Quantification of vesicoureteral reflux using machine learning.

INTRODUCTION: The radiographic grading of voiding cystourethrogram (VCUG) images is often used to determine the clinical course and appropriate treatment in patients with vesicoureteral reflux (VUR). However, image-based evaluation of VUR remains highly subjective, so we developed a supervised machine learning model to automatically and objectively grade VCUG data. STUDY DESIGN: A total of 113 VCUG images were gathered from public sources to compile the dataset for this study. For each image, VUR severity was graded by four pediatric radiologists and three pediatric urologists (low severity scored 1-3; high severity 4-5). Ground truth for each image was assigned based on the grade diagnosed by a majority of the expert assessors. Nine features were extracted from each VCUG image, then six machine learning models were trained, validated, and tested using 'leave-one-out' cross-validation. All features were compared and contrasted, with the highest-ranked then being used to train the final models. RESULTS: F1-score is a metric that is often used to indicate performance accuracy of machine learning models. When using the highest-ranked VCUG image features, F1-scores for the support vector machine (SVM) and multi-layer perceptron (MLP) classifiers were 90.27 % and 91.14 %, respectively, indicating a high level of accuracy. When using all features combined, F1 scores were 89.37 % for SVM and 90.27 % for MLP. DISCUSSION: These findings indicate that a distorted pattern of renal calyces is an accurate predictor of high-grade VUR. Machine learning protocols can be enhanced in future to improve objective grading of VUR.

Urologist validation of an artificial intelligence-based tool for automated estimation of penile curvature.

INTRODUCTION: Severity of penile curvature (PC) is commonly used to select the optimal surgical intervention for hypospadias, either alone or in conjunction with other phenotypic characteristics. Despite this, current literature on the accuracy and precision of different PC measurement techniques in hypospadias patients remains limited. PURPOSE: Assess the feasibility and validity of an artificial intelligence (AI)-based model for automatic measurement of PC. MATERIAL AND METHODS: Seven 3D-printed penile models with variable degrees of ventral PC were used to evaluate and compare interobserver agreement in estimation of penile curvatures using various measurement techniques (including visual inspection, goniometer, manual estimation via a mobile application, and an AI-based angle estimation app. In addition, each participant was required to complete a questionnaire about their background and experience. RESULTS: Thirty-five clinical practitioners participated in the study, including pediatric urologists, pediatric surgeons, and urologists. For each PC assessment method, time required, mean absolute error (MAE), and inter-rater agreement were assessed. For goniometer-based measurement, the lowest MAE achieved was derived from a model featuring 86° PC. When using either UVI (unaid visual inspection), mobile apps, or AI-based measurement, MAE was lowest when assessing a model with 88° PC, indicating that high-grade cases can be quantified more reliably. Indeed, MAE was highest when PC angle ranged between 40° and 58° for all the investigated measurement tools. In fact, among these methodologies, AI-based assessment achieved the lowest MAE and highest level of inter-class correlation, with an average measurement time of only 22 s. CONCLUSION: AI-based PC measurement models are more practical and consistent than the alternative curvature assessment tools already available. The AI method described in this study could help surgeons and hypospadiology researchers to measure PC more accurately.

Impact of bilateral ureteral reimplantation at the time of complete primary repair of bladder exstrophy on reflux rates, renogram abnormalities and bladder capacity.

