Exstrophy Research Articles

Delivery in a Female Bladder Exstrophy Patient With Ileal W Pouch Continent Cutaneous Stoma

Propose: To deliver a case report of female bladder exstrophy patients who experienced pregnancy until the birth process.
 Methods: A 28-year-old woman with a history of bladder exstrophy, which received inadequate treatment during childhood, had bladder shrinkage and uterine prolapse. The patient underwent reconstruction of continent cutaneous stoma and sacrocolpopexy with mesh in her adulthood. She achieved complete continence by intermittent self-catheterization and gained a better quality of life. She later got married and became pregnant. Pregnancy monitoring was done to evaluate the condition of the patient and her fetus. A collaborative team was built to discuss the best option for delivering the procedure. At 38–39 weeks of gestational age, an elective cesarian section was performed to deliver the baby.
 Results: Despite having a greater risk of complications, a woman with a history of bladder exstrophy could undergo pregnancy and give birth. The choice of delivery, either vaginal or cesarean section, is feasible for delivery.
 Conclusions: An adult female with bladder exstrophy underwent pregnancy and gave birth. Prior surgery and increased risk of obstetrical complications must be considered.

Bladder exstrophy: An atypical journey of a rare case of an adult

Bladder exstrophy is a rare and complex malformation. Often associated with diverse deformations, an accurate diagnosis is a must for adequate management. In the African setting, especially in a remote area, delivery rarely occurs within a healthcare structure, thus reducing the chance of an early diagnosis. Due to a low density of specialists, people in rural areas refer to traditional healers for healthcare problems, the thing that delays the time to diagnosis. We report, according to the CARE guidelines (https://www.equator‐network.org/reporting‐guidelines/care/), a case of an 18‐year‐old male patient who was transferred to us for better management of a reddish hypogastric mass leaking urine. Initially considered as a congenital wound, his parents applied traditional products to heal it. Following the poor outcomes, the parents will consult several general physicians; unfortunately, no one gave a clear diagnosis. The clinical examination on admission revealed a good general condition and noted the presence of a reddish, ovoid structure of about 9 centimeters of the minor axis and 11 centimeters of the major axis in the hypogastric region allowing urine to flow in its upper part. The external genitalia examination revealed a retracted and short penis with urine exit at its base through the reddish structure. The patient was transferred outside the country for better management due to a lack of dedicated equipment. Although vesical exstrophy is a rare disease, an early diagnosis allows adequate management and good outcome. Thus, a well‐performed neonatal examination is required to avoid later diagnosis and complications.

Quality of life of children operated for bladder exstrophy.

Bladder exstrophy is a rare malformation with functional and psychological issues affecting children's quality of life. This study aims to evaluate the urinary continence and the quality of life of patients operated for bladder exstrophy in our institution. This is a retrospective study including fifteen patients operated for bladder exstrophy between 1995 and 2015. All patients underwent a physical examination and an interview with a psychiatrist. Urinary continence was evaluated by dryness periods. Quality of life was evaluated by the QoL SF-36 scale. All patients underwent primary early bladder closure. Posterior osteotomy was performed initially in 6 cases, and during the redo surgery in 7 cases. Leakage was recorded in 4 cases, fistula in 9 patients. Twelve patients had one or more redo surgery. Final bladder closure success rate was of 80%. Additional continence surgical procedures were performed by a Young Dees technique in 8 cases and with bladder enlargement associated with Mitrofanoff continent derivation in 5 cases. Only two patients over fifteen did not use diapers. All the items of QoL score according SF-36 were under the normal value (75%) except physical functioning and physical limitation. These scores go worst with age. Scores were better for continent boys in both physical and psychological items, but the difference was not significant However, significant difference was observed between boys having less than 3 surgeries and those having more than three procedures. Psychological and social scores were better for girls than for boys and differences were significant. The risk of urinary incontinence is high in children managed for bladder exstrophy. Boys are more affected than girls with subsequent psychological and social repercussions. In our study, quality of life depends more on number of surgery than continence results. Psychological problems should be screened early for accurate treatment. 3.

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.

