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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 [ABSTRACT FROM AUTHOR]

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60–120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60--120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy. [ABSTRACT FROM AUTHOR]

The penis in exstrophy–epispadias complex (EEC) males is diminutive and patients are often dissatisfied with appearance and length. Due to an increased risk of psychosexual dysfunction, patients desire penile reconstruction. Surgical options include penile lengthening with a full thickness skin graft (SG) or tissue expansion (TE) used for cutaneous coverage or neophalloplasty using a radial forearm free flap or pedicled antero-lateral thigh flap. The aim of this study was to assess sexual health outcomes before and after SG or TE assisted lengthening and neophalloplasty. Patients aged 18 years or older, who underwent penile reconstruction were identified using an institutionally approved database of EEC patients. Patient perception of penile appearance, sexual function, their frequency of sexual intercourse and overall satisfaction with reconstruction were assessed using a survey consisting of validated and non-validated questions. Eighty-three patients underwent penile reconstruction, 57 met the inclusion criteria and 28 responded to the survey (49.1%). Fourteen were reconstructed using a SG, 6 with TE and 8 underwent neophalloplasty. Median time from reconstruction to survey completion was 4.4 years (range 1.2–13.2) Four patients had a diagnosis of epispadias, 1 cloacal exstrophy, 23 classic bladder exstrophy (CBE). Before reconstruction patients were dissatisfied with their penile appearance with a median penile perception score of 4.5 compared to 7.5 after surgery (p = 0.0034, Fig. 1). Twenty-three patients were dissatisfied with penile length, with 18 reporting an improvement following reconstruction (p = 0.0002). There was no correlation in time after reconstruction with PPS or satisfaction with penile length (p = 0.86 and p = 0.55, respectively). Overall, the median Sexual Health Inventory for Men (SHIM) score was 19, with no difference between the surgical groups (p = 0.33). Nine patients engaged in sexual intercourse before reconstruction which increased to 17 afterwards. All three surgical methods improved patient perception of penile appearance and length. Patients who previously never engaged in intercourse were able to and those who had were doing so more frequently, of which a greater proportion were in a relationship. The results are encouraging though must be interpreted with caution due to the small number of responders and possible risk of bias. These findings provide vital information for future patients and routine psychological assessment will help manage patient expectations to improve satisfaction. [ABSTRACT FROM AUTHOR]

The term exstrophy-epispadias complex refers to a group of midline defects ranging from epispadias to cloacal exstrophy. Bladder exstrophy is the most frequent malformation of this spectrum and it can present as a classical or a variant form. We report a case of a hybrid bladder exstrophy variant having some characteristics of both a duplicate bladder exstrophy and a superior vesical fistula.

The term exstrophy-epispadias complex refers to a group of midline defects ranging from epispadias to cloacal exstrophy. Bladder exstrophy is the most frequent malformation of this spectrum and it can present as a classical or a variant form. We report a case of a hybrid bladder exstrophy variant having some characteristics of both a duplicate bladder exstrophy and a superior vesical fistula. [ABSTRACT FROM AUTHOR]

Purpose of Review: Adult males with exstrophy-epispadias complex (EEC) are commonly dissatisfied with their genitalia and desire greater sexual normality. These patients have typically had several previous genital/pelvic procedures and there are specific considerations that must be taken into account if operative intervention is being considered. Genital reconstruction in the form of phalloplasty presents an option for improving sexual function. Recent Findings: The majority of studies have focused on the radial free forearm flap (RFFF) phalloplasty with subsequent insertion of penile prosthesis (PP). Long-term cosmetic and functional satisfaction is high. Early urethral complications are common and PP-related complications are higher than in other patient populations. Summary: Genital reconstruction is a safe procedure for EEC males with good long-term functional and cosmetic satisfaction. RFFF phalloplasty represents the most complete surgical option and should be considered in carefully selected patients. [ABSTRACT FROM AUTHOR]

Objectives: To study the presentation and management of the exstrophy-epispadias complex in adolescents and adults and to evaluate the outcome of the repair. Subjects and methods: This retrospective review of our medical records of adult patients with an exstrophy-epispadias complex managed between January 2010 and June 2015 included one female and 7 male patients. Only patients aged above 10 years who had not undergone any surgical treatment during childhood were included in the study. All patients were subjected to preoperative biopsy from the urinary bladder plate. None of them underwent cystectomy. Seven patients underwent urinary bladder augmentation using ileocystoplasty, bladder neck reconstruction with para-urethral tissue closure using a wedge of pubic bone, and single-stage epispadias repair. In one patient epispadias repair was not done due to intraoperative hemodynamic instability. Results: All patients are continent but one in whom epispadias repair was not done and who is waiting for epispadias repair. One patient has developed a urethro-cutaneous fistula at the base of the penis. Two patients developed urinary bladder stones, and both of them were managed by minimally invasive methods. All the patients are on clean intermittent self-catheterization. They are all doing well socially and psychologically. Conclusion: Though exstrophy-epispadias in adolescents and adults is a rare entity, the described modified surgical technique provides good results and improves the patients’ social and psychological well being.