INTRODUCTION: Bilateral ureteral reimplantation at the time of the complete primary repair of bladder exstrophy (BUR-CPRE) has been proposed and has demonstrated favorable outcomes in the past. However, the potential benefits, including prevention of vesicoureteral reflux (VUR) and renal scarring must be tempered with any risks of reimplantation, persistent VUR, and the potential for overtreatment. We aimed to determine the impact of BUR-CPRE on reflux rates, renogram findings and bladder capacity. METHODS: An IRB approved registry of children treated for bladder exstrophy epispadias complex (BEEC) during a long-term international collaboration hosted in a region with high prevalence of BEEC was queried. Children undergoing primary CPRE for bladder exstrophy (BE) were identified. Surgical procedure and outcome measures nuclear medicine dimercaptosuccinic acid (DMSA) scintigraphy scans, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were assessed for presence and degree of VUR, renogram abnormalities, and bladder capacity. RESULTS: A total cohort of 147 patients with BEEC was queried; 52 children (37 males, 71%) underwent primary CPRE for BE between 2009 and 2019 at median age of 1.1 years (IQR 0.6-1.9 years) with median follow up 4.4 years (IQR 2.4-6.4 years). BUR-CPRE was performed in 22/52 (42%). After BUR-CPRE, children were less likely to have VUR (any VUR present in 9 of 20 with imaging (45%) compared to 23 of 26 with imaging (82%) in the CPRE alone group (p = 0.007)). VUR in the BUR-CPRE group tended to be unilateral and lower grade in comparison to the CPRE alone group. DMSA abnormalities were less common in the BUR-CPRE group (4/19 (21%) vs.12/27 (44%)), although the difference did not reach statistical significance (p = 0.1). At 4 years follow-up, the BUR-CPRE group had a larger bladder capacity (p = 0.016). DISCUSSION: After BUR-CPRE, children had a lower rate of VUR, and when present, VUR was more often unilateral and lower grade compared to the CPRE alone group. Fewer numbers of children in the BUR-CPRE group depicted DMSA abnormalities. No children developed obstruction after BUR-CPRE and none have undergone repeat reimplantation. We documented a larger bladder capacity at the time of maximum follow-up available (4 years)-but further data are needed to confirm this observation. CONCLUSION: BUR-CPRE decreases the incidence and severity of VUR after CPRE, but the clinical significance of this remains unclear. We are encouraged by these initial results, but since BUR-CPRE does not uniformly eliminate VUR, we continue to proceed carefully in the well selected patient.

Plate Objective Scoring Tool (POST); An objective methodology for the assessment of urethral plate in distal hypospadias.

BACKGROUND: Estimation of the quality of the urethral plate (UP) seems to be important when assessing postoperative outcomes of hypospadias repair, but its evaluation remains subjective. We developed an objective model aiming to standardize this assessment, proposing a methodology that could be adopted in future studies designed to evaluate outcomes in the treatment of hypospadias. OBJECTIVES: To evaluate the inter and intra observer reliability of a method to assess the quality of the urethral plate (UP) in hypospadias (POST - Plate Objective Scoring Tool) based on elements of glans characteristics. The reliability of such scoring methodology was compared to an analog accepted tool: the Glans-Urethral Meatus-Shaft (GMS) score. A secondary goal was to compare some characteristics of the UP in GMS score to POST values; aiming to find the threshold between favorable and unfavorable plates. METHODS: Data were prospectively obtained from prepubertal boys who underwent primary hypospadias repair between January 2018 and November 2019. Intrinsic elements of the glanular UP (A: distal midline mucocutaneous junction; B: Glanular knob where the mucosal edges of the UP's edge change direction; C: Glanular/coronal junction) were marked and the AB/BC ratio calculated. The "G" and "M" components of the GMS score were measured electronically three times by four different reviewers with variable pediatric urology experience and blinded to each other values. Favorable UP, according to the GMS score was compared to measurements obtained by POST (AB/BC ratio). RESULTS: 84 subjects were enrolled. The POST score had an excellent inter-observer agreement for the evaluation of the UP in distal hypospadias (Kappa = 0.832) while the "G" parameter of the GMS correlated poorly among the observers. The Inter-Class Correlation (ICC) analysis to examine the intra-rater agreement value was 0.914 (95% CI 0.87, 0.95.). AB/BC ratio values ranged from 0.6 to 1.6, with a mean of 1.12. The POST cut-off value for "favorable" UP was 1.2 (AUC = 0.62) 95% CI (0.52-0.84) (sensitivity 80% and specificity 60%). CONCLUSIONS: The POST score has higher inter-observer reliability and functions favorably when compared to the GMS. In addition, it demonstrated a high intra-observer reliability among observers of variable experience. Our findings suggest that the POST score adds to evaluation of the UP and could be incorporated as an additional criterion to assess outcomes in distal hypospadias repairs.

