Skip to main content

Bladder Exstrophy-Epispadias Complex

The bladder exstrophy-epispadias complex (BEEC) is a rare congenital spectrum of genitourinary malformations (prevalence about 1 in 10,000 births in the sources cited here) that requires lifelong multidisciplinary care. The EAU 2026 paediatric guideline gives a lower prevalence of 1 in 30,000–50,000 live births for epispadias plus classic exstrophy, with male predominance; estimates differ with how the spectrum is defined. As childhood survivors reach adulthood in growing numbers, adult and transitional urology carries a larger share of their management.[1][2][45]

A European consensus favors specialist centers for patients needing lifelong congenital urologic care; the Transitional Urology hub addresses the broader care handoff.[2]

Spectrum and Overview​

BEEC spans a range of severity: isolated epispadias (mildest), classic bladder exstrophy (CBE), and cloacal exstrophy / OEIS complex (most severe). The defect involves the urinary tract, abdominal wall, musculoskeletal pelvis, pelvic floor, and genitalia, and sometimes the spine and anus.[1][3] Management is primarily surgical, beginning in the neonatal period, with the staged goals of abdominal-wall closure, urinary continence, renal preservation, and genital reconstruction.[1] The 2024 German S3 BEEC guideline recommends latex-free examination and treatment (grade A; evidence level 3), with dermatologic allergy evaluation when the history includes coexisting atopy and previous allergic reactions.[66]

What the primary newborn repair means for the adult patient. Two primary-repair strategies have been described, and knowing which one a given adult patient had helps interpret their anatomy and bladder history. A staged approach closes the bladder and posterior urethra first, repairs epispadias at about 6-12 months, and defers bladder-neck reconstruction until capacity and development allow it;[60] complete primary repair instead combines bladder closure with penile disassembly and urethral reconstruction in a single newborn operation. When the reconstructed urethra cannot reach the glans in that single stage, a ventral hypospadias is left and matured for a later graft urethroplasty, so an adult can present with a hypospadias-pattern meatus or urethroplasty scar that traces back to this staged completion rather than to a separate hypospadias diagnosis.[61][62] Separately, a longitudinal cohort found that delaying primary bladder closure beyond the newborn period was associated with lower achieved bladder capacity than neonatal closure, which matters when an adult's childhood closure was delayed and a continence procedure now depends on that capacity.[63]

Urinary Continence and Lower Urinary Tract Function in Adults​

Continence is the dominant issue in transitional care, and most patients require multiple procedures over a lifetime.

  • Few void normally per urethra. Only about 25% of classic bladder exstrophy patients are expected to void per urethra without catheterization or diversion.[4] A French long-term follow-up study of 42 questionnaire respondents among 63 eligible patients (median follow-up 22 years) found that 21.4% voided spontaneously per urethra while 76.2% performed clean intermittent self-catheterization (CISC).[4][5]
  • The need for augmentation or diversion rises with age. The estimated probability was 50.7% by age 10 and 70.1% by age 18 in a 36-year multi-institutional cohort; among 76 adults without diversion, 85.5% performed CIC.[6]
  • LUTS are common but under-recognized. In a 32-patient questionnaire study, moderate-to-severe symptoms were reported by 12/15 males (80%) and 6/9 females; most became apparent only on a detailed questionnaire.[7]
  • Continence differs by procedure. In one institutional cohort, continence was 93% after bladder neck closure with a continent catheterizable stoma (most patients also had augmentation) and 64% after isolated bladder neck reconstruction.[4]

Continence Surgery — Approach and Outcomes​

For an incontinent adolescent or adult with BEEC seeking continence surgery, the 2024 German S3 guideline recommends assessment of the upper urinary tract, bladder by urodynamics and cystoscopy, and psychosocial situation (grade A; evidence level 4), alongside prior reconstruction, patient goals, and ability to catheterize. After physiological continence age, bladder capacity <85 mL calls for BNR or BNC only with augmentation and a stoma to protect the upper tracts (grade A; evidence level 3).[66]

ProcedureContinenceNotes
Isolated bladder neck reconstruction (BNR) — techniques not separated in the cited cohort≈ 64% (91/142; dry interval of ≥3 hours without nighttime leakage)Final continence procedure in 162/432 (37.5%) of the cited cohort. In a separate redo-BNR series, failure of initial BNR may reflect inadequate capacity / compliance; no redo-BNR patient voided per urethra without CIC[4][8]
BNR + augmentation cystoplasty ± continent catheterizable stoma38/62 (61.3%) in the cited classic-exstrophy cohort after BNR with augmentation and/or a catheterizable stoma, versus 91/142 (64.1%) after isolated BNR[4]In a 30-patient redo-BNR series, the authors suggested that initial failure may reflect inadequate capacity or compliance and recommended considering augmentation with redo BNR.[8]
Bladder neck closure (BNC) + continent catheterizable stoma93% (124/133; 95% CI 87–97%)Higher observed continence in the cited cohort; 165/173 in this final-procedure group also underwent augmentation. Requires reliable lifelong catheterization and a safe reservoir; counsel men before BNC about possible ejaculatory and erectile dysfunction (2024 German S3 expert consensus).[4][66]
Endoscopic bulking (dextranomer/hyaluronic acid)In this pediatric cohort, 5-year dry survival was 44% (male epispadias) and 23% (female epispadias/exstrophy); failure-free survival, also counting significant improvement, was 70% and 45%Fifty-eight children received 105 injections; 33/58 (57%) received multiple injections. After prior BNR, 5-year failure-free survival was 58% in the female epispadias/exstrophy group and 75% in male epispadias; complications occurred in 9%.[10]
Artificial urinary sphincter (AS792)10/11 totally continent (91%; 1983 series)Historical result in a small implanted cohort; not a contemporary comparative estimate[11]

Isolated BNR has been described as removing approximately 30 mL of bladder capacity; on that basis it has been favored for a growing, reasonably capacious bladder with low residual urine, with consideration of whether the patient can store urine for roughly 2-3 hours afterward and empty regularly against the reconstructed outlet. These are the cited authors' planning estimates rather than a validated numerical eligibility threshold; current bladder safety, prior repairs, emptying ability and patient goals remain decisive.[54]

Continent Urinary Diversion — Options and Long-Term Complications​

When the native bladder cannot be preserved or is inadequate, continent diversion is performed. These reservoirs commit the patient to lifelong surveillance.

