of hypogonadism, metabolic and lipid profile, side effects, safety aspects. RESULTS: We included 19 studies, comprising four randomized controlled trials and 15 observational studies, resulting in 1642 patients. Seventeen studies were included in the meta-analysis, with a total of 1279 patients. Therapy and follow-up duration varied between one and a half and 52 months. Total testosterone increased with 2.60 (95% CI 1.82-3.38) during clomiphene citrate treatment. An increase was also seen in free testosterone, luteinizing hormone, follicle stimulating hormone, sex hormone-binding globulin and estradiol. Different symptom scoring methods were used in the included studies. The most frequently used instrument was the Androgen Deficiency in Aging Males questionnaire, whose improved during treatment. Reported side effects were only prevalent in less than 10% of the study populations and no serious adverse events were reported. CONCLUSION: Clomiphene citrate is an effective therapy for improving both biochemical as well as clinical symptoms of males suffering from hypogonadism. Clomiphene citrate has few reported side effects and good safety aspects.

Do previous urethral endoscopic procedures and preoperative self-dilatation increase the risk of stricture recurrence after urethroplasty?

OBJECTIVE: To evaluate the relation between clinically relevant stricture recurrence after first urethroplasty and prior endoscopic treatments (dilatation and/or direct visual internal urethrotomy) or intermittent self-dilatation (ISD). METHODS: Patients with bulbar urethral strictures treated with first urethroplasty between 2011 and April 2019 were included in a prospectively gathered database with standardized follow-up. Stricture recurrence was defined as any need for reintervention. Primary outcome was the analysis of recurrence risk after first urethroplasty in relation with the number of prior endoscopic treatments or performance of ISD. Univariate and multivariate statistical analyses were performed. RESULTS: Overall, 106 patients were included with a median follow-up of 12 months (interquartile range 8-13]. Reintervention was necessary in 16 patients (15%). Recurrence was more prevalent in patients with ≥3 prior endoscopic treatments (28%, P = .009). No increased risk of recurrence was found in patients with 1 or 2 prior endoscopic treatments. The prevalence of prior ISD was twice as high in the stricture recurrence group (56% vs 26%, P = .014), and ISD was performed in 61% of the patients with ≥3 prior endoscopic treatments (P < .001). The number of prior endoscopic interventions and performance of ISD were no independent predictors for recurrence in the multivariable analysis. CONCLUSIONS: This study shows that the risk of recurrence after first urethroplasty is increased in patients with ≥3 prior endoscopic treatments and in those who performed ISD. Patients performing ISD more often had ≥3 prior endoscopic treatments. Prior endoscopic treatment and performance of ISD were not independent predictors of stricture recurrence.

The use of local therapy in preventing urethral strictures: A systematic review.

BACKGROUND: Urethral stricture disease is a common problem amongst men in Western countries often leading to a decreased quality of life. Current endoscopic treatment procedure shows an unsatisfying stricture recurrence rate which could be improved by addition of local therapies. OBJECTIVES: To provide an overview of both preclinical and clinical studies in order to investigate current level of evidence on the addition of local therapy to improve urethral stricture recurrence rates after endoscopic procedures. METHODS: We performed a literature search in December 2020 and August 2021 using Cochrane, Embase, PubMed, Scopus and Web of Science and identified articles through combinations of search terms for 'urethral stricture disease', 'stricture formation' and 'local interventions'. We used the SYRCLE, RoB-2 and ROBINS-I tools to assess risk of bias across included studies. We did not perform a meta-analysis due to methodological differences between studies. RESULTS: We included 32 articles in the qualitative analysis, 20 of which were preclinical studies and 12 clinical studies. Regarding preclinical articles using an animal model, nearly all interventions showed to have a positive effect on either urethral fibrosis, urethral stricture formation and/or fibrotic protein expression levels. Here, immunosuppressants and chemotherapeutics seemed most promising for possible clinical purposes. Regarding clinical studies, mitomycin-C and hyaluronic acid and carboxymethylcellulose showed positive effects on urethral stricture recurrence rates with low to intermediate risk of bias across studies. However, the positive clinical effects of mitomycin-C and steroids seemed to decrease in studies with a longer follow-up time. CONCLUSION: Although local adjuvant use of mitomycin-C or hyaluronic acid and carboxymethylcellulose may carry clinical potential to improve urethral structure recurrence rates after endoscopic procedures, we believe that a large, well-designed RCT with a yearlong follow-up time is necessary to identify the true clinical value.

The effect of meatal correction on daytime urinary incontinence in girls with an anterior deflected urinary stream.