Modified staged repair of bladder exstrophy: a strategy to prevent penile ischemia while maintaining advantage of the complete primary repair of bladder exstrophy.

INTRODUCTION: Penile ischemic injury is a reported catastrophic complication after complete primary repair of exstrophy (CPRE). Aiming to improve the bladder exstrophy-epispadias repair outcomes, the study institution adopted a modified staged exstrophy repair to incorporate the advantages of CPRE by avoiding concurrent epispadias repair and adding bilateral ureteral re-implantation and bladder neck tailoring (staged repair of bladder exstrophy with bilateral ureteral re-implantation [SRBE-BUR]) at the initial repair. It was hypothesized that such modifications minimize penile complications and prevent upper tract deterioration while enhancing bladder resistance and consequent capacity. Here, a comparative series of outcomes between CPRE and SRBE-BUR is reported. METHODS: A retrospective cohort study including all exstrophy-epispadias male neonates managed in the study institution from January 2000 to December 2014 was performed. Patients were divided into those who underwent CPRE-BUR (group 1) and SRBE-BUR (group 2) (Figure). Baseline characteristics, peri-operative data, and long-term surgical outcomes were collected and analyzed for between-group comparison. Fisher exact and Mann-Whitney U tests were performed for statistical analysis. RESULTS: A total of 21 eligible patients were included: 10 in group 1 and 11 in group 2. Baseline characteristics were comparable. Two patients in group 1 had intra-operative penile ischemic injury (one with subsequent penile tissue loss), whereas none of the group 2 patients had intra-operative complications. No significant difference between the groups was noted for operative time; however, significantly lesser blood loss was noted in group 2. Comparable long-term surgical outcomes such as additional surgical intervention, urinary continence, bladder capacity, vesicoureteral reflux, hydronephrosis and recurrent urinary tract infections (UTIs) were noted. In addition, although subjective, better penile length and cosmesis were achieved by staging the repair (Figure). CONCLUSION: The SRBE with bilateral ureteral re-implantation is a safe alternative for the repair of the exstrophy-epispadias repair as it prevents the catastrophic complication of penile tissue loss, while having comparable long-term outcomes with the CPRE. Delaying epispadias repair avoids penile injury besides possible improvement of its overall cosmesis.

Long-term follow-up after traditional versus modified perineal approach in the management of female epispadias.

OBJECTIVE: Isolated female epispadias (IFE) is a rare congenital anomaly. The defect extends to the bladder neck, which is usually incompetent. The traditional surgical approach includes urethral and genital reconstruction in the first year, followed by bladder neck reconstruction (Young-Dees-Leadbetter cervicoplasty (YDL)) at the age of social continence. An alternative single-stage technique includes urethral, bladder neck and clitoris repair by a perineal approach. The aim of the present study was to describe long-term follow-up of patients who underwent the traditional vs alternative approach. MATERIALS AND METHODS: A retrospective review was performed of all female epispadias cases managed between 2000 and 2013. The YDL procedure (Group 1) vs single-stage perineal approach (Group 2) cases were followed and compared. Collected variables included: patients' demographics, age at diagnosis and surgery, presence of associated anomalies, clinical presentation, presence of vesicoureteral reflux (VUR), and pre-operative and postoperative continence. RESULTS: A total of 12 cases of female epispadias were managed and followed between 2000 and 2013. No major complications occurred in either group. Urinary continence evaluated in seven children showed that none (0/3) and 4/7 (57%) were continent following the initial procedure in Group 1 and Group 2, respectively. All patients in Group 1 failed to achieve continence and required re-intervention. CONCLUSIONS: Female epispadias could be successfully repaired using a single-stage modified perineal approach that achieved good continence with volitional voiding, good cosmetic results and compared favorably with the ones repaired with the YDL technique. The additional step of performing bladder neck tailoring to achieve a funneling configuration seemed to be useful in improving continence.

