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

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

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

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

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

"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

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

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