Penopubic Epispadias Research Articles

Isolated Male Epispadias with Continence in a 40 Year Old: A Case Report

Epispadias is part of the bladder exstrophy-epispadias complex; it has a defective dorsal wall of the urethra with potential incompetence of the urinary continence mechanism. Isolated male epispadias is due to failure of the urethral plate to tubularize on the dorsum of the penis. It is rare with an incidence rate of 1 in 117,000 live births in males. The severity depends on the position of the urethral opening and ranges from peno-pubic to penile and glandular. Epispadias is usually repaired within the first year of life though some patients have presented in their second decade. Epispadias is classically associated with bladder exstrophy in over 90% of the cases while isolated epispadias with continence is very rare constituting less than 10% of cases. Separation of pelvic bones is seen in 70% of peno-pubic epispadias affecting the bladder neck and external sphincter leading to incontinence and stress urinary dribbling. The goals of repair include; achieving a cosmetically acceptable and functional penis, which is straight and adequate in length, enabling penetrative sexual intercourse and urinary continence. There have been reports of isolated epispadias in adults but none was as old as 40 years. This patient has been married for 20 years and has been unable to impregnate his spouse. He had modified Cantwell-Ransley procedure after a detailed clinical and psychological evaluation to achieve the goals of repair. Adults presenting with isolated continent peno-pubic epispadias are rare. Presenting at this age and after having been married for two decades could have a derogatory effect on body image, self-confidence, psycho-sexual and reproductive life. Surgical correction of this anomaly as we did has the potential to lead to acceptable outcomes as demonstrated in this index case.

Bladder Neck Surgery is not Routinely Needed to Achieve Urinary Continence in Patients with Primary Epispadias

BackgroundThis study aims at investigating the continence outcome in primary epispadias patients treated at a tertiary center. The authors hypothesized that additional continence procedures following primary epispadias repair is not routinely needed. MethodsPatients treated for primary epispadias at the authors’ institution between 2007 and 2019 and toilet trained, were identified from a prospective maintained database. Males underwent chordee correction, urethroplasty and glanuloplasty. Females underwent genitoplasty with reduction urethroplasty. If continence was not achieved by 4–5 years of age, pelvic floor muscle (PFM) biofeedback therapy was performed. Other continent procedures were discussed with family/patient if still incontinent. Primary outcome: urinary continence. Secondary outcomes: PFM biofeedback therapy, continence surgery, hydronephrosis. Type of epispadias, age at repair and follow-up presented as median was also reported. ResultsThirty-three patients (29 males) were included. Twelve had penopubic epispadias, 13 glanular/penile, 4 duplicated urethra, 4 females. Median age at repair: 2 years (IQR 1–3), at follow-up: 8 years (IQR 6–10). Daytime continence: 100 % in penile/glanular; 33 % in penopubic and 75 % in duplicated urethra. Nighttime continence: respectively 92 %, 50 % and 100 %. 24 % of males were intermittently incontinent. All patients except one voided urethrally. One patient underwent bladder neck closure, ileocystoplasty and Mitrofanoff. One girl achieved daytime continence, 2 were intermittently incontinent, one continuously incontinent. All were enuretic. 38 % of boys and 100 % of girls had biofeedback therapy. None had hydronephrosis/renal impairment. ConclusionsMost children with primary epispadias can achieve social urinary continence spontaneously or with the support of PFM biofeedback therapy. Other continence procedures should be reserved for patients who do not attain satisfactory continence. Level of EvidenceTreatment study – level IV.

