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Abstract Purpose To report our early experience of a novel surgical approach for penile urethral strictures after hypospadias repair, using a lateral incision to keep the ventral tissue and vasculature of the penis intact and to avoid the need for tissue interposition. Patients and methods A total of 21 patients underwent lateral incision 1-stage urethroplasty with oral mucosal graft. The median age of the patients was 21 years old (range, 13–47). The median number of prior procedures for hypospadias repair was 3 (range, 1–9) with 18 of 21 patients (85.7%) undergoing greater than 1 prior reconstructive procedure. The mean length of the penile urethral strictures was 4.5 ± 1.7 cm, with a range of 1.0 to 8.0 cm. Selection criteria for lateral incision 1-stage urethroplasty include: non-obliterative stricture, no or mild penile curvature and no urethrocutaneous fistula. Results Median follow-up was 30 months (range, 6–73). Success was achieved in 17 of 21 patients (80.9%). The 4 (19.0%) patients with treatment failure developed recurrent urethral strictures. Of the 4 men with recurrent strictures, 3 were ultimately treated successfully by DVIU (2) or two-stage urethroplasty (1), and one patient chose repeated dilation. Conclusions For patients with penile urethral stricture after hypospadias repair with non-obliterative stricture, no significant penile curvature and no urethrocutaneous fistula, a lateral approach with oral mucosal graft is a simple technique that avoids the need for tissue interposition and keeps the penile ventral tissue and vasculature intact, resulting in a low risk of complications.

Purpose: To report our early experience of a novel surgical approach for penile urethral strictures after hypospadias repair, using a lateral incision to keep the ventral tissue and vasculature of the penis intact and to avoid the need for tissue interposition. Patients and methods: A total of 21 patients underwent lateral incision 1-stage urethroplasty with oral mucosal graft. The median age of the patients was 21 years old (range, 13–47). The median number of prior procedures for hypospadias repair was 3 (range, 1–9) with 18 of 21 patients (85.7%) undergoing greater than 1 prior reconstructive procedure. The mean length of the penile urethral strictures was 4.5 ± 1.7 cm, with a range of 1.0 to 8.0 cm. Selection criteria for lateral incision 1-stage urethroplasty include: non-obliterative stricture, no or mild penile curvature and no urethrocutaneous fistula. Results: Median follow-up was 30 months (range, 6–73). Success was achieved in 17 of 21 patients (80.9%). The 4 (19.0%) patients with treatment failure developed recurrent urethral strictures. Of the 4 men with recurrent strictures, 3 were ultimately treated successfully by DVIU (2) or two-stage urethroplasty (1), and one patient chose repeated dilation. Conclusions: For patients with penile urethral stricture after hypospadias repair with non-obliterative stricture, no significant penile curvature and no urethrocutaneous fistula, a lateral approach with oral mucosal graft is a simple technique that avoids the need for tissue interposition and keeps the penile ventral tissue and vasculature intact, resulting in a low risk of complications. [ABSTRACT FROM AUTHOR]

Introduction:  The aim of this study was to evaluate whether prolonged stenting reduces the risk of urethral stricture after proximal hypospadias (PH) with severe curvature (SC) repair., Materials and Methods:  We prospectively studied a cohort of patients with PH with SC repair who underwent urethral plate transection and urethroplasty between January 2010 and December 2020. According to the duration of stenting, the patients were divided into 2-, 4-, and 6-week groups. Postoperative complications and time of urethral stricture occurrence were analyzed., Results:  In total, 665 patients were included in the analysis. The overall incidence of complications was 26.6% ( n  = 177), including 42 cases of urethral strictures: 27 (64.3%) cases of urethral stricture occurred between 4 and 6 weeks after urethroplasty, 7 cases occurred between 7 weeks and 6 months after urethroplasty, 7 cases occurred more than 6 months after urethroplasty, and 1 case occurred at 3 weeks after urethroplasty. The incidence of urethral stricture in the 6-week group (1.8%) was significantly lower than that in the 4- (5.8%) and 2-week groups (10.9%) ( p  < 0.05)., Conclusion:  Prolonged stenting reduces the risk of urethral stricture in PH with SC repair. Four to six weeks after PH with SC repair may be the key period for the formation of early urethral strictures., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

The aim of the study was to compare the efficacy of Mathieu combined urethral plate incision (Mathieu-IP) versus onlay island flap urethroplasty in patients with distal hypospadias complicated by urethral stenosis. The clinical data of 70 patients with distal hypospadias complicated by urethral plate stenosis treated in the Department of Urology, Anhui Provincial Children's Hospital (Hefei, China), from May 2019 to May 2022, were retrospectively analyzed. Thirty-eight patients underwent Mathieu-IP (Mathieu-IP group) and 32 underwent onlay island flap urethroplasty (Onlay group). Follow-ups at 1 month, 6 months, and 12 months postoperatively assessed operative time, complications, urethral meatus morphology, and family satisfaction. The Mathieu-IP group had significantly shorter operative time (mean ± standard deviation [s.d.]: 81.58 ± 5.18 min) versus the Onlay group (mean ± s.d.: 110.75 ± 6.05 min; P < 0.05). Surgical success rates were 78.9% (Mathieu-IP group) and 75.0% (Onlay group), with no significant difference ( P > 0.05). Complications were comparable between the groups. The Mathieu-IP group resulted in a vertical slit-shaped urethral meatus in 89.5% versus 13.8% in the Onlay group ( P < 0.05). Family satisfaction with general penile appearance and skin shape showed no significant differences, but the Mathieu-IP group had higher satisfaction with meatal position ( P < 0.05). Mathieu-IP offers simplicity, safety, and shorter operative time compared to Onlay. Both the techniques effectively treat urethral plate stenosis in distal hypospadias, with reduced postoperative complications compared to tubularized incised plate urethroplasty. Mathieu-IP results in a vertical slit-shaped urethral meatus which enhances urinary stream, indicating its potential for broader adoption., (Copyright © 2024 Copyright: ©The Author(s)(2024).)

Introduction: Evidence regarding the potentiating effects of intravenous dexamethasone on peripheral regional anesthesia in children is sparse. The objective of the current study was to investigate the potentiating effect of intravenous dexamethasone upon pudendal block during surgical correction of hypospadias using Snodgrass technique., Methods: The study consisted of a monocentric, randomized controlled, double-blinded study. Patients were randomized to receive either intravenous dexamethasone 0.15 mg.kg - 1 (D group) or a control solution (C group). Both groups received standardized anesthesia including a preemptive pudendal block performed after the induction of anesthesia. The primary outcome was the proportion of patients needing rescue analgesia. Secondary outcomes were other pain outcomes over the first 24 postoperative hours., Results: Overall, 70 patients were included in the study. Age were 24 [24; 36] and 26 [24; 38] months in the D and C groups, respectively (p = 0.4). Durations of surgery were similar in both groups (60 [30; 60], p = 1). The proportion of patients requiring rescue analgesia was decreased in the D group (23% versus 49%, in D and C groups respectively, p = 0.02). The first administration of rescue analgesia was significantly delayed in the D group. Postoperative pain was improved in the D group between 6 and 24 h after surgery. Opioid requirements and the incidence of vomiting did not significantly differ between groups., Conclusion: Associating intravenous dexamethasone (0.15 mg.kg - 1 ) to pudendal block during hypospadias surgery improves pain control over the first postoperative day. Further studies are needed in order to confirm these results., Gov Identifier: NCT03902249. A., What Is Already Known: dexamethasone has been found to potentiate analgesia obtained with regional anesthesia in children. B. WHAT THIS ARTICLE ADDS: intravenous dexamethasone was found to improve analgesia with a preemptive pudendal block during hypospadias surgery. C. IMPLICATIONS FOR TRANSLATION: results of this study indicate that intravenous dexamethasone could be used as an adjunct to pudendal block., (© 2024. The Author(s).)