A survey of management preferences for bladder exstrophy and cloacal anomalies among Canadian pediatric surgeons and urologists

PurposeThis study examined the current referral patterns and preferences of Canadian Association of Paediatric Surgeons (CAPS) and Pediatric Urologists of Canada (PUC) members for management of bladder exstrophy and cloacal anomalies (BECA). MethodsWe invited CAPS and PUC members to participate in an online survey using RedCap. Demographic variables, years in practice, current referral patterns and local expertise at the participants’ institution were collected. Participants’ preferences towards three distinct referral models were assessed using Likert scales: no centralization of care, centralization in one or two national centres of excellence, or a consortium-based approach. ResultsThere were 82 survey respondents (2/3 were CAPS members, 35.4% female, 72% in practice for >10 years). Although >90% of participants agreed/somewhat agreed that surgical volumes impact outcomes, 58% reported not referring out BECA patients for treatment; about 50% recognized the existence of a local dedicated expert. In terms of referral preferences, 84% of participants favoured identification of a few centres with expertise based on geographic location (a consortium-based approach), while only 7% chose a one or two national centres of excellence model. Over half of participants agreed/somewhat agreed with participating in trials of a consortium-based approach in Canada. ConclusionMost CAPS and PUC members do not refer BECA patients elsewhere for treatment. Nonetheless, most surgeons recognize the importance of volume to improve outcomes and show willingness to participate in trials to concentrate experience; most participants favour a consortium-based approach through identification of a few centres of excellence based on geographic location.

Bladder exstrophy: Modern staged repair experience in our institution.

Introduction:Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem in Sub-Saharan Africa. While outlining our challenges, we report our management experience to improve our results.Subjects and Methods:We retrospectively reviewed the records of patients undergoing surgical repair of classic bladder exstrophy at our department between January 2010 and December 2019 (10 years). Epidemiological, clinical, therapeutic and evolution data were analysed.Results:Twenty-five children with classic bladder exstrophy were treated. Our series included 16 boys and 9 girls with a sex ratio of 1.7. Age ranged from 0 day to 6 years. Twenty-five bladder closures were performed, associated to pelvic osteotomy in 11 cases. Epispadias repair was performed on nine boys. Eight cases of bladder neck reconstruction and three cases of bladder enlargement were performed. We observed six bladder fistulas, four wound dehiscence, of which three partial, two parietal suppurations and six cases of urinary tract infection. Eight children had a continence of 1–2 h.Conclusion:The treatment of bladder exstrophy in our context is still limited because of financial difficulties encountered by the population and the insufficient technical platform in our country.

Abdominal wall closure in adult patients with untreated exstrophy of bladder.

Introduction:Bladder exstrophy is an anomaly, which if not treated early in the age, poses a surgical challenge in providing an adequate abdominal wall closure. We report our experience in patients with untreated exstrophy of the bladder, who underwent cystectomy, ileal conduit, and abdominal reconstruction using the anterior rectus sheath turndown flap and paired inguinal skin flaps.Materials and Methods:Ten previously unoperated adult patients with exstrophy with epispadias, who underwent surgery at our institute from January 2010 till January 2021, were included in this study to evaluate the adequacy of abdominal wall closure with our technique of retrograde turndown anterior rectus sheath flap with paired inguinal flap, and to document immediate and delayed complications, especially incisional hernia.Results:The mean follow-up period of the study was 16.5 months. We found that our technique provided adequate local tissue for a sturdy two-layered closure of the lower abdominal wall defect. Out of the ten patients, only one required an additional flap for abdominal wound closure. There were no stoma-related complications or incisional hernia.Conclusion:Abdominal wall reconstruction, for skin and fascial defects, in such complex cases can be performed by local skin and fascia using a relatively simple, safe, easy, and affordable technique as we have described. Our technique avoids the use of synthetic mesh, thereby reducing the chances of infection in such chronic open wounds.

How We Made Exstrophy Bladder Continent and Preserved the Renal Functions.