  • Continent catheterizable pouches (Penn, Indiana, Mainz): in a 25-patient untreated-adult series, 18 selected a continent pouch and all were continent at mean 6.5 years.[12] A separate 33-patient benign-indication Indiana-pouch cohort, not specific to BEEC, had mean revision-free survival of 198 months, but 67% required at least one revision over median 258 months.[12][13]
  • Continent anal diversion. In a 45-year experience, 32/57 eligible patients were assessed at median 23.9 years: 97% were daytime continent and 41% received prophylactic alkaline substitution; secondary rectal-reservoir malignancies and adverse sexual-function outcomes were also reported.[14] For BEEC patients with ureters implanted into bowel, the 2024 German S3 guideline recommends complete removal of the ureteral stump during ureteral reimplantation or diversion conversion (expert consensus).[66]
  • Ileal conduit. The simplest option for patients with limited healthcare access, inability or preference not to perform self-catheterization, or significant comorbidity.[12][15] Neoplasia has been reported after urinary diversion in exstrophy, chiefly when urine and feces mix.[20]
  • Stomal stenosis is the most common long-term stomal complication (25% at median 1.9 years). Monofilament rather than multifilament umbilicoplasty suture was associated with less stenosis in the retrospective study (p = 0.009); scar contractures respond to stomal incision, keloids to excision and local tissue rearrangement.[16]
  • Bladder/pouch calculi occurred in 24/91 (26%) patients with continent diversion at mean six-year follow-up, alongside stomal stenosis in 21/91; stones recurred in nine.[17] In a 2024 BEEC series of 259 augmented patients, 56 (21.6%) formed bladder stones and 74% of stone formers required a second stone operation, a median 1.4 years after the first.[69]
  • Bladder perforation was reported in 2/91 patients in the same series.[17]
  • A mixed-diagnosis cohort of 173 catheterizable channels had a 53% revision rate at median 12.4 years; revision does not necessarily mean loss of the channel.[9]

Augmentation Cystoplasty — Metabolic and Renal Monitoring​

Any bowel segment incorporated into the urinary tract obliges lifelong metabolic surveillance:[18]

  • Hyperchloremic metabolic acidosis. The most common metabolic complication, particularly with ileal or colonic segments; monitor bicarbonate and treat clinically relevant acidosis; routine prophylaxis is not necessary for every reconstruction.
  • Vitamin B12 deficiency. Risk depends on ileal location/length, prior resection and time since surgery; no single length threshold excludes risk. Monitor B12 over lifelong follow-up.
  • Bone demineralization. Chronic acidosis is associated with bone loss; consider DEXA.
  • Renal function. In a 43-patient predominantly pediatric cross-sectional study (median age 9), 74% had at least one sign of kidney injury (elevated BP, reduced eGFR, or proteinuria); recurrent UTI/bacteriuria in 44% and kidney or bladder stones in 29%.[19] In a five-center classic-exstrophy cohort, 3/201 developed end-stage kidney disease at a median age of 23.4 years, and survival analysis estimated 5% risk by age 30; among 141 with eGFR data, 8% of adults had CKD stage 3 or higher.[67] A separate retrospective series of 22 children after primary closure and isolated BNR found an average eGFR decline of 30 mL/min/1.73 m² before age 18, with five meeting CKD criteria; 16 later underwent augmentation or diversion over median 16-year post-BNR follow-up.[68] Two older cohorts give a concrete sense of how often the surveillance findings above are actually seen: in a 23-patient cohort followed after complete primary repair, 5 had episodes of pyelonephritis and 5 had cortical defects on renal scan; in a 57-patient cohort followed after bladder neck procedures (27 with closure and 38 with augmentation), renal scarring was found in 14, and one patient each developed renal insufficiency or progressed to renal transplantation.[48][49]
  • EAU/ESPU neurogenic-bladder guidance for children and adolescents (an adjacent population) lists physical exam, renal ultrasound, blood gas (pH and base excess), renal function, and vitamin B12 if ileum was used.[18]

See also the broader augmentation surveillance discussion on the transitional-urology hub.

Malignancy Risk and Surveillance​

Adults with BEEC have an elevated bladder-malignancy risk. A historical 103-patient BEEC cohort born before 1964, with complete follow-up for only 61, produced the often-cited nearly 700-fold relative bladder-malignancy estimate.[20][21]