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46,XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.

Proximal hypospadias: A persistent challenge. Single institution outcome analysis of three surgical techniques over a 10-year period.

INTRODUCTION: The optimal treatment of proximal hypospadias remains controversial. Several techniques have been described, but the best approach remains unsettled. OBJECTIVE: To evaluate and compare the complication rates of proximal hypospadias with and without ventral curvature (VC), according to three different surgical techniques: tubularized incised plate (TIP) uretroplasty, dorsal inlay graft TIP (DIG), and staged preputial repair (SR). It was hypothesized that SR performs better than TIP and DIG for proximal hypospadias. METHODS: Single-center, retrospective chart review of all patients with primary proximal hypospadias reconstructed between 2003 and 2013. The DIG was selectively employed in cases with narrow urethral plate (UP) and deficient spongiosum. Extensive urethral plate (UP) mobilization (UPM), dorsal plication (DP) and/or deep transverse incisions of tunica albuginea (DTITA) were selectively performed when attempting to spare transecting the UP. Division of UP and SR was favored in cases with severe VC (>50°), which was often concurrently managed with DTITA if intrinsic curvature was present. For SR, tubularization of the graft was performed 6 months later. RESULTS: A total of 140 patients were included. Tubularized incised plate (TIP), DIG, and SR techniques were performed in 57, 23, and 60 patients, respectively. The TIP and DIG techniques achieved similar success rates, although DIG was performed in cases of narrow and spongiosum-deficient plates. Reoperation rates with TIP and DIG techniques was 52.6% and 52.1% (NS). Urethro-cutaneous fistulas were seen in 31.5% and 13% of TIP and DIG techniques, respectively. Staged repair accomplished better results than both TIP and DIG techniques, despite being performed in the most unfavorable cases (reoperation rate 28%). After technical modifications, the DIG technique achieved similar outcomes of SR. CONCLUSIONS: Proximal hypospadias remains challenging, regardless of the technique utilized for its repair. Urethro-cutaneous fistulas were more commonly seen after long TIP repairs. Approximately half of the patients undergoing long TIP and DIG procedures needed re-intervention, although the percentage decreased significantly with late modifications in the DIG group. Recurrence of VC after TIP and DIG techniques seemed to be a significant and under-reported complication. Staged repairs, despite being performed for the most severe cases, resulted in overall better outcomes.

Pre-transplant management of valve bladder: a critical literature review.

OBJECTIVE: Indications, timing and problems related to augmentation cystoplasty (AC), in the context of posterior urethral valves (PUV) and renal transplantation (RT) are ill defined. Associated bladder dysfunction (BD) is not a stable condition and may cause the loss of the renal graft. Polyuria, accentuates BD and seems to improve after RT. The objective of this research is to critically review the available literature, aiming to rationalize the treatment of PUV with BD in the context of end stage renal disease (ESRD). MATERIALS AND METHODS: A thorough literature review was performed. Pertinent papers were, critically analyzed and classified according to the level of evidence. RESULTS: Data relating to PUV, RT and AC showed low levels of evidence. Results of RT in PUV cases with adequate management of BD were comparable to those suffering from other causes of ESRD. Bladder function can recover spontaneously after urinary undiversion. There were no established criteria to indicate AC in the context of ESRD and PUV or to define the ideal protocol to treat associated vesicoureteral reflux (VUR). Urinary tract infections (UTIs) were more frequent in transplanted PUV patients; this is possibly related to the inadequate control of BD, especially after AC. AC is feasible after RT with outcomes comparable to preemptive ones. CONCLUSION: AC increases the risk of UTI after RT. Preemptive AC should be constructed only if the risks associated with increased bladder pressures exceed those associated with AC. Adequate management of BD is essential to improve bladder function and to minimize UTIs. AC is feasible after RT, with complication rates similar to the ones performed beforehand. Since a considerable number of PUV patients with high-pressure bladders eventually develop myogenic failure, it seems logical to postponing AC in this population, as long as they are under close surveillance.

