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.

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.

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.

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.

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.

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.

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.

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.

Gender Identity and Sex Role of Patients Operated on for Bladder Exstrophy-Epispadias.

PURPOSE: We evaluated whether genital deformity has an impact on gender identity and sex role in patients operated on for bladder exstrophy-epispadias complex. MATERIALS AND METHODS: A total of 62 adolescents and adults operated on for bladder exstrophy-epispadias complex were mailed questionnaires evaluating gender identity (Gender Identity/Gender Dysphoria Questionnaire for Adolescents and Adults) and sex role (Bem Sex Role Inventory). Of the patients 33 responded and the results were compared with 99 gender matched controls. RESULTS: On the gender identity questionnaire female patients had median scores similar to those of their gender matched controls (4.93 vs 4.89, p = 0.412) but in males the score was lower compared to controls (4.87 vs 4.96, p = 0.023), indicating somewhat more conflicted gender identity. However, no patient had gender dysphoria. Female sex role index was higher in female patients vs controls (5.9 vs 5.3, p = 0.003) but was comparable between male patients and controls (5.2 vs 5.0, p = 0.459). Masculine sex role indices were comparable between female patients and controls as well as between male patients and controls. Of 32 patients 17 were considered to have androgynous sex role, as were 24 of 97 controls (p = 0.004). The exact diagnosis (bladder exstrophy or epispadias) or dissatisfaction with appearance of the genitals had no impact on gender identity or on sex role indices. CONCLUSIONS: Male patients had lower gender identity scores compared to controls and female sex role was enhanced among female patients. Androgynous sex role was more common in patients vs controls. Gender dysphoria was not noted in any patient.

Managing adult urinary incontinence from the congenitally incompetent bladder outlet.

PURPOSE OF REVIEW: Treatment of urinary incontinence in adult patients with congenital incompetent bladder outlet (exstrophy-epispadias complex, cloacal anomalies, or neurogenic bladder secondary to myelomeningocele) is a challenging surgical problem. In this review article, we summarize and highlight recent studies in the management of incontinence in this patient population. RECENT FINDINGS: The literature regarding management of urinary incontinence in this patient population is scarce. Injection of bulking agents to the bladder neck, artificial/autologous slings, artificial urinary sphincters, bladder neck reconstruction, bladder neck closure, or a combination of these are the cornerstone of management. Augmentation cystoplasty is a major adjunct procedure that can help increase continence rate and success of surgery in select patients. The level of evidence on bladder neck procedures for this patient population is low because of significant limitations, including small sample, heterogeneity of primary diagnosis/surgical techniques, variable definitions of continence, and the retrospective nature of most studies in this field. SUMMARY: Standard options for treatment of urinary incontinence in the congenitally incompetent bladder outlet procedure remain unchanged. There is no single reproducible procedure to accomplish the goal of renal preservation and continence in these patients, and often patients require multiple procedures to achieve continence. Most importantly, the pediatric and adult urologist should continue to work toward achieving a well tolerated and efficient transition of care. There is a need to standardize data acquisition and reporting of outcomes. Although randomized control studies would be ideal, because of the small number of patients with these conditions, this may not be practical. Collaboration and continued discussion among experts in the field is needed to gain a better understanding of the optimal management strategy in this growing patient population.

A wet dressing for male genital surgery: A phase II clinical trial.

PURPOSE: This study was to confirm the safety and efficacy of BC dressing when used in surgical male wound healing at the urogenital area. METHODS: Open, non-controlled clinical study of phase II. A total of 141 patients, among those children, adolescents and adults with hypospadias (112), epispadias (04), phymosis (13) and Peyronie's disease (12) that had a BC dressing applied over the operated area after surgery. A written informed consent was obtained from all participants. Study exclusion criteria were patients with other alternative treatment indications due to the severity, extent of the injury or the underlying disease. The outcomes evaluated were efficacy, safe and complete healing. The costs were discussed. RESULTS: In 68% patients, the BC dressing fell off spontaneously. The BC was removed without complications in 13% of patients at the outpatient clinic during the follow-up visit and 17% not reported the time of removal. In 3% of the cases, the dressing fell off early. Complete healing was observed between 8th and 10th days after surgery. The BC dressings have shown a good tolerance by all the patients and there were no reports of serious adverse events. CONCLUSION: The bacterial cellulose dressings have shown efficacy, safety and that can be considered as a satisfactory alternative for postoperative wound healing in urogenital area and with low cost.

Sexual Health Outcomes in Adults with Complete Male Epispadias.

PURPOSE: Complete male epispadias is a rare congenital anomaly characterized by failed closure of the entire penopubic dorsal urethra. Epispadias repair is typically performed during infancy, and resultant genitourinary abnormalities can have a marked impact on adult life. We assess long-term post-reconstruction sexual health and fertility outcomes in adults with complete male epispadias. MATERIALS AND METHODS: A total of 132 patients 18 years or older with complete male epispadias who had undergone reconstruction were identified from a prospectively maintained, institutionally approved database. Patients who could be contacted were asked to complete a telephone survey regarding sexual function. Reconstructive history and clinical details were obtained by chart/database review. RESULTS: Of 132 patients with complete male epispadias 74 met inclusion criteria and 15 (20%) completed the questionnaire. Seven patients (47%) reported currently being in a relationship. Although 12 patients (80%) reported overall satisfactory sexual intercourse, 11 (73%) admitted to 1 or more problems with sexual function, including abnormal ejaculation (53%), diminished sensation (20%) and difficulty maintaining an erection (20%). When questioned regarding the importance of fertility on a scale of 0 to 5 using a Likert-type item the response of 10 patients (67%) was 4 points or greater. Five patients (33%) reported having impregnated a sexual partner. Although 4 patients (27%) had suspicion of fertility problems, only 2 (13%) reported having abnormal semen analyses. CONCLUSIONS: This is one of few studies examining post-reconstruction sexual health and function in adults with complete male epispadias. Although small, our study demonstrates that patients are able to engage in relationships, participate in sexual intercourse and impregnate their partners. These results highlight sexual concerns and outcomes that may be of use when counselling patients with complete male epispadias and their families.

