De novo variants identified by trio whole exome sequencing of bladder exstrophy epispadias complex.

Bladder exstrophy epispadias complex (BEEC) encompasses a spectrum of conditions ranging from mild epispadias to the most severe form: omphalocele-bladder exstrophy-imperforate anus-spinal defects (OEIS). BEEC involves abnormalities related to anatomical structures that are proposed to have a similar underlying etiology and pathogenesis. In general, BEEC, is considered to arise from a sequence of events in embryonic development and is believed to be a multi-etiological disease with contributions from genetic and environmental factors. Several genes have been implicated and mouse models have been generated, including a knockout model of p63, which is involved in the synthesis of stratified epithelium. Mice lacking p63 have undifferentiated ventral urothelium. MNX1 has also been implicated. In addition, cigarette smoking, diazepam and clomid have been implied as environmental factors due to their relative association. By in large, the etiology and pathogenesis of human BEEC is unknown. We performed de novo analysis of whole exome sequencing (WES) of germline samples from 31 unrelated trios where the probands have a diagnosis of BEEC syndrome. We also evaluated the DECIPHER database to identify copy number variants (CNVs) in genes in individuals with the search terms "bladder exstrophy" in an attempt to identify additional candidate genes within these regions. Several de novo variants were identified; however, a candidate gene is still unclear. This data further supports the multi-etiological nature of BEEC.

Ensuring our exstrophy-epispadias complex patients and families thrive.

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative.

Dextranomer Endoscopic Injections for the Treatment of Urinary Incontinence in Bladder Exstrophy-epispadias Complex.

PURPOSE: Since bladder neck dextranomer/hyaluronic acid copolymer (Deflux) injections can improve urinary incontinence of various etiologies, we hypothesized that incontinent children with bladder exstrophy-epispadias complex would benefit from dextranomer/hyaluronic acid copolymer. We aimed to analyze dextranomer/hyaluronic acid copolymer efficacy and predictors of treatment success in bladder exstrophy-epispadias complex. MATERIALS AND METHODS: Incontinent bladder exstrophy-epispadias complex children aged >4 years undergoing dextranomer/hyaluronic acid copolymer injections in our hospital between October 1997 and January 2021 were included. Medical history, continence, and bladder capacity before injections were recorded. Postoperatively, patients were categorized as "dry," "significantly improved," or "failure." Postoperative complications, bladder emptying mode, and bladder capacity were reported. Failure-free survival was estimated by Kaplan-Meier models. RESULTS: Altogether, 58 patients (27 male epispadias, 9 female epispadias, 22 bladder exstrophy) underwent 105 injections at median age of 8.5 (interquartile range 5.8-12) years. Previous bladder neck reconstruction had been performed in 38 (66%) and 33 (57%) received multiple injections. Complications occurred in 9%. Five-year failure-free survival was 70% (standard error 9.1) in males epispadias compared to 45% (9.0) in females and exstrophy patients (P = .04). Previous bladder neck reconstruction associated with improved 5-year failure-free survival in females and exstrophy patients (58%, SE 11) but not in male epispadias (75%, SE 11). CONCLUSIONS: Dextranomer/hyaluronic acid copolymer injections provided satisfactory and lasting continence in over half of bladder exstrophy-epispadias complex patients. While injections were successful in male epispadias regardless of bladder neck reconstruction timing, results were better in bladder exstrophy and female epispadias after previous bladder neck reconstruction.

Exstrophy-epispadias complex: are the kidneys and kidney function spared?