Buccal grafts for urethroplasty in pre-pubertal boys: what happens to the neourethra after puberty?

OBJECTIVE: Buccal mucosa grafts (BMG) are often used in complex urethral reconstruction. Following pubertal endogenous androgen stimulation (EAS) in prepubertal boys, there are concerns that the neourethra may not grow proportionally to the phallus. To address the paucity of literature on the topic, this article reports on data for post-pubertal follow up after pre-pubertal BMG urethroplasties (BMGU). PATIENTS AND METHODS: Retrospective chart review of boys who underwent staged BMGU before the age of 12 years at a single referral center between 2000 and 2010 and who were followed up until after puberty. Demographic information, initial meatal location, quality of graft before tubularization, flow rate parameters (FRP) and complications were captured. RESULTS: Of the 137 patients who underwent staged BMGU during the study period, 10 satisfied the inclusion criteria. Mean patient age at first stage BMGU was eight years (range five to eleven years). The mean follow-up was 40.6 months (9-66 months). The grafts were harvested from the cheek and lower lip in seven and three cases, respectively. The mean interval between the first and second stage was 15.8 months (6-87 months). Complications included one urethro-cutaneous fistula and two cases of glanular dehiscence. The final position of the meatus was glanular in nine boys and coronal in one. Importantly, no recurrent ventral curvature (VC) was found during the second stage BMGU or reported after puberty. All patients demonstrated normal maximum flow after puberty (mean 25.7 ml/s). CONCLUSION: Buccal mucosa grafts appear to grow proportionally to the phallus after pubertal EAS. No recurrent VC or inadequate FRP were observed in this series. Despite the small number of subjects, the results are reassuring and support continued use of BMG in the pediatric pre-pubertal population.

Single-center experience with botulinum toxin endoscopic detrusor injection for the treatment of congenital neuropathic bladder in children: effect of dose adjustment, multiple injections, and avoidance of reconstructive procedures.

OBJECTIVE: Since 2007, intra-detrusor OnabotulinumtoxinA (OnabotA) injections have been selectively offered at our institution for cases in which maximal anticholinergic therapy failed or was not tolerated. Herein we present our experience with this approach. MATERIALS AND METHODS: We prospectively obtained data on 17 patients who underwent OnabotA injections over a 4-year period. Demographic information, number of injections, and dose delivered were captured. Children were monitored with baseline and post-injection renal ultrasound, urodynamics, and assessed for side effects, satisfaction, and symptom improvement. RESULTS: Forty-three sessions were performed with injections given every ∼ 6 months. Mean patient age was 10.7 years (range, 3-17). Compared with baseline, after the first injection, mean bladder capacity adjusted for age and compliance improved by 27% (p = 0.039) and 45.2% (p = 0.041), respectively. After subsequent injections, these values increased to 35.7% (p = 0.043) and 55.1% (p = 0.091), respectively. Out of 13 symptomatic patients, ≥ 50% improvement was reported in ten (76.9%) and complete resolution in seven (53.8%). However, all three patients in whom the maximum dose of OnabotA was reduced from 300 to 200 units complained of recurrent symptoms. Fourteen children avoided surgical reconstruction as a second line of treatment. Overall patient/parental reported satisfaction rate was 70.6% (12/17). CONCLUSIONS: Intra-detrusor OnabotA injection is a promising intervention for management of neuropathic bladder in selected patients. Our data demonstrate improvement in symptoms and urodynamic parameters. Although an optimal dose has not been determined for children, we found optimal response with a maximum administration of OnabotA up to 300 units.