We describe the case of a bladder exstrophy–epispadias complex (BEEC), in which a “split” appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. Split appendix had been used before for simultaneous Mitrofanoff and Malone's antegrade contrast enema channels to manage neurogenic bladder and bowel. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC.

A successful abdominal wall and bladder closure is critical in the management of cloacal exstrophy (CE). This study examines closure outcomes and practices over the last 4 decades at the authors' institution. Beginning in 1995, the authors' institution standardized CE closure and management with the Dual-Staged Pathway (DSP). The DSP consists of a staged bladder closure, a staged or concurrent osteotomy, and postoperative immobilization with external fixation. The authors hypothesize that the DSP has provided better outcomes in CE closures. A prospective database of 1332 Exstrophy–Epispadias Complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. The DSP consists of a staged osteotomy and a staged bladder closure in CE patients with a diastasis greater than 4 cm. To evaluate the DSP, outcomes of closure at the authors' institution were compared between two equal, twenty-year periods before and after its implementation. Data on timing of closure, postoperative management, surgical complications, and outcomes were collected. There are 142 CE patients in the database. In this study, 49 CE patients with 50 closures met inclusion criteria. The overall success rate of closures from 1975 to 1994 was 88% (14 of 16), while the success rate of the DSP was 100% (n = 34), p = 0.098. Twenty-two (65%) primary and 12 (35%) secondary closures were performed using the DSP. Overall complication rates of the DSP remained similar to previous closures, (29% vs 19%, p = 0.508). Since incorporation of the DSP, patients referred for closure have generally had a larger preclosure diastasis (7.2 cm vs 5.1 cm, p = 0.011). The standardized DSP closure has proven successful in 34 primary and reoperative cloacal closures in the past 20 years. With this approach, the authors feel that the DSP offers greater patient safety and better outcomes. Level III, retrospective comparative study. [ABSTRACT FROM AUTHOR]

Abstract Purpose To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure. Methods A prospectively maintained database of 1332 exstrophy-epispadias complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. Univariate and multivariable analyses were performed to identify significant factors associated with CE primary bladder closure. Results Of 143 CE patients identified, 99 patients met inclusion criteria. Median follow-up time was 8.82 [IQR 5.43–14.26] years. In the multivariable model, the odds of having a successful closure are about 4 times greater for the staged cloacal approach compared to the 1-stage approach (OR, 3.7; 95% CI 1.2–11.5; p-value = 0.023). Also, having an osteotomy increases the chance of a successful closure by almost six-fold (OR, 5.8; 95% CI 1.7–19.6; p-value = 0.004). Conclusions Using the staged approach with a pelvic osteotomy is paramount to a successful primary closure in CE. The authors strongly recommend using the staged approach and osteotomy as these factors independently increase the chance for a successful primary bladder closure. Study Type Therapeutic study. Level of Evidence Level III, Retrospective comparative study. [ABSTRACT FROM AUTHOR]

Abstract Aim The aim of this report is to present a brief review of the current literature on the management of EEC. Case Report A term male neonate presented at birth with classic bladder exstrophy, a variant of the exstrophy-epispadias complex (EEC). The defect was covered with sterile silicon gauzes and waterproof dressing; at 72 hours of life, primary closure without osteotomy of bladder, pelvis, and abdominal wall was successfully performed. Discussion EEC incidence is approximately 2.15 per 1,00,000 live births; several urological, musculocutaneous, spinal, orthopedic, gastrointestinal, and gynecological anomalies may be associated to EEC. Initial medical management includes use of occlusive dressings to prevent air contact and dehydration of the open bladder template. Umbilical catheters should not be positioned. Surgical repair stages include initial closure of the bladder and abdominal wall with or without osteotomy, followed by epispadias repair at 6 to 12 months, and bladder neck repair around 5 years of life. Those who fail to attain continence eventually undergo bladder augmentation and placement of a catheterizable conduit. Conclusion Modern-staged repair of EEC guarantees socially acceptable urinary continence in up to 80% of cases; sexual function can be an issue in the long term, but overall quality of life can be good.