Congenital penile pathology is associated with abnormal development of the dartos muscle: a prospective study of primary penile surgery at a tertiary referral center.

PURPOSE: Pathophysiological mechanisms leading to chordee in patients with hypospadias and to the hidden state of buried penis in the prepubic fat remain unclear. Resection of dartos tissue usually makes the penis straight in patients with hypospadias and corrects it in those with buried penis, suggesting a common pathophysiology related to dartos tissue. MATERIALS AND METHODS: Tissue samples from 113 children undergoing primary penile surgery for hypospadias (94 patients), epispadias (1) or buried penis (18) were collected between November 2011 and September 2013. Tissue samples from 79 children undergoing circumcision for nonmedical reasons served as controls. All samples were stained with smooth muscle actin and analyzed by the same pathologist, who was blinded to indication for surgery. Chi-square and Fisher exact tests were applied. RESULTS: Three different dartos tissue patterns were observed. Pattern I (normal) consisted of smooth muscle fibers of dartos tissue organized in a parallel configuration in the subcutaneous tissue. Pattern II was characterized by poorly developed and hypotrophic smooth muscle fibers. Pattern III was determined by randomly distributed smooth muscle fibers in the subcutaneous tissue, without parallel configuration. Pattern I was observed in 45 circumcision specimens (64%). Of buried penis cases 78% were considered abnormal (pattern II in 4 cases and III in 10, p = 0.001). Of hypospadias cases 70% were considered abnormal (pattern II in 31 cases, III in 32, and mixed II and III in 3, p < 0.001). The only epispadias case was designated pattern II. CONCLUSIONS: Congenital penile pathology (hypospadias, buried penis) is associated with structural anomalies in dartos tissue. Further research is needed to unveil the pathophysiology of the condition.

Sexual Function in Patients Operated on for Bladder Exstrophy and Epispadias.

PURPOSE: Bladder exstrophy and epispadias complex is a rare congenital malformation that may have detrimental effects on sexual function. We evaluated sexual function of patients with bladder exstrophy and epispadias complex using validated questionnaires and compared the results with age matched controls. MATERIALS AND METHODS: Patients with bladder exstrophy and epispadias complex treated between 1956 and 1992 were identified from our hospital operative database. A total of 63 patients were mailed questionnaires up to 3 times, resulting in 32 replies (51%). Men were mailed the International Index of Erectile Function-15 questionnaire and women were mailed the Female Sexual Function Index questionnaire, and all patients were asked auxiliary questions regarding children, satisfaction with external genitalia and urinary continence. RESULTS: There were no differences in sexual function between sexually active men with bladder exstrophy and epispadias complex and age matched controls on different erectile function domain scores according to the International Index of Erectile Function-15 questionnaire. The Female Sexual Function Index questionnaire among women yielded comparable results on different domain scores, except for median total score, which was higher in patients (33.6, IQR 29.4 to 34.9) than in controls (30.1, IQR 26.4 to 32.4, p = 0.049), suggesting better sexual function in patients with bladder exstrophy and epispadias complex. A greater proportion of patients with bladder exstrophy and epispadias complex had not become sexually active, compared to controls (35% vs 11%, p = 0.008). Patients with bladder exstrophy and epispadias complex were less likely to have children than controls (22% vs 45%, p = 0.021). CONCLUSIONS: Men and women with bladder exstrophy and epispadias complex had good long-term outcomes on erectile and general sexual function tests. However, there is a likelihood that these individuals start their sexual life later than the general population, and fewer have children compared to controls.

Skin cover in epispadias repair by dorsal transposition of a ventral island flap: a modification of the Pippi Salle technique.

PURPOSE: To describe a technique for a reliable and cosmetic skin cover for epispadias repair. This is achieved using a ventral flap of prepuce and the penile skin. This is a modification of the technique described by Pippi Salle, who wrapped the penile shaft with the same flap, from one side with a resultant suture line on to the contralateral side of the penis. METHODS: Thirty-five children (18 with pure epispadias and 17 with exstrophy epispadias complex) who underwent epispadias repair by the described technique in our hospital were reviewed. In our technique, a ventral flap of prepuce and penile skin, as described by Pippi Salle, is developed and a button hole is made in the dartos fascia. This button hole is utilized to deliver the shaft ventrally which effectively brings the flap onto the dorsal aspect of the penis. The skin flap is then wrapped in toto around to provide a complete cover on the penile shaft with suture line on the ventral aspect. RESULTS: All the 35 children had satisfactory skin cover with a scarless dorsum of penis. Two children had immediate post-operative ventral suture line breakdown, one requiring resuturing and the other healed spontaneously. CONCLUSION: The 'button hole' technique with dorsal transposition of the prepuce and skin flap effectively prevents the dorsal scar contracture by completely avoiding a longitudinal suture line dorsally. On the other hand, the healed ventral suture line is continuous with the scrotal midline raphe, giving a natural look. The overall result is a sturdy repair with a cosmetic appearance of the penis.