- Open Access
Current management of classic bladder exstrophy in the modern era
African Journal of Urology volume 29, Article number: 27 (2023)
Classic bladder exstrophy is a complex, multi-system congenital malformation affecting formation of the genitourinary system, pelvis, and abdominal wall.
Historically children with this abnormality were consigned to poor outcomes and quality of life. Modern advancements in the diagnosis and management of this disorder have resulted in low mortality rates and shifted clinical focus toward optimizing quality of life.
Modern techniques in addition to recent discoveries in the diagnosis and delayed management of this disorder have enabled high rates of urinary continence, genital cosmesis, and an improved quality of life. This is an updated overview of the pathology, diagnosis, and management of this rare disorder.
The bladder exstrophy-epispadias complex (BEEC) is a rare spectrum of defects affecting the genitourinary and gastrointestinal tracts, musculoskeletal system, pelvic floor musculature, and bony pelvis. The BEEC spectrum ranges from mild isolated epispadias, presenting with a dorsally open urethral meatus, mild pubic diastasis, and closed abdominal wall and bladder. In contrast, the most severe form of cloacal exstrophy, or OEIS (omphalocele, exstrophy, imperforate anus, and spinal abnormalities) syndrome, consists of debilitating multisystemic anomalies of the genitourinary, musculoskeletal, and gastrointestinal systems. The most common presentation of BEEC, classic bladder exstrophy (CBE) presents with a wide pubic diastasis, abdominal wall defect exposing an open bladder and urethra with an epispadias opening.
Surgical management of CBE requires multiple reconstructive surgeries starting with closure of the bony pelvis, bladder, and anterior abdominal wall, followed later by epispadias repair. Recent trends have shifted management toward scheduled delayed closure in the 6-8th week of life and utilization of pelvic osteotomy and lower extremity immobilization to ensure complete approximation and sufficient deepening of the pelvis for anatomic placement of the bladder. While current techniques achieve reasonable success in preservation of renal function, continence, and cosmesis, there are still discoveries that are needed to improve quality of life even more.
Within the USA, the incidence of bladder exstrophy is estimated to be 2.15 cases per 100,000 live births [1, 2]. In a broader international population, Cervellione et al. reported an incidence of 1 in 46,000 live births . Most recently, an increased live prevalence of CBE was reported amidst the German population at 1 in 30,675 live births . Historically, CBE is most common among boys with a male-to-female ratio ranging between 2–5: 1 [5,6,7]. Risk factors include Caucasian race, young maternal age, advanced paternal age, maternal multiparity, and use of assisted-reproductive technologies (i.e., in vitro fertilization) [1, 8, 9]. In relevant studies, no meaningful associations have been made between CBE incidence and periconception maternal exposure to alcohol, drugs, radiation, or infections . The risk of bladder exstrophy in the offspring of individuals with bladder exstrophy is approximately 500-times greater, 1 in 70 births, than baseline risk in the general population .
While the granular causes of BEEC are not completely understood, the predominately held theory popularized by Marshall and Muecke traces the basic defect to an abnormal overdevelopment of the cloacal membrane in the fourth week of gestation . The cloacal membrane is a bilaminar layer situated at the caudal end of the germinal disk which occupies the infraumbilical abdominal wall. The overdevelopment of the cloacal membrane prevents mesenchymal migration between the ectoderm and endoderm. The effect of this overdevelopment causes (1) limited development of lower abdominal musculature and pelvic bones and (2) a propensity for early rupture of the cloacal membrane due to innate instability. The timing and location of rupture of the cloacal membrane are thought to dictate the patient’s presentation along the Bladder Exstrophy-Epispadias spectrum [12, 13]. Epispadias occurs if the rupture produces a division or nonunion at the distal end of the urinary tract. CBE results if the rupture occurs after the urorectal septum which divides the gastrointestinal from the genitourinary tracts while CE (cloacal exstrophy) results if the rupture occurs before this separation .