Introduction Abnormalities of the bony pelvis in exstrophy-epispadias complex (EEC) and their possible relation to hip disease are well described. However, there is a lack of information about long-term orthopedic consequences and hip function in patients with EEC. Therefore, we investigated clinical and radiological results in an EEC patient cohort after long-term follow-up. Patients and Methods We conducted a cross-sectional study using standardized radiography, clinical investigation, and the Harris hip score. Seventeen postpuberty consecutive unselected EEC patients (3 female, 14 male; mean age 18.2 years) that presented to our clinic due to urological procedures or routine check-up from 2010 to 2011 were included. All had undergone symphysis approximation with a traction bandage without osteotomy in early childhood. Radiological analysis was conducted offline by two independent investigators. Results Radiological analysis showed a mean pubic diastasis of 5.1 cm (range 2.8-8.5 cm). Borderline hip dysplasia was present in four patients, one of them having had co-occurring developmental hip dysplasia in previous history. No severe dysplasia, subluxation, or luxation of the hip was found; however, one patient showed early hip arthrosis. Clinical examination revealed no relevant restriction of range of motion, although rotation and abduction were slightly altered in five patients. None of the EEC patients complained about pain or restriction in sports or daily activities. Harris hip score was perfect for all but one study participants. Conclusion Despite EEC-specific hip morphology, long-term hip function is not impaired in patients after symphyseal approximation without osteotomy in the newborn period. The symphysis diastasis after this procedure is comparable to available postosteotomy data. The large majority of EEC patients did not show dysplastic or degenerative hip disease. Functional hip score results confirmed reasonable age-related hip function in nearly all examined patients. However, postnatal ultrasound hip screening is recommended to prevent and adequately treat potential co-occurring developmental hip dysplasia. [ABSTRACT FROM AUTHOR]

Introduction and Aim Female epispadias is a rare congenital anomaly associated with significant urinary incontinence. This study aims to evaluate the results of its treatment in terms of continence, sexuality, and impact of incontinence on quality of life. Materials and Methods We retrospectively reviewed nine patients (5-39 years) treated of female epispadias in our hospital during the period 1976-2013. Urinary continence (ICIQ-SF), sexuality, impact of incontinence on quality of life (Potenziani-14-CI-IO-2000-QOL), and overall quality of life (SF-36) were evaluated. Results All nine women were treated by bladder neck reconstruction (Young-Dees-Leadbetter) and genitoplasty; four received 2.5 (R = 1-5) endoscopic bladder neck injections; one required enterocystoplasty, urinary diversion, and bladder neck closure because of persistent incontinence; five performed pelvic floor rehabilitation; and two took anticholinergics. Three achieved continence; five had mild urinary incontinence; and 1 had moderate urinary incontinence and was awaiting an endoscopic injection. Of the seven who were older than 18 years, five answered the questionnaires of quality of life and sexuality. All had a general quality of life (SF-36) that does not differ from the normal population and had a slight impact (7 [0-15] points) of incontinence on quality of life (Potenziani-14-CI-IO-QOL-2000). Four had a normal sex life, and the fifth had lack of self-confidence due to her incontinence. Conclusion Patients with female epispadias have good long-term results regarding quality of life and sexuality, despite having some degree of urinary incontinence. [ABSTRACT FROM AUTHOR]

We describe the case of a bladder exstrophy–epispadias complex (BEEC), in which a "split" appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. Split appendix had been used before for simultaneous Mitrofanoff and Malone's antegrade contrast enema channels to manage neurogenic bladder and bowel. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC. [ABSTRACT FROM AUTHOR]

Bladder exstrophy is a complex congenital birth defect ranging from complete epispadias to complete bladder exstrophy. Though not included in the exstrophy-epispadias complex (EEC) disease spectrum, cloacal exstrophy is a more severe form which includes bowel segments in the defect and has many of the same defects as the EEC. Initial care of these complex patients focuses on the primary repair of the defect, a repair which is the most important of the many operations these children will have in their lifetime. A failure of primary closure is associated with worse outcomes across the board. The most essential goal in treatment of the exstrophy patient is renal protection. Once that can be assured, social continence then becomes the most important goal. Given the complex nature of this disorder, these children should be managed at speciality centers with orthopedic, urologic, and nursing experience with these patients. [ABSTRACT FROM AUTHOR]

