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.

The Engagement of the Pelvic Floor Muscles to the Urethra, Does Variation in Point of Action Exist?

Purpose: Lower urinary tract dysfunction (LUTD) occurs frequently in girls and may display a spinning top urethra (STU) on voiding cysto-urethrogram (VCUG) in case of dysfunctional voiding. A STU presents as a narrowing of the urethra caused by a lack of relaxation of the pelvic floor musculature during micturition and may vary in length between the proximal and the distal urethra. Although a STU has been recognized since 1960 as a pathological entity on VCUG, no reports exist on the different levels of engagement of the pelvic floor muscles to the urethra as expressed by the varying length of the phenomenon. The aim of our study is to demonstrate the wide anatomical variation in the level of engagement of the pelvic floor musculature to the urethra. Materials and Methods: Dynamic ultrasound videos of pelvic floor musculature of 40 girls with LUTD were reassessed by three observers, looking for the level of engagement of the puborectalis muscle (PRM) to the urethra during coughing, Valsalva and hold-up maneuver. Three levels were defined, for the level of engagement of the pelvic floor to the urethra, proximal, mid, and distal. Intra- and inter-rater variability was analyzed using Cohen's kappa statistics. Results: A wide range of points of action was found on the assessed ultrasound videos. Intra- and inter-rater agreement showed different levels of conformity, varying over a wide spectrum (intra-rater kappa 0.145-0.546; inter-rater kappa -0.1030.724). Throughout the assessed videos, all not-corresponding intra-rater observations differed maximal one category. Of the not-corresponding inter-rater observations, 90% differed maximal one category. Conclusion: An anatomical variation in levels of engagement of the PRM to the urethra does exist. The clinical value of this finding, whether the point of engagement influences symptomatology or treatment success of LUTD, is currently being studied.

Short-term Complications After Pyeloplasty in Children With Lower Urinary Tract Anomalies.

OBJECTIVE: To investigate whether children with lower urinary tract (LUT) anomalies are at greater risk for postoperative complications after laparoscopic pyeloplasty stented with a double-J catheter (JJC). MATERIALS AND METHODS: Prospectively collected data of laparoscopic pyeloplasty (LP) performed between 2006 and 2015 were analyzed. Inclusion criteria are (1) toilet-trained child and (2) unilateral dismembered pyeloplasty stented with a JJC done by the same surgeon. Our pyeloplasty protocol includes cystoscopy and retrograde pyelography. JJC is left in for 3weeks. Asymptomatic patients with infravesical LUT anomalies (a-LUTA) and those with history of LUT symptoms (LUTS) were identified. Any short-term complication was classified according to Clavien-Dindo. Fisher's exact test was used for statistical analysis. RESULTS: Fifty-four children (mean 9.8 years) were included. Ten of 54 patients had LUTS. In 4 of those 10, anatomical infravesical anomaly was found during cystoscopy. Accidental urethral anomaly was found in 11 patients (a-LUTA). The control group (CG) consisted of 33 patients. Postoperative hospital stay ranged from 1 to 8 days (mean 2 days). Overall complication rate was 8 of 54 (14%). Grade 1 complications occurred in 3 patients in the CG. Five patients had grade 3 complications (2 needed replacement of bladder catheter, and 3 had diversion of the upper tract). Those problems occurred in 1 of 10 patients with LUTS and 3 of 11 patients with a-LUTA compared to 1 of 33 in the CG. This difference was statistically significant (P < .05). CONCLUSION: Careful history should be taken in toilet-trained children before pyeloplasty. If any infravesical abnormality is discovered, internal diversion should probably be avoided. Special attention must be paid to bladder function in the postoperative period.