Outcome analysis of staged preputial graft technique for primary proximal hypospadias with and without post-operative vacuum physiotherapy.

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.

Functional recoverability post-pyeloplasty in children with ureteropelvic junction obstruction and poorly functioning kidneys: Systematic review.

BACKGROUND: The management of poorly functioning kidneys (PFK) associated with ureteropelvic junction obstruction (UPJO) is controversial. There is contradictory information about how to best manage these cases: pyeloplasty or nephrectomy? OBJECTIVE: To systematically summarize the available evidence concerning the effects of pyeloplasty on the differential renal function of PFK in children with unilateral UPJO, highlighting the ongoing challenges in their definition, management, and long-term follow-up. In addition, we aim to verify potential predictors of renal functional recoverability that could help clinicians choose candidates for pyeloplasty. METHODS: We searched several databases including PubMed, Embase, and Cochrane Library CENTRAL until August 20, 2021, according to the PRISMA guidelines. The following concepts were searched: pediatric, ureteropelvic junction obstruction, UPJO, pyeloplasty, recovery, split renal function, and differential renal function. We enrolled studies where the PFK was defined as preoperative differential renal function (DRF) ≤30% by renal scintigraphy. Potential predictors of renal functional recoverability were assessed and compared among studies. The quality of the included studies was evaluated using a modified version of the Newcastle-Ottawa scale (NOS). RESULTS: 1499 citations perceived as relevant to screening were retrieved. After screening, 20 studies were included, comprising a total of 625 cases. The number of patients in each study varied between 5 and 84, while the average post-surgical follow-up duration ranged between 3 months and 180 months. The most significant preoperative predictive factor for postoperative functional recoverability was the baseline DRF, especially when antenatally diagnosed. The quality was considered average in a significant portion of included studies. CONCLUSION: A significant proportion of PFK showed an increase of DRF post-pyeloplasty. However, no consistent predictive factors for functional recoverability have yet been determined apart from preoperative DRF. Until further evidence appears, pyeloplasty should be considered a valid option in the armamentarium of UPJO management in PFK.

Orchio-Septopexy: A new technique to cover and fix detorsed testis undergoing fasciotomy of tunica albuginea

ABSTRACT Purpose Compartment Syndrome (CS) has been recognized as a potential factor that worsens testicular viability after detorsion, especially in borderline cases of prolonged ischemia. Fasciotomy of the testicular tunica albuginea to relieve the pressure associated with CS has been proposed to accommodate edema after detorsion, embracing the raw fasciotomy area with tunica vaginalis flap (TVF) or graft. Fashioning the TVF can be tedious in cases of severe scrotal edema. Herein we present a technique that facilitates and expedites the procedure, maintaining the fasciotomy area decompressed. Materials and Methods In testicular torsion, where the testis remains with dark coloration and questionable viability after detorsion a longitudinal releasing incision is made in the tunica albuginea (fasciotomy) to decrease compartmental pressure. If signs of parenchymal recovery (bleeding points, better color) are seen an orchio-septopexy is performed, suturing the incised albuginea's edges to the septum with a running suture, avoiding CS as well as re-torsion. Results Orchio-septopexy was performed in 11 cases with a mean age of 11.9 years (3-17). All cases had clinic follow-up and testicular Doppler US with a mean of 9.5 months (6-24). 6/11 cases (54%) were salvaged, with good vascularity in the Doppler US and maintained more than 50% testicular volume compared to the contralateral side. Conclusion Orchio-septopexy after testicular fasciotomy is a simple and fast technique that can be utilized in cases of prolonged testicular ischemia and questionable viability. More than half of the testes recovered, encouraging us to propose its utilization as well as its validation by other surgeons.

Orchio-Septopexy: A new technique to cover and fix detorsed testis undergoing fasciotomy of tunica albuginea.