Crucial requirement of exstrophy bladder repair is to make patients continent as well as to preserve kidney functions. We analyzed our patients' data retrospectively to study their continence and to find out the justification behind continence and preservation of renal functions. We selected files of 18 fully continent patients from 52 patients operated. Eleven out of 18 patients were presented from beginning and 7 were referred after around 8 to 14 years, as incontinent bladder following good repair of bladder neck and posterior urethra. Eleven were operated with complete primary repair of exstrophy along with pubic osteotomy minimal and were kept on cystostomy track (CT) till augmentation to vent out vesical pressure. In seven patients, we did reduction of caliber of posterior urethra and bladder neck along with CT followed by augmentation after 6 months. All 18 patients are maintaining dry period for 24 h. Two patients had enuresis but are manageable with partial fluid restriction from evening. Seventeen out of 18 patients are maintaining their renal functions. No tension abdominal wall closure with rectus muscle apposition is essential to preserve repaired bladder exstrophy. Osteotomy prevents lateral drag to overcome failure of whole reconstruction. Increased "systolic" vesical pressure from contraction of small bladder might destroy the mechanism of continence and renal functions. Hence, venting of vesical pressure through CT is obligatory till augmentation which is of necessity to be done as early as possible to create a low-pressure continent system.

Our Neonatal Surgery Experiences in Somalia.

Purpose:The aim of this study is to report surgical outcomes of the neonates who have undergone various surgical procedures.Materials and Methods:In this retrospective study, 39 neonates who have undergone a surgical procedure in Mogadishu Somalia Turkey Recep Tayyip Erdoğan Training and Research Hospital, between October 2018 and March 2019 were included. Data regarding age, gender, diagnosis, surgical procedure, length of hospital stay, mortality, and cause of mortality were recorded.Results:Of 39 neonates, 12 were female (30.7%) and 27 were male (69.3%). The mean age of the neonates at admission was 7.7 ± 7.6 days (1–30 days) days. The most common diagnoses were anal atresia (n = 12, 30.8%), esophageal atresia (n = 9, 23.1%), and pyloric stenosis (n = 5, 12.8%). The most common surgical procedures were colostomy creation (n = 10, 25.6%), esophageal anastomosis (n = 9, 20.5%), primary closure of anterior abdominal wall defects including bladder exstrophy (n = 6, 15.4%), and pyloromyotomy (n = 5, 12.8%). Mortality rate was 17.9%, and mortality causes were sepsis (n = 4, 57.1%) and congenital heart disease (n = 3, 42.9%). Neonates with the highest mortality by underlying primary surgical diagnosis were esophageal atresia (n=4, 57.1%).Conclusion:The mortality rate from the surgical procedures of the neonates in Somalia is extremely high when compared with the developed countries. Employment of experienced pediatric surgeons and well-trained nurses, strict attention to the sanitary measures and shortening the time from birth to presentation might improve the surgical outcomes of the neonates in Somalia.

The Short-Term Complications of Augmentation Cystoplasty: A Single Center Experience

Objective: To evaluate the short-term complications of various cystoplasty techniques in pediatric population and determine the predicting factors. Material and Methods: The data of 206 patients (94 males, 112 females) who underwent augmentation cystoplasty between 1991-2016 were analysed retrospectively. Short-term complications within 30 days were evaluated according to the modified Clavien classification system. Grade III, IV, and V were grouped major complications. Chronic renal Disease (CRD) was defined as increasing serum creatinine levels above the normal age-adjusted values. Clinical factors and associated procedures to the surgery (Mitrofanoff procedure, bladder outlet procedures, ureteral anti-reflux surgery) were evaluated to predict overall and major complications. Results: The mean age was 9.8±4.7 years and female/male ratio was 112/94. Overall complication rate was 32% (66 patients) and distributed to Clavien class I, II, IIIb, IVa as 18 (8,7%), 33(16%), 14 (6,81%), 1 (0.48%) patients; respectively. There was no mortality. Age, PUV aetiology and CRD were significant predictor of complication on univariate analysis (p=0.026, p=0.048 and p=0.045, respectively). Age and preoperative CRD were significant on multivariable analysis (p=0.015, OR=0.918 and p=0.046, OR=2.134). Major complications occurred in 15 (7.3%) patients. Bladder exstrophy epispadias complex (BEEC), preoperative CRD, anti-reflux surgery, ileal augmentation were significant parameters on univariate analysis (p=0.007, p=0.003, p=0.043, and p=0.022, respectively). For major complications where BEEC, CRD and ileal AC were significant parameters on multivariate analysis (p=0.009, OR=7.837; p=0.003, OR=9.649, p=0.047, OR=4.241, respectively). Conclusion: Augmentation cystoplasty and associated procedures have acceptable complication rate for pediatric group. Preoperative renal disease and younger age are associated with complications. For major complications, Bladder exstrophy epispadias complex, ileal augmentation and preoperative renal disease are risk factors.