BACKGROUND: Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients. METHODS: This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017-2020. RESULTS: Forty-three patients (29 males), median age 9 years (interquartile range 6-19), were included. Eleven (26%) used clean intermittent catheterization (CIC) for bladder drainage. At least one sign of kidney injury was identified in 32 (74%) patients; elevated BP, decreased kidney function (estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2), and proteinuria/albuminuria were detected in 29%, 12%, and 36% of patients, respectively. Urinary tract dilatation (UTD) was found in 13 (37%) ultrasound examinations. Parenchymal kidney defects were suspected in 46% and 61% of ultrasound and DMSA scintigraphy, respectively. UTD was significantly associated with DMSA-proven kidney defects (p = 0.043) and with elevated BP, 39% vs. 20% in those without UTD. Decreased eGFR and elevated BP were less frequent among patients on CIC than among patients who voided spontaneously: 10% vs. 14% and 18% vs. 36%, respectively. Recurrent UTIs/bacteriuria and nephro/cystolithiasis were reported by 44% and 29% patients, respectively. CONCLUSION: The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams. A higher resolution version of the Graphical abstract is available as Supplementary information.

Spectrum and classification of megameatus anomalies.

OBJECTIVE: To define and classify megameatus anomalies, the parameters of a considerable number of cases were investigated and compared with those of normal children. METHODS: A total of 1150 normal babies were examined during routine nonmedical circumcision, and another 750 boys referred with hypospadias were examined during the previous 3 years. All patients were evaluated and assessed for the size, location, and configuration of the urinary meatus, and penile length and girth were measured. Children with normal size and location of the meatus were considered control group A, and 42 cases of different forms of megameatus were considered group B. Other penoscrotal, urinary, and general anomalies were examined and investigated accordingly. All data were analyzed by the SPSS 9.0.1 statistical package and compared by paired t tests. RESULTS: Forty-two uncircumcised patients aged from 1 month to 4 years (mean 18 months) were diagnosed with a urinary meatus that engrossed the whole ventral or dorsal aspects of the glans, exceeding half the width of the glans or penile girth with the complete vanishing of the glans closure in most cases. Megameatus is usually associated with the abnormal meatal position as hypospadiac, orthotopic, or epispadic. Additionally, megameatus may be associated with a normally intact or deficient prepuce. Consequently, we had four categories of megameatus, and the intact prepuce orthotopic megameatus subcategory has not been described before. Megameatus was also detected with deficient prepuce, and this was considered a hypospadiac variant. CONCLUSION: Megameatus is diagnosed precisely with penile biometry and is classified into 4 groups: hypospadiac, epispadic, and orthotopic or central, either with or without intact prepuce. This classification is applicable for expansion to other centers.

Introduction of a modified single stage reconstruction technique of male penopubic epispadias.

OBJECTIVE: To represent the long-term outcomes of our modified single-stage technique for the reconstruction of isolated penopubic epispadias in male patients. PATIENTS AND METHODS: Data from 113 patients were obtained from bladder-exstrophy-epispadias database of our tertiary center. A total of seven boys with isolated penopubic epispadias with no prior history of surgery and any other anomaly underwent our modified surgical approach from February 1997 to September 2019. The mean ± SD age at surgery was 6.5 ± 2.4 years. Volitional voiding status and cosmetic appearance were evaluated at each follow-up interval. Postoperative follow-up was performed at quarterly intervals in the first year and once a year in subsequent years. RESULTS: The mean ± SD of follow-up was 8.5 ± 6 years. All boys who were incontinent achieved urinary control and the ability of normal transurethral micturition following the surgery. Four boys became completely dry, and the other three attained social dryness. Postoperative mean (SD) bladder capacity was significantly increased from 54.5 (11) to 124 (40.0) within 6 months, and to 194 (47.5) at 18 months after surgery. Dorsal curvature has been resolved in all cases, and no postoperative complications were noted except for surgical site infection in one patient treated with antibiotics and bilateral vesicourethral reflux resolved after injection of bulking agents. Four patients had normal erectile function and ejaculation, while the others have not reached puberty yet. Moreover, none of the patients developed urethrocutaneous fistula, stricture, or penile ischemia. CONCLUSION: The present findings suggest the safety and effectiveness of the combination of single-stage urethro-genitoplasty, bladder neck plication, and fat pad pedicled flap in management of boys with isolated penopubic epispadias that can lead to the achievement of urinary control, acceptable sexual function, and cosmetically satisfactory genitalia. Minimal morbidity, low complication rate, and promising outcomes are essential factors, supporting the notion of introducing this technique as a valid option for management of this entity.