  • Adenocarcinoma is the most common histology, though squamous cell and urothelial carcinoma also occur.[20][22] A systematic review cited by the EAU 2026 paediatric guideline found adenocarcinoma in 68% of tumors, a male-to-female ratio of 2.7:1, and diagnosis at age 45–67 in 56% and before age 45 in 37%.[45] The guideline states that no standardized surveillance protocol has been established.[45]
  • Risk is highest with ureterosigmoidostomy or other diversions mixing urine and feces (38% benign or malignant neoplasia in the historical cohort). The reported 3.3% malignant-neoplasia rate in the group without mixing included bladder and kidney cancer; it is not a 3.3% bladder-cancer estimate.[20]
  • Severe epithelial changes such as cystitis glandularis and intestinal metaplasia were found in up to 62% of exstrophic bladders several years after primary closure; their malignant potential remains uncertain.[23]
  • A matched congenital-bladder study (153 augmented and 153 controls) found no statistically significant cancer-incidence difference (4.6% versus 2.6%).[24] Transplant immunosuppression was associated with more malignancies.[24]
  • Lifelong specialist follow-up is warranted, with prompt evaluation of hematuria, unexplained recurrent symptomatic infection, pain or other concerning changes. Individualize malignancy surveillance by reconstruction and symptoms: the 2024 German S3 BEEC guideline recommends annual bladder endoscopy beginning 10–15 years after bowel augmentation and annual endoscopic inspection of the ureteral implantation site from postoperative year 10 after continent anal diversion (expert consensus).[66] For a colorectal reservoir exposed to both urine and feces, the cited cohort supports annual endoscopic colorectal surveillance.[20]
  • Evidence from one enterocystoplasty surveillance cohort. A single-center cohort of 65 patients placed on annual cytology and endoscopy beginning 10 years or more after enterocystoplasty illustrates the tradeoff: over the first 5 years, 10.5% of cytology results were suspicious for cancer but no malignancy was found on further evaluation (specificity 90%; sensitivity could not be determined because no tumors developed), and endoscopy identified 4 lesions in 250 examinations, none malignant (1 adenomatous polyp, 1 squamous metaplasia, 2 nephrogenic adenomas).[59] Citing the low event rate, false-positive burden and cost, the authors recommended discontinuing routine annual cytology and endoscopy after the initial evaluation period while continuing annual clinical follow-up; no tumor developed over a median 15-year surveillance interval in this cohort.[59] A later EAU/ESPU neurogenic-bladder guideline, addressing an adjacent population, says annual cystoscopy may be considered after 10–15 years but that cytology is not useful after bowel incorporation.[18][59]

Abdominal Wall Reconstruction​

Closing the anterior abdominal-wall defect in adults is a substantial challenge:

  • Closure without osteotomy is possible in selected adults. Small series describe closure without pelvic osteotomy using the vesical-plate muscular coat and/or multilayered flap reconstruction.[25][26]
  • Multilayered reconstruction with superficial fascial and myofascial flaps (rectus anterior sheath, external oblique aponeurosis) gave reliable closure with no recurrent dehiscence in a 12-patient series.[26]
  • Primary abdominal-wall closure was reported in all but three patients in one 25-adult untreated-exstrophy series.[12]

Genital Reconstruction in Males​

Penile inadequacy is a major adult concern:

  • Genitoplasty (including corporeal mobilization, chordee correction, and dermal grafting) was followed by parent/patient-rated esthetic satisfaction in 60/65 patients (92%) in one series; 19 revision procedures were reported, with four patients undergoing multiple revisions.[27]
  • Substitution phalloplasty is used for severe inadequacy when local tissue is insufficient. The radial forearm free flap is the dominant technique (89% of cases in a systematic review), in 47 patients across seven low-quality studies. The reported 15% flap/procedure figure must not obscure urethroplasty complications in 12/22 (54%) and prosthesis complications in 8/32 (25%), mainly erosion.[28]
  • An 11-patient institutional phalloplasty series (8 CBE, 3 cloacal exstrophy) reported survival of all six radial forearm and five pedicled anterolateral thigh flaps, with inflatable penile prostheses placed in five; among the thigh-flap recipients, one died of pulmonary embolism and the other four required at least one debulking procedure.[29]
  • Recurrent chordee can appear with growth. One author's follow-up discussion of a childhood functional epispadias repair warns that later penile growth can reveal recurrent upward chordee that was not apparent at the original operation; no denominator is given for this observation, but it supports reassessing curvature at puberty and in adulthood rather than assuming a childhood repair is durably straight.[64]

Sexual Function and Fertility​

  • In a questionnaire survey with 32 of 63 BEEC invitees responding, erectile-function domains among sexually active men and sexual-function domains among responding women were broadly comparable to controls (women had a higher median total FSFI score); 35% of BEEC respondents versus 11% of controls had not become sexually active, and 22% versus 45% had children.[30] A separate 17-adult BEEC cohort found broadly similar sexual-function scores except for the female pain domain.[31]
  • Male fertility is frequently impaired. Oligoasthenoteratozoospermia was found in 71% of a selected 39-man follow-up cohort with a history of urinary diversion[32], and 50% of the men who fathered children in a separate cohort (31.6% of respondents were fathers) used assisted reproductive technology[33]. Early sperm banking should be discussed.[32][33]
  • In one selected 29-patient series that included four women, both women who tried to conceive succeeded (2/2); obstetric management must still account for pelvic anatomy and prior surgery.[34]
  • In a selected 29-patient series (25 men, four women), 96% of men underwent genital surgery (84% penoplasty, 52% phalloplasty) and 25% of women underwent introitoplasty.[34] An older, small series of eight women with bladder exstrophy who underwent vaginal-orifice enlargement, with bifid-clitoris reapproximation in five, reported dyspareunia requiring a repeat enlargement procedure in one; the authors advised against extending the perineal incision too far posteriorly, given a possible link to later uterine prolapse, and noted postoperative dilator therapy as a useful adjunct.[52]

Pregnancy and Obstetric Management​

Pregnancy in women with BEEC is high-risk and requires multidisciplinary planning:

  • Fertility estimates vary considerably between small, selected cohorts; assess the individual reproductive anatomy and history.[35][34]
  • In a selected cross-sectional cohort of 52 women with classic exstrophy, 19 reported 57 pregnancies, including three twin gestations; 21 miscarriages and four stillbirths or neonatal deaths were recorded.[35]
  • Mode of delivery. In a 52-woman classic bladder exstrophy series, the authors considered planned cesarean the most appropriate mode in most cases.[35] The 2024 German S3 BEEC guideline recommends cesarean after lower urinary tract reconstruction to protect pelvic-floor support (grade B; evidence level 3).[66] Vaginal delivery has been reported in selected cases.[36] A 2024 case report describes multidisciplinary delivery planning around reconstructed urinary anatomy.[37]
  • A systematic review covering 292 pregnancies across several reconstructed-bladder diagnoses, including exstrophy, reported febrile UTI in approximately 33% and upper-tract drainage in 11%; these are not BEEC-only estimates.[38] Uterine prolapse is an additional concern: pelvic organ prolapse was found in 20/67 (29.9%) postpubertal women with classic bladder exstrophy in one series[46], while 37/130 (28.5%) women in a social-media-recruited cohort reported treatment for uterine prolapse[47]. These selected cohorts measured different outcomes and should not be pooled as a single prolapse prevalence. In the larger cohort, pelvic osteotomy was not associated with a lower prolapse risk on multivariate analysis; only the degree of pubic diastasis was, with a wider diastasis carrying higher risk.[46][47] An MRI comparison of 10 postpubertal women with classic bladder exstrophy and 20 controls offers an anatomic explanation for this vulnerability: a smaller proportion of the levator ani lay in the anterior compartment in the exstrophy group, alongside a wider symphyseal diastasis and a wider ileococcygeal angle, leaving relatively less support for the anterior compartment containing the bladder, vagina and uterus.[55] Surgery for vaginal narrowing sufficient to interfere with tampon use or penetrative intercourse was reported by 56.9% (74/130) of women in the same patient-reported cohort, a higher rate than estimates drawn only from surgical series.[35][38][47]
  • Catheterizable-channel access late in pregnancy. The gravid uterus can stretch or distort a continent catheterizable channel or reservoir efferent limb, causing tortuosity and difficulty with clean intermittent catheterization in the third trimester and the first weeks postpartum. In the 292-pregnancy systematic review above, catheterization difficulty was reported in about half of pregnancies with a continent catheterizable channel, more often with a right-iliac-fossa stoma than an umbilical stoma, though no site-specific denominator is available; a temporary indwelling catheter through the channel can relieve the problem, with intermittent catheterization usually resumed within a few weeks after delivery.[38] In a separate single-center series of 25 women who became pregnant after urinary diversion performed at a young age (37 pregnancies, a mix of continent cutaneous diversion, continent anal diversion and colonic conduit; not exstrophy-only), 4 of 25 required an indwelling catheter because of difficulty with intermittent catheterization. Treat this as an individualized contingency rather than an expected course.[53]
  • Urine pregnancy testing after enterocystoplasty. Urine hCG tests can give a false-positive result in patients with an enteric reservoir, possibly because reservoir mucus interferes with the test reagent; an early study of 23 patients with an enterocystoplasty found a positive over-the-counter urine test in 14 (one was a true pregnancy), a false-positive rate of 57%.[56] More recent evidence complicates a blanket warning: false positives appear to depend on the test's hCG sensitivity threshold (a pediatric cohort found 75% false positives with an ultra-sensitive over-the-counter test, but none with a less sensitive one)[57], and a 10-year single-center review of 244 in-hospital urine pregnancy screens in patients with bowel-containing urinary reconstruction found zero false positives.[58] When pregnancy status must be confirmed or excluded with confidence, a serum hCG test remains the more reliable option, particularly for a home or over-the-counter urine result.[56][57][58]
  • Delivery should occur at a tertiary center with urology coverage, with incision type and awareness of reconstructed reservoirs planned with the urological team in advance.[37][38]

Psychosocial and Quality of Life​

  • Generic QoL scores approximated population norms in small adult BEEC surveys, whereas a 21-study systematic review found lower pooled health-related QoL in the five studies eligible for meta-analysis.[31][39][40]
  • Mental-health concerns. In one survey of patients operated for bladder exstrophy or epispadias, 7 of 32 respondents (about 20%) had high psychiatric symptom-screen scores suggesting possible mental-health problems.[39] Depressive symptoms and anxiety were reported in a separate 27-patient series[41], and low self-esteem about sexual relationships was reported in a male cohort.[33] Dissatisfaction with genital appearance and urinary incontinence were associated with reduced QoL in the cited studies.[39][41][42]
  • Caregiver burden is significant (mean Zarit score 36.4), underscoring family-centered support.[41]
  • Multidisciplinary psychosocial support (behavioral health, peer support, educational consultation, and formal transition planning) is recommended in a review of family-centered exstrophy care.[43]
  • Orthopedic sequelae of the underlying bony pelvic anomaly, and of childhood pelvic osteotomy, persist into adulthood: gait analysis in 19 patients with bladder exstrophy (mean age 14 years) showed abnormal trunk, spine, pelvis, knee and foot kinematics compared with controls[50], and a survey answered by 33 of 63 adult patients found more low-back pain and reduced running ability in bladder exstrophy than in isolated epispadias, with hip pain reported by 45% of osteotomized bladder-exstrophy respondents (mainly women) versus none of those without osteotomy[51]. Awareness of this non-urologic burden supports orthopedic referral as part of multidisciplinary lifelong care.[50][51]

Management of Previously Untreated Adults​

In resource-limited settings, some patients present with untreated BEEC in adulthood:

  • Continent catheterizable pouch (e.g., Penn pouch): preferred by most patients, with excellent continence.[12]
  • Cystectomy with ileal conduit is a simpler option for patients with limited healthcare access or inability or preference not to perform self-catheterization.[12][15]
  • Bladder preservation with ileocystoplasty and bladder-neck reconstruction is feasible if random biopsies show no significant dysplasia, with an individualized lifelong malignancy follow-up plan; this evidence is limited to four reported patients.[44]

Lifelong Surveillance​

DomainFrequencyMethod
Renal function + upper-tract imagingIndividualized for BEEC; use NLUTD risk strata when applicableAssess renal function with creatinine/eGFR and the upper tracts with ultrasound; pediatric BEEC and neurogenic-bladder sources support monitoring but establish no universal adult BEEC interval.[18][19] For stable adult NLUTD, AUA/SUFU recommends annual renal assessment and imaging every 1–2 years at moderate risk, or both annually at high risk (Moderate Recommendation; Grade C).[65]
Metabolic panel (if bowel incorporated)Lifelong; interval individualizedRenal function, electrolytes/bicarbonate and B12 when ileal risk is present; blood gas if needed to clarify acid-base status[18]
Malignancy surveillanceReconstruction-specificIndividualized BEEC plan and symptom-triggered investigation; annual colorectal endoscopy for urine–feces reservoirs[20]; the 2024 German S3 BEEC guideline recommends annual bladder endoscopy beginning 10–15 years after bowel augmentation and annual endoscopic inspection of the ureteral implantation site from year 10 after continent anal diversion (expert consensus).[66]
Stomal assessmentEach visitInspect stoma, troubleshoot catheterization[16]
Sexual / reproductive healthPeriodicallyCounseling; early sperm banking for males; semen analysis if fertility desired[32][33]
Obstetric planningChildbearing agePre-pregnancy multidisciplinary planning; tertiary-center delivery[37]
Psychosocial / mental healthPeriodicallyScreening + validated QoL instruments[40][43]

Key Principles​

  • Catheterization and further reconstruction are common. In two cohorts, about 21–25% voided per urethra without catheterization; in a separate multi-institutional cohort, 67.4% used CIC at last follow-up and the survival-analysis estimate for augmentation or diversion reached 70.1% by age 18.[4][5][6]
  • At last follow-up, 124/133 evaluated patients (93%) were continent after bladder neck closure plus a catheterizable channel, versus 91/142 (64%) after isolated BNR (continence: at least 3 dry hours without nighttime leakage).[4]
  • Bowel reconstruction requires lifelong follow-up for metabolic, stone and other complications. BEEC-related cancer risk is not attributable solely to bowel incorporation.[18][20]
  • Selected adult abdominal-wall defects can be closed without osteotomy; small series cannot determine the best approach for every pelvis.[25][26]
  • Discuss fertility early. Male fertility can be impaired: among 39 men with classic bladder exstrophy included after urinary diversion, 11 had fathered children.[32] In a separate survey of 38 men with classic bladder exstrophy or epispadias, 50% of the parents had used ART; the authors proposed early sperm storage.[33] In a 52-woman classic-bladder-exstrophy cohort, fertility was impaired and pregnancy was high-risk; the authors advised delivery at a tertiary obstetric unit with urology cover.[35][37] The 2024 German S3 BEEC guideline recommends cesarean after lower urinary tract reconstruction to protect pelvic-floor support (grade B; evidence level 3).[66]
  • Genital appearance and incontinence are associated with quality of life. Address them proactively alongside mental-health screening and caregiver support.[41][43]

See Also​

Videos​

Minimally Invasive Ancillary Surgery After Adult Primary Exstrophy Closure
Kalra S, et al. Urology Video Journal 2024 (CC BY-NC-ND 4.0)
Open source videoRead the article

References​

1. Ebert AK, Reutter H, Ludwig M, Rösch WH. "The Exstrophy-Epispadias Complex." Orphanet J Rare Dis. 2009;4:23. doi:10.1186/1750-1172-4-23

2. Wood D, Baird A, Carmignani L, et al. "Lifelong Congenital Urology: The Challenges for Patients and Surgeons." Eur Urol. 2019;75(6):1001-1007. doi:10.1016/j.eururo.2019.03.019

3. Lee T, Borer J. "Exstrophy-Epispadias Complex." Urol Clin North Am. 2023;50(3):403-414. doi:10.1016/j.ucl.2023.04.004

4. Maruf M, Manyevitch R, Michaud J, et al. "Urinary Continence Outcomes in Classic Bladder Exstrophy: A Long-Term Perspective." J Urol. 2020;203(1):200-205. doi:10.1097/JU.0000000000000505

5. Abdellaoui S, Cazzorla F, Morel-Journel N, et al. "Long-Term Urinary Outcomes in Classic Bladder Exstrophy: Results of an Extensive Follow-Up." BJU Int. 2025;135(6):1018-1024. doi:10.1111/bju.16680

6. Szymanski KM, Fuchs M, McLeod D, et al. "Probability of Bladder Augmentation, Diversion and Clean Intermittent Catheterization in Classic Bladder Exstrophy: A 36-Year, Multi-Institutional, Retrospective Cohort Study." J Urol. 2019;202(6):1256-1262. doi:10.1097/JU.0000000000000552

7. Taskinen S, Suominen JS. "Lower Urinary Tract Symptoms (LUTS) in Patients in Adulthood With Bladder Exstrophy and Epispadias." BJU Int. 2013;111(7):1124-1129. doi:10.1111/j.1464-410X.2012.11756.x

8. Burki T, Hamid R, Duffy P, et al. "Long-Term Followup of Patients After Redo Bladder Neck Reconstruction for Bladder Exstrophy Complex." J Urol. 2006;176(3):1138-1141. doi:10.1016/j.juro.2006.04.055