Purpose: Mid-shaft/proximal (msp) and penoscrotal/scrotal/perineal (pssp) hypospadias treated by urethroplasty (UP) terminating at the corona (UPC) or UP terminating at the tip of the glans (UPG) were compared., Methods: UP performed at a single institute (n = 234) between 2003 and 2020 were grouped as: msp-UPC (n = 23), msp-UPG (n = 75), pssp-UPC (n = 81), and pssp-UPG (n = 55) to compare data obtained from medical records for post-UP complications (PUC; urethral stenosis, urethrocutaneous fistula, diverticulum formation, and bleeding; n = 234), post-UP uroflowmetry (PUF; average flow rate (Qave), maximum flow rate (Qmax), voiding time (VT), voided volume (VV) and urine flow curves) in 57 UP patients [msp-UPC (n = 5), msp-UPG (n = 12), pssp-UPC (n = 32), pssp-UPG (n = 8)] and 9 controls, and post-UPC esthetics (EST; n = 104). P < 0.05 was considered significant., Results: Mean ages at UP (years) were: msp-UPC (3.1 ± 3.0), msp-UPG (3.3 ± 1.4), pssp-UPC (4.6 ± 2.4), and pssp-UPG (3.4 ± 1.4); p < 0.0001 by ANOVA test. Overall, there were significantly more PUC in pssp-UPG compared with pssp-UPC except for bleeding. Ages at PUF assessment were similar. Mean Qave (mL/s) for pssp-UPG (4.0 ± 1.0) was significantly less than pssp-UPC (5.9 ± 2.5; p < 0.05) and both were significantly less than controls (6.9 ± 1.8; p < 0.05, p < 0.01, respectively). Mean Qmax (mL/s) for pssp-UPC (11.4 ± 4.8) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.05) and for controls (14.9 ± 4.4) was significantly better than pssp-UPG (7.8 ± 2.3; p < 0.01). All VT (seconds) were similar to controls; all urine flow curves were normal. For EST in UPC (n = 104), 3 cases requested meatal advancement., Conclusions: UPC may be a valid option for treating pssp hypospadias because of significant differences in PUC/PUF and minimal EST issues compared with UPG., Level of Evidence: Prognosis Study Level-Ⅱ., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: The prostatic utricle (PU) consists of the caudal remnant of the Müllerian duct and the urogenital sinus. The term "vagina masculina" is used if other Müllerian structures are associated with the PU. This work aims to investigate the incidence, management, and follow up of enlarged PUs and Müllerian remnants in males with posterior hypospadias., Patients and Methods: This study presents a retrospective review of cases presented with posterior hypospadias over a 5-year period. Prior to hypospadias repair, retrograde urethrograms were used to investigate enlarged PU. Subsequently, they were classified according to the Ikoma score and further assessed by karyotyping and cystoscope. Surgical excision was indicated in cases with symptomatic utricles or vagina masculina., Results: Thirty patients were included in the study in the period between 2015 and 2020 (Table). All cases were asymptomatic initially. Twelve patients were diagnosed with enlarged PU; three of them had vagina masculina. One case with perineal hypospadias had a separate perineal opening for PU. Following hypospadias repair, three of the eight cases treated conservatively turned symptomatic., Discussion: The incidence of enlarged PU and Müllerian remnants varied among different studies. However, it increased as the severity of hypospadias increased. Preoperative urethrogram was helpful in the diagnosis and classification of PU, but it had its limitations. Cystoscope was more advantageous in diagnosing vagina masculina. Although most cases were asymptomatic, some turned symptomatic after hypospadias repair. Some cases with perineal hypospadias had PU with a separate perineal opening., Conclusion: The incidence of enlarged PUs or Müllerian remnants was 40%. Although cases were asymptomatic before hypospadias surgery, some cases turned into symptomatic after hypospadias repair. In some cases, the PU or Müllerian remnants had a separate perineal opening. They can be classified as a particular form of Ikoma grade III necessitating surgical intervention., Competing Interests: Conflict of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Abstract: Male congenital urethrocutaneous fistula is an extremely rare anomaly that is commonly associated with chordee or anorectal malformations. It is characterised by an abnormal urethral opening on the ventral aspect of the penis with well-formed distal urethra and meatus at the tip of the glans. The treatment is individualised according to the site of the fistula, associated anomalies and condition of the distal urethra. The principles of hypospadias surgery should be strictly followed., (Copyright © 2023 Copyright: © 2023 African Journal of Paediatric Surgery.)

Aim of the Study: To assess the effect of Preoperative Hormone Stimulation (PHS) on glans size in proximal hypospadias with chordee and small glans, and to determine if PHS is associated with increased postoperative complications., Patients & Methods: Between 2014 and 2021, 101 cases of proximal hypospadias with small glans (12 mm or less) were operated upon in our hospital and are the basis of this cohort. All patients underwent a standard two-stage surgical repair, undergoing a correction of the chordee in the first operation and urethroplasty in the second operation. All patients included were operated by the same surgeon. They were classified into two groups; Group A: 50 children were operated upon between 2014 and 2017 and did not receive PHS and Group B: 51 children operated between 2018 and 2021 and received PHS. Glans dimensions including Dorsal Longitudinal Length (DLL) and Glans Width (GW) were measured during the first operation. PHS was given 1-2 months before the second operation in Group B. The glans dimensions were measured again during the second operation after PHS. Follow up period ranged from 2 to 9 years (mean 5 years)., Results: Following PHS a statistically significant increase in glans length (p = 0.042) and glans width (p = 0.011) was observed at the second operation, with 36 patients (70%) showing a mean glans width increase of 2.78 mm (range 2-8 mm) after receiving PHS. There was no statistically significant difference in the complication rates between the two groups (p = 0.556) with a fragility index of zero (FI = 0)., Discussion: The present study separates itself from most other studies in the literature, in the fact that it has included only a specific group of proximal and perineal hypospadias with severe chordee and glans width of 12 mm or less and that glans dimensions were always objectively and accurately measured under general anesthesia. The other major difference is that PHS in our study was given after and not before the first operation. Furthermore, the fact that all patients prior to 2018 did not receive PHS and all patients after 2018 received PHS, indicates that there was no selection bias., Conclusion: This study shows that PHS results in an increase the size of the glans in 70% of patients with hypospadias and a small glans without an increase in postoperative complications., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Management of proximal hypospadias remains challenging. We assessed the results of staged preputial graft repairs (SPG) for proximal hypospadias and hypothesize that post-operative vacuum physiotherapy (VP) improves graft suppleness and overall outcomes., Materials and Methods: Retrospective analysis of n = 71 patients with proximal hypospadias and severe ventral penile curvature (PC) of ≥50° after degloving. PC was corrected using ventral transverse incisions of the tunica albuginea (VTITA) without applying a tourniquet, taking care to avoid injuring the underlying erectile tissue. The ventral raw area at the penile shaft, including VTITA, were covered with either divided and partially mobilized urethral plate, or with the inner preputial graft itself. During the second stage, a tunica vaginalis flap was often used to cover the tubularized neourethra. Outcomes and post-op complications were assessed after each stage, comparing patients who received vacuum physiotherapy (VP+, n = 49) with those who did not (VP-, n = 22)., Results: Mean PC was 66°, average follow-up duration was 13.01 months, and overall complication rate was 22.5%. Only 6 of 49 VP + patients experienced complications (12.24%; 4 fistulas; 2 urethral strictures) and no recurrence of PC after second stage was observed in this group. VP- patients displayed a significantly higher rate of complications, with 10 of 22 cases (45.45%) exhibiting fistula development (n = 5) and glans dehiscence (n = 5). Recurrence of mild PC after first-stage repair was comparable between patient groups (12% VP+, 18% VP-) and easily corrected by simple graft tubularization or dorsal plication during second-stage repair., Conclusions: Staged repair using VTITA is effective for correcting proximal hypospadias with severe chordee. VP appears to promote and expedite graft suppleness and significantly improves patient outcomes., Competing Interests: Conflicts of interest All co-authors have nothing to declare., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: To investigate the clinical phenotypes, genetic characteristics, and pathological features of children with disorders of sex development (DSD)., Methods: A retrospective analysis was conducted on epidemiological, clinical phenotype, chromosomal karyotype, gonadal pathology, and genotype data of 165 hospitalized children with DSD at Children's Hospital of Hebei Province and Tangshan Maternal and Child Health Hospital from August 2008 to December 2022., Results: Among the 165 children with DSD, common presenting symptoms were short stature (62/165, 37.6%), clitoromegaly (33/165, 20.0%), cryptorchidism (28/165, 17.0%), hypospadias (24/165, 14.5%), and skin pigmentation abnormalities/exteriorized pigmented labia majora (19/165, 11.5%). Chromosomal karyotype analysis was performed on 127 cases, revealing 36 cases (28.3%) of 46,XX DSD, 34 cases (26.8%) of 46,XY DSD, and 57 cases (44.9%) of sex chromosome abnormalities. Among the sex chromosome abnormal karyotypes, the 45,X karyotype (11/57, 19%) and 45,X/other karyotype mosaicism (36/57, 63%) were more common. Sixteen children underwent histopathological biopsy of gonadal tissues, resulting in retrieval of 25 gonadal tissues. The gonadal tissue biopsies revealed 3 cases of testes, 3 cases of dysplastic testes, 6 cases of ovaries, 11 cases of ovotestes, and 1 case each of streak gonad and agenesis of gonads. Genetic testing identified pathogenic/likely pathogenic variants in 23 cases (23/36, 64%), including 12 cases of 21-hydroxylase deficiency congenital adrenal hyperplasia caused by CYP21A2 pathogenic variants., Conclusions: Short stature, clitoromegaly, cryptorchidism, hypospadias, and skin pigmentation abnormalities are common phenotypes in children with DSD. 45,X/other karyotype mosaicism and CYP21A2 compound heterozygous variants are major etiological factors in children with DSD. The most commonly observed gonadal histopathology in children with DSD includes ovotestes, ovaries, and testes/dysgenetic testes.

Objective: To investigate the potential association between paternal health and male genital malformations in the offspring., Materials and Methods: We analyzed data from 2007 to 2016 derived from the IBM MarketScan Research database, which reports on reimbursed private healthcare claims in the United States. The association between paternal comorbidities (defined as individual and combined measures) and genital malformations in male offspring was analyzed., Results: Of 376,362 male births, 22% of fathers had at least one component of metabolic syndrome (≥1) prior to conception. Totals of 2880 cases of cryptorchidism (0.77%) and 2651 cases of hypospadias (0.70%) were identified at birth. While 0.76% of sons born to fathers with no metabolic syndrome components were diagnosed with cryptorchidism, 0.82% of sons with fathers with multiple metabolic syndrome components had cryptorchidism. Similarly, 0.69% versus 0.88% of sons had hypospadias when fathers had 0 or 2+ components of metabolic syndrome. After adjusting for maternal and paternal factors, the odds of a son being diagnosed with hypospadias increased with two or more paternal metabolic syndrome components (Odds ratio [95% confidence interval]: 1.27 [1.10-1.47]). Specific components of paternal metabolic syndrome were not generally more associated with a son's genital malformations. When we performed a subgroup analysis where genital malformations were defined based on surgical correction, the association with hypospadias persisted., Conclusions: Fathers with multiple components of metabolic syndrome in the preconception period were observed to be at increased risk for having sons born with hypospadias. The results support the association between a man's andrological and overall health., (© 2023 American Society of Andrology and European Academy of Andrology.)