Urothelial Differences in the Exstrophy-Epispadias Complex: Potential Implications for Management.

PURPOSE: The authors examined the urothelium of exstrophy-epispadias complex spectrum patients for histological differences and expression of terminal markers of urothelial differentiation. MATERIALS AND METHODS: Between 2012 and 2017 bladder biopsies were obtained from 69 pediatric exstrophy-epispadias complex patients. These specimens were compared to bladder specimens from normal controls. All bladder specimens underwent histological assessment followed by immunohistochemical staining for uroplakin-II and p63. Expression levels of uroplakin-II and p63 were then assessed by a blinded pathologist. RESULTS: Forty-three classic bladder exstrophy biopsies were obtained (10 newborn closures, 22 delayed closures, and 11 repeat closures). Additional biopsies from 18 cloacal exstrophy patients and 8 epispadias patients were also evaluated. These specimens were compared to 8 normal control bladder specimens. Overall, uroplakin-II expression was lower in exstrophy-epispadias complex patients compared to controls (p <0.0001). Among classic bladder exstrophy patients, there was reduced expression of uroplakin-II in the delayed and repeat closures in comparison to newborn closures (p=0.045). Expression of p63 was lower in patients with exstrophy-epispadias complex compared to controls (p <0.0001). Expression of p63 was similar among classic bladder exstrophy patients closed as newborns when compared to delayed or repeat closures. Classic bladder exstrophy patients had a higher rate of squamous metaplasia when compared to controls (p=0.044). Additionally, there was a higher rate of squamous metaplasia in the patients undergoing delayed closure in comparison to those closed in the newborn period (p <0.001). CONCLUSIONS: The urothelium in the exstrophy-epispadias complex bladder is strikingly different than that of healthy controls. Uroplakin-II expression is greatly reduced in exstrophy-epispadias complex bladders and is influenced by the timing of bladder closure. Reduced uroplakin-II expression and increased rates of squamous metaplasia in exstrophy-epispadias complex patients undergoing delayed closure suggests that exposure of the urothelium may induce these changes. These findings shed light on the molecular changes in exstrophy-epispadias complex bladders and may have implications on the appropriate timing of primary bladder closure, as those closed in the newborn period appear to have a greater potential for growth and differentiation.

Pedicled Anterolateral Thigh and Radial Forearm Free Flap Phalloplasty for Penile Reconstruction in Patients With Bladder Exstrophy.

PURPOSE: Male patients with exstrophy-epispadias complex have a congenitally short penis. Adolescent and adult patients often desire penile reconstruction to enable penetrative intercourse. When penile lengthening using local tissue and/or skin graft is unsuitable, phalloplasty is performed. We provide updated experiences using radial forearm free flap and pedicled anterolateral thigh flap phalloplasty techniques over a 6-year period in exstrophy-epispadias complex patients. MATERIALS AND METHODS: Consecutive patients who underwent radial forearm free flap or pedicled anterolateral thigh flap phalloplasty between 2013 and 2019 were reviewed. A total of 11 patients were identified, including 8 with classic bladder exstrophy and 3 with cloacal exstrophy. In each case, flap dimensions, donor and recipient vessels and nerves, complications, outcomes and subsequent surgical procedures are reported. RESULTS: A radial forearm free flap was performed in 6 patients and a pedicled anterolateral thigh flap in 5. Flap survival was observed in all cases and 5 patients subsequently received an inflatable penile prosthesis. Among patients undergoing a pedicled anterolateral thigh flap, there was 1 mortality from acute pulmonary embolism, and the other 4 required at least 1 debulking procedure. CONCLUSIONS: Phalloplasty is an important procedure in patients with exstrophy-epispadias complex with severe penile inadequacy. Both techniques have favorable long-term outcomes, although the pedicled anterolateral thigh flap frequently requires a later debulking procedure. The radial forearm free flap is preferred but if the radial forearm is overly thin and the risk of inflatable penile prosthesis extrusion is high, the pedicled anterolateral thigh flap is an acceptable alternative.

Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications and Outcomes.

BACKGROUND: Males born with bladder exstrophy-epispadias complex generally have a shorter phallus, split corpora with dorsal curvature, and a scarred and flattened glans, so substitution phalloplasty is often required. AIM: The aim of this study was to review the techniques, complications, and outcomes of substitution phalloplasty in bladder exstrophy-epispadias complex patients to determine the ideal surgical procedure and gauge the risks and benefits for the patient. METHODS: A systematic review of the literature was performed using PubMed/MEDLINE and the Cochrane Library with the following terms: ("phalloplasty"); (("epispadias") OR ("bladder exstrophy") OR ("cloacal exstrophy")). We included only full-text articles reporting data about techniques and outcomes of substitution phalloplasty in patients with bladder exstrophy-epispadias complex. OUTCOMES: To determine whether patients with bladder exstrophy-epispadias complex might benefit from substitution phalloplasty. RESULTS: We selected 7 studies involving 47 patients. All the studies were characterized by a low level of evidence and a heterogeneous approach during treatment and outcome assessment. The free radial forearm flap was the most commonly performed technique (89%) with an overall complication rate of 15%. Urethroplasty was performed in 22 of 47 (47%) patients, and in most cases (20/22) a "tube-within-the-tube" technique was performed simultaneously with the phalloplasty (20/47). Urethroplasty complications were recorded in 12 of 22 (54%) patients with 6 fistulae and 6 stenoses. A penile prosthesis was implanted in 32 of 47 (68%) patients and complications occurred in 8 of 32 (25%) patients with 6 erosion. Aesthetic, sexual, and psychological outcomes were satisfactory, but none of the studies used validated instruments for the final assessment. CLINICAL IMPLICATIONS: It was not possible to formulate any recommendations based on a high level of evidence regarding substitution phalloplasty in patients with bladder exstrophy-epispadias complex. STRENGTH & LIMITATION: To our knowledge, this is the first review to address bladder exstrophy-epispadias complex patients only. The limitations are mainly represented by the small number of cases because of the rarity of this disease and by the fact that no studies used validated instruments. CONCLUSION: Substitution phalloplasty in patients with bladder exstrophy-epispadias complex can achieve good functional, aesthetic, psychological, and sexual outcomes. It requires multiple procedures and carries a high complication rate. Multicentric studies including the assessment of patients by means of a validated questionnaire which investigates both sexual function and psychosexual satisfaction are required. Berrettini A, Sampogna G, Gnech M, et al. Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications, and Outcomes. J Sex Med 2021;18:400-409.

Fertility and sexuality issues in congenital lifelong urology patients: male aspects.

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.

Fertility and sexuality issues in congenital lifelong urology patients: female aspects.

PURPOSE: With advances in treatment modalities and medical knowledge, girls with congenital urologic disorders are living well into adulthood. Although, sexual and reproductive function in this population is still poorly understood. The aim is to review existing literature about fertility and sexuality in women with congenital genitourinary disorders, including spina bifida (SB), bladder exstrophy-epispadias complex (BEEC) and congenital adrenal hyperplasia (CAH). METHODS: This review represents the joint SIU-ICUD (Société Internationale d'Urologie-International Consultation on Urological Disease) consultation on congenital lifelong urology. The results of this analysis were first presented at a joint consultation of the SIU and ICUD at the 2018 SIU annual conference in Seoul, South Korea. Appropriate experts were asked to write specific sections regarding sexuality and reproductive function in female patients with these complex congenital urogenital disorders. Each expert performed their own literature review which was reviewed by GDW, AFS, Hadley M. Wood and Dan Wood. Expert opinion was obtained where data are non-existent. RESULTS: Only about half of the individuals with SB express a satisfactory sex life. In women with BEEC, cosmetic concerns surrounding genital appearance and function may increase psychological distress, including severe depression, suicide and sexual dysfunction. Professional health care is key for improving self-esteem and to interact in the biopsychosocial model of the quality of life. Patients with SB and BEEC should be informed about all the potential risks and difficulties before, during and after pregnancy. Screening for pelvic organ prolapse is important as it can exacerbate their already existing sexual dysfunction, difficulties achieving pregnancy and challenges with clean intermittent catheterization. CONCLUSIONS: Lifelong multidisciplinary follow-up and management are complex but necessary. As these patients grow into their adolescence, they may have the desire to become involved in personal relationships and have sexual interactions. Their healthcare team needs to be increasingly sensitive to these aspects.