PURPOSE: Compartment Syndrome (CS) has been recognized as a potential factor that worsens testicular viability after detorsion, especially in borderline cases of prolonged ischemia. Fasciotomy of the testicular tunica albuginea to relieve the pressure associated with CS has been proposed to accommodate edema after detorsion, embracing the raw fasciotomy area with tunica vaginalis flap (TVF) or graft. Fashioning the TVF can be tedious in cases of severe scrotal edema. Herein we present a technique that facilitates and expedites the procedure, maintaining the fasciotomy area decompressed. MATERIALS AND METHODS: In testicular torsion, where the testis remains with dark coloration and questionable viability after detorsion a longitudinal releasing incision is made in the tunica albuginea (fasciotomy) to decrease compartmental pressure. If signs of parenchymal recovery (bleeding points, better color) are seen an orchio-septopexy is performed, suturing the incised albuginea's edges to the septum with a running suture, avoiding CS as well as re-torsion. RESULTS: Orchio-septopexy was performed in 11 cases with a mean age of 11.9 years (3-17). All cases had clinic follow-up and testicular Doppler US with a mean of 9.5 months (6-24). 6/11 cases (54%) were salvaged, with good vascularity in the Doppler US and maintained more than 50% testicular volume compared to the contralateral side. CONCLUSION: Orchio-septopexy after testicular fasciotomy is a simple and fast technique that can be utilized in cases of prolonged testicular ischemia and questionable viability. More than half of the testes recovered, encouraging us to propose its utilization as well as its validation by other surgeons.

Hydronephrosis Classifications: Has UTD Overtaken APD and SFU? A Worldwide Survey.

Objective: To collect baseline information on the ultrasonographic reporting preferences. Method: A 13-multiple choice questionnaire was designed and distributed worldwide among pediatric urologists, pediatric surgeons, and urologists. The statistical analysis of the survey data consisted of 3 steps: a univariate analysis, a bivariate and a multivariate analysis. Results: Three hundred eighty participants responded from all the continents. The bivariate analysis showed the significant differences in the geographical area, the years of experience and the volume of cases. Most of the physicians prefer the SFU and APD systems because of familiarity and simplicity (37 and 34%, respectively). Respondents noted that their imaging providers most often report findings utilizing the mild-moderate-severe system or the APD measurements (28 and 39%, respectively) except for North America (SFU in 50%). Multivariate analysis did not provide significant differences. Conclusion: Our study evaluates the opinions regarding the various pediatric hydronephrosis classification systems from a large number of specialists and demonstrates that there is no single preferred grading system. The greatest reported shortcoming of all the systems was the lack of universal utilization. The observations taken from this study may serve as basis for the construction of a common worldwide system. As APD and SFU are the preferred systems and the UTD a newer combination of both, it is possible that with time, UTD may become the universal language for reporting hydronephrosis. This time, based on the result of this survey, seems not arrived yet.

Segmental Testicular Infarction Associated to Torsion: First Case Report in Childhood.

Testicular torsion in children is not uncommon emergency problem which occur due to twist in the spermatic cord leading to ischemia or infarction to testicle. Hemorrhagic infarction can occur following testicular torsion is globally; however, in extremely rare situation, such infarction can be segmental. Segmental testicular infarction (STI) was reported in an infant due to epididymitis and a newborn with STI in 1 testicle with complete infarction in the contralateral testicle due to birth trauma. To best of our knowledge, our case of STI in a child associated with testicular torsion is the first described in the literature.

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.

Upper lip graft (ULG) for redo urethroplasties in children. A step by step video.

PURPOSE: Lower lip and cheek are commonly used sources of buccal mucosa grafts for urethroplasty. In recent years, aiming to improve the donor site morbidity, our preference changed to the use (ULG). The aim of this video is to illustrate the technical details of the ULG harvesting for children. MATERIAL AND METHODS: The inner surface of the upper lip is exposed by two stay sutures. The frenulum is spared, the mucosa to be harvested is marked and local submucosal infiltration is done with a solution of bupivacaine plus epinephrine. The edges are incised ant the submucosa plane created with a scissor. The graft is detached, defatted, and applied with quilting stitches over the recipient site with the standard technique. Hemostasis is secured and the donor site is left open. RESULTS: From 2015 to 2018, 25 ULG harvests were done in 24 patients. Only one (5%) presented local pain associated to the procedure in the first 24 h. After minimum 2 months after surgery, none of the patients presented perioral nubmness, difficulty with mouth opening, contraction of the donor site or changes in salivation. CONCLUSIONS: ULG harvest is easy and a suitable alternative source of oral mucosa for urethroplasty in children.