Клинический случай создания гетеротопического мочевого резервуара с выведением континентной стомы у пациента с экстрофией мочевого пузыря

This article presents a clinical case of a 32-year-old patient who underwent surgery for bladder exstrophy (uretero-ileostomy) at the age of 1 year 8 months. By the time of admission, the patient had already undergone percutaneous nephrolithotripsy twice on the right side for staghorn stone in the right kidney, suffered from recurrent chronic pyelonephritis, bilateral ureteral strictures and bilateral ureterohydronephrosis. Conversion of ureterosigmostomy to heterotopic urinary reservoir with continent stoma diversion was performed with satisfactory results.

Delayed primary closure of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to neonatal surgery

BackgroundSuccessful primary closure of bladder exstrophy is of utmost importance for bladder capacity and urinary continence. We evaluated our concept of delayed primary closure that challenges the role of neonatal surgery, pelvic osteotomy, and perioperative pain management. Material and MethodsWe reviewed the medical records of patients with classic bladder exstrophy (CBE) who had undergone delayed primary closure without osteotomy at our institution between January 2008 and May 2020. Data to be analyzed included patient demographics, intraoperative pelvic laxity, blood transfusion, postoperative ventilation time, requirement of pain medication, time to full feeds, length of ICU stay, postoperative complications, and total hospital stay. Results66 patients (44 boys) met the inclusion criteria. Mean age at surgery was 64.8 days (SD±24.7). Pelvic approximation < 5 mm was possible in 66 (100%) patients. Blood transfusion was required by 31 (47%) patients. 14 (21.2%) patients needed postoperative ventilation for a mean time of 2.7 h. 45 (68.2%) children required intravenous opioids in addition to an epidural catheter. Oral feeding started on average 17.6 h after surgery. Mean ICU stay was 1.3 day. The initial success rate of delayed closure was 93.9%. None of the patients had bladder dehiscence. Girls developed more often minor postoperative complications than boys (m/f: 12 [27.3%] vs. 8 [36.4%]. Mean overall time of hospitalization was 19 days (13–34 d). ConclusionDelayed primary closure of CBE without osteotomy but with continuous epidural blockage is a safe and promising procedure that has crucial advantages in the pre- and postoperative management of CBE. Level of EvidenceLevel III.

Delayed surgical treatment of children with exstrophy without approximation of the pubic bones, the use of displaced flaps and immobilization of the patient

BACKGROUND: Bladder exstrophy is one of the most difficult for the reconstruction of malformations.&#x0D; AIM: This study aimed to evaluate the outcomes of delayed bladder closure in exstrophy in comparison with procedures in newborns and to test the hypothesis that the reliability of bladder closure does not decrease without closing the fascial defect between the rectus abdominis muscles and that the postoperative period is more favorable.&#x0D; MATERIALS AND METHODS: Delayed closure of exstrophy without osteotomy, convergence of pubic bones, or flap movement was performed in 46 patients with classic bladder exstrophy. Among them, 34 were primary patients, and the remaining patients, who had a complete relapse of exstrophy as a result of suture divergence, had undergone unsuccessful surgery in other clinics 14 times (n = 12). In total, 25 were boys and 21 were girls who underwent surgery from 2006 to 2021.&#x0D; RESULTS: Delayed bladder closure was successful in all 46 children (100%). No signs of acute pain were noted. Serious complications such as dehiscence of the sutures in the bladder and proximal urethra were not observed. Minor complications occurred in five patients, which resolved promptly.&#x0D; DISCUSSION: In the treatment of exstrophy, successful primary bladder closure is critical to achieving future urinary continence. The elimination of the defect between the rectus abdominis muscles with the convergence of the pubic bones has significant drawbacks, including an increase in the operating time and trauma, need for blood transfusion, risk of orthopedic and neurological complications, and a more difficult postoperative period. The vast majority of the patients develop rediastasis. The authors proposed a more reliable method of delayed closure of exstrophy.&#x0D; CONCLUSIONS: The presented method of bladder closure was 100% successful. The procedure was simplified technically, the surgery time was reduced, and the postoperative period proceeded more easily without prolonged pain syndrome, prolonged use of analgesics, and, in most cases, without blood transfusions.