INTRODUCTION: Girls with an anterior deflected urinary stream (ADUS) are known to wet the toilet rim and their buttocks while voiding. This deviation may prevent adopting an ideal toileting position and can thus develop into a functional voiding disorder. Although surgical correction of the urinary stream by a meatotomy is part of standard care in girls with ADUS and lower urinary tract symptoms (LUTS) at our center, little is known about the effect of this procedure on daytime urinary incontinence (DUI). OBJECTIVE: To assess the effect of meatal correction on incontinence in girls with ADUS and DUI, and to find predictors for therapy success. STUDY DESIGN: A retrospective chart study including all girls with ADUS and DUI who underwent a dorsally directed meatal correction at our tertiary referral center between 2005 and 2018 (n = 274). The main outcome measurement was continence according to the International Children's Continence Society criteria, expressed as the percentage of children that were continent at post-surgical follow-up (complete response). Multivariate logistic regression was used to identify predictors of continence. RESULTS: In 93% of girls, the direction of the urinary stream was no longer anterior deflected. 29% (n=79) of all girls were continent at post-surgical follow-up. We could not find a convincing predicting factor for achieving continence. DISCUSSION: Although meatal correction is successful on normalizing the direction of the urinary stream, less than one-third of girls became continent. In addition, we were unable to indicate which girls will profit from this procedure in terms of continence. Since not every girl with ADUS develops incontinence complaints, it may also be questionable whether ADUS and incontinence are directly related. Since other, less invasive and more effective treatment options for DUI are available, meatal correction should therefore be considered a last-resort option in the treatment of pediatric DUI in girls with ADUS. CONCLUSION: Of the 274 girls with ADUS and DUI, 29% did benefit from a meatotomy. No convincing indicator for therapy success could however be found. Therefore, we discourage a meatotomy as standard treatment in girls with ADUS and DUI.

A Retrospective Analysis of Female Müllerian Duct Anomalies in Association With Congenital Renal Abnormalities.

STUDY OBJECTIVE: Müllerian (paramesonephric) duct anomalies (MDAs) are associated with several coexisting congenital abnormalities, including renal abnormalities. Although congenital renal abnormalities may remain asymptomatic, the consequences should not be underestimated. In both the literature and clinical practice, it remains necessary to improve awareness of the co-occurrence of different congenital renal abnormalities in women with MDAs. To gain further insight into this co-occurrence and to estimate whether guidelines for women with MDAs should be optimized, this study was performed. DESIGN: A descriptive retrospective analysis. SETTING: University Medical Centre Utrecht in the Netherlands. PARTICIPANTS: Women with MDAs diagnosed or treated between 1980 and 2015. INTERVENTIONS: None. MAIN OUTCOME MEASURES: The prevalence of the co-occurrence of congenital renal abnormalities in women with MDAs. RESULTS: Renal status was recorded in 186 of 255 women (72.9%), and the other women (27.1%) did not have a retrievable renal status. Congenital renal abnormalities were present in 90 of 186 women (48.4%) and were observed most frequently in women having a duplex uterus with obstructed hemivagina. The most common renal abnormality was unilateral renal agenesis, which was observed in 58 of 90 women (64.4%). CONCLUSIONS: MDAs are highly associated with different congenital renal abnormalities, and these results emphasize that women with MDAs should be routinely screened for their co-occurrence. However, these results also highlight that there remains a lack of awareness of this association. Whether all women with congenital renal abnormalities should be routinely screened for MDAs requires further investigation.

Transition from childhood to adolescence: steps to be taken.

PURPOSE OF REVIEW: The aim of this article is to give an overview of recent literature on transitional care of children with complex urological disease. RECENT FINDINGS: Most studies on transitional care concerned spina bifida patients. Assessment of current practices revealed that formalized clinics for transitional care may improve healthcare. However, there is still little consensus among healthcare providers on how to organize transitional care. A widely recognized problem is a lack of interested, dedicated, and well-trained staff for transitional care and lifelong follow-up of complex urological patients and also a lack of funding. The timing of the pediatric urologist's transfer to adult care may be helped by measuring transitional readiness in adolescent patients. Outcomes could also expose specific issues as were patients need assistance or education. Introduction of a transitional protocol made patients feel more ready for transition. However, even in established transitional clinics, there is a lack of disease-specific awareness of sexuality and fertility issues among patients. SUMMARY: Although there is growing awareness of the importance of well-organized and protocolled transitional care for young patients with complex urological disease, this is by no means sufficiently implemented. Furthermore, there is shortage of dedicated and experienced healthcare providers and a lack of funding.

Gel Casting as an Approach for Tissue Engineering of Multilayered Tubular Structures.