Summary Hypospadias is commonly assessed and repaired by pediatric urologists. Mild, distal hypospadias is generally more a cosmetic problem than a functional one and is more frequently encountered than severe, proximal hypospadias. Outcomes following repair, especially of mild phenotypes, are important to understand, but range widely in timing and measurability. Surgical complications, postoperative satisfaction of parents, patients, surgeons and even lay observers, urinary function, sexual function, and quality of life all may be considered as relevant outcomes of hypospadias repair. Existing studies examining these outcomes are diverse in their conclusions, but are important to understand when counseling parents and patients prior to any surgical intervention. [ABSTRACT FROM AUTHOR]

Background: To describe a modification of PATIO repair for urethrocutaneous fistula repair and evaluate its outcome. Methods: We studied 15 boys who underwent modified PATIO repair from Jan 2010 to Sept 2015. Parameters studied included age, type of hypospadias, age at first urethroplasty, hypospadias repair technique, number of urethroplasties required, location of fistula, time gap between urethroplasty and fistula repair, method of fistula repair, and outcome of fistula repair. Results: Mean age of the studied patients was 67.6 months (38-139). Type of hypospadias was Coronal = 3, subcoronal = 8, mid-penile = 2, prominal penile = 1, and penoscrotal = 1. Ten patients had single urethroplasty, while two patients had two urethroplasties, details not available for three patients. Average age at urethroplasty was 43.4 months (18-110). 12 patients had Snodgrass repair, Mathieu = 1 patient, tubularised plate repair = 1 patient, and unknown = 1 patient. Location of fistula was coronal in nine patients and subcoronal in six patients. Average operative time was 47.2 min (30-68). Follow-up is available for 12 patients out of which 2 patients had recurrent fistula, one of which was successfully treated by the redo modified PATIO method. Conclusion: Modified PATIO method is technically easy method for urethrocutaneous fistula repair, with less operating time and good postoperative outcomes. [ABSTRACT FROM AUTHOR]

Purpose: Testosterone administration prior to hypospadias repair is common practice among pediatric urologists; however, its impact on surgical outcomes remains controversial. We hypothesize that testosterone administration prior to distal hypospadias repair with urethroplasty significantly decreases postoperative complications., Materials and Methods: We queried our hypospadias database for primary distal hypospadias repairs with urethroplasty from 2015 to 2021. Patients undergoing repair without urethroplasty were excluded. We collected information on patient age, procedure type, testosterone administration status, initial visit and intraoperative glans width, urethroplasty length, and postoperative complications. To determine the role of testosterone administration on incidence of complications, a logistic regression adjusting for initial visit glans width, urethroplasty length, and age was performed., Results: A total of 368 patients underwent distal hypospadias repair with urethroplasty. One hundred thirty-three patients received testosterone and 235 did not. Initial visit glans width was significantly larger in the no-testosterone vs testosterone group (14.5 mm vs 13.1 mm, P = .001). Testosterone patients had significantly larger glans width at the time of surgery (17.1 mm vs 14.6 mm [no-testosterone group], P = .001). On multivariable logistic regression analysis after controlling for age at surgery, preoperative glans width, testosterone status, and urethroplasty length, testosterone administration did show significant association with reduced odds of postoperative complications (OR 0.4, P = .039)., Conclusions: This retrospective review of patients shows that on multivariable analysis there is significant association between testosterone administration and decreased incidence of complications in patients undergoing distal hypospadias repair with urethroplasty. Future studies on testosterone administration should focus on specific cohorts of patients with hypospadias as benefits of testosterone may be more evident in some subgroups than others.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To summarize the available evidence and to quantitatively evaluate the global results of different waterproofing layers in substantiating the UCF repair., Material and Methods: After defining the study protocol, the review was conducted according to the PRISMA guidelines by a team comprising experts in hypospadiology, systematic reviews and meta-analysis, epidemiology, biostatistics and data science. Studies published from 2000 onwards, reporting on the results of UCF closure after hypospadias repair were searched for on PUBMED, Embase and Google Scholar. Study quality was assessed using Joanna Briggs Checklist (JBI) critical appraisal tool. The results with different techniques were compared with the two samples independent proportions test with the help of Microsoft Excel, MedCalc software and an online calculator., Results: Seventy-three studies were shortlisted for the synthesis; the final analysis included 2886 patients (71 studies) with UCF repair failure in 539. A summary of various dimensions involved with the UCF repair has been generated including time gap after last surgery, stent-vs-no stent, supra-pubic catheterization, suture material, suturing technique, associated anomalies, complications, etc. The success rates associated with different techniques were calculated and compared: simple catheterization (100%), simple primary closure (73.2%), dartos (78.8%), double dartos flaps (81%), scrotal flaps (94.6%), tunica vaginalis (94.3%), PATIO repair (93.5%), biomaterials or dermal substitutes (92%), biocompatible adhesives (56.5%) and skin-based flaps (54.5%). Several techniques were identified as solitary publications and discussed., Conclusions: Tunica vaginalis and scrotal flaps offer the best results after UCF closure in the synthesis. However, it is not possible to label any technique as ideal or perfect. Almost all popular waterproofing layers have depicted absolute (100%) success sometimes. There are a vast number of other factors (patient's local anatomy, surgeon's expertise and technical perspectives) which influence the final outcome., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To correlate age at hypospadias repair with early post-operative complications and highlight need for adaptation to post-operative care in older children., Methods: Anecdotal evidence suggests boys with delayed surgery for hypospadias suffer increased rates of early post-operative complication. Hence, a retrospective analysis was conducted of all patients undergoing hypospadias repair between March 2019 and 2022., Results: Ninety eight patients were divided into Group A (< 2years of age at first surgery) or Group B (> 2years). While patients in Group A encountered no early post-operative complications, seven in Group B (11%) suffered a range of complications including dislodged stents (3/7), significant spasmodic pain requiring prolonged hospital stay (2/7) and urinary retention (2/7). More than half of these children required emergency supra-pubic catheter insertion., Conclusion: Significantly more children undergoing hypospadias surgery after the age of 2 years suffered complications within the early post-operative period. This resulted in prolonged hospital stays and a number returning to theatre for insertion of a supra-pubic catheter. We recommend a tailored approach to the post-operative care of older children undergoing hypospadias repair, including strict parental education regarding dressing/stent care and medication compliance, as well as efforts to enhance robustness of dressings and stent anchorage in children likely to pull at stents., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Hypospadias is a common condition and familiar, though sometimes challenging territory for the pediatric urologist. This review is a summary of hypospadias incidence, epidemiology, surgery, research, and complications intended for the non-specialist. It outlines the history, the principles of surgery for hypospadias, and the long-term outcomes., (© 2022. The Author(s), under exclusive licence to Springer Nature Limited.)

Importance: Proton pump inhibitors (PPIs) are increasingly used during pregnancy; however, several observational studies have raised concerns about an increased risk of specific types of congenital malformations., Objective: To examine the association between PPI exposure during early pregnancy and the risk of congenital malformations., Design, Setting, and Participants: This population-based cohort study used data from the National Health Insurance Service-National Health Information Database of South Korea (2010-2020); sibling-controlled analyses were conducted to account for familial factors. A total of 2 696 216 pregnancies in women aged 19 to 44 years between June 1, 2011, and December 31, 2019, and their live-born infants were identified. Pregnant women who were exposed to known teratogens or who delivered infants with chromosomal abnormalities or genetic syndromes were excluded. Data on participant race and ethnicity were not collected because the National Health Information Database does not report this information., Exposures: Proton pump inhibitor use during the first trimester., Main Outcomes and Measures: Primary outcomes were major congenital malformations, congenital heart defects, cleft palate, hydrocephalus, and hypospadias. The subtypes of major congenital malformations and congenital heart defects were evaluated as exploratory outcomes. Propensity score fine stratification was used to control for potential confounders, and a weighted generalized linear model was used to estimate relative risks with 95% CIs., Results: Of 2 696 216 pregnancies (mean [SD] maternal age, 32.1 [4.2] years), 40 540 (1.5%; mean [SD] age, 32.4 [4.6] years) were exposed to PPIs during the first trimester. The absolute risk of major congenital malformations was 396.7 per 10 000 infants in PPI-exposed pregnancies and 323.4 per 10 000 infants in unexposed pregnancies. The propensity score-adjusted relative risks were 1.07 (95% CI, 1.02-1.13) for major congenital malformations, 1.09 (95% CI, 1.01-1.17) for congenital heart defects, 1.02 (95% CI, 0.72-1.43) for cleft palate, 0.94 (95% CI, 0.54-1.63) for hydrocephalus, and 0.77 (95% CI, 0.51-1.17) for hypospadias. In the sibling-controlled analyses, no associations were observed between PPI use and primary outcomes, including major congenital malformations (odds ratio, 1.05; 95% CI, 0.91-1.22) and congenital heart defects (odds ratio, 1.07; 95% CI, 0.88-1.30). A range of sensitivity analyses revealed results that were similar to the main findings., Conclusions and Relevance: In this cohort study, the use of PPIs during early pregnancy was not associated with a substantial increase in the risk of congenital malformations, although small increased risks were observed for major congenital malformations and congenital heart defects; findings from sibling-controlled analyses revealed that PPIs were unlikely to be major teratogens. These findings may help guide clinicians and patients in decision-making about PPI use in the first trimester.