9. Polm PD, Christiaans CHH, Dik P, Wyndaele MIA, de Kort LMO. "Continent Catheterizable Urinary Channels: Lessons for Lifelong Urological Care From a Comparative Analysis of Very Long-Term Complications and Revision-Free Survival of Three Different Types." Neurourol Urodyn. 2024;43(5):1083-1089. doi:10.1002/nau.25350

10. Fiorenza V, Hukkinen M, Alova I, et al. "Dextranomer Endoscopic Injections for the Treatment of Urinary Incontinence in Bladder Exstrophy-Epispadias Complex." J Urol. 2023;209(3):591-599. doi:10.1097/JU.0000000000003086

11. Light JK, Scott FB. "Treatment of the Epispadias-Exstrophy Complex With the AS792 Artificial Urinary Sphincter." J Urol. 1983;129(4):738-740. doi:10.1016/s0022-5347(17)52336-8

12. Kiran PS, Panaiyadiyan S, Singh P, et al. "Management of Untreated Classical Bladder Exstrophy in Adults: A Single-Institutional Experience." Urology. 2020;146:293-298. doi:10.1016/j.urology.2020.09.009

13. Polm PD, Wyndaele MIA, de Kort LMO. "Very Long-Term Follow-Up of Indiana Pouches Proves Durability." Neurourol Urodyn. 2024;43(5):1090-1096. doi:10.1002/nau.25344

14. Rubenwolf PC, Hampel C, Roos F, et al. "Continent Anal Urinary Diversion in Classic Bladder Exstrophy: 45-Year Experience." Urology. 2017;100:249-254. doi:10.1016/j.urology.2016.11.026

15. Venkatramani V, Chandrasingh J, Devasia A, Kekre NS. "Exstrophy-Epispadias Complex Presenting in Adulthood: A Single-Center Review of Presentation, Management, and Outcomes." Urology. 2014;84(5):1243-1247. doi:10.1016/j.urology.2014.06.063

16. Harris TGW, Haffar A, Crigger CB, et al. "Stomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management." Urology. 2024;191:110-118. doi:10.1016/j.urology.2024.07.003

17. Surer I, Ferrer FA, Baker LA, Gearhart JP. "Continent Urinary Diversion and the Exstrophy-Epispadias Complex." J Urol. 2003;169(3):1102-1105. doi:10.1097/01.ju.0000044921.19074.d0

18. Stein R, Bogaert G, Dogan HS, et al. "EAU/ESPU Guidelines on the Management of Neurogenic Bladder in Children and Adolescent Part II: Operative Management." Neurourol Urodyn. 2020;39(2):498-506. doi:10.1002/nau.24248

19. Cleper R, Blumenthal D, Beniamini Y, et al. "Exstrophy-Epispadias Complex: Are the Kidneys and Kidney Function Spared?" Pediatr Nephrol. 2023;38(8):2711-2717. doi:10.1007/s00467-023-05889-y

20. Smeulders N, Woodhouse CR. "Neoplasia in Adult Exstrophy Patients." BJU Int. 2001;87(7):623-628. doi:10.1046/j.1464-410x.2001.02136.x

21. Woodhouse CR, North AC, Gearhart JP. "Standing the Test of Time: Long-Term Outcome of Reconstruction of the Exstrophy Bladder." World J Urol. 2006;24(3):244-249. doi:10.1007/s00345-006-0053-7

22. Rieder JM, Parsons JK, Gearhart JP, Schoenberg M. "Primary Squamous Cell Carcinoma in Unreconstructed Exstrophic Bladder." Urology. 2006;67(1):199. doi:10.1016/j.urology.2005.07.008

23. Rubenwolf PC, Eder F, Ebert AK, et al. "Persistent Histological Changes in the Exstrophic Bladder After Primary Closure — a Cause for Concern?" J Urol. 2013;189(2):671-677. doi:10.1016/j.juro.2012.08.210

24. Higuchi TT, Granberg CF, Fox JA, Husmann DA. "Augmentation Cystoplasty and Risk of Neoplasia: Fact, Fiction and Controversy." J Urol. 2010;184(6):2492-2496. doi:10.1016/j.juro.2010.08.038

25. Mansour AM, Sarhan OM, Helmy TE, et al. "Management of Bladder Exstrophy Epispadias Complex in Adults: Is Abdominal Closure Possible Without Osteotomy?" World J Urol. 2010;28(2):199-204. doi:10.1007/s00345-009-0436-7

26. Azhati B, Dilixiati D, Reheman A, Li W, Yu Y. "An Analysis of the Efficacy of Multilayered Repair and Reconstruction Using Combined Tissue Pedicle Flaps for Abdominal Wall Defects in Adult Bladder Exstrophy Patients." Ann Plast Surg. 2024;92(4):437-441. doi:10.1097/SAP.0000000000003844

27. VanderBrink BA, Stock JA, Hanna MK. "Esthetic Outcomes of Genitoplasty in Males Born With Bladder Exstrophy and Epispadias." J Urol. 2007;178(4 Pt 2):1606-1610. doi:10.1016/j.juro.2007.03.192

28. Berrettini A, Sampogna G, Gnech M, et al. "Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications and Outcomes." J Sex Med. 2021;18(2):400-409. doi:10.1016/j.jsxm.2020.10.007

29. Harris TGW, Manyevitch R, Wu WJ, et al. "Pedicled Anterolateral Thigh and Radial Forearm Free Flap Phalloplasty for Penile Reconstruction in Patients With Bladder Exstrophy." J Urol. 2021;205(3):880-887. doi:10.1097/JU.0000000000001404