PD06-04 THE SUB SYMPHYSEAL APPROACH TO THE BLADDER NECK FOR CHILDREN BORN WITH INCONTINENT EPISPADIAS

You have accessJournal of UrologyCME1 Apr 2023PD06-04 THE SUB SYMPHYSEAL APPROACH TO THE BLADDER NECK FOR CHILDREN BORN WITH INCONTINENT EPISPADIAS Moneer Hanna, Mohammed El Menshawy, and Ashraf Fahmy Moneer HannaMoneer Hanna More articles by this author , Mohammed El MenshawyMohammed El Menshawy More articles by this author , and Ashraf FahmyAshraf Fahmy More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003230.04AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: To review the outcome of a less invasive surgical approach for plication/ reconstruction of the bladder neck to treat urinary incontinence which may be combined with partial penile disassembly in boys born with epispadias. METHODS: Between 2010 and 2022 a total of 9 children, 6 boys born with penopubic epispadias and 3 girls with incontinent epispadias, all had no prior surgery were operated via the sub symphyseal approach. Their age varied between 3 and 11 years. In boys the urethral plate was first dissected and lifted from the corporal bodies down to the glans penis distally and the wide bladder neck proximally i.e partial penile disassembly. In girls the skin wedge between the crura of the clitoral bodies was raised and tabularized to be used for retraction and further proximal dissection behind the pubic symphysis. The urethra was tubularized over 10 French catheter 10 French catheter, and the bladder neck was plicated over the catheter in 6 children using non absorbable sutures. In 3 children (one boy and 2 girls) the bladder neck was too wide, and a triangular wedge of tissue was excised followed by 2-layer closure with long lasting absorbable sutures around the catheter. The bladder was filled with saline and the water tightness of the suture line and/or the competence of the plicated bladder neck were tested. The corporal bodies are rotated and sutured together to correct the dorsal chordee and the penis is resurfaced by skin. In girls the crural bodies are approximated followed by monsplasty. RESULTS: The follow up ranged between 6 months and 5 years. All boys and 2 girls who were incontinent achieved dryness for 3+ hours and achieved volitional voiding over 6 months postoperatively. One girl exhibited stress incontinence which improved considerably following periurethral injection of bulking agent. The bladder capacity increased from 50- 120 ml preoperatively to 150-230 ml. post-surgery with gradual increase of the bladder capacity over the years. 6/18 renal units showed vesicoureteral reflux (Grade II-III) prior to surgery and were all corrected successfully by endoscopic bulking agent injections. CONCLUSIONS: The sub symphyseal exposure to achieve continence by plicating and/or reconstructing the bladder neck provides a safe and effective surgical approach for children born with incontinent epispadias. Furthermore, in boys it can be combined with penile disassembly. Source of Funding: none © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e156 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Moneer Hanna More articles by this author Mohammed El Menshawy More articles by this author Ashraf Fahmy More articles by this author Expand All Advertisement PDF downloadLoading ...

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

ObjectiveTo represent the long-term outcomes of our modified single-stage technique for the reconstruction of isolated penopubic epispadias in male patients.Patients and methodsData 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.ResultsThe 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.ConclusionThe 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.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium

Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3h, 9 (13%) patients have dry intervals of 1-3h and 44 (61%) patients had dry intervals <1h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p=0.3). In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Pelvic Floor Anatomical Variations in Children With Exstrophy-Epispadias Complex Using Magnetic Resonance Imaging

ObjectiveTo quantitatively measure the anatomical variations of the pelvic floor in children with exstrophy-epispadias complex using magnetic resonance imaging. Materials and MethodsSix cases of classic bladder exstrophy (CBE), 5 cases of penile epispadias (PE) and 11 cases of penopubic epispadias (PPE) were included. Another 8 cases with the testicular tumor were taken as the controls. A series of measurements obtained from the pelvic floor magnetic resonance imaging were analyzed, and the measurements with significant differences were obtained by ANOVA. ResultsThe pelvic floor of the CBE was significantly different from that of controls in measurements including wider pubic diastasis (P <.001), greater posterior anal distance (P = .019), greater posterior bladder neck distance (P = .004), larger iliac wing angle (P <.001), diminutive ischial angle (P <.001), bigger puborectalis angle (P <.001), larger ileococcygeous angle (P = .002) and shortened anterior corporal length (P <.001). For the PE, the posterior bladder neck distance (P = .038) was greater than that of controls. In the PPE, the posterior bladder neck distance (P = .001) and puborectalis angle (P = .026) was greater than that of controls, respectively. ConclusionCBE shows severe anatomical variations of the pelvic floor. The bladder neck moves more anteriorly both in PE and PPE than the control. The enlarged puborectalis angle resulting from wider pubic diastasis and more anterior position of the anorectal canal is also noticed in PPE.

V03-09 ANATOMICAL REPAIR OF EPISPADIAS

INTRODUCTION AND OBJECTIVE:Most boys with penopubic epispadias have varying degree of incontinence. We describe the technique of anatomical repair of epispadias through this short video presentatio...