Objective: To assess the incidence of genital pain in patients with urethral stricture and examine the impact of urethroplasty. Genital pain is a common and challenging urological condition and potentially associated with urethral stricture., Methods: From 2011-2019, patients were offered enrollment in a prospective single-center study assessing patient-reported genital pain pre- and 6-months posturethroplasty. Genital pain was assessed with the question, "Do you experience genital (scrotum or penis) pain?" answered on a five-point scale ["Never" (1), "Occasionally" (2), "Sometimes" (3), "Most of the Time" (4) or "All of the Time" (5)]. Responses of 3, 4, or 5 were considered clinically significant. Wilcoxon signed-rank test was used to compare pre- and postoperative states and logistic regression was used to evaluate the association between genital pain and clinical variables., Results: Of the 387 patients completing enrollment, 36.4% (141/387) reported genital pain preoperatively. Patients with panurethral stricture reported higher rates (57.1%) of pain (Odds Ratio 2.93, 95%CI 1.32-6.50; P = .008). Posturethroplasty, pain scores improved with an incidence of 14.2% (P < .0001). In patients reporting preoperative pain, 88.7% (125/141) experienced improvement, 8.5% were unchanged and 2.8% reported worsening pain. On logistic regression, patients with penile strictures (O.R. 0.24, 95%CI 0.06-0.91; P = .04), hypospadias (O.R. 0.14, 95%CI 0.02-0.88; P = .04), and staged reconstruction (O.R. 0.22, 95%CI 0.05-0.90; P = .04) were less likely to report improvement., Conclusion: Genital pain is common in patients with urethral stricture and improves in the majority of patients undergoing urethroplasty but less so in patients with penile strictures, hypospadias and staged reconstruction., (Copyright © 2022 Elsevier Inc. All rights reserved.)

EEC syndrome is an autosomal dominant genetic disease with incomplete penetrance characterized by ectrodactyly, ectodermal dysplasia, and cleft lip/palate; these manifestations can differently occur in the affected subjects and can also be associated with other anomalies, such as in the urogenital tract.We reported the case of a newborn with prenatal diagnosis of EEC type 3 associated with severe cardiac abnormalities (Tetralogy of Fallot), high esophageal atresia with fistula and penoscrotal hypospadias.

Objective: The aim: In this study, we present and evaluate the vest-over-pants technique as a simple way to correct urethrocutaneous fistulas after hypospadias., Patients and Methods: Materials and methods: Between October 2018 and June 2020, twenty male patients aged 5 to 20 years came to us with post hypospadias repair fistula, these patients underwent vest-over-pant repair of their fistula. The size of fistula was ranging between 2.5-5 mm. The distribution of fistula was coronal (3 patients), distal penile (9 patients), midshaft (2 patients) and proximal penile (6 patients). In 14 patients there were single fistula and 6 patients had more than one fistula. Eleven of patients were exposed to a previous failed fistula repair procedure., Results: Results: Six months after the operation, the fistula recurred only in 2 patients, and our operation was successful in 90% of cases without complications., Conclusion: Conclusions: The vest-over-pants technique is a simple and effective way to treat penile fistulas after hypospadias in properly selected patients. It is a technically simple procedure with a short learning curve and no major postoperative complications.

Background: The use of barrier layers between the neourethra and skin is associated with lower rates of post-operative urethrocutaneous fistula (UCF) following hypospadias surgery. Recent studies have evaluated the ability of biologic adjuvant urethral coverings (BAUCs) - namely acellular matrix (AM), tissue adhesives (TAs), and autologous platelet-rich plasma or fibrin (PRP/PRF) - to prevent wound complications following hypospadias surgery. In general, however, these studies are small and conducted at single institutions., Objective: To assess the effect of BAUCs on the rate of UCF following single-stage primary hypospadias repair., Methods: We conducted a systematic review of studies reporting the rate of postoperative UCF in pediatric patients undergoing single-stage, primary hypospadias repairs using either AM, TA, or PRP/PRF as a layer interposed between the neourethra and skin. We then performed a pooled proportional meta-analysis of post-operative UCF. Patients within each study who underwent comparable surgery but did not receive a BAUC were used as controls., Results: 10 studies were included in our review. The meta-analysis included 280 patients from 7 studies who underwent hypospadias repairs with BAUCs. The pooled incidence of UCF was 10% (95% CI 6-14%). Mean follow-up ranged 5-23.5 months in the 5/7 studies reporting specific durations, and ≥6 month and 14-30 months, respectively, in the other two studies. Patients in whom a BAUC was used had significantly lower odds of UCF than control patients (OR 0.39, 95% CI 0.24-0.64, p = 0.0002). In subgroup analyses, significant superiority held for AM and TA; proximal or penoscrotal cases; transverse preputial island flap (TPIF) technique; when both cases and controls had local flaps; and when neither cases nor controls had flaps., Discussion: The use of BAUCs was associated with decreased rates of post-operative UCF in single-stage primary hypospadias repairs and may be most beneficial in more severe cases and when used in addition to local flaps or when using a flap is not possible. In 2/3 studies of PRP/PRF and 2/4 studies of tubularized incised plate (TIP) technique, dartos flaps were used in controls but not BAUC patients, which may explain the lack of benefit demonstrated for these subgroups. This meta-analysis is limited by the quality of evidence in the included studies, which are not uniformly randomized. Furthermore, the follow-up durations and methods for assessing complications are not standardized between included studies., Conclusion: The meta-analysis herein suggests that using BAUCs may reduce UCF rates following hypospadias surgery. Rigorous prospective evaluation is needed to validate this benefit., Competing Interests: Conflicts of interest The authors have no conflicts of interest to disclose., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Distal hypospadias repair has several operative strategies such as tubularized incised plate (TIP); Transverse preputial onlay flap (TPOF) and Modified Mathieu Procedure. The aim of our work is to compare between TIP and TPOF techniques in the outcome., Material and Method: We conducted this comparative study on 66 patients with different types of distal hypospadias divided in two groups A and B for TIP and TPOF respectively. We reported outcome and complications for both TIP and TPOF repair. Our results revealed mean operative time was significantly higher in group B (123.1 ± 6.8 min) than in group A (93.73 ± 3.9 min, P value < 0.001). Postoperative urinary fistula was reported in group A (15.2%) while no fistula in group B (p value = 0.05). The vertical slit appearance of meatus was better in group A (54.5%) than in group B (24.2%, P value 0.023). As regard to hypospadias objective scoring evaluation (HOSE) (Summary Figure) the meatal shape score was higher in group A (1.55 ± 0.51) than in group B (1.24 ± 0.44, p value = 0.0 l), the urinary stream score was significantly higher in group A (1.82 ± 0.39) than in group B (1.97 ± 0.17, p value = 0.04) and the urinary fistula score was significantly higher in group A (3.79 ± 0.60) than in group B (4 ± 0, p value = 0.04)., Discussion: Complications are more common after TIP than in TPOF. As regard the meatus shape, the naturally looking vertical slit like meatus was achieved more in the TIP than in TPOF. We advocate further studies with a large number of patients with a long follow up period., Conclusion: TPOF is a great option to consider in cases of distal hypospadias because it is an effective technique for primary distal hypospadias repair with a good cosmetic outcome and a low associated complication., Competing Interests: Conflicts of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Duplication of urethra is a very rare congenital disorder. Several types of this anomaly have been reported around the world, and are also discussed in this report. However, the mechanism of this anomaly is still unclear., Case: A 45-year-old Persian man with a complaint of recurrent urinary tract infection was referred to our clinic. He had a history of repairing penoscrotal hypospadias in childhood along with obstructive and irritating symptoms in adolescence. On his last voiding cystourethrogram and retrograde urethrogram, stenosis was observed in the proximal bulbar urethra along with a double urethra in the dorsal region of the main urethra. The double urethra was removed with operation, and he was followed for 1 month after surgery. He had no complaints of recurrence or urinary incontinence., Conclusions: This report shows the different classification systems, types of double urethra, and approach and management, which mainly involves surgery; however, surgical management should be done according to the anatomical findings of the abnormality., (© 2022. The Author(s).)

Background: Heterozygous de novo variants in SAMD9 cause MIRAGE syndrome, a complex multisystem disorder involving Myelodysplasia, Infection, Restriction of growth, Adrenal hypoplasia, Genital phenotypes, and Enteropathy. The range of additional clinical associations is expanding and includes disrupted placental development, poor post-natal growth and endocrine features. Increasingly, milder phenotypic features such as hypospadias in small for gestational age (SGA) boys and normal adrenal function are reported. Some children present with isolated myelodysplastic syndrome (MDS/monosomy 7) without MIRAGE features., Objective: We aimed to investigate: 1) the range of reported SAMD9 variants, clinical features, and possible genotype-phenotype correlations; 2) whether SAMD9 disruption affects placental function and leads to pregnancy loss/recurrent miscarriage (RM); 3) and if pathogenic variants are associated with isolated fetal growth restriction (FGR)., Methods: Published data were analyzed, particularly reviewing position/type of variant, pregnancy, growth data, and associated endocrine features. Genetic analysis of SAMD9 was performed in products of conception (POC, n=26), RM couples, (couples n=48; individuals n=96), children with FGR (n=44), SGA (n=20), and clinical Silver-Russell Syndrome (SRS, n=8), (total n=194)., Results: To date, SAMD9 variants are reported in 116 individuals [MDS/monosomy 7, 64 (55.2%); MIRAGE, 52 (44.8%)]. Children with MIRAGE features are increasingly reported without an adrenal phenotype (11/52, 21.2%). Infants without adrenal dysfunction were heavier at birth (median 1515 g versus 1020 g; P < 0.05) and born later (median 34.5 weeks versus 31.0; P < 0.05) compared to those with adrenal insufficiency. In MIRAGE patients, hypospadias is a common feature. Additional endocrinopathies include hypothyroidism, hypo- and hyper-glycemia, short stature and panhypopituitarism. Despite this increasing range of phenotypes, genetic analysis did not reveal any likely pathogenic variants/enrichment of specific variants in SAMD9 in the pregnancy loss/growth restriction cohorts studied., Conclusion: MIRAGE syndrome is more phenotypically diverse than originally reported and includes growth restriction and multisystem features, but without adrenal insufficiency. Endocrinopathies might be overlooked or develop gradually, and may be underreported. As clinical features including FGR, severe infections, anemia and lung problems can be non-specific and are often seen in neonatal medicine, SAMD9-associated conditions may be underdiagnosed. Reaching a specific diagnosis of MIRAGE syndrome is critical for personalized management., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Suntharalingham, Ishida, Del Valle, Stalman, Solanky, Wakeling, Moore, Achermann and Buonocore.)