Purpose Urinary continence is the goal of exstrophy–epispadias complex (EEC) reconstruction. Patients may require a continent urinary diversion (CUD) if they are a poor candidate for bladder neck reconstruction or are receiving an augmentation cystoplasty (AC) or neobladder (NB). This study was designed to identify the incidence of surgical complications among various bowel segments typically used for CUD. Methods A prospectively kept database of 1078 patients with EEC at a tertiary referral center from 1980 to 2012 was reviewed for major genitourinary reconstruction. Patient demographics, surgical indications, perioperative complications, and outcomes were recorded. Results Among reviewed EEC patients, 134 underwent CUD (81 male, 53 female). Concomitant AC was performed in 106 patients and NB in 11. Median follow up time after initial diversion was 5 years. The most common CUD bowel segments were appendix and ileum. The most common surgical complications after CUD were small bowel obstruction, post-operative ileus, and intraabdominal abscess. There was a significantly increased risk in the occurrence of pelvic or abdominal abscess when colon was used as a conduit compared to all other bowel segments (OR = 16.7, 95% CI: 1.16–239) and following NB creation compared to AC (OR = 39.4, 95% CI: 3.66–423). At postoperative follow-up, 98% of patients were continent of urine via their stoma. Conclusion We report the largest series to date examining CUD in the EEC population. The increased risk of abdominal and pelvic abscesses in patients who receive a colon CUD and undergo NB compared to AC indicates that while surgical complications following major genitourinary reconstruction are rare, they do occur. Practitioners must be wary of potential complications that are best managed by a multi-disciplinary team approach. [ABSTRACT FROM AUTHOR]

Objective To present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy–epispadias complex (EEC) population. Methods Retrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed. Results Among the EEC patients reviewed, 133 underwent CS (80 male, 53 female). Mean follow-up time after initial continent stoma was 5.31 years (range: 6 months to 20 years). Appendix and tapered ileum were the primary bowel segments used for the continent channel and stoma in the EEC population. The most common stomal complications in this population were stenosis, incontinence, and prolapse. Seventy-nine percent of EEC CS patients underwent AC primarily done with sigmoid colon or ileum. Eleven patients (8%) underwent neobladder creation with either colon or a combination of colon and ileum. Bladder calculi, vesicocutaneous fistula, and pyelonephritis were the most common non-stomal complications. Stomal ischemia was significantly increased in Monti ileovesicostomy compared to Mitrofanoff appendicovesicostomy in classic bladder exstrophy patients ( p = 0.036). Furthermore, pyelonephritis was more than twice as likely in colonic neobladder than all other reservoir tissue types in the same cohort (OR = 2.53, 95% CI: 1.762–3.301, p < 0.001). Conclusions To the best of the authors' knowledge, this is the largest study examining catheterizable stomas in the exstrophy population. While Mitrofanoff appendicovesicostomy is preferred to Monti ileovesicostomy because it is technically less challenging, it may also confer a lower rate of stomal ischemia. Furthermore, even though ileum or colon can be used in AC with equally low complication rates, practitioners must be wary of potential urologic complications that should be primarily managed by an experienced reconstructive surgeon. [ABSTRACT FROM AUTHOR]

Bladder exstrophy is a rare malformation characterized by an infra-umbilical abdominal wall defect, incomplete closure of the bladder with mucosa continuous with the abdominal wall, epispadias, and alterations in the pelvic bones and muscles. It is part of the exstrophy-epispadias complex, with cloacal exstrophy on the severe and epispadias on the mild ends of the spectrum. Bladder exstrophy is the most common of these entities and is more common in boys. The goal of this paper is to describe common methods of repair and to provide an imaging review of the postoperative appearances. [ABSTRACT FROM AUTHOR]

The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy-epispadias complex, the modern management of bladder exstrophy, and potential surgical complications. [ABSTRACT FROM AUTHOR]