The learning curve for robotic-assisted pyeloplasty in children: Our initial experience from a single center.

BACKGROUND: Robotic-assisted pyeloplasty surgery has become the preferred approach of ureteropelvic junction obstruction (UPJO) in pediatrics. However, to our knowledge, there is limited data on the learning curve for robotic-assisted pyeloplasty in children and no similar study from Saudi Arabia. AIMS: The objective of the study was to evaluate the progression of the surgical team performing robotic-assisted laparoscopic pyeloplasty (RALP) and to assess the feasibility of the RALP in children, since it is having been recently started in the Kingdom. SETTINGS AND DESIGN: Retrospective charts and surgical videos review at the tertiary care centre. SUBJECTS AND METHODS: After approval from the internal review board (IRB), we reviewed the surgical video recording of the RALP procedure of 15 patients presented with UPJO from January 2016 to October 2017. Statistical analysis was done for the variables includes dissection time, pyelotomy, anastomosis on both sides, and total surgery time and calculated in minutes. Renal ultrasound reviewed to assess any change in grade. RESULTS: Fifteen patients with UPJO underwent RALP. Of 15 cases, nine were primary and six cases as secondary UPJO. The median age was 8 (3-15) years. Out of 15 cases, 13 and 2 patients diagnosed as Society for Fetal Urology grades of 4 and 3, respectively. Total operative time was prolonged in secondary group as compared to primary pyeloplasty group (mean [standard deviation (SD)]: 166.3 [35.1], range: 125-223, P = 0.0028 versus mean (SD): 149.17 (30.4), range: (114-207), P = 0.0008). The success rate was 100% in primary and 84% in secondary cases. The median length of follow-up was 12.0 (7.0-18.0) and 10.0 (8.0-12.5) months in primary and secondary cases, respectively. The overall complication rate was 13% (2/15) (Clavien grade: 1-2). CONCLUSIONS: The evaluation of the learning curve of RALP for this group of patients concluded that total operative time for RALP, performed by the pediatric urology team, steadily decreased with collective surgical experience.

Outcome of salvage ureteral reimplantation after endoscopic treatment failure for high-grade vesicoureteral reflux compared to primary ureteral reimplantation.

INTRODUCTION: Surgical treatment of vesicoureteral reflux is required after conservative treatment has failed. However, there is a controversy if fibrosis related to previous attempts of dextranomer/hyaluronic acid (Dx/Ha) injection increases the risk of surgical difficulty and postoperative complications. Therefore, the purpose of our study was to compare the outcome of salvage ureteral reimplantation (SUR), after failed endoscopic therapy, to that of primary ureteral reimplantation in patients with high-grade primary vesicoureteral reflux (VUR). MATERIALS AND METHODS: We conducted a retrospective analysis of children, <14 years old, treated for Grade IV or V VUR, between 1998 and 2014. Cases were classified into the SUR or the PUR group. Cases of secondary VUR were excluded. All patients were treated using a cross-trigonal ureteral reimplantation technique by two surgeons. The following demographic and clinical variables were included in the analysis: presentation, reflux severity, scarring on imaging, age at endoscopic injection, total amount of Dx/Ha injected, operative time, postoperative hospital stay, operative complications, incidence of febrile urinary tract infections (UTIs) after surgery, and persistent VUR. Between the groups, differences were evaluated using Fisher's exact test. RESULTS: Twenty-six patients were included, 19 in the SUR and 7 in the primary ureteral reimplantation (PUR) group. In the SUR group, 12 cases had a bilateral VUR and 7 had a unilateral VUR, with 4 bilateral and 3 unilateral VUR cases in the PUR group. In the SUR group, 13 patients had received one Dx/Ha injections, with the other 6 receiving two injections, of 0.5 ml of Dx/Ha (range, 0.5-2.0 ml). A bilateral reimplantation was performed in 14/19 patients in the SUR group and 4/7 in the PUR group. The median age at surgery was 4 years in the SUR group and 3 years in the PUR group (P < 0.02). The median operative time was comparable between the groups (120 and 140 min for the SUR and PUR groups, respectively, P = 0.73), with a comparable length of hospital stay (5 and 6 days, respectively, P = 0.061). Blood loss was generally <10 ml, except in three cases in the SUR group, due to difficult dissection. Over the median follow-up of 1 year, persistent Grade III SUR was identified in only one patient in the SUR group, with no occurrence of febrile UTIs postoperatively. CONCLUSION: SUR for high-grade primary VUR after failed Dx/Ha injection has the same success rate as PUR, with no significant complication rate, although the necessary dissection may be more difficult.