Primary urinary bladder adenocarcinoma with vaginal invasion

Primary urinary bladder adenocarcinoma is not a common tumor, it comprises between 0.5 and 2% of all bladder tumors. It has been linked to bladder exstrophy, chronic irritation, and pelvic lipomatosis. Next, the case of a 62-year-old female patient with primary urinary bladder adenocarcinoma and vaginal wall invasion is presented.

Histopathology of intersymphyseal band and its correlation with continence in bladder exstrophy repair

BackgroundUrinary incontinence is a major problem in operated exstrophy patients. Most of the repairs described in literature stress on the importance of dividing the intersymphyseal band (ISB) to place the bladder in the pelvis. But the origin of this band and its importance has hardly been discussed in literature. The purpose of this study is to establish the nature of tissue the ISB is composed of. This can be used to determine its role in the surgical management of exstrophy epispadias complex (EEC) patients.ResultsThirty out of 33 operated patients demonstrated smooth muscle with/without fibrous tissue in the sections taken through the ISB.A significant percentage of patients (χ2= 38.319, p < 0.0001) in whom this band was reconstructed around the bladder neck gradually became continent/partly continent with an increase in the dry interval with time.ConclusionIt can be a considerable factor to pay attention to the step of wrapping the ISB around the bladder neck during EEC repair. This serves to function as the smooth muscle of the bladder neck as proven histologically in our operated patients. It may have a role to support future continence in these patients.

Rare Variants of Bladder Exstrophy-Epispadias Complex: A Report of Two Cases

Exstrophy-epispadias complex encompasses a spectrum of pathologies ranging from isolated epispadias to classical bladder exstrophy to cloacal exstrophy as the most severe and the rarest variant, are quite uncommon and constitute about 8% of all the cases. The treating surgeon should be aware of all the variants, their diagnosis and modalities of treatment for the same. We present two such unique variants and successful surgical management of the same.

Bladder exstrophy: We need to improve. A lot

Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients' point of view are needed to get a real-life perspective on the problem. A self-developed questionnaire concerning sexual, psychosexual and psychosocial outcomes was sent to the adult members of the Brazilian Exstrophy Group. Fifty out of 67 adults from the group (74.5%) responded to the questionnaire. Failure of initial bladder closure attained 62%. Almost ¾ of the patients had augmentation cystoplasty. Bladder lithiasis was common. Esthetic procedures were frequently done. Repetitive UTI (n=32, 64%) and kidney scars/disease (n=20, 40%) were frequent. Most (88%) patients either depend on CIC or remain incontinent. Sexual problems predominated in males. Surgery for continence often failed, requiring re-operations, but the prognosis without these procedures was comparatively worse. Continent patients underwent more surgeries (mean 18, 13 and 9 procedures in continent, imperfectly continent and incontinent patients, respectively). Augmented patients more frequently achieved dryness (p=0.0035). Two-thirds of the women underwent vaginoplasties, but dyspareunia/feeling of "tight" vagina still affected a quarter of them. Four women (15.4%) delivered healthy children. 91.7% of the males reported "normal" erections, but sexual inhibition was common due to feeling of having a small penis (n=18, 75%). Persistent dorsal curvature and abnormal ejaculation were common (58.3% and 77.1%, respectively). Patients' comments related mainly to mental health issues/need for specialized care, limitations of medicine to cure/treat their disease, unavailability of experts, especially adult specialists, embarrassment over deformities and insufficient information about disease/treatment/prognosis. Most BE patients are well-integrated into society, but feelings of sadness and low self-esteem are common. Most welcome procedures to become dry, despite self-catheterization. The results of bladder neck reconstruction are far from perfect, despite multiple attempts and bladder augmentation was often necessary. Volitional voiding is uncommon. Sexual problems are worse for males, and sexual avoidance is common. Sexual function and self-image are inter-related. It seems reasonable to offer selective esthetic procedures to improve social/sexual interaction. Obstetric complications are common, especially UTI, need for ureteral and/or conduit stenting, abnormal fetal positioning, uterine prolapse, technical problems during surgical deliveries and prematurity. Continence/dryness in BE was mostly eventually achieved, usually depending on multiple interventions, bladder augmentation and self-catheterization. Despite multiple surgeries many adults remain incontinent. Sexual problems and avoidance are the rule in males, due to the feelings of penile inadequacy. Pregnant females deserve expert obstetric care.