Several urological structures, such as the male urethra, have a tubular organization consisting of different layers. However, in severe urethral disease, urologists are limited to replacing solely the epithelial layer. In case of severe hypospadias and urethral stricture disease, the underlying supporting structure (the corpus spongiosum) is either absent or fibrotic, causing suboptimal vascularization and therefore increasing the risk of graft failure. Recapitulating the multilayered architecture of the urethra, including supporting structure with tissue engineering, might minimize urethral graft failure. However, current tissue engineering applications for complex multilayered tubular constructs are limited. We describe a gel casting method to tissue engineer multilayered tubular constructs based on fiber-reinforced cell-laden hydrogels. For this, a multichambered polydimethylsiloxane mold was casted with fiber-reinforced hydrogels containing smooth muscle cells (SMCs) and a coculture of endothelial cells and pericytes. The cell-loaded hydrogels were rolled, with the fiber mesh as guidance, into a tubular construct. In the lumen, urothelial cells were seeded and survived for 2 weeks. In the tubular construct, the cells showed good viability and functionality: endothelial cells formed capillary-like structures supported by pericytes and SMCs expressed elastin. With a graft produced by this technique, supported with subepithelial vascularization, urethral reconstructive surgery can be improved. This approach toward tissue engineering of multilayered tubular structures can also be applied to other multilayered tubular structures found in the human body. Impact Statement Recapitulating the multilayered architecture of tubular structures found in the human body might minimize graft failure. Current tissue engineering applications for complex multilayered tubular constructs are limited. Here we describe a gel casting approach based on fiber-reinforced cell-laden hydrogels. A multichambered polydimethylsiloxane mold was casted with cell-loaded, fiber-reinforced hydrogels, with the fiber mesh as guidance, into a tubular construct. A graft produced by this technique can improve reconstructive surgery by providing subepithelial vascularization and thereby can reduce graft failure.

Urological, Sexual, and Quality of Life Evaluation of Adult Patients With Exstrophy-Epispadias Complex: Long-term Results From a Dutch Cohort.

OBJECTIVE: To assess urological function, sexual function, and quality of life in patients with exstrophy or epispadias. Little is known regarding these outcomes in adult patients; our aim is to determine where improvements are needed for long-term management. METHODS: The study population comprised adult (>18 years) patients. Demographic data were gathered and patients were asked to fill out 4 validated questionnaires: (1) International Consultation on Incontinence Questionnaire urinary incontinence form (ICIQ-UI) regarding continence; (2) International Prostate Symptom Score (IPSS) for men and International Consultation on Incontinence Questionnaire-Female Lower Urinary Tract Symptoms (ICIQ-FLUTS) for women regarding lower urinary tract symptoms; (3) 12-Item Short Form Health Survey regarding quality of life; (4) International Index of Erectile Function for men and Female Sexual Function Index for women regarding sexual function. RESULTS: Seventeen patients were included (9 men and 8 women) with a median age of 36 years (range 19-73). Median score on ICIQ-UI was 5/21. Median IPSS score was 7/35 and median quality of life score was 1 (=pleased). Median scores per domain within ICIQ-FLUTS were 7 for storage, 0 for voiding, and 6 for urinary incontinence with bother scores of 4, 0, and 2.8, respectively. Scores for 12-Item Short Form Health Survey in the study population were comparable with those of the Dutch population, except for Physical Component Summary in women. For sexual function, no difference was found between those in the general population and our participants except for the domain "pain" in Female Sexual Function Index. CONCLUSION: Adult patients with exstrophy or epispadias have a high rate of incontinence and lower urinary tract symptoms with relatively low to some degree of bother. When compared with the general population, quality of life, and sexual function of our patients were more or less similar.

Coping styles in patients with hypospadias.

OBJECTIVE: The objective of this study is to investigate the coping styles used by patients with hypospadias. Long-term hypospadias studies generally show satisfying outcomes, but some report a lower quality of life (QoL) or poorer psychosocial adjustment, particularly in patients with proximal hypospadias. A lower QoL or psychosocial adjustment was found to be associated with passive coping styles in other pediatric patient populations. Hypothetically, patients with hypospadias also develop different coping styles due to medical experiences in childhood, leading to the lower QoL outcomes on the long term. However, coping styles of patients with hypospadias have never been analyzed. PATIENTS AND METHODS: Adult men with hypospadias repair in childhood were recruited (n = 55; aged 19.9 [IQR 19.2-22.1]). Coping styles were determined with the Utrecht Coping List (UCL) and results compared with a reference group of male students (n = 55, age 20-30 years, no medical history). Sub analysis of coping styles of the hypospadias groups was done based on three items: severity of hypospadias, time of last hypospadias surgery and occurrence of postoperative complications. RESULTS: Compared to the reference groups, patients with hypospadias had higher scores on Avoidance (P < 0.05), particularly patients who had ≥1 postoperative complication or proximal hypospadias. Patients with proximal hypospadias also had lower scores on Seeking Social Support compared to the reference group (P < 0.05). Within the hypospadias group, coping style scores did not differ based on severity of hypospadias, timing of surgery or postoperative complications. CONCLUSION: Patients with hypospadias, particularly those with proximal hypospadias or a postoperative complication, more often use an avoiding coping style compared to a reference population. Attention to coping styles during the follow-up of patients with hypospadias might help to improve the QoL in these patients.