Telehealth for the Assessment of Patients Referred for Pediatric Urological Care: A Preliminary Cost Savings Analysis and Satisfaction Survey.

Background: Almost one-fifth of the population of Saudi Arabia lives in rural areas and may still lack access to specialty healthcare. Because of the growing demand for telehealth services, we initiated the virtual clinic (VC) concept for new referrals to the pediatric urology clinic (PUC), the primary tertiary care unit in Riyadh. Methods: We conducted a retrospective analysis of costs and timing involved with the VC practice for a 1-year period. The cost analysis is based on savings realized by patients as a result of the VC evaluation. A 15-question nonvalidated satisfaction survey was also conducted by an independent observer. Results: Of 105 patients assessed through the VC program, 44 were accepted for surgery and further investigation. The number of trips to the center saved by the virtual encounter were 203, resulting in a savings of 740,950 Saudi Arabian Riyals (SAR) minus the cost of the VC. The cost of conducting most of the investigations at the referring hospital instead of the PUC represented a savings of 639,970 SAR. Thus, the overall cost savings to patient and family and to the PUC was 1,311,570 SAR. An additional benefit was the reduction in the time for treatment from 6.6 to 3.9 months. The overall satisfaction rate was 89%. Conclusions: Although preliminary, our study indicates that telemedicine can achieve a cost savings without compromising the safety or adversely affecting patient management. Further studies should more clearly define the benefits and any limitations, and reveal how the technology could be used most effectively.

Timing of orchidopexy at a tertiary center in Saudi Arabia: reasons for late surgery.

BACKGROUND: Orchidopexy should be performed during the first 18 months of life to decrease the risk of infertility and tumor formation. In our center, the timing of surgical correction varies depending on the availability of an operating room. OBJECTIVES: Evaluate whether orchidopexy performed for patients referred to our center is done within the recommended time period and to determine causes for delay. DESIGN: Retrospective descriptive study. SETTING: Pediatric urology department of a tertiary care center. SUBJECTS AND METHODS: We retrospectively reviewed the charts of patients charts who underwent orchidopexy at our center from 2000 to 2010. We assessed referral time and waiting list time, which were subdivided as follows: from referral to first visit and from first visit to surgery. We included patients younger than 14 years and excluded pa.tients with comorbidities that affected the timing of referral and surgi.cal treatment. MAIN OUTCOME MEASURES: Referral time period and waiting list time for surgical correction of patients presented with undescended testis. SAMPLE SIZE: 128 RESULTS: After exclusion of 32 patients because of comorbidities, we describe 128 who underwent surgery for cryptorchidism at our center. The median (interquartile range, minimum-maximum) for age at sur.gery was 46.7 months (24.4-83.4, 3.1-248.6]). The median (IQR) referral occurred at an age of 25.3 months (4.1-65.5). The median (IQR) waiting list time was 15.2 months (8.1-23.3). The median (IQR) waiting time from referral to the first visit was 4.1 months (1.0-8.2). The median wait.ing time from the first visit to surgery was 8.1 months (3.8-17.5). CONCLUSIONS: The age at the time of surgery at our center was far from ideal because of late referrals. A structured program offered by our National Health Service to educate referring physicians is necessary. Community health initiatives must emphasize prompt referral to reduce the impact of delayed surgery. LIMITATIONS: Lack of data on the type of referring physician (i.e., general practitioner, pediatrician, surgeon, urologist). CONFLICT OF INTEREST: None.

Robotic versus open pyeloplasty in pediatric patients: a single center experience in Saudi Arabia.