Long-term outcomes in primary male epispadias

The authors present a case series of 30 male patients who presented with primary epispadias between 1989 and 2002 and looked at their long-term outcomes. (1) What procedures and operations did these patients require following their original surgery? (2) What were their outcomes as adults in terms of continence, cosmesis, and sexual function? Primary male epispadias patients who had gone through transition into Adolescent and Adult Urology services were identified retrospectively from electronic patient records. The authors identified 30 male patients with a median follow-up of 18.5 years. Twenty-four had penopubic epispadias, and six had penile epispadias. All initial surgery took place between 9 and 48 months. Twenty-eight patients needed further surgery over the follow-up period, 26 had surgery for continence, and 12 required revision surgery. At follow-up, 15 patients were continent voiding per urethra, nine patients reported stress leakage with volitional urethral voiding, six patients were using a Mitrofanoff to void, and four of these had an ileocystoplasty. Nineteen patients had documentation on their feelings towardcosmetic outcome; 17 expressed concern. Twenty-one patients had documentation about sexual function; 20 had normal erections with six reporting chordee and nine reporting retrograde ejaculation. No patients were recorded to have fathered any children. There are only a few published studies looking at long-term outcomes of genitourinary reconstruction in primary male epispadias and their sexual function in adulthood. The majority of this patient cohort required surgery to improve their continence and had more than one continence procedure. There is limited data on continence outcomes in the literature with small cohorts and rates varying between 40 and 100% at 10-year follow-up. Despite corrective surgery, nearly all the patients were concerned about their genital appearance. Other studies have shown similar outcomes in terms of patient satisfaction and sexual function. The limitations of this work are that the information was gathered retrospectively from the electronic patient record and validated instruments on outcomes were not used. The study outcomes will be useful for clinicians who are counseling patients and parents regarding what to expect as adults.

Use of small intestinal submucosa for corporal body grafting in cases of epispadias and epispadias/exstrophy complex

Male epispadias is a rare congenital urogenital anomaly in which the meatus is ectopically located along the dorsal midline of the penile shaft. In cases associated with severe curvature, functional and cosmetic outcomes could be accomplished by lengthening the shorter dorsal surface with the use of corporal grafting. Various graft materials have been used in the past for hypospadias repair including tunica vaginalis, dermis, and small intestinal submucosa (SIS). The use of SIS grafting for corporoplasty during epispadias repair has rarely been described in the literature. To report the experience in the management of dorsal corporal body grafting using SIS in children with severe penile curvature due to epispadias. The authors retrospectively reviewed the charts of all patients with epispadias or bladder exstrophy/epispadias complex and severe dorsal chordee (>40°) who underwent epispadias repair with single-layer SIS for corporal body grafting. Clinical variables, surgical technique, and outcomes were analyzed. A total of nine consecutive patients underwent staged epispadias repair with dorsal corporal single-layer SIS grafting (summary figure). Of these, four (44.4%) had primary penopubic epispadias, one (11.1%) had mid-shaft epispadias, and four (44.4%) had bladder exstrophy/epispadias complex. The mean age at surgery was 13.4±6 months. After phalloplasty with SIS grafting, there were no reported complications related to the graft during the post-operative period or follow-up visits. Although traditional techniques for epispadias repair allow some degree of corporal lengthening, they also result in abrupt medial rotation of the corporal bodies leading to torqueing and potential unsatisfactory cosmetic results. In contrast, the authors use single-layer SIS for corporal body grafting, and this study technique results in a more gradual inward rotation thus allowing more anatomical accuracy. Furthermore, an advantage of the use of SIS over other grafting materials is that there is no need to harvest an autologous graft such as tunica vaginalis or dermis. Epispadias repair using single-layer SIS corporal body grafting is an effective, safe, and feasible method, which provides satisfactory cosmesis and correction of dorsal curvature in congenital epispadias in children. Furthermore, a more normal penis appearance, without a decrease in the corporal length or diameter, is achieved with this technique.

Urethral duplication in male epispadias: a very uncommon association.