Objective: To present our experience with urethral plate (UP) transection to treat hypospadias with severe chordee and examined the effects of this procedure on penile length. The technique involved transecting the UP to correct curvature, facilitating elongation of penile length and reconstruction of the urethra., Methods: We prospectively studied a cohort of patients with hypospadias with severe chordee who underwent UP transection and urethroplasty at our institution between February 2012 and March 2020. The length of the UP defect (length of penile elongation), complications, and patient and urologist satisfaction with penile length were analyzed., Results: A total of 510 children were included in our study: 78 cases of distal hypospadias and 432 cases of proximal hypospadias. The mean age at initial surgery was 34.84 ± 20.10 months. The length of the UP defect after transection was 2.51 ± 0.92 cm (1.6-8.0 cm). The length of the UP defect gradually increased with age (P < .05). Seven patients were "poorly satisfied" with their penile length after urethroplasty. The median follow-up duration was 32 months. Of the 510 patients, 108 (21.1%) had complications after urethroplasty, including urethral fistulas in 86 cases, urethral strictures in 9 cases, urethral diverticulum in 8 cases and glans dehiscence in 5 cases. During the follow-up period, 7 patients had mild chordee., Conclusion: Transecting the UP for hypospadias with severe chordee can elongate penile length, reduce the recurrence of curvature, and improve patient satisfaction., Competing Interests: CONFLICTS OF INTEREST The authors declare no conflicts of interest., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Hypospadias surgery undertaken in early life often continues to impose challenges as patients age. Little is known about the natural history of uncorrected hypospadias persisting into adulthood., Objective: To describe presenting symptoms and management strategies in men with uncorrected hypospadias referred to our national tertiary transitional clinic for congenital urological conditions., Materials and Methods: Patients with uncorrected hypospadias older than 16 years at the time of referral were identified by searching the electronic patient record system for ICD-10 hypospadias codes. Data were extracted over a 10-year period according to a predefined protocol., Results: Among 201 referrals, 65 men with hypospadias (glanular n = 12, coronal n = 26, subcoronal n = 9, corporal n = 4, penoscrotal n = 2 and MIP n = 12) had never previously had reconstructive surgery undertaken. Obstructive symptoms predominated (n = 30) and the risk of symptoms increased with advancing age (Figure). Presenting complaints varied across the age span; cosmetic issues (n = 11) and coital pain (n = 5) were primarily seen in youth as opposed to urinary obstructive symptoms that were increasingly more frequent with age (p = 0.002) (Figure). Management included reconstructive surgery (n = 24), minor procedures (preputioplasty, circumcision, meatoplasty, dilatation/urethrotomy, total n = 28) as well as counselling (n = 12). The management strategies were independent of age and hypospadias type., Discussion: The current cohort delineates the dynamic nature of hypospadias in itself. We speculate that the distinction in the primary complaint leading to referral between the extremes of age may relate to the vanity and insecurity of youth while older patients first come forward when other symptoms arise. Dissatisfaction with genital appearance is uncommon in previous smaller studies on men with uncorrected hypospadias unlike in our study, where 11 patients were assessed mainly for cosmetic concerns. Obstruction is the main symptom encountered in adult hypospadias patients operated in early life, and a similar picture was observed in our cohort of unoperated cases. Urethral dilatation and internal urethrotomy are temporizing procedures but were successful in immediate alleviation of obstructive symptoms in patients not willing to consign themselves to formal surgery. The study is limited by its retrospective design, and our symptomatic cohort may also represent the extreme end of the hypospadias spectrum., Conclusion: Medical issues vary across the age span in men with unrepaired hypospadias. Minor surgical procedures as well as counselling play an equally important role as reconstructive hypospadias surgery in the management of unrepaired hypospadias in adulthood., Competing Interests: Conflicts of interest The third author is a statistical advisor for Journal of Pediatric Urology. All other authors have no conflicts of interest., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: The major influencing factors for complications during hypospadias repair are the original location of the meatus, previous urethral surgery, surgical technique, and surgeon’s experience. While the effect of the technique of regional analgesia on complication rates has been considered in some studies, this issue remains controversial., Aims: To determine the effect of caudal block and penile block on complication rates of hypospadias repair in patients with subcoronal hypospadias., Study Design: Retrospective cross-sectional study., Methods: Data of children who underwent hypospadias repair between 2011 and 2019 in our clinic (n = 770) were reviewed retrospectively. Only patients with subcoronal hypospadias and who underwent tubularized incised plate urethroplasty, performed by the same two experienced surgeons (n = 279), were included in the study. The exclusion criteria were incomplete data and follow-up time shorter than 12 months. Data of 279 patients were analyzed. Patients were divided into two groups according to the analgesia type: caudal block (n = 95) and penile block (n = 184), and complication rates were compared between these groups., Results: The median age was 36 months in the caudal block group and 30 months in the penile block group ( P = 0.390). The median follow-up times were 54 and 42.7 months in the caudal and penile block groups, respectively. Total complication rates did not differ significantly between the groups and were determined as 10.5% and 12.5% in the caudal and penile groups, respectively. Urethrocutaneous fistula rates were observed as 2.1% and 4.3% (p=0.50). None of the patients had penile chordee and no penile plication was performed in the whole group., Conclusion: This study shows that there is no increase in post-repair complications in patients with subcoronal hypospadias who underwent caudal block.

Background: Hypospadias is one of the most common congenital diseases of the genitourinary system in children. The European Association of Urology (EAU) Guidelines recommend that children undergoing hypospadias surgery should be between 6 and 18 months. In China, where many children have hypospadias, it remains unknown whether clinical characteristics, socioeconomic factors and COVID-19 were associated with delayed surgery in children with hypospadias., Methods: We retrospectively analyzed children with hypospadias who underwent primary surgery at the Department of Pediatric Urology in Guangzhou Women and Children's Medical Center between January 2010 and October 2021. Patients who had two-stage surgery or a second round of surgery due to complications were excluded to eliminate data duplication. The clinical characteristics and demographic information were collected. We defined delayed surgery as primary surgery performed after 18 months following the EAU Guidelines., Results: A total of 4439 children diagnosed with hypospadias were included in the study. The median age (29.1 ± 16.7 months) of surgery for hypospadias in our study was much higher than the recommended age reported in the EAU guidelines, and 76.6% of the children underwent surgery after the age of 18 months. Children without comorbidities including cryptorchidism (odds ratio [OR] = 1.562; 95% confidence interval [CI] 1.199-2.034; p = 0.001), prostatic cyst (OR = 2.613; 95% CI 1.579-4.324; p < 0.001), penile hypoplasia (OR = 1.778; 95% CI 1.225-2.580; p = 0.002), inguinal hernia (OR = 2.070; 95% CI 1.394-3.075; p < 0.001), and penoscrotal transposition (OR = 4.125; 95% CI 1.250-13.619; p = 0.020) were more likely to receive delayed surgery. Living in a low economic area (OR = 1.731; 95% CI 1.068-2.806; p = 0.026) or not close to a main medical center (OR = 1.580; 95% CI 1.370-1.824; p < 0.001) was highly associated with delayed surgery. The proportion of children undergoing delayed surgery and the median age of surgery during the COVID-19 pandemic were significantly higher than those before the COVID-19 pandemic (p = 0.004 and < 0.001, respectively)., Conclusions: Most children with hypospadias received delayed surgery (surgical age > 18 months). Comorbidities, living in a low economic area, too far from a main medical center and the COVID-19 pandemic were highly associated with delayed surgery. It is vital to improve the public awareness of hypospadias and strengthen the re-education of primary community doctors to reduce delayed surgery., (© 2022. The Author(s).)