Abstract Background : Because exstrophy–epispadias complex is uncommon and satisfactory surgical reconstruction outcomes are difficult to achieve, the surgical repairs by one surgeon (PS) were analysed over a 14-year period. Methods : Retrospective analysis was performed on 13 patients with the complex who underwent surgery between January 1986 to August 2000. Cosmesis and continence were evaluated. Complications including wound dehiscence and urethrocutaneous fistula were reported. Results : Of six patients with classical exstroply who underwent functional bladder closure, all had good cosmesis except one who had partial dehiscence. Four patients with isolated epispadias had satisfactory cosmesis. Urethrocutaneous fistula was found in one boy in the classical exstrophy group. Continence was achieved in three out of four patients who had bladder neck reconstruction. One girl whose bladder neck was severely obstructed after functional bladder closure, had continent catheterizable stoma. Conclusion : Functional bladder closure yielded satisfactory cosmetic outcome. Bladder neck reconstruction made the patient dry in 75% of cases. [ABSTRACT FROM AUTHOR]

We report a new method of repair of exstrophy-epispadias complex (EEC) at a second stage by using flaps from the skin between the penis and scrotum (the clitoris and labia majora in females) used in seven patients. The non-pigmented skin between the penis and scrotum in males or a band of skin between the clitoris and labia majora in female is mobilized in two flaps, which are rotated superiorly. Five patients were treated primarily by this method and two patients at the time of secondary reconstruction. There were six boys and one girl. Two patients were post-pubertal, one male and one female. Follow-up (6–12 months) revealed good cosmetic and functional results. If EEC is repaired in a single stage, the entire pelvic diaphragm is visualized from inside and the sphincters can be reconstructed around the bladder neck. This procedure gives better exposure of attachments of the corporal bodies to the pubic rami, allowing easier mobilization. The bulbospongiosus muscle can be reconstructed. A normal penoscrotal relation is achieved in male patients. The root of the scrotum, which is splayed out and wide, narrows. Rotation of the flaps superiorly normalizes the symphyseal area. In female patients the appearance of the mons pubis is satisfactory. In post-pubertal children the pubic hair distribution appears normal. [ABSTRACT FROM AUTHOR]

Objective To evaluate the surgical procedures required for anatomical reconstruction of the bladder and penis in the exstrophy–epispadias complex. Patients and methods All primary exstrophy–epispadias repairs carried out by one surgeon between 1987 and 1997 were reviewed. Bladder closure consisted of full extraperitoneal mobilization, transpositional omphaloplasty, drainage with ureteric and urethral catheters and immobilization with a ‘frog-leg’ plaster-cast or ‘mermaid’ dressings. Osteotomies were always performed when bladder closure was attempted after 37 h of age. Before 1990 the osteotomies were posterior vertical iliac (one patient) and subsequently anterior oblique iliac (10 patients). Pre-peritoneal herniotomies, in the absence of a clinical hernia, were included in the primary procedure after 1992. A modified Cantwell technique was used for epispadias repair and this was undertaken at a median of 16 months after bladder closure (range 6–30). Results Thirty-four patients (27 male) were reviewed; one patient had a chromosomal abnormality, a deletion in the short arm of chromosome 4. The male infants required a median of four procedures (range 2–5) for bladder closure, epispadias reconstruction and herniotomies, while the females needed a median of two (range 2–5). Complete bladder dehiscence, requiring re-closure with osteotomies, occurred in three cases (9%, two male). There were no dehiscences in the primary osteotomy group. Fistulae after epispadias repair occurred in four patients (17%). The bladder capacity increased to >60 mL in 10 of 15 males by 36 months after epispadias repair. Only two of seven female infants attained a capacity of >60 mL. Of the 15 infants who did not undergo herniotomy at primary closure, 13 subsequently developed inguinal hernias (one uni- and 11 bilateral) with incarceration occurring in two. Twelve infants underwent herniotomy at primary closure and six... [ABSTRACT FROM AUTHOR]

Summary This video provides a case report of a 3 year old girl with epispadia and the highlights of the surgery. A cystoscopic guided bladder neck plication was performed to achieve continence. Key points include: (1) Skin incision planning; (2) Cutaneous flap liberation to create a new urethra; (3) Complete bladder neck release to allow a controlled plication; (4) Use of cystoscopy to achieve the ideal bladder neck closure; (5) Bladder neck manipulation to achieve continence. [ABSTRACT FROM AUTHOR]

Patients with prior bladder exstrophy could achieve pregnancy successfully and without major adverse events. [ABSTRACT FROM AUTHOR]