Urethral duplication associated with epispadias is a rare malformation. Few cases are described in Literature. We report the experience of two centers to add to the literature. A retrospective study was conducted in two Italian Centers. All patients with urethral duplication associated with epispadias, treated from 1997 to 2017 were included. The preoperative work-up included renal-urinary ultrasonography and voiding cystourethrogram. All patients underwent surgery according to the Mitchell-Caione technique. Cosmetic result, urinary continence and satisfaction degree of patients at the last follow-up were evaluated as outcomes. Six male patients with urethral duplication in epispadias were included. Two patients presented penile epispadias and four penopubic epispadias. Only one patient had urinary incontinence as presenting symptomatology. The diagnosis of urethral duplication was accidental during preoperative evaluation in the remaining five patients. At last follow-up (mean 8.3 years) all patients but one presented good cosmetic result, one patient presented mild stress urinary incontinence, one presented nocturnal enuresis. The physical genital appearance was improved in all patients. Urethral duplication in association with epispadias is a rare urogenital abnormality. No classification is universally accepted. Based on our experience, we believe that the presence of any duplication should be carefully searched during surgery for male epispadias.

Complete penile disassembly in epispadias repair.

To report current results of complete penile disassembly technique in epispadias repair. In ten years, we have preformed 31 complete penile disassembly for proximal epispadias repair. Twenty-four patients had epispadias after primary repair of bladder exstrophy and 7 isolated penopubic epispadias. The age of the patients ranged from 10months to 6years (median 3years). The shortening of urethral plate was found in 30 patients (97% of cases), and this shortening varied between 6 and 16mm. However, in one patient we found a lengthening of the urethral plate of 8mm. The narrowing of urethral plate was found in all patients, and this narrowing varied between 30 and 50% of the width of the plate. Postoperative complications encountered in our patients were dominated by fistulas and dehiscence, particularly in patients who had bladder exstrophy-epispadias complex. After dehiscence and fistulas repair, the cosmetic results were satisfying in 25 patients (80.5% of cases) with conical glans and meatus in orthotopic position without any necrosis of the glans. However, the urinary continence ≥ 1h was observed in 6 patients (19% of cases) and only 3 patients (9.7% of cases) had a urinary continence ≥ 3h. The mean follow-up was 61 months. The complete penile disassembly remains one of the best techniques for epispadias repair. However, we noticed a reappearance of the dorsal curvature of the penis in a large number of patients treated for isolated epispadias and the impact of this technique on urinary incontinence remains uncertain.

Is there a shortening of the urethral plate in complete penile disassembly used in epispadias repair, and what is its impact on the final outcomes?

PurposeTo assess the importance of shortening of the urethral plate that occurred with complete penile disassembly technique in epispadias repair and its impact on cosmetic and functional results (on urinary incontinence). MethodsFrom January 2009 to December 2016, 26 boys underwent complete penile disassembly technique for proximal epispadias repair. Twenty-one patients had epispadias after primary repair of bladder exstrophy, and 5 patients had isolated penopubic epispadias. The age of the patients ranged from 11 months to 6 years (median 3 years). ResultsAfter disassembling the penis in three parts, the shortening and narrowing of urethral plate were found in all patients; the shortening varied between 2 and 16 mm. However, in isolated epispadias, the urethral plate is easily extensible. The cosmetic results (after dehiscence and fistulas repair) were found to be satisfactory in 24 patients with conical glans and meatus in the orthotopic position without any necrosis of the glans. However, 18 patients (81.8% of cases) who initially had a bladder exstrophy presented a dehiscence or fistula. The urinary continence ≥ 1 h was observed in 5 patients (19% of cases), and only 3 patients (11.5% of cases) had a urinary continence ≥ 3 h. ConclusionsThe complete penile disassembly procedure restores the normal anatomy of the penis. Despite the shortening and narrowing of the urethral plate, the cosmetic results were good in the majority of patients. However, its functional outcomes on urinary incontinence, particularly for epispadias with bladder exstrophy, remain uncertain.

