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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 m 2 ), 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., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Introduction: 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., Objective: The aim of this study was to assess sexual health outcomes before and after SG or TE assisted lengthening and neophalloplasty., Study Design: 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., Results: 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., Conclusion: 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., Competing Interests: Conflict of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: The exstrophy-epispadias complex (EEC) ranges from distal epispadias to cloacal exstrophy, with serious repercussions on the quality of life of patients. However, reconstructive surgery offers the opportunity to reach adulthood and consider motherhood.The objective of this work is to assess the uro-gynecological characteristics and the risks that pregnant women have with EEC. MATERIALS AND METHOD: Retrospective study of 50 patients diagnosed with EEC and treated in a reference center for this pathology, born between 1968 and 2000. Their medical records were reviewed and all demographic, pathological and gynecological data were collected. RESULTS: 37 patients have the inclusion criteria and of these 8 achieved 17 pregnancies (90% spontaneous and 10% through IVF). 10 were successful (50% at term) and 7 were abortions, 87.5% of which were in the first trimester. Urinary tract infection (UTI) was the most frequent complication (41.6%) and the most severe was intestinal occlusion. None of the patients presented renal function impairment during the gestation or dilation of the pathological upper urinary tract (UUT). 62.5% of the patients presented genital prolapses after pregnancies, 80% of which were grade III and IV. 87.5% were dry in the follow-up after their pregnancies. CONCLUSION: Pregnancy in the EEC patients is high risk and it is crucial that the follow-up is carried out by a specialized and integrated multidisciplinary team to minimize complications.

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.)

Introduction: 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., Methods: 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., Results: 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)., Conclusion: 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 of Evidence: Level III, retrospective comparative study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2015 Elsevier Inc. All rights reserved.)

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.

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., (Copyright © 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)