Background: Children affected by 45,X/46,XY mosaicism have a wide spectrum in their phenotypes. Although surgery has a role in management, there is no universally agreed strategy., Methods: We conducted a retrospective review in a tertiary center on the surgical management and its long-term outcomes in children with the karyotype 45,X/46,XY or its variants over a 20-year study period. Patients were divided into 4 groups based on their phenotypes., Results: 22 children(female=13, male=9) were included in the study, and were grouped as I)female phenotype(n = 11), II)ambiguous external genitalia(n = 2), III)undervirilized male with hypospadias(n = 6), and IV)normal male phenotype(n = 3). 19/22(86%) underwent gonadal surgeries(bilateral gonadectomy=15; unilateral gonadectomy=1; testicular biopsy=3) and  8/22(36%) underwent genitalia reconstructive surgeries(hypospadias repair=6; feminizing surgery=2). Gonadal germ cell tumors were detected in 55%, 50% and 50% of Group I, II and III respectively. 3/6 patients required reoperations for complications after hypospadias repairs. None of the patients had recurrence of germ cell tumors after gonadectomy. All the patients who had reached late adolescence or adulthood maintained their genders as their sex of rearing., Conclusions: Majority of 45,X/46,XY children benefit from timely gonadal surgery while genitalia reconstructive surgery can be considered in selected patients. Surgical management can be strategized by patients' phenotypes., Competing Interests: Declaration of Competing Interest All of the authors have no competing interest to declare., (Copyright © 2021. Published by Elsevier Inc.)

Purpose: We present our modified technique of using the inner preputial flap to cover the penile shaft, while removing the subcutaneous tissue of the distal part of the flap to act similar to graft. Herein, we present our experience with modified two stage inner preputial flap for repair of proximal hypospadias with chordee., Patients and Methods: The current study was a single-institution retrospective study between January 2016 and December 2020. Thirty-one patients with proximal hypospadias with chordee were included and underwent our modified technique. We excluded re-operative hypospadias and incomplete follow-up cases (<6 month of follow up). Patient demographics, outcomes and complications in the form of fistula formation, diverticulum, metal stenosis, stricture formation and glans dehiscence were reviewed., Results: A total of 31 patients were included in the study and underwent our modified technique. The median age was 18 months (9-60) & IQR 15-25). The median follow up was 40 months. Overall, success was achieved in 24 cases (77.4%). Complications occurred in seven cases (22.6%) and included urethrocutanous fistula in three patients (9.7%), diverticulum in two patients (6.5.%), metal stenosis in one patient (3.2%) and glans dehiscence in one patient (3.2%)., Conclusion: Our technique provides a favourable outcome with a low complication rate for repair of proximal severe hypospadias., Level of Evidence: Case Series Study (Level IV)., Competing Interests: Declaration of Competing Interest All authors declare no conflict of interest and no financial support., (Copyright © 2021. Published by Elsevier Inc.)

Introduction: Meatal stenosis is a common acquired urological problem facing pediatric surgeons and urologists. It can affect both the circumcised and non-circumcised boys with a higher incidence in the non-circumcised boys because of the increased risk of penile inflammatory conditions, most commonly lichen sclerosis and urinary tract infection, and in the most recent meta-analysis study, the incidence of meatal stenosis following circumcision is only 0.65%. Urethral meatal web is a common cause of meatal stenosis after circumcision (Fig. 1). Symptoms of meatal stenosis are not evident in non-toilet trained infants. However, in older boys, an abnormal upward direction of urine stream while urinating with a long narrow stream, dysuria, burning at the meatus, blood spot at the meatus, frequency, prolonged urination and recurrent urinary tract infection are the common presenting symptoms. Surgical correction of meatal stenosis is done by either meatotomy or meatoplasty., Objective: We describe a novel modification in the classical urethral meatotomy technique, which is wedge urethral meatotomy, and assess the outcome of this procedure in managing meatal stenosis secondary to a meatal web., Patients and Method: A prospective study was conducted on 60 cases of symptomatic meatal stenosis due to a thin ventral web treated in the pediatric surgical centre in Mosul city from October 2018 until April 2021., Exclusion Criteria: Patients with meatal stenosis not accompanied by web, patients with a thick ventral web (requiring suturing technique), and those with hypospadias or other associated urinary disorders (like VUR) were excluded from the study., Surgical Technique: In all cases, the procedure was performed under general anaesthesia and by a single surgeon (the author). A wedge (V-shaped) 2-3 mm long excision of the thin ventral meatal web made after applying a hemostat in three directions (ventral midline and two lateral crushes) (Figs. 2 and 3). The procedure did not require suturing or Foley catheterization. The operative time was ranged from 4 to 6 min (mean 5 min). Postoperatively, The parents were instructed to separate both edges of the meatus three times daily for ten days, together with applying Fucidin cream locally with oral cefixime and paracetamol., Results: The age of the 60 patients ranged between 6 months and 10 years old (median age 38 months) (Fig. 4). Two (3%) patients developed mild meatal stricture, which did not require further intervention as their urine stream remained straight with accepted calibre. The remaining 58 (97%) cases did well postoperatively apart from temporary dysuria in 23 (38%) patients, evident in the first 2-3 days postoperatively. They had a single straight urinary stream while urinating with a good calibre meatus (Figs. 2(D), 3(D) and 5), and during short term follow up, there is a substantial reduction in the frequency of urinary tract infections., Discussion: Although two (3%) cases developed mild postoperative meatal stricture, none of them required redo surgery compared with a study done by Godley SP in 2015, which showed a reoperation rate after formal meatotomy of 2%. Cartwright in 1996 had a 2% reoperation rate after office meatotomy. Another study in 2018 also reported a reoperation rate of 2.2%. The reason for restenosis that necessitates revision surgery in the above studies may be refusion of the cut edge of the web after meatotomy, which was not a concern in wedge urethral meatotomy in which the web excised and not incised. In 2017, Özen MA applied the same procedure on 13 boys complaining from meatal web after circumcision. The technique involved excision of the web, and two stitches were inserted at 5 and 7 o'clock using 7/0 Polydiaxonone suture with excellent final results. In our study, suturing was not applied, so spraying or divided urinary stream (although temporary) was not observed in the postoperative period as seen in meatoplasty or in cases of meatotomy that require suturing technique. This point was satisfactory for patients and parents., Conclusion: Wedge urethral meatotomy represents a novel modification in the formal meatotomy technique, which can be applied in meatal stenosis secondary to a thin ventral web with satisfactory results, as reoperation after this procedure was not recorded., Competing Interests: Conflict of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction and Objectives: Proximal hypospadias repair is a challenge in the pediatric urology field. Although the previous reports showed that the Belt-Fuqua staged procedure is reliable with a low complication rate for midshaft hypospadias, this procedure has not been generally applied to more proximal hypospadias with severe ventral curvature due to technical limitations. To solve these technical limitations, we developed a modified Belt-Fuqua procedure using an asymmetric long skin sleeve looking like a Japanese long-sleeved Kimono called a "furisode". The aim of this study was to evaluate the outcomes of this new modification of the Belt-Fuqua procedure for the repair of proximal hypospadias with severe curvature in children., Study Design: The study retrospectively reviewed consecutive patients with proximal hypospadias with severe curvature underwent this new technique. The major modifications included are proximal degloving beyond the urethral meatus, asymmetrical ventral transposition of dorsal preputial skin like a furisode sleeve, and circumferential proximal anastomosis of preputial skin to native meatus in the first stage. Hypospadias severity was evaluated objectively in two ways: preoperatively by the Glans-Urethral Meatus-Shaft score and intraoperative direct measurement of ventral curvature. The primary outcome was urethroplasty complications., Results: A total of 66 patients completed both stages of the furisode technique. The median Glans-Urethral Meatus-Shaft score was 11. Overall, 60 (91%) patients showed ventral curvature greater than 60 degrees after degloving, and 35 (53%) underwent ventral grafting with a dermal graft in the first stage. Median urethral length constructed at the second stage was 41.5 mm. The median follow-up period was 25 months. Complications occurred in 9 patients (14%); 7 had urethral diverticula, 1 showed a fistula and 1 had a urethral stricture. Neither glans dehiscence nor meatal stenosis occurred., Discussion: There are a couple of advantages of this furisode technique to other tunneled flap techniques like the Ulaanbaatar procedure. The first is the ease of glans reshaping with a Firlit collar at the first stage because of no urethra in the glans. The second is that a long neourethra could be made by the same skin flap with one anastomosis to the native urethra.The weakness of this procedure was that urethral diverticula were prone to occur less than one year after urethroplasty., Conclusion: The furisode technique, a new modification of the Belt-Fuqua procedure, provides an alternative staged repair of proximal hypospadias. This technique was easily applied for hypospadias with severe curvature requiring ventral grafting., Competing Interests: Conflict of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Abstract: Objective: To compare the outcomes of three different urethroplasty techniques (onlay, buccal mucosa, Koyanagi type I) used in the reconstruction of severe hypospadias. Patients and methods: Over 10 years (1997–2007), 300 severe hypospadias cases were treated with a mean follow up of 2 years (1–105 months); 203 were operated by the same surgeon of whom 184 completed follow up. Three main techniques were used according to the quality of the urethral plate: onlay urethroplasty (133), buccal graft urethroplasty (25) and Koyanagi type I (26). The mean age at surgery was 36 months (8–298); 76 required preoperative androgen stimulation (onlay 37, buccal 11, Koyanagi 26); 18 required a corporoplasty to straighten the penis (onlay 13, buccal 3, Koyanagi 2). Results: Thirty-eight onlay (28.5%); 14 buccal (56%); 16 Koyanagi (61.5%) urethroplasties had a complication. The fistula rate was 15% for the onlay group; 32% for the buccal mucosa group; 19.2% for the Koyanagi cases. The dehiscence rate was, respectively, 11.3%, 20% and 42.3%. The stricture rate was, respectively, 1.5%, 20% and 34.6%. Urethrocele was found in seven Koyanagi patients. Final functional and cosmetic results were satisfactory in 126/133 (94.7%) onlay, 20/25 (80%) buccal and 14/26 Koyanagi (53.8%) urethroplasties. Primary cases had better results (89%) than redo cases (75.9%). Patients submitted to preoperative androgen therapy developed more complications (onlay: 40.5% vs 23.9%; buccal: 70% vs 43.7%). Conclusion: Two striking results are the low number of severe hypospadias cases requiring an additional corporoplasty, and the increased complication rate found in androgen-stimulated patients. The excellent results of the onlay procedure could be related to the use of dorsal preputial tissue, which in hypospadias is characterized by a well-balanced protein platform compared to the ventral tissues. [Copyright &y& Elsevier]