30. Suominen JS, Santtila P, Taskinen S. "Sexual Function in Patients Operated on for Bladder Exstrophy and Epispadias." J Urol. 2015;194(1):195-199. doi:10.1016/j.juro.2015.01.098

31. Zhu X, Klijn AJ, de Kort LMO. "Urological, Sexual, and Quality of Life Evaluation of Adult Patients With Exstrophy-Epispadias Complex: Long-Term Results From a Dutch Cohort." Urology. 2020;136:272-277. doi:10.1016/j.urology.2019.10.011

32. Rubenwolf P, Thomas C, Thüroff JW, Stein R. "Sexual Function, Social Integration and Paternity of Males With Classic Bladder Exstrophy Following Urinary Diversion." J Urol. 2016;195(2):465-470. doi:10.1016/j.juro.2015.08.076

33. Reynaud N, Courtois F, Mouriquand P, et al. "Male Sexuality, Fertility, and Urinary Continence in Bladder Exstrophy-Epispadias Complex." J Sex Med. 2018;15(3):314-323. doi:10.1016/j.jsxm.2018.01.004

34. Sinatti C, Waterschoot M, Roth J, et al. "Long-Term Sexual Outcomes in Patients With Exstrophy-Epispadias Complex." Int J Impot Res. 2021;33(2):164-169. doi:10.1038/s41443-020-0248-2

35. Deans R, Banks F, Liao LM, et al. "Reproductive Outcomes in Women With Classic Bladder Exstrophy: An Observational Cross-Sectional Study." Am J Obstet Gynecol. 2012;206(6):496.e1-6. doi:10.1016/j.ajog.2012.03.016

36. Lachica R, Chan Y, Uquillas KR, Lee RH. "Vaginal Delivery After Dührssen Incisions in a Patient With Bladder Exstrophy and Uterine Prolapse." Obstet Gynecol. 2017;129(4):689-692. doi:10.1097/AOG.0000000000001938

37. Hosiani A, Smet ME, Nayyar R. "A Road Map for the Management of a Pregnancy Complicated by Maternal Bladder Exstrophy." BMC Pregnancy Childbirth. 2024;24(1):195. doi:10.1186/s12884-024-06316-2

38. Bey E, Perrouin-Verbe B, Reiss B, et al. "Outcomes of Pregnancy and Delivery in Women With Continent Lower Urinary Tract Reconstruction: Systematic Review of the Literature." Int Urogynecol J. 2021;32(7):1707-1717. doi:10.1007/s00192-021-04856-1

39. Taskinen S, Suominen JS, Mattila AK. "Health-Related Quality of Life and Mental Health in Adolescents and Adults Operated for Bladder Exstrophy and Epispadias." Urology. 2015;85(6):1515-1519. doi:10.1016/j.urology.2015.02.020

40. Dellenmark-Blom M, Sjöström S, Abrahamsson K, Holmdahl G. "Health-Related Quality of Life Among Children, Adolescents, and Adults With Bladder Exstrophy-Epispadias Complex: A Systematic Review of the Literature and Recommendations for Future Research." Qual Life Res. 2019;28(6):1389-1412. doi:10.1007/s11136-019-02119-7

41. Bakır AC, Emül HM, Eliçevik M, et al. "Psychological and Sexual Outcomes in Patients With Bladder Exstrophy and Their Caregivers." J Pediatr Surg. 2026;61(3):162832. doi:10.1016/j.jpedsurg.2025.162832

42. Traceviciute J, Zwink N, Jenetzky E, et al. "Sexual Function and Quality of Life in Adult Male Individuals With Exstrophy-Epispadias Complex — a Survey of the German CURE-Network." Urology. 2018;112:215-221. doi:10.1016/j.urology.2017.08.063

43. Haddad E, Hayes LC, Price D, et al. "Ensuring Our Exstrophy-Epispadias Complex Patients and Families Thrive." Pediatr Nephrol. 2024;39(2):371-382. doi:10.1007/s00467-023-06049-y

44. Pathak HR, Mahajan R, Ali NI, Kaul S, Andankar MG. "Bladder Preservation in Adult Classic Exstrophy: Early Results of Four Patients." Urology. 2001;57(5):906-910. doi:10.1016/s0090-4295(01)00959-1

45. European Association of Urology. EAU Guidelines on Paediatric Urology: Transitional Urology (section 27.4.4) and Foetal Urology (bladder exstrophy-epispadias complex). 2026 limited update, March 2026. Guideline.

46. Anusionwu I, Baradaran N, Trock BJ, Stec AA, Gearhart JP, Wright EJ. "Is Pelvic Osteotomy Associated With Lower Risk of Pelvic Organ Prolapse in Postpubertal Females With Classic Bladder Exstrophy?" J Urol. 2012;188(6):2343-2346. doi:10.1016/j.juro.2012.08.034

47. Canalichio KL, Ahn J, Artigas P, et al. "Patient-Reported Outcomes in Adult Females With Bladder Exstrophy: A Study of Long-Term Sexual, Reproductive and Urinary Outcomes Using Social Media." J Pediatr Urol. 2020;16(5):567.e1-567.e7. doi:10.1016/j.jpurol.2020.06.020

48. Borer JG, Gargollo PC, Hendren WH, et al. "Early Outcome Following Complete Primary Repair of Bladder Exstrophy in the Newborn." J Urol. 2005;174(4 Pt 2):1674-1678. doi:10.1097/01.ju.0000175942.27201.59

49. Bolduc S, Capolicchio G, Upadhyay J, Bagli DJ, Khoury AE, McLorie GA. "The Fate of the Upper Urinary Tract in Exstrophy." J Urol. 2002;168(6):2579-2582. doi:10.1016/S0022-5347(05)64220-6

50. Petrarca M, Zaccara A, Marciano A, et al. "Gait Analysis in Bladder Exstrophy Patients With and Without Pelvic Osteotomy: A Controlled Experimental Study." Eur J Phys Rehabil Med. 2014;50(3):265-274.