BACKGROUND: Robotic surgery in pediatric patients is performed in our center since 2013. This study aims to analyze the evolution of robot-assisted laparoscopic pyeloplasty (RALP) in our center to investigate its feasibility and safety compared with open pyeloplasty (OP) technique. METHODS: In this retrospective study, patients aged 2 to 14 years who underwent pyeloplasty procedure for ureteropelvic junction obstruction were divided into two groups according to the type of surgical approach (RALP and OP). The median age, weight, duration of the procedure, length of hospital stays, complication according to the Clavien grading system and success rates were recorded. We determined any trends in RALP activity, the length of hospital stay, and the patients' weight. We also compared the results of two equally divided periods (January 2015 to March 2016 and April 2016 to June 2017). RESULTS: Forty-one pyeloplasty were performed in this study. RALP and OP were performed in 26 (57.8%) and 15 (33.3%) cases, respectively. RALP was more utilized than OP in the second period (19 vs. 7). Comparing the two periods, a reduction in the median weight in the RALP group was observed (P=0.039); in the OP group, the weight remained almost unchanged (15 vs. 18 kg). The median duration of OP was shorter than that of RALP (P<0.04). The length of hospital stays reduced in the RALP group (P=0.013). CONCLUSIONS: RALP is safe and feasible and has a promising potential in pediatric urology. The rapid and favorable evolution in the studied indicators shows encouraging results as the indications for RALP are expanded and the length of hospital stay shortened.

Long-term Outcomes of Augmentation Cystoplasty in a Pediatric Population With Refractory Bladder Dysfunction: A 12-Year Follow-up Experience at Single Center.

PURPOSE: Augmentation cystoplasty (AC) is a surgical procedure used in adults and children with refractory bladder dysfunction, including a small bladder capacity and inadequate bladder compliance, and in whom conservative and medical treatment has failed. This study was aimed to determine the long-term outcomes of AC in children. METHODS: A retrospective analysis was conducted of 42 patients (31 males; mean age, 14.2±6.2 years) who underwent AC for neurogenic and nonneurogenic bladder dysfunction, with a median 12.0±1.5 years of follow-up. All patients underwent AC using the ileum with or without continent reconstruction. Pre-AC, concurrent, and post-AC procedures and complications were analyzed. Patients who underwent ureterocystoplasty, were lost to follow-up, or had less than 10 years of follow-up were excluded. The primary outcomes were the complication and continence rates, the post-AC linear rate of height and weight gain, and renal function. The Student t-test was used to evaluate between-group differences and the paired t-test was used to evaluate longitudinal changes in measured variables. RESULTS: Renal function was stable or improved in 32 of 42 patients (76.2%), with a post-AC continence rate of 88.1%. Thirty patients (71.4%) required 72 procedures post-AC. There was no statistically significant difference in the mean percentile of height (P=0. 212) or weight (P=0.142) of patients in the pre- and post-AC periods. No cases of bladder perforation or malignancy were detected. CONCLUSION: We consider AC to be a safe and effective procedure that does not negatively affect future physical growth, while achieving a good rate of stable renal function. Patients need long-term follow-up to address long-term complications.

Environmental, parental and gestational factors that influence the occurrence of hypospadias in male patients.

OBJECTIVE: Hypospadias is a congenital defect, which affects normal development of the male urogenital external tract. In this malformation, the urethral orifice of the penis is positioned ventrally, thus interfering with normal urination and creating, in some adults, problems during sexual intercourse. Heritability of hypospadias has been shown in some reports, and the abnormality has been associated with the presence of mutations in one of the genes involved in urogenital development. However, even for patients who were born in families with a higher incidence rate of this defect, no evident genetic alteration could be identified in known genes, indicating that the list of loci involved is still incomplete. To further complicate matters, recent reports also underline that epigenetic changes, without any identifiable gene sequence mutation, may be involved in gene function impairment. Therefore, the inheritance of most hypospadias cases is not evident, suggesting that the genetic background is not the only cause of this malformation; indeed, the majority of hypospadias cases are classified as sporadic and idiopathic. MATERIALS AND METHODS: Evidence has accumulated highlighting the role of the environment and of its relationships with the genome in the etiology of this abnormality. In particular, the interaction between some chemicals, which are able to mimic endogenous molecules such as sexual hormones--for this reason called endocrine disrupting compounds (EDC)--and specific receptors has been extensively investigated during the pregnancy. Additionally, several articles have shown that parental and gestational factors play a significant role too. Indeed, physiological alterations, such as body weight of the mother and/or of the newborn, mother's diabetes, impaired father fertility, and exposure of one parent to job-related pollutants, show in many cases a direct correlation with hypospadias incidence. The overall prevalence of this condition has been studied in many countries, suggesting that at least in some periods and/or in specific populations there are detectable fluctuations, probably mirroring the different natural environments. However, many articles present data that do not agree with these findings and, consequently, most causes of hypospadias are still highly debated. RESULTS: In this review, we summarize the developmental steps involved in urogenital tract formation, with a particular emphasis on the genes that most frequently are associated with this condition, or that are subject to environmental stress, or that may be the targets of hormone-like, exogenous molecules. Then, we make an overview of the identified factors able to impair the function of important genes, even in the absence of their mutations, including those for which contradictory reports have been published. Finally, we propose an explanation of sporadic cases of hypospadias that reconciles these contradictions and suggest some steps for moving forward in the research focused on this condition. CONCLUSION: We hypothesize that most patients develop hypospadias because of gene-environment interactions acting on polymorphic genes that, in the absence of environmental stimuli, would otherwise cause no developmental anomaly during urogenital development.