Long-term outcome of urethral and genital reconstruction in hypospadias and exstrophy-epispadias complex.

PURPOSE OF REVIEW: Although immediate surgical outcomes of genitourinary reconstruction used to be the main focus in hypospadias and exstrophy-epispadias complex (EEC), recent research demonstrates rising concerns about long-term functional and psychosexual outcomes. Recent results about long-term outcomes of complex genitourinary reconstruction in those children transitioning into adulthood are summarized and discussed in this comprehensive review. RECENT FINDINGS: Long-term outcomes in hypospadias focus on psychosexual satisfaction and decisional regret. Interestingly, uncomplicated hypospadias repair results in equal satisfaction rates to those found in controls. Most adult patients are happy with their parents' decision to have them undergo surgery during childhood. No decisional regret was found in parents. Reinterventions were associated with a decline in satisfaction, as was a decrease in perceived penile length. Long-term, males with EEC are more dissatisfied with penile length than with continence problems. In cases of severe penile insufficiency, phalloplasty shows promising psychosexual outcomes at the price of a high complication rate. Female EEC patients show higher rates of pregnancy complications, portending a higher risk of miscarriage. SUMMARY: Psychosexual satisfaction of patients with repaired hypospadias transitioning into adulthood demonstrates equal satisfaction rates to those found in controls, without decisional regret associated with surgery during childhood. In EEC, more complications are seen when transitioning into a sexually active adult life.

[Complete penile rehabilitation of a patient with microphallia after correction of bladder exstrophy].

Bladder exstrophy is a complex of urogenital malformations, varying in severity from epispadias to classical bladder exstrophy. The main aim of correcting bladder exstrophy in childhood is to achieve urine continence with the prevention of upper urinary tract disorders and to provide penile reconstruction. However, by the time of puberty, it is not always possible to achieve optimal anatomical features of the penis, and usually, by the end of puberty, the penile length is no more than 7 cm, which subsequently leads to psychosocial and sexual problems during puberty. Neither patient, nor urologist in most cases do not satisfy final result of the treatment of exstrophy and a number of epispadias forms. A clinical case of the surgical rehabilitation of a patient after undergoing multi-stage surgical treatment for a congenital malformation of the genitourinary system is presented in the article. MATERIALS AND METHODS: At the admission, the size of the penis was 3 cm (microfallia), urethral meatus was located in the area of the penoscrotal junction. From 2016 to 2019, the patient underwent neofallos formation, urethroplasty, and implantation of prostheses in the neophallos. RESULTS: According to the results, urinary and sexual function was completely restored, as well as anatomical relationships and dimensional parameters of the genitals.

Health-related quality of life among children, adolescents, and adults with bladder exstrophy-epispadias complex: a systematic review of the literature and recommendations for future research.