While several genetic studies are underway, the majority of BEEC cases are sporadic without Mendelian inheritance. Some evidence suggests an association between CBE and the CASPR3 gene, p63 tumor suppressor gene, and 22q11.2 duplications [9, 15,16,17]. The p63 gene, a member of the p53 tumor suppressor gene family, may have the strongest association with CBE as it is highly expressed within the bladder and overlying skin . Animal studies consisting of p63 knockout in mice resulted in CBE-like anomalies [19, 20].
4 Anatomic considerations
4.1 Urogenital anomalies
In CBE, the bladder and posterior urethra are exposed anteriorly through a triangular abdominal defect. Histologically, the bladder appears immature as demonstrated by significantly reduced myelinated nerves . Further, the exstrophied bladder presents with an increased ratio of collagen to smooth muscle compared to normal controls and may correct following a successful closure [22,23,24,25]. Historically, neonatal closures of the bladder soon after birth were the standard of care except in cases where delayed closure were necessary. Delayed closure was indicated whenever hamartomatous polyps are present on the bladder mucosa, a bladder template < 3 cm in diameter, fibrosis of the bladder template, or patient referral is delayed . In the modern era, scheduled delayed closure for patients has become the new standard of care even for patients who could be candidates for neonatal closure.
Sufficient bladder growth for continent bladder neck reconstruction occurs in approximately 60% of successfully closed CBE patients . If bladder growth does not reach sufficient capacity, a bladder augmentation cystoplasty may be required . In a subset of patients with extremely small or excessively fibrotic bladders, cystectomy with urinary diversion (continent catheterizable pouch or orthotopic neobladder) is favored over augmentation [29, 30].
The upper urinary tract is generally normal at birth, but anomalies do occur. Approximately 3% of patients will have an associated renal anomaly (duplicated system, solitary kidney, ureteropelvic junction obstruction, etc.) [31, 32]. The entry course of the ureteral section terminating into the bladder predispose all CBE patients to vesicoureteral reflux following bladder closure [33, 34]. If the reflux does not cause upper tract changes, patients can be managed conservatively until reimplantation can be paired with continence surgeries.
In the male CBE patient, the phallus is shorter and wider than normal controls with the open urethral plate on the dorsal surface. Most cases will also present with significant dorsal chordee. The short and broad phallus is influenced by pubic diastasis and the lateralization of corporal bodies .
In the female CBE patient, the dorsal urethra remains open at the distal aspect creating a patulous bladder neck. The vagina and introitus are displaced anteriorly with a flattened and lateralized mons pubis. Female CBE frequently presents with a bifid clitoris located in the anterior vaginal wall surrounded with divergent labia. The vagina is shallow and stenotic . Mullerian anomalies are more commonly associated with the more severe cloacal exstrophy condition but have been reported with CBE patients [37, 38].
4.2 Bony pelvis and spinal defects
Among CBE patients, the bony pelvis presents with a characteristic widening of the pubic symphysis (pubic diastasis), rotational, and dimensional anomalies . Pubic diastasis is secondary to malrotation of the innominate bones which evert, or externally rotate, the pubic rami at their junction with the iliac bones. Classic bladder exstrophy patients have an average pubic diastasis between 4 and 5 cm [39, 40]. Additional rotational anomalies include external rotation of the anterior pelvic segment, coronal rotation of the sacroiliac joint, acetabular retroversion, convergence of iliac wings, and femoral retroversion. The bony pelvis of the CBE patient has a 30% shortened anterior pubic segment and increased intertriradiate cartilage distance . The summation of these bony anomalies increases distance between the hips and accounts for waddling gait and outward rotation of lower limbs in children with CBE. The functional outcomes derived from the bony pelvis cause minimal disability and will self-correct to a small degree overtime .
Spinal anomalies are relatively uncommon among patients with classic bladder exstrophy. In a popularized 1997 study of