Comparison between laparoscopic and open radical nephrectomy for the treatment of primary renal tumors in children: single-center experience over a 5-year period.

OBJECTIVES: To compare the outcomes of laparoscopic nephrectomy (LN) with open radical nephrectomy (ORN) in the management of consecutive pediatric neoplasms. PATIENTS AND METHODS: Retrospective cohort study of consecutive children treated for primary renal tumors between 2006 and 2011, segregated based on surgical modality (LN/ORN). Pre-, intra- and postoperative data and outcomes were collected. RESULTS: Demographics from the 45 patients (13 LN, 32 ORN) were similar, and tumors in the LN group were smaller [6.59 ± 1.8 cm vs. 10.99 ± 2.99 cm ORN (p < 0.05)]. Six patients had preoperative chemotherapy (two LN, four ORN). No tumor ruptures occurred with either technique. Wilms tumor (seven LN, 24 ORN) was the most common diagnosis, followed by renal cell carcinoma (four LN, four ORN). Procedure length was similar between groups (282 ± 79 LN, 263 ± 81 min ORN). Mean length of stay was significantly shorter for LN (2.9 vs. 5.9 days; p = 0.002). Postoperative narcotic requirements and use of nasogastric tube were higher in the ORN group. After a median follow-up of 18 (LN) and 33 months (ORN), 1 and 4 recurrences occurred, respectively. CONCLUSIONS: LN is an attractive alternative to open surgery in carefully selected cases of pediatric renal tumors. Procedure length and incidence of intra-operative rupture were not increased, while post-operative recovery and hospital stay were shorter for LN. Longer follow-up is mandatory to confirm comparable oncological outcomes to ORN.

Urethral compliance in hypospadias operated by tubularized incised urethral plate (TIP) with and without a dorsal inlay graft: an experimental controlled study.

OBJECTIVE: Abnormal uroflowmetries are common after tubularized incised plate urethroplasties (TIP), perhaps due to low compliance. We hypothesized that (1) abnormal uroflowmetries after TIP might be caused by segmental lower compliance; (2) by adding a graft to the raw area in the incised plate (TIPG), compliance might be improved by preventing secondary intention healing of the dorsal incision. METHODS: A standardized penectomy was performed in 27 adult male rabbits: 9 normal non-operated controls (G1), 6 weeks after TIP (G2: n = 9) or TIPG (G3: n = 9). A standardized isolated segment (including the whole urethroplasty in G1 and G2) was progressively distended with air (1, 2 and 3 ml) in the 3 groups. The respective intraluminal pressures were measured with a tensiometer. RESULTS: Pressure measurements were feasible and reproducible for this model. Mean pressures tended to be higher in the experimental groups (G1: 59.7 mmHg vs. G2: 79.6 mmHg vs. G3: 100.1 mmHg for 1 ml injections; G1: 233.1 mmHg vs. G2: 241 mmHg vs. G3: 308.4 mmHg for 2 ml injections and G1: 457.3 mmHg vs. G2: 429 mmHg vs. G3: 520 mmHg for 3 ml injections) without reaching the statistical significance. CONCLUSION: In this model, the elasticity of the TIP or TIPG neourethras tended to be reduced when compared to controls. The placement of an inlay graft on the dorsal incised area did not increase the compliance. This model allows the measurement of segmental intraluminal urethral pressures generated by controlled air distension and may be a useful tool to evaluate the experimental urethroplasty models.

Congenital megalourethra: prenatal diagnosis and postnatal/autopsy findings in 10 cases.

