In patients with EEC, the issues such as sexuality, sexual function and fertility gain more importance once theses patients advance from puberty to adulthood. The aim of this review is to critically examine the available evidence on these issues. A systemic literature search was performed in Medline over the last 25 years using the key words: Exstrophy, sexual function and pregnancy. Search results were limited to studies of patients with exstrophy published in English literature. A total of 1500 publications were found and subsequently screened by title and when appropriate by abstracts. Of these, 40 publications pertinent to the subject were included for the analysis. The publications were supplemented by an additional 15 publications obtained from their bibliographies. The studies were rated according to the guidelines published by the US department of health and human services. Heterosexuality is usually expressed in both the sexes and most of them have adequate sexual function. Urinary diversion in some series seems to result in better ejaculatory hence fertility outcome in male patients. Recent series have shown equally good results with primary reconstruction. Most of the female patients have normal fertility while male patients have significantly low fertility. Most of the male and female patients with EEC have adequate sexual function. Most of the female patients have normal fertility while most of the male patients have significantly low fertility.

PURPOSE: Definitions of continence following surgery in children with exstrophy-epispadias complex vary widely. We assessed the most common definitions of continence and evaluated the clinical significance of usage patterns for those definitions. MATERIALS AND METHODS: We searched MEDLINE and EMBASE (2000 to 2011) for English language reports describing postoperative continence outcomes in children with exstrophy-epispadias complex. Articles were evaluated and data were abstracted by 2 reviewers. We assessed patient level factors such as age, preoperative diagnoses and use of additional therapies, as well as study level factors such as continence definition(s), country of origin and method of data collection. RESULTS: We identified 884 articles, of which 87 met inclusion criteria. In total these studies included continence outcomes data on 2,681 patients (57% male). Only 59 studies (68%) clearly defined the term "continence." The most common definition of continence was dry with voiding/catheterization every 3 hours (used in 23 studies, or 39%, defining continence). There was no association between publication date (p = 0.17), study location (p = 0.47) or study size (p = 0.81) and continence definition. There was a trend toward improved reporting of methods for continence ascertainment in more recent years (p = 0.02). Of the 2,681 children included 1,372 (51%) were dry by the definition used in their study. CONCLUSIONS: The most frequent definition of continence was "dryness with voiding or catheterization at 3-hour intervals." However, definitions were highly variable and many authors did not define continence at all. To better define outcomes, we recommend that a standardized definition of continence be established and used in future reports.

Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life.

BACKGROUND: Epispadias is a rare condition. Epispadias in females is two times less common than in males. Female epispadias range from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed through careful physical examination of the genital. Surgery is the management of epispadias. Surgical management of epispadias is quite tricky and requires expertise. The literature that discusses female epispadias is challenging to be found. In this paper, we would like to report surgical management of isolated female epispadias in Cipto Mangunkusumo Hospital, Jakarta. CASE PRESENTATION: A 7-year-old girl was admitted with a chief complaint of urinary incontinence since birth, during daytime and nighttime. Urinary incontinence was not induced by activities nor worsened by eating/drinking. Physical examination showed that the patient's external genitalia has underdeveloped labia minora, patulous urethra, bifid clitoris, and multiple hypopigmentation lesions. Laboratory results were in the normal range. The voiding cystourethrography (VCU) result revealed urine leaks during the filling phase. The bladder wall was normal, and no vesicoureteral reflux (VUR) appeared. The urethrocystoscopy shows a more vertical OUE, a wide-open bladder neck, and a urethral length of 1.5 cm. The patient underwent single-stage surgical procedures that consist of epispadias repair and bladder neck reconstruction through a subsymphyseal perineal approach. No complications occurred intra-operative and post-operative. At the 1-week and 6-months follow-up, the patient achieved urinary continence, and the surgical wound healed normally. DISCUSSION: Epispadias is a rare condition that could occur in various degrees, from mild to severe degrees. To a severe degree, there is a split at the entire urethral and involves the bladder neck causing constant incontinence for the patient. Epispadias cases are quite challenging to diagnose. The physician needs to separate the labia majora and perform the physical examination carefully. The objective goals of surgical management of epispadias are to achieve urinary continence, restoration of anatomy, function, and cosmetic appearance of urethra and genitalia. Single-stage reconstruction is the current surgical method used to repair isolated female epispadias. CONCLUSION: Females epispadias is a rare congenital anomaly that is quite often to miss diagnosed. The single-stage technique is the recommended surgical technique for isolated female epispadias. This patient has achieved urinary continence, and no complications occurred during and post-surgery.

From 1978 to 1993, 180 boys with epispadias (85) and the exstrophy/epispadias complex (95) underwent epispadias reconstruction at our institution. The 180 patients were divided into 4 groups: group 1--2-stage epispadias reconstruction with dissection of the corporo (30 patients), group 2--pedicled preputial tube urethroplasty either alone or with a lyophilized human dural patch to the corpora for correction of penile deformity (35), group 3--pedicled preputial tube urethroplasty and corporeal rotation (40), and group 4--a modified Cantwell epispadias repair incorporating complete tubularized urethroplasty, cavernocavernostomy and corporeal rotation. In 46 patients from groups 1 to 3 the primary outcome was not considered satisfactory and they underwent radical penile revision with the modified Cantwell technique. Followup ranges from 1 to 15 years (mean 6 years). We conclude that a good cosmetic result can be achieved in almost all cases using the modified Cantwell technique as a primary procedure in experienced hands. This technique has a low complication rate and can be used as a salvage procedure following previous unsuccessful epispadias reconstruction using other techniques.

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

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.

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.

A 58-year-old woman was admitted to our hospital. At 10 years old, she had undergone bilateral uretero-sigmoid anastomosis for congenital epispadias, and at 57 years old, she had received transverse colostomy. Biochemical tests showed marked metabolic acidosis. Computed tomography showed urine stagnation in the sigmoid colon, leading to a diagnosis of metabolic acidosis associated with transverse stoma after bilateral uretero-sigmoid anastomosis. Her bone mineral density was below normal, and the bone metabolic marker levels were high, indicating high-turnover osteoporosis. Both metabolic acidosis and bone metabolism were stabilized by treatment with a transanal urinary catheter, sodium bicarbonate, and vitamin D.