Aims: Hypogonadism is associated with cardiovascular disease. However, the cardiovascular impact of hypogonadism during development is unknown. Using hypospadias as a surrogate of hypogonadism, we investigated whether hypospadias is associated with vascular dysfunction and is a risk factor for cardiovascular disease., Methods and Results: Our human study spanned molecular mechanistic to epidemiological investigations. Clinical vascular phenotyping was performed in adolescents with hypospadias and controls. Small subcutaneous arteries from penile skin from boys undergoing hypospadias repair and controls were isolated and functional studies were assessed by myography. Vascular smooth muscle cells were used to assess: Rho kinase, reactive oxygen species (ROS), nitric oxide synthase/nitric oxide, and DNA damage. Systemic oxidative stress was assessed in plasma and urine. Hospital episode data compared men with a history of hypospadias vs. controls. In adolescents with hypospadias, systolic blood pressure (P = 0.005), pulse pressure (P = 0.03), and carotid intima-media thickness standard deviation scores (P = 0.01) were increased. Arteries from boys with hypospadias demonstrated increased U46619-induced vasoconstriction (P = 0.009) and reduced acetylcholine-induced endothelium-dependent (P < 0.0001) and sodium nitroprusside-induced endothelium-independent vasorelaxation (P < 0.0001). Men born with hypospadias were at increased risk of arrhythmia [odds ratio (OR) 2.8, 95% confidence interval (CI) 1.4-5.6, P = 0.003]; hypertension (OR 4.2, 95% CI 1.5-11.9, P = 0.04); and heart failure (OR 1.9, 95% CI 1.7-114.3, P = 0.02)., Conclusion: Hypospadias is associated with vascular dysfunction and predisposes to hypertension and cardiovascular disease in adulthood. Underlying mechanisms involve perturbed Rho kinase- and Nox5/ROS-dependent signalling. Our novel findings delineate molecular mechanisms of vascular injury in hypogonadism, and identify hypospadias as a cardiovascular risk factor in males., (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology.)

Objectives: Penile urethral stricture disease not associated with hypospadias is rare, and there is a wide range of commonly used surgical repair techniques for this disease. We sought to compile a multi-institutional database of patients who had surgical correction of strictures in the penile urethra not limited to the meatus, and who had no history of hypospadias, for analysis using the Trauma and Urologic Reconstructive Network of Surgeons length, urethral segment and etiology classification system., Methods: A retrospective database from 13 institutions was compiled of patients who had undergone surgical correction of Trauma and Urologic Reconstructive Network of Surgeons length, urethral segment and etiology urethral stricture segments S2b/S2c and excluding E5, with a minimum follow-up time of 4 months. Failure was defined as cystoscopically confirmed recurrence of a stricture measuring less than 16-Fr., Results: We analyzed 222 patients with a median age of 57 years and a follow-up of 49 months. The overall surgical success rate was 80.2%. On multivariate analysis, the two variables identified that were predictive of surgical success were stricture length ≤2 cm as well as use of a buccal mucosa graft as compared to use of a fasciocutaneous flap, which had success rates of 83% and 52%, respectively (P = 0.0004). No statistically significant differences were found based on incisional approach or surgical technique, nor were outcomes different based on etiology or preoperative patient demographics., Conclusions: Surgical repair of penile urethral strictures of non-hypospadiac origin have a favorable overall success rate, at 80.2%. Regardless of incisional approach or surgical technique, all operations appear to have similar outcomes other than repairs using fasciocutaneous flap, which were statistically less successful than those using buccal mucosa graft., (© 2022 The Japanese Urological Association.)

Background: Use of prophylactic antibiotics after stented hypospadias repair is very common, but most research has not identified any clinical benefits of this practice. Only one study has found that postoperative prophylaxis reduces symptomatic urinary tract infections (UTIs). Data from the same trial suggested that prophylaxis may also reduce urethroplasty complications. No studies on this subject have been placebo-controlled., Objective: We performed a randomized, double-blind, placebo-controlled study to evaluate the effect of postoperative prophylactic antibiotics on the incidence of infection or urethroplasty complications after stented repair of midshaft-to-distal hypospadias., Study Design: Boys were eligible for this multicenter trial if they had a primary, single-stage repair of mid-to-distal hypospadias with placement of an open-drainage urethral stent for an intended duration of 5-10 days. Participants were randomized in a double-blind fashion to receive oral trimethoprim-sulfamethoxazole or placebo twice daily for 10 days postoperatively. The primary outcome was a composite of symptomatic UTI, surgical site infection (SSI), and urethroplasty complications, including urethrocutaneous fistula, meatal stenosis, and dehiscence. Secondary outcomes included each component of the primary outcome as well as acute adverse drug reactions (ADRs) and C. difficile colitis., Results: Infection or urethroplasty complications occurred in 10 of 45 boys (22%) assigned to receive antibiotic prophylaxis as compared with 5 of 48 (10%) who received placebo (relative risk [RR], 2.1; 95% confidence interval [CI], 0.8 to 5.8; p = 0.16). There were no significant differences between groups in symptomatic UTIs, SSIs, or any urethroplasty complications. Mild ADRs occurred in 3 of 45 boys (7%) assigned to antibiotics as compared with 5 of 48 (10%) given placebo (RR, 0.6; 95% CI, 0.2 to 2.5; p = 0.72). There were no moderate-to-severe ADRs, and no patients developed C. difficile colitis., Conclusions: In this placebo-controlled trial of 93 patients, prophylactic antibiotics were not found to reduce infection or urethroplasty complications after stented mid-to-distal hypospadias repair. The study did not reach its desired sample size and was therefore underpowered to independently support a conclusion that prophylaxis is not beneficial. However, the result is consistent with most prior research on this subject., Gov Identifier: NCT02096159., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias., Methods: A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD)., Results: The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P<0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P>0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular., Conclusion: In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results., Level of Evidence: III., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Introduction: Urethrocutaneous fistula (UCF) development following primary hypospadias repair is a common complication with high rates of recurrence despite attempts at repair. A novel technique for the management of these fistulae, the PATIO (preserve the tract and turn it inside out) repair, has been described and has shown encouraging outcomes in previous reports., Objective: The aim of this study was to evaluate fistula repair outcomes in patients undergoing the PATIO technique compared with standard repair., Study Design: A retrospective chart-based review was performed for pediatric patients undergoing UCF repair from January 2005 to July 2018. Data including: age, follow-up, meatal location, meatal stenosis, number of fistulae and repairs, UCF location, complications, and outcomes was obtained. Cases were categorized into PATIO repair, standard repair, and PATIO repair following prior standard repair. Surgical outcome with respect to freedom from fistula recurrence was determined., Results: In total, 586 patients underwent hypospadias surgery with 44 patients developing 52 UCF cases that required repair during the study period for a fistula rate of 8.9%. Mean age at repair was 19 months. Median follow-up time was 28 months. For PATIO repair alone, 21/26 (81%) had success. For standard repair alone, 8/18 (44%) had success and for standard repair followed by PATIO repair, 8/8 (100%) were successful. A statistically significant difference was noted for success when comparing standard repair with PATIO repair (p = 0.023, p < 0.05) and PATIO repair following standard repair (p = 0.010, p < 0.05). There was a statistically non-significant difference between PATIO repair and PATIO repair following standard repair (p = 0.309, p < 0.05). Failure following PATIO repair was found in cases where the procedure was early in implementation and experience was limited., Discussion: UCF repair using the PATIO technique has shown encouraging results in the short-term, with a majority of patients achieving a successful outcome compared with standards techniques. As this procedure continues to be used and experience develops, a larger sample of cases will become available for analysis and longer follow-up will prove necessary in examining the long-term outcomes of this procedure. The outcomes examined have demonstrated consistency with previously reported outcomes in the literature. Limitations include small sample size, short-term follow up, and the retrospective nature of the review., Conclusions: The findings of this study have provided further support to the use of this technique in conventional UCF repair as a means to decrease the risk of recurrence and provide durable results in the short-term. Ongoing follow up will prove necessary to examine success in the long-term., Competing Interests: Conflicts of interest The author of this paper does not have any conflicts of interest to disclose., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Our study examined the benefit of an alternative interposition urethroplasty (IU) procedure for glandular hypospadias (GH) with severe penile curvature (SPC). The technique involved transecting and reconstructing the urethra to preserve the distal glandular and coronal urethra and correct the curvature. We compared procedural characteristics, outcomes, and surgical complications for the single-stage and staged IU techniques., Methods: We retrospectively studied 44 patients with GH with SPC who underwent single-stage or staged IU between March 2005 and June 2020. Demographics, operative details, complications, and uroflometry findings were analyzed., Results: The median age at initial surgery was 37.5 months. Ten patients underwent single-stage IU repair, and 34 patients underwent staged IU repair. The median length of the interposition neourethra was 3.2 cm (2.2-4.3). The median follow-up duration was 58 months, and the overall complication rate was 13.6%. Complications were noted in 30% (3/10) and 8.8% (3/34) of patients in the single-stage and staged IU groups, respectively (p > 0.05). Fistula formation was noted in one and three patients in the single-stage and staged groups, respectively (8.8% vs. 10%, p > 0.05). Two cases of urethral stricture were documented in the single-stage group only. No chordee recurrence or urethral diverticula was noted in any of the patients., Conclusion: IU is a reliable and durable technique for GH with SPC. It avoided penile shortening, preserved the distal urethra, and reduced the risk of chordee recurrence. The staged IU technique had more superior outcomes compared to the single-stage IU technique., (© 2021. The Author(s).)