Penile prosthesis implantation (PPI) is a definitive treatment option for patients with medically refractory erectile dysfunction (ED). It is a safe, reliable operation with high patient satisfaction and few complications. We report a novel case of an adult patient with exstrophy-epispadias complex who underwent PPI for ED and discuss the surgical challenges presented by the unique anatomic constraints of this condition. Sotimehin AE, Burnett AL. Penile Prosthesis Implantation in an Exstrophy-Epispadias Complex Patient: A Case Report. Sex Med 2019;7:540-542., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Abstract: The complete penile disassembly technique is increasingly employed during the surgical repair of classic bladder exstrophy. We describe the complication in a newborn boy of loss of the glans penis following complete primary exstrophy repair. The possible causes and prevention of this injury are discussed. [Copyright &y& Elsevier]

Summary Introduction With advances in genitourinary reconstructive surgery, women with exstrophy–epispadias complex (EEC) have improved health and quality of life, and may reach reproductive age and consider pregnancy. Despite literature suggesting impaired fertility and higher risk with pregnancy, childbirth is possible. Medical comorbidities, including müllerian anomalies, contribute to increased risk of obstetric and urologic complications during pregnancy. Objectives We reviewed our experience with EEC patients who achieved pregnancy to investigate (1) urological characteristics of women who achieved pregnancy; (2) pregnancy management, complications, and delivery; and (3) neonatal outcomes. We developed recommendations for managing pregnancy in women with EEC. Study design/Results This was a retrospective chart review of 36 female patients with EEC seen at our institution between 1996 and 2013. Female patients less than 18 years, and patients who did not have documented pregnancy were excluded. This resulted in a total of 12 patients with 22 pregnancies. All women with successful pregnancy had bladder exstrophy. The majority had undergone prior bladder augmentation (75%) and were on self-catheterization programs (92%). Thirty-six percent had symptomatic urinary tract infections (UTIs) during pregnancy. Five women had more than one pregnancy. There were four terminations of pregnancy. Of 18 desired pregnancies, there were four spontaneous abortions (SABs) (22%) and 16 live births (78%). The cesarean delivery (CD) rate was 100% (14/14), of which the majority were vertical (classical) uterine incisions with a paramedian skin incision. With the exception of one patient, there were no CD surgical complications. The mean gestational age at delivery was 36 weeks (Range 25 4/7 to 39 4/7 weeks) among eight pregnancies with known gestational age. There were no stillbirths, one neonatal death and no birth defects. Discussion Women with EEC can have successful pregnancies, though at increased risk for preterm delivery and SABs. In our cohort, the rate of SAB is similar to that described in prior studies. Symptomatic UTIs likely due to self-catheterization were common. Cesarean delivery using a paramedian skin incision and classical uterine incision were not associated with major complications in this cohort. Limitations include reliance on retrospective data and small sample size. The strength of this study is the longitudinal detailed management of pregnancies in EEC women by a single team over time. A multidisciplinary approach to providing a continuum of care from pediatrics through adolescence to adulthood optimizes successful transitions, reproductive health, and successful pregnancies. Based on our experience, an algorithm providing guidance for pregnancy management was developed. Table Pregnancy resulting in live birth versus spontaneous abortion or termination. Outcomes of 22 pregnancies in 12 EEC patients Outcome Total pregnancies ( n = 22) Bladder exstrophy pregnancies ( n = 20) Cloacal exstrophy pregnancies ( n = 2) Total pregnancies 22 (100%) 20 2 Spontaneous abortion <24 weeks 4 (18%) 3 (15%) 1 (50%) Terminations 4 (18%) 3 (15%) 1 (50%) Live births 14 (64%) 14 (70%) 0 [ABSTRACT FROM AUTHOR]

Abstract: The authors report a rare variant of exstrophy-epispadias complex, a duplicate bladder with normal bladder communicating with an exstrophic bladder by a fistula, in a girl with no genital malformation except for a duplicated clitoris. This variant could be a hybrid form of duplicate bladder exstrophy and superior vesical fistula. It seems easier to repair and has a better prognosis than classic bladder exstrophy. [Copyright &y& Elsevier]

Cloacal exstrophy is the most severe and rare form of the exstrophy-epispadias complex, presenting with exposed bladder halves extruding through an abdominal wall defect and variable genitourinary, gastrointestinal, musculoskeletal, and neurological defects. The authors report magnetic resonance imaging findings of a neurologically-intact, 24-month-old female with cloacal exstrophy who presented with anterior spinal dysraphism and diastematomyelia and duplicate pelvic floor musculature. The constellation of defects suggests a common genetic, biochemical, and embryological origin for duplication of the bladder, spinal cord, and pelvic floor muscles occurring in the fourth week of gestation.