The Role of Human Acellular Dermis in Preventing Fistulas After Bladder Neck Transection in the Exstrophy-epispadias Complex

To evaluate human acellular dermis (HAD) as an adjunct during bladder neck transection (BNT) by comparing surgical outcomes with other types of tissue interposition. A prospectively maintained institutional database of exstrophy-epispadias complex (EEC) patients was reviewed for those who underwent a BNT with at least 6 months follow-up. The primary outcome was the occurrence of BNT-related fistulas. In total, 147 EEC patients underwent a BNT with a mean follow-up time of 6.9 years (range 0.52-23.35 years). There were 124 (84.4%) classic exstrophy patients, 22 (15.0%) cloacal exstrophy patients, and 1 (0.7%) penopubic epispadias patient. A total of 12 (8.2%) BNTs resulted in fistulization, including 4 vesicoperineal fistulas, 7 vesicourethral fistulas, and 1 vesicovaginal fistula. There were 5 (22.7%) fistulas in the cloacal exstrophy cohort and 7 (5.6%) fistulas in the classic bladder exstrophy cohort (P = .019). Using either HAD or native tissue flaps resulted in a lower fistulization rate than using no interposed layers (5.8% vs 20.8%; P = .039). Of those with HAD, the use of a fibrin sealant did not decrease fistulization rates when compared to HAD alone (6.5% vs 8.8%, P = .695). There was no statistical difference in surgical complications between the use of HAD and native flaps (8.6% vs 5%, P = .716). Use of soft tissue flaps and HAD is associated with decreased fistulization rates after BNT. HAD is a simple option and an effective adjunct that does not require harvesting of tissues in patients where a native flap is not feasible.

A Model for Sustained Collaboration to Address the Unmet Global Burden of Bladder Exstrophy-Epispadias Complex and Penopubic Epispadias

International collaboration to alleviate the massive burden of surgical disease is recognized by World Health Organization as an urgent need, yet the surgical mission model to treat reconstructive surgical challenges is often constrained in ensuring adequate patient follow-up, optimal outcomes, and sustainability. To determine whether a collaboration predicated on long-term commitment by surgeons returning to the same institution annually combined with an experienced host surgical team and infrastructure to ensure sustained patient follow-up could provide surgical care with acceptable outcomes to treat bladder exstrophy-epispadias complex (BE) and penopubic epispadias (PE). In this prospective, observational study, long-term collaboration was created and based at a public hospital in Ahmedabad, India, between January 2009 and January 2015. The entire postoperative cohort was recalled in January 2016 for comprehensive examination, measurement of continence outcomes, and assessment of surgical complications. Seventy-six percent of patients (n = 57) who underwent complete primary repair of exstrophy during the study interval returned for annual follow-up in 2016 and formed the study cohort: 23 patients with primary BE, 19 patients with redo BE, and 11 patients with PE repair. Demographics, operative techniques, and perioperative complications were recorded. A postoperative protocol outlining procedures to ensure monitoring of study participants was followed including removal of ureteral stents, urethral catheter, external fixators, imaging, and patient discharge. Of the 57 patients, 4 were excluded because they underwent ureterosigmoidostomy. Median age at time of surgery was 3 years (primary BE), 7 years (redo BE), and 10 years (PE), with median follow-up of 3 years, 5 years and 3 years, respectively; boys made up more than 70% of each cohort (n = 17 for primary BE, n = 15 for redo BE, and n = 9 for PE). All BE and 3 PE repairs (27%) were completed with concurrent anterior pubic osteotomies. Seventeen of 53 patients (32%) experienced complications. Only 1 patient with BE (4%) had a bladder dehiscence and was repaired the following year. A unique surgical mission model consisting of an international collaborative focused on treating the complex diagnoses of BE and PE offers outcomes comparable with those in high-income countries, demonstrating a significant patient retention rate and an opportunity to rigorously study outcomes over an accelerated interval owing to the high burden of disease in India. Postoperative care following a systematized algorithm and rigorous follow-up is mandatory to ensure safety and optimal outcomes.

Epispadias repair with tunica vaginalis flap

Primary epispadias is a rare congenital malformation that afflicts about 1 in 80,000 children. The surgical repair, originally described by Cantwell over 100 years ago, is fundamentally sound and incorporates several important steps to achieve an optimal result. A 9-month-old male with penopubic epispadias presented for surgical repair. Pre-operative imaging included a normal renal ultrasound and voiding cystourethrogram that demonstrated a moderate-sized bladder, partially competent bladder neck, and no vesicoureteral reflux. A modified Cantwell-Ransley approach to the repair was performed without complete disassembly of the penis. Established surgical principles of identification of laterally placed neurovascular bundles, preservation of the ventral mesentery to the urethral plate, mobilization of the urethral plate off the corporal bodies with ventral relocation, and dorsal medial rotation of the corporal bodies are demonstrated in this video. This procedure was performed as an outpatient. The patient had an unremarkable postoperative course. The #6-French urethral stent was removed on postoperative day 10. This video demonstrates a modified Cantwell-Ransley technique for epispadias repair, which employs a tunica vaginalis flap to reinforce the urethral repair. The adherence to surgical principles described by Cantwell in the late 1800s for children with primary epispadias continues to lead optimal surgical outcomes.