Failed hypospadias in paediatric patients.

Failed hypospadias refers to any hypospadias repair that leads to complications or causes patient dissatisfaction. The complication rate after hypospadias repairs ranges from 5-70%, but the actual incidence of failed hypospadias is unknown as complications can become apparent many years after surgery and series with lifelong follow-up data do not exist. Moreover, little is known about uncomplicated repairs that fail in terms of patient satisfaction. Risk factors for complications include factors related to the hypospadias (severity of the condition and characteristics of the urethral plate), the patient (age at surgery, endocrine environment, and wound healing impairment), the surgeon (technique selection and surgeon expertise), and the procedure (technical details and postoperative management). The most important factors for preventing complications are surgeon expertise (number of cases treated per year), interposition of a barrier layer between the urethroplasty and the skin, and postoperative urinary drainage. Major complications associated with failed hypospadias include residual curvature, healing complications (preputial dehiscence, glans dehiscence, fistula formation, and urethral breakdown), urethral obstruction (meatal stenosis, urethral stricture, and functional obstruction), urethral diverticula, hairy urethra, and penile skin deficiency.

Maternal exposures to endocrine disrupting chemicals and hypospadias in offspring.

BACKGROUND: Prenatal exposures to endocrine-disrupting chemicals (EDCs) are suspected risk factors in the etiology of hypospadias. The aim of this case-control study was to test the hypothesis of an association between maternal environmental exposures to EDCs and hypospadias in the offspring. METHODS: Detailed questionnaire data on occupational and dietary exposures to EDCs in the perinatal period were collected from 80 mothers with hypospadiac infants and from 80 mothers with healthy controls within 24 months of childbirth. Maternal exposure to selected EDCs was also ascertained by measuring the concentration of dichlorodiphenyldichloroethylene, hexachlorobenzene, and several polychlorinated biphenyl congeners in the serum of primiparous mothers of 37 cases and 21 controls. RESULTS: The risk to bear an hypospadiac infant was associated with perinatal maternal occupational exposures to EDCs evaluated by a job-exposure matrix: jobs with exposure to one class of EDCs (odds ratios [OR](crude), 2.83; 95% confidence intervals [CI], 1.32-6.07; OR(adjusted), 2.44; 95% CI, 1.06-5.61) and jobs with exposure to more than one group of EDCs (OR(crude), 4.27; 95% CI, 1.43-12.78; OR(adjusted), 4.11; 95%CI, 1.34-12.59). Increase in risk was also found among mothers consuming a diet rich in fish or shellfish (OR(crude), 3.41; 95% CI, 1.42-8.23; OR(adjusted), 2.73; 95%CI, 1.09-6.82). Serum hexachlorobenzene concentration above the median of all subjects was significantly associated with the risk of hypospadias (OR(adjusted), 5.50; 95% CI, 1.24-24.31). CONCLUSIONS: This study, although based on a limited number of cases, for the first time provides evidence of an association between maternal exposure to EDCs, in particular elevated plasma hexachlorobenzene concentration, and the development of hypospadias in the offspring.