51. Suominen JS, Helenius I, Taskinen S. "Long-Term Orthopedic Outcomes in Patients With Epispadias and Bladder Exstrophy." J Pediatr Surg. 2012;47(10):1821-1824. doi:10.1016/j.jpedsurg.2012.04.023

52. Damario MA, Carpenter SE, Jones HW Jr, Rock JA. "Reconstruction of the External Genitalia in Females With Bladder Exstrophy." Int J Gynaecol Obstet. 1994;44(3):245-253. doi:10.1016/0020-7292(94)90174-0

53. Huck N, Schweizerhof S, Honeck P, et al. "Pregnancy After Urinary Diversion at Young Ages-Risks and Outcome." Urology. 2017;104:220-224. doi:10.1016/j.urology.2017.01.033

54. Gupta AD, Gearhart JP. Approach to the Exstrophy Patient. In: Wood HM, Wood D, eds. Transition and Lifelong Care in Congenital Urology. Springer; 2015:27–33. doi:10.1007/978-3-319-14042-1_3

55. Anusionwu I, Tekes A, Stec AA, Gearhart JP, Wright EJ. "Comparison of Musculoskeletal Anatomic Relationships, Determined by Magnetic Resonance Imaging, in Postpubertal Female Patients With and Without Classic Bladder Exstrophy." BJU Int. 2013;112(2):E195-200. doi:10.1111/j.1464-410X.2012.11753.x

56. Nethercliffe J, Trewick A, Samuell C, Leaver R, Woodhouse CR. "False-Positive Pregnancy Tests in Patients With Enterocystoplasties." BJU Int. 2001;87(9):780-782. doi:10.1046/j.1464-410x.2001.02187.x

57. Vasquez E, Dillon H, Hannallah A, et al. "False Positive Pregnancy Tests in Pediatric Patients With Augmentation Enterocystoplasty." J Pediatr Urol. 2022;18(5):615.e1-615.e6. doi:10.1016/j.jpurol.2022.09.008

58. Venna A, Valovska MT, Estrada CR, Borer JG, Nelson CP. "False-Positive Urine Pregnancy Screening Tests Are Uncommon in the Hospital Setting Among Patients With Bowel-Containing Urinary Tract Reconstruction." J Pediatr Urol. 2023;19(3):312.e1-312.e4. doi:10.1016/j.jpurol.2023.02.001

59. Higuchi TT, Fox JA, Husmann DA. "Annual Endoscopy and Urine Cytology for the Surveillance of Bladder Tumors After Enterocystoplasty for Congenital Bladder Anomalies." J Urol. 2011;186(5):1791-1795. doi:10.1016/j.juro.2011.07.028

60. Purves JT, Gearhart JP. Modern Staged Repair of Exstrophy/Epispadias. In: Montague DK, Gill IS, Angermeier KW, Ross JH, eds. Textbook of Reconstructive Urologic Surgery. Informa Healthcare; 2008:235-243.

61. Grady RW. Rationale for Use: Complete Primary Exstrophy Repair Technique. In: Montague DK, Gill IS, Angermeier KW, Ross JH, eds. Textbook of Reconstructive Urologic Surgery. Informa Healthcare; 2008:244-252.

62. Bush NC, Snodgrass WT. Congenital Penile Abnormalities. In: Muneer A, Arya M, Jordan GH, eds. Atlas of Male Genitourethral Surgery: The Illustrated Guide. Wiley-Blackwell; 2014:25-42.

63. Wu WJ, Maruf M, Manyevitch R, et al. "Delaying Primary Closure of Classic Bladder Exstrophy: When Is It Too Late?" J Pediatr Urol. 2020;16(6):834.e1-834.e7. doi:10.1016/j.jpurol.2020.09.003

64. Datar G. Functional Repair of Epispadias. In: Joshi PM, Aubé-Peterkin M, Kulkarni SB, Datar G, eds. Surgical Atlas of Urethroplasty. Springer; 2024:233-274. doi:10.1007/978-981-97-5196-9_26

65. Ginsberg DA, Boone TB, Cameron AP, et al. "The AUA/SUFU Guideline on Adult Neurogenic Lower Urinary Tract Dysfunction: Diagnosis and Evaluation." J Urol. 2021;206(5):1097-1105. doi:10.1097/JU.0000000000002235

66. Deutsche Gesellschaft für Urologie e. V. S3-Leitlinie Epidemiologie, Diagnostik, Therapie und Nachsorge des Blasenekstrophie-Epispadie Komplex (BEEK). Langversion 1.0. 2024. AWMF 043-058. Guideline.

67. Roth JD, Bowen D, Fuchs ME, et al. "End-stage and chronic kidney disease in classic bladder exstrophy: A retrospective muti-institutional cohort study." J Pediatr Urol. 2025;21(1):41-46. doi:10.1016/j.jpurol.2024.10.016

68. Batra NV, Meldrum KK, Misseri R, et al. "Chronic kidney disease in classic bladder exstrophy following primary closure and then isolated bladder neck repair." J Pediatr Urol. 2026 Apr 11:105952. doi:10.1016/j.jpurol.2026.105952

69. Maxon V, Haffar A, Michel CA, et al. "A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient." Urology. 2024;192:100-104. doi:10.1016/j.urology.2024.07.001