OBJECTIVE: Congenital megalourethra is a rare urogenital malformation characterized by dilation and elongation of the penile urethra associated with absence or hypoplasia of the corpora spongiosa and cavernosa. Postnatal complications include voiding and erectile dysfunction as well as renal insufficiency and pulmonary hypoplasia. To date, only a few prenatally diagnosed cases have been reported. We report on 10 cases diagnosed prenatally and their postnatal/autopsy findings. METHODS: The study involved retrospective chart review of all cases diagnosed antenatally in three tertiary care centers over 5 years. Antenatal ultrasound images and medical records from obstetrics, genetics, urology and nephrology were reviewed. RESULTS: Ten fetuses with megalourethra were identified at a median gestational age of 19 (range, 13-24) weeks and all were confirmed postnatally or at autopsy. Three pregnancies were terminated and seven continued. All cases presented with a distended bladder and megalourethra and all cases had normal karyotype. Of seven liveborn babies, one died neonatally of pulmonary hypoplasia. All six infants alive at the time of writing had a dysfunctional urethra and three suffered from impaired or end-stage renal disease. Associated anomalies were found in half of the cases. CONCLUSION: Congenital megalourethra is caused by abnormal development or hypoplasia of the penile erectile tissue, secondary to distal urethral obstruction. When the amniotic fluid volume is normal, survival is possible. However, all liveborn infants have voiding and renal dysfunction and sexual dysfunction is expected. Megalourethra should be considered in all male fetuses presenting prenatally with megacystis and detailed fetal ultrasonography should look for an elongated and/or distended phallic structure as well as any associated anomalies.

Congenital adrenal hyperplasia: a critical appraisal of the evolution of feminizing genitoplasty and the controversies surrounding gender reassignment.

Although congenital adrenal hyperplasia (CAH) is the most common cause of genital ambiguity, its surgical treatment remains controversial and often times difficult, especially in cases of high urethro-vaginal confluence. Many aspects are still uncertain, such as the timing of feminizing genitoplasty and type of surgical technique. The objective of this study is to provide a thorough review of the surgical management of children with CAH, discussing the evolution of feminizing genitoplasty and its different techniques, and also to critically appraise the available literature on the timing of surgical intervention and gender reassignment. Prospective long-term studies evaluating the results of modern feminizing genitoplasty techniques are needed as current evidence is based on outdated operations that are no longer used. To date, there have been no studies comparing early and delayed feminizing genitoplasty with regard to psychological outcomes. All families should be counseled regarding the controversies and treatment options, including the watchful waiting approach.

Patency of the "third inguinal ring" in children with unilateral cryptorchidism: fact or fiction?

BACKGROUND: The entrance to the scrotum, or the so-called "third inguinal ring" (3rd IGR), was thought to be an important finding and etiological factor for cryptorchidism at the beginning of the 20th century. Historical reports of its association with undescended testis suggest that it was considered by many to be a true anatomical entity. As the understanding of testicular descent has changed, the description of this anatomical passage has vanished from textbooks and publications. In order to evaluate the findings that led to its original report, we sought to assess the patency of the expected testicular path of descent into the scrotum in children with unilateral cryptorchidism. METHODS: Two hundred consecutive children who underwent unilateral orchidopexy were prospectively evaluated at the time of surgery to determine the anatomical patency of the area thought to represent the 3rd IGR. We also evaluated its association with the patient's age at surgery, the affected side, position of the undescended testis, macroscopic epididymal anomalies (MEA), and the patency of the processus vaginalis (PV). RESULTS: The mean age at surgery was 5.2 +/- 3.0 years, ranging from 1 to 13 years. The 3rd IGR was closed in 118 boys (59.0 %) and open in 82 (41.0 %). A closed 3rd IGR was found significantly more frequently in patients with intra-abdominal testes (p < 0.01). No significant association was observed between patency of the 3rd IGR and other evaluated factors. CONCLUSION: Our results suggest that the so-called 3rd IGR may represent the passage point of the testicle into the scrotum, which was not patent during the intraoperative examination in (2/3) of children with unilateral cryptorchidism. This observation by surgeons at the beginning of the 20th century may represent an anatomical curiosity rather than an important factor in the pathogenesis of cryptorchidism.