Isolated Male Epispadias: Anatomic Functional Restoration Is the Primary Goal.

Background. Isolated male epispadias (IME) is a rare congenital penile malformation, as often part of bladder-exstrophy-epispadias complex (BEEC). In its isolated presentation, it consists in a defect of the dorsal aspect of the penis, leaving the urethral plate open. Occurrence of urinary incontinence is related to the degree of dorsal displacement of the meatus and the underlying underdevelopment of the urethral sphincter. The technique for primary IME reconstruction, based on anatomic restoration of the urethra and bladder neck, is here illustrated. Patients and Methods. A retrospective database was created with patients who underwent primary IME repair between June 1998 and February 2014. Intraoperative variables, postoperative complications, and outcomes were assessed. A descriptive statistical analysis was performed. Results and Limitations. Eight patients underwent primary repair, with penopubic epispadias (PPE) in 3, penile epispadias (PE) in 2, and glandular epispadias (GE) in 3. Median age at surgery was 13.0 months [7–47]; median follow-up was 52 months [9–120]. Complications requiring further surgery were reported in two patients, while further esthetic surgeries were required in 4 patients. Conclusion. Anatomical restoration in primary IME is safe and effective, with acceptable results given the initial pathology.

V10-09 ROBOT-ASSISTED LAPAROSCOPIC BLADDER NECK SLING IN MALE EPISPADIAS IN THE PEDIATRIC PATIENT

You have accessJournal of UrologyRobotics – Benign Disease1 Apr 2015V10-09 ROBOT-ASSISTED LAPAROSCOPIC BLADDER NECK SLING IN MALE EPISPADIAS IN THE PEDIATRIC PATIENT Patricia Cho, Ashley Wietsma, Richard Yu, and Marc Cendron Patricia ChoPatricia Cho More articles by this author , Ashley WietsmaAshley Wietsma More articles by this author , Richard YuRichard Yu More articles by this author , and Marc CendronMarc Cendron More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2015.02.2456AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Male epispadias represents the least severe phenotypic defect of the exstrophy-epispadias complex. Its presentation is variable, but can be associated with significant functional problems due to bladder neck incompetence. Though there are no reliable medical treatments for this condition, there are several options for surgical intervention including bladder neck sling placement. The advantage of the sling procedure is its relative straightforward application. Typically, this procedure is performed using an open approach in children. However, a robotic approach would provide advantages for access and mobility to deep pelvic structures. We aim to demonstrate the application and safety of a robotic approach to the bladder neck sling procedure in the pediatric population. METHODS A 5 year old male (23kg) with penopubic epispadias status post repair at 5 months of age was found to have persistent urinary incontinence for >1 year. Voiding cystourethrogram was performed and demonstrated bladder neck incompetence. The patient underwent robot-assisted laparoscopic bladder neck sling placement, utilizing dorsal lithotomy positioning and two working ports with a single assistant port. A suprapubic tube (SPT) was placed at the time of the procedure. Clamp trials were started 2 weeks post-operatively and the SPT was removed 4 weeks post-operatively after documentation of adequate voiding and bladder emptying. RESULTS There were no immediate complications to the procedure, and operative time was 3.5 hours. The patient was discharged to home on post-operative day 4. In the 6 months following surgery, the patient reported subjective improvement in urinary incontinence with increased dry intervals and quality of life. CONCLUSIONS The robot-assisted laparoscopic sling procedure appears to be safe and technically feasible in small children. This procedure provides a good option for managing bladder neck incompetence in pediatric males with epispadias. This use of the robotic approach demonstrates versatility, as this can be applied to other bladder neck or deep pelvic procedures in pediatric patients. Long-term studies will be needed to validate this technique and to assess durability of symptom improvement. © 2015 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 193Issue 4SApril 2015Page: e847 Advertisement Copyright & Permissions© 2015 by American Urological Association Education and Research, Inc.MetricsAuthor Information Patricia Cho More articles by this author Ashley Wietsma More articles by this author Richard Yu More articles by this author Marc Cendron More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Double breasting of bladder neck and posterior urethra for continence in isolated peno-pubic epispadias.