PURPOSE: Bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of genitourinary malformations. Children risk long-term urinary and genital dysfunctions. To achieve a comprehensive understanding, this study aimed to review the literature on generic and disease-specific health-related quality of life (HRQOL) in BEEC patients, and methodologies used. METHODS: A literature search was conducted in Pubmed/CINAHL/Embase/PsycINFO/Cochrane, from inception to May 2018. A meta-analysis of HRQOL in BEEC patients compared to healthy references was performed. RESULTS: Twenty-one articles (published 1994-2018), describing HRQOL of children and adolescents (n = 5) and adults only (n = 5), or integrated age populations (n = 11), were identified (median sample size 24, loss to follow-up 43%, response rate 84%). Overall HRQOL was reduced in BEEC patients compared to healthy references in 4/4 studies. Impaired physical or general health in BEEC patients has been described in 9 articles, diminished mental health in 11, restricted social health in 10, and sexual health/functioning or body perception impairments in 13 articles. Urinary incontinence was the most common factor related to worse HRQOL (12 studies). In six studies, HRQOL was better than healthy norms. In eligible studies (n = 5), the pooled estimate of the effect of BEEC indicated worse HRQOL for children and adults (0 > effect sizes < 0.5). Thirty-six HRQOL assessments were used, none developed and validated for BEEC. CONCLUSIONS: HRQOL in BEEC patients may be negatively impacted, particularly considering mental and social HRQOL. Sexual health/functioning or body perception impairments may be present in adolescents and adults. However, HRQOL is heterogeneously assessed and subsequent findings are differently reported. Additional research is warranted and can be improved.

Anatomy of Classic Bladder Exstrophy: MRI Findings and Surgical Correlation.

PURPOSE OF REVIEW: The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation. RECENT FINDINGS: Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors' experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy. Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve.

Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex.

BACKGROUND: The bladder exstrophy-epispadias complex is a rare congenital malformation associated with severe dysfunction of the genital and urinary tracts and requiring a staged surgical reconstruction. AIM: The primary aims of this study were to report the sexuality, infertility, and urinary incontinence outcomes in a cohort of men born with bladder exstrophy-epispadias complex. The secondary aim was to highlight some predictive factors of infertility in this population. METHODS: We conducted a descriptive, cross-sectional study of men diagnosed with classic presentations of bladder exstrophy or epispadias. OUTCOMES: Patients were asked to complete 4 validated questionnaires: the International Index of Erectile Function (IIEF)-5, the Erection Hardness Score (EHS), the Self-Esteem and Relationship, and the International Consultation Incontinence modular Questionnaire-Short Form. Fertility potential was assessed with semen analysis and a non-validated questionnaire. RESULTS: 38 Patients 18-64 years old (M [mean] = 32.2) completed the questionnaires. The average IIEF-5 score was 18.1/25 (ranging from 3-25; SE = 7.62), with results indicating that 55% of the sample had normal erectile function. Results also showed higher scores for patients with normal spermatozoa concentration (M = 22.75, SE = 1.89, P = .08) than for those with oligospermia (M = 17.30, SE = 8.53, P = .08). Results on the IIEF-5 also indicated higher scores for patients who conceived children without assisted reproductive technologies (ART) (M = 22.83, SE = 2.317, P = .02) than for patients without children (M = 15.76, SE = 8.342, P = .02). The average EHS was 3.43/4 (ranging from 1-4, SE = 0.9). EHS was higher for patients who had reconstruction than for patients who had cystectomy (M = 3.88, SE = 1.07 and 2.78, SE = 1.09, P = .02). The average total Self-Esteem and Relationship score was 67.04/100 (ranging from 10.71-96.43, SE = 22.11). The average total International Consultation Incontinence modular Questionnaire-Short Form score was 4.97/21 (ranging from 0-18, SE = 5.44), higher score indicating more urinary incontinence. Among the patients surveyed, 31.6% were parents at the time of study and 50% of them benefited from ART. With regards to the 14 semen analyses performed, only 7.1% produced normal results and 44.7% indicated that ejaculation was weak and dribbling. CLINICAL TRANSLATION: Erectile function appears to be decreased and psychological aspects of sexuality indicate low self-esteem about sexual relationship. Although ethical problems could not allow prospective spermograms, our cohort is large enough to provide significant data. CONCLUSIONS: Early sperm storage for future ART, sexual medicine management, and complementary genital reconstruction in adulthood constitute potential treatment options for this population. Reynaud N, Courtois F, Mouriquand P, et al. Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex. J Sex Med 2017;15:314-323.