Hypospadias is a common congenital disorder of male genital formation. Children born small for gestational age (SGA) present a high frequency of hypospadias of undetermined etiology. No previous study investigated the molecular etiology of hypospadias in boys born SGA using massively parallel sequencing. Our objective is to report the genetic findings of a cohort of patients born SGA with medium or proximal hypospadias. We identified 46 individuals with this phenotype from a large cohort of 46,XY DSD patients, including 5 individuals with syndromic features. DNA samples from subjects were studied by either whole exome sequencing or target gene panel approach. Three of the syndromic patients have 5 main clinical features of Silver-Russell syndrome (SRS) and were first studied by MLPA. Among the syndromic patients, loss of DNA methylation at the imprinting control region H19/IGF2 was identified in 2 individuals with SRS clinical diagnosis. Two novel pathogenic variants in compound heterozygous state were identified in the CUL7 gene establishing the diagnosis of 3M syndrome in one patient, and a novel homozygous variant in TRIM37 was identified in another boy with Mulibrey nanism phenotype. Among the non-syndromic subjects, 7 rare heterozygous variants were identified in 6 DSD-related genes. However, none of the variants found can explain the phenotype by themselves. In conclusion, a genetic defect that clarifies the etiology of hypospadias was not found in most of the non-syndromic SGA children, supporting the hypothesis that multifactorial causes, new genes, and/or unidentified epigenetic defects may have an influence in this condition., (© 2021 S. Karger AG, Basel.)

Background: To explore whether opening the external urethral orifice in the coronal sulcus can reduce the incidence of epididymitis after operating on hypospadias with prostatic utricle cyst (PUC) connecting to the vas deferens. Group A consisted of 3 patients with severe hypospadias and PUC undergoing cystostomy, hypospadias correction and urethroplasty, along with the relocation of the external orifice of the urethra to the coronal sulcus. Group B consisted of 4 patients having initial hypospadias repaired with meatus in the orthotopic position in the glans, presenting with multiple epididymitis after hypospadias surgery and unsuccessful conservative treatment. MR confirmed that all the Group B patients had PUC connecting to the vas deferens. Group B patients underwent urethral dilatation along with urethral catheterization, cutting of the original corpus cavernosum that encapsulated the urethra, and extension of the position of the external urethral orifice to the coronal sulcus., Results: In group A, 3 children underwent bladder fistula removal 2 weeks after the operation. The penis developed normally without any complications. Four children in group B underwent stent removal 12 weeks after operation, and one patient was still stenosed and dilated again. All patients in group B were followed without epididymitis recurrence., Conclusions: For patients with hypospadias complicating with a PUC, connecting to one side of the vas deferens, the positioning of the external urethral orifice in the coronary sulcus would be helpful to reduce the occurrence of epididymitis., (© 2021. The Author(s).)

Purpose: We identified the incidence of acquired cryptorchidism among patients with proximal and mid shaft hypospadias, predictors of acquired cryptorchidism, and the prevalence of testis-epididymis nonfusion with ascended testes. We hypothesized that proximal hypospadias would be associated with higher incidence of acquired cryptorchidism than mid shaft hypospadias, and that ascended testes would exhibit increased prevalence of testis-epididymis nonfusion similar to anatomical findings in an undescended testis., Materials and Methods: A retrospective cohort study of patients who underwent primary proximal and mid shaft hypospadias repair from 2010 to 2016 was conducted. Clinical and operative notes were reviewed. Patients with congenitally undescended testes or differences of sex development were excluded., Results: A total of 175 patients were identified. Those with proximal hypospadias (14/104, 13%) were more likely than those with mid shaft hypospadias (1/71, 1%) to develop acquired cryptorchidism (p=0.04). Among proximal hypospadias patients, increased risk of acquired cryptorchidism was associated with pre-term birth (p <0.01) and penoscrotal transposition (p=0.01) but not with testis position on initial examination (p >0.99). In the 14 proximal hypospadias patients with acquired cryptorchidism, 21 ascended testes underwent orchiopexy. Operative notes adequately described testis-epididymis anatomy for 8/21 ascended testes. Testis-epididymis nonfusion was described in 6/8 ascended testes., Conclusions: Risk of acquired cryptorchidism is increased among patients with proximal hypospadias. Operative notes revealed a high rate of epididymal nonfusion with ascended testes, suggesting these testes morphologically resemble undescended testes. Close followup of testis position is needed in these patients, and the threshold to perform orchiopexy may need to be lower in select patients.

Purpose: To report long-term results and patient reported outcomes of staged anterior urethroplasties, and isolate risk factors for recurrence., Methods:  We reviewed urethroplasty database for all patients who underwent staged urethroplasty from 2000 to 2017. Follow-up included a cystoscopy 4 months after their 2nd stage to assess early success, and then annual follow-up thereafter with post-void residual and symptom assessment. Stricture characteristics, etiology and graft type were analyzed with regards to success., Results:  Forty-nine patients were eligible for inclusion. The median stricture length was 7 cm (3-17 cm). The early success rate demonstrated by cystoscopy at 4 months was 100%. Long-term success was 96.4% in buccal graft (BMG) only patients; however, long-term success fell considerably to 53% in patients requiring any use split thickness skin graft (STSG) in the first stage. Median follow up time was 57 months (6-240 months). On analysis, age, increased stricture length and especially the use of STSG all appeared to be associated with late recurrence. The recurrence group had longer stricture length and were more likely to be panurethral. All recurrences occurred after the initial 4-month cystoscopy with a median time to recurrence of 78 months., Conclusion: Staged repairs that are amenable to BMG-only repairs have high long-term success rates. Increasing stricture length and the addition of split-thickness skin graft were associated with lower success rate in staged urethral reconstruction. Patients requiring staged repairs often experience recurrence in a very delayed fashion reinforcing the need for close, long-term follow up., (© 2021. The Author(s).)

Purpose: We assessed the long-term surgical, functional urinary and sexual outcomes of adolescent and young adult men who underwent childhood hypospadias repair., Materials and Methods: Men born with nonsyndromic hypospadias and healthy male controls aged 16-21 years old were recruited, and their surgical, urinary, sexual functional and aesthetic outcomes assessed. Good outcome was defined as a patent and orthotopic meatus without fistulas, and straight erections (<30 degree curvature) without erectile or ejaculatory problems. Statistics included regression analyses, chi-square/Fisher exact tests and Student's t/Mann-Whitney U and Kruskal-Wallis tests., Results: A total of 193 patients and 50 controls participated 16.4 years (range 8.2-21.2) after initial repair. At least 1 reintervention was performed in 39.2%. The highest reintervention rate was found in those younger than 12 months at initial repair, even when excluding proximal hypospadias cases. A disturbed urinary and/or suboptimal sexual functional outcome was seen in 52.9% of cases. Suboptimal voiding was found in 22.1%, although few had relevant residual urine. More reinterventions and proximal hypospadias cases were associated with suboptimal urinary outcome, and the latter also with impaired sexual function. Poor inter-observer agreements were found between physician and patient genital appraisal., Conclusions: In 52.9% of cases, at least 1 concern was identified that required long-term followup. Hypospadias repair below 12 months was associated with more reinterventions. Adopting a restrictive attitude toward aesthetic refinement, unless on the patient's own request, could improve urinary outcomes.

Purpose: To review existing literature about fertility and sexuality of boys born with complex congenital genitourinary anomalies., Methods: A Pubmed review was performed in December 2018 to identify the most relevant original manuscripts regarding male complex congenital conditions affecting the urogenital system in male patients including spina bifida (SB), bladder exstrophy-epispadias complex (BEEC) and hypospadias. A comprehensive review was drafted exploring sexual dysfunction from a medical, psychosexual, surgical and reproductive point of view during transition from childhood (or adolescence) to adulthood., Results: About 75% of men with SB have erectile dysfunction (ED) (Gamé et al. in Urology 67(3):566-570, 2006; Diamond et al. in 58(4):434-435, 1986). Most SB patients have impaired sexual development mainly due to diminished self-esteem, dependence on caregivers and lack of privacy (Blum et al. in Pediatrics 88(2):280-285, 1991). Men with BEEC have fewer intimate relationships than women because of the greater difficulties with issues regarding their genitalia and sexual activities (Deans et al. in Am J Obstet Gynecol 206(6):496.e1-496.e6, 2012). However, a good quality of life is achievable with the effective use of coping strategies (Deng et al. in Transl Androl Urol 7:941, 2018; Rikken et al. in BMC Womens Health 18(1):163, 2018; Friedler et al. in Reprod Biomed Online 32(1):54-61, 2016). Chordee occurs in 25% of all hypospadias patients. More severe hypospadias is related to a greater risk for complications. The long-term sexual quality of life (QoL) in men who underwent hypospadias surgery is influenced by a lot of factors. Therefore, an interactive and dynamic biopsychosocial model of sexual QoL was proposed., Conclusions: The care of patients with congenital urologic conditions becomes a challenge especially in the period of 'transition'. The goal of follow-up is a holistic management viewed from a medical, psychosexual, surgical end reproductive point. All patients should be asked for specific urinary, fecal or sexual concerns.