The objectives of surgical repair of epispadias include the achievement of urinary continence, cosmetically acceptable genitalia with correction of curvature and normal genital function. To achieve all the aforementioned objectives, patients usually undergo two- or multiple-stage surgeries. Traditionally, the patients undergo epispadias repair in the first stage through modified Cantwell-Ransley or Mitchell-Bagli procedure. Subsequently, in the second stage, bladder neck repair is performed to achieve continence, the most common procedure being modified Young-Dees-Leadbetter. There is no reported single-stage technique of epispadias repair achieving both cosmesis and continence in isolated incontinent epispadias. The objectives of the study were to assess continence and cosmesis with partial penile disassemble and double breasting of bladder neck and posterior urethra in isolated peno-pubic epispadias. A retrospective analysis of surgical outcome of seven cases of primary isolated incontinent peno-pubic epispadias repair from July 2008 to July 2012 was carried out. Patients' age varied from 10 months to 16 years. Penile de-gloving is done with mobilization of urethral plate from ventrum to dorsum, distally till mid-glans and proximally up to pubic symphysis with preservation of blood supply at both ends. Partial mobilization of corporal bodies from its attachment and division of peno-pubic ligament are done to lengthen the penis. A mucosal strip of 5-7 mm is excised to denude the mucosa for double breasting. Tubularization of urethral plate with double breasting from the region of bladder neck to posterior urethra is done to increase the outlet resistance and then tubularization of distal urethral plate. Approximation of mobilized pelvic floor muscles is done to complete sphincteroplasty. Spongioplasty along the entire length and corporoplasty with medial rotation of corporeal bodies is done. Glanuloplasty with meatoplasty is done to bring the meatus ventrally and then skin cover to penis is done with rotation of ventral flaps or z plasty. Preoperatively, three patients (42.8%) had moderate and four (58.2%) had severe chordee. Three (42.8%) of the seven patients had mild torque toward right. Six patients became fully continent and had excellent cosmesis postoperatively, while one was partially continent with a dry interval of 2 h and required anticholinergics. None of the patients developed fistula, stricture, wound dehiscence or necrosis in a follow-up period of 1-5 years; however, one patient had mild residual chordee but did not require any additional surgery. Double breasting of bladder neck and posterior urethra with sphincteroplasty and partial penile disassembly produces a reliable tubularized neourethra with complete chordee correction with emphasis on achievement of continence and near-normal-appearing penile morphology through a single-stage surgery. Mobilization of urethral plate and proximal urethra up to bladder neck helps correction of chordee as well as torsion. Partial penile disassembly keeping the urethra attached to glans maintains the dual blood supply, thus preventing stricture and fistula. This small series is a preliminary study, and more studies at different centers may authenticate it by reproducing the results.

Modified Cantwell-Ransley epispadias repair in children our experience

Introduction: We retrospectively evaluated our experience with modified Cantwell-Ransley epispadias repair at our center to determine the complications and long-term results. Materials and Methods: We retrospectively reviewed the case records of 43 male children with a mean age of 9.13 ± 1.94 years who underwent primary epispadias repair at our center. The results of epispadias repair were assessed by both physical and endoscopic examination. All children who were old enough to opine as well as all parents/guardians were interviewed during the follow-up visits. Results: Urethrocutaneous fistulae occurred in 17.85% (5/28) children of the classic bladder exstrophy group and in 13.33% (2/15) children with penopubic epispadias. Postoperative cystoscopy done 12 weeks after repair revealed a smooth urethral tube in 81.39% (35/43) of children. With the patient in a standing position, the penis was dangling downward or in a horizontal position in 88.37% (38) of children, 85% of the patients ≥18 years of age were satisfied with both the functional and cosmetic outcome, as assessed by short form 36 and 93.02% (40/43) of the patients were continent during the daytime with voided volumes of more than 200 ml. Conclusion: In our experience, Cantwell-Ransley repair creates a functionally and cosmetically acceptable penis and produces a reliably tubularized neourethra with acceptable complication rates.

Modified Cantwell-Ransley repair in penopubic epispadias with continent bladder

Modified Cantwell-Ransley repair in penopubic epispadias with continent bladder