Duplicate Bladder Exstrophy With Fistula: A Rare Variant of Exstrophy-Epispadias Complex

Variants of exstrophy are rare anomalies seen in the spectrum of bladder exstrophy-epispadias complex. A seven day old male neonate was admitted in our department with a rare variant of duplicate bladder communicating with exstrophy bladder with fistula. Patient presented with patch of exstrophic bladder just below the low set umbilicus with a normal sized phallus and normally positioned urethral meatus. The baby was managed successfully with surgery and is under regular follow up.

Rare Variants of Bladder Exstrophy-Epispadias Complex: A Report of Two Cases

Exstrophy-epispadias complex encompasses a spectrum of pathologies ranging from isolated epispadias to classical bladder exstrophy to cloacal exstrophy as the most severe and the rarest variant, are quite uncommon and constitute about 8% of all the cases. The treating surgeon should be aware of all the variants, their diagnosis and modalities of treatment for the same. We present two such unique variants and successful surgical management of the same.

A Prevalence Estimation of Exstrophy and Epispadias in Germany From Public Health Insurance Data

Introduction: The prevalence of rare diseases is very important for health care research. According to the European Surveillance of Congenital Anomalies (EUROCAT) registers, the live prevalence for exstrophy and/or epispadias (grades 1–3) is reported with 1:23,255 (95% CI: 1:26,316; 1:20,000). A Europe-wide prevalence evaluation based on reports from excellence centers estimates a prevalence for exstrophies of 1:32,200 and for isolated epispadias of 1:96,800 in 2010. However, the frequency of exstrophy [International Statistical Classification of Diseases and Related Health Problems revision 10 (ICD-10): Q64.1] and epispadias (ICD-10: Q64.0) treated in different age groups in Germany remains unclear.Material and Method: Public health insurance data from 71 million people (approximately 87% of the population) were provided by the German Institute for Medical Documentation and Information (DIMDI) in accordance to the German Social Insurance Code for this research purpose. DIMDI analyzed the data source for the ICD diagnoses exstrophy and epispadias between 2009 and 2011. As provided data were robust over the years, averaged data are mentioned. Detailed subgroup analysis of small numbers was forbidden due to privacy protection.Results: Annually, 126 persons of all ages with epispadias and 244 with exstrophy are treated as inpatients. In the observed population, 34 infants (<1 year of age) with epispadias and 19 with exstrophy (58% male) are treated as outpatients each year. This corresponds to an estimated live prevalence of 1:11,000 (95% CI: 1:14,700; 1:8,400) for EEC (exstrophy–epispadias complex), more specifically a prevalence of 1:17,142 for epispadias and of 1:30,675 for exstrophy. The male-to-female ratio for exstrophy is 1.4:1 for infants and 1.6:1 for all minors. In children and adolescents, 349 epispadias and 393 exstrophies (up to the age of 17) are treated annually, whereas adults with exstrophy and even more with epispadias make comparatively less use of medical care.Conclusion: With the help of DIMDI data, the live prevalence of bladder exstrophy and epispadias in Germany could be estimated. The prevalence of epispadias was higher than in previous reports, in which milder epispadias phenotypes (grade 1 or 2) may not have been included. These analyses might enlighten knowledge about nationwide incidence and treatment numbers of rare diseases such as the EEC.