Lessons learned from the management of adults who have undergone augmentation for spina bifida and bladder exstrophy: Incidence and management of the non-lethal complications of bladder augmentation.

The present review provides clinical insights and makes recommendations regarding patient management garnered by the long-term follow up of patients undergoing enteric bladder augmentation for the management of congenital anomalies. A prospectively maintained database on 385 patients that have experienced an enteric bladder augmentation, using either the ileum or colon, was reviewed. Evaluations included methods used to prevent bladder calculi formation and recurrence, the incidence and etiology of renal calculi development, the incidence and treatment of vitamin B12 deficiency, and the complications and need for surgical revisions for continent catheterizable stomas. A significantly increased risk for continent catheterizable stomal complications occurred after Monti-Yang tube formation, 70% (21/30 patients), compared with appendicovesicostomy, 41% (27/66 patients), P = 0.008. Both procedures had significantly more complications than continent catheterizable stomas using tapered ileum with a reinforced ileal-cecal valve, 21% (13/63 patients), P < 0.0001 and P < 0.013, respectively. Approximately 50% of the patient population developed a body mass index ≥30 during adulthood. The onset of obesity resulted in significantly more complications developing in patients with a Monti-Yang tube (87%; 13/15 patients) or appendicovesicostomy (55%; 18/33 patients) compared with a tapered ileum with a reinforced ileal-cecal valve (27%, 8/30 patients), P < 0.00015 and P < 0.025, respectively, with a median follow-up interval of 16 years, range 10-25 years. Long-term follow-up evaluations on patients undergoing an enteric bladder augmentation are necessary to prevent the long-term sequela of this procedure. The key to improving patient prognosis is the nutritional management of the patient as they mature, especially if a continent abdominal stoma is going to be carried out.

Preoperative care of Polypoid exposed mucosal template in bladder exstrophy: the role of high-barrier plastic wraps in reducing inflammation and polyp size.

OBJECTIVE: To assess the role of high-barrier plastic wrap in reducing the number and size of polyps, as well as decreasing the inflammation and allergic reactions in exstrophy cases, and to compare the results with the application of low-barrier wrap. MATERIALS AND METHODS: Eight patients with bladder exstrophy-epispadias complex (BEEC) that had used a low density polyethylene (LDPE) wrap for coverage of the exposed polypoid bladder in preoperative care management were referred. The main complaint of their parents was increase in size and number of polyps. After a period of 2 months using the same wrap and observing the increasing pattern in size of polyps, these patients were recommended to use a high-barrier wrap which is made of polyvinylidene chloride (PVdC), until closure. Patients were monitored for the number and size of polyps before and after the change of barriers. The incidence of para-exstrophy skin infection/inflammation and skin allergy were assessed. Biopsies were taken from the polyps to identify histopathological characteristics of the exposed polyps. RESULTS: The high barrier wrap was applied for a mean ± SD duration of 12±2.1 months. Polyps' size and number decreased after 12 months. No allergic reaction was detected in patients after the usage of PVdC; three patients suffered from low-grade skin allergy when LDPE was applied. Also, pre-malignant changes were observed in none of the patients in histopathological examination after the application of PVdC. CONCLUSION: Polyps' size and number and skin allergy may significantly decrease with the use of a high-barrier wrap. Certain PVdC wraps with more integrity and less evaporative permeability may be more "exstrophy-friendly".

[Total epispadias of duplicated urethra in boys].

The article reports on two cases of the total epispadias of duplicated urethra with and without penile duplication. The authors describe the clinical presentation, diagnosis, and treatment. Duplicated urethra with epispadias both with and without penile duplication must be removed. At the same time, it is necessary to restore the patency of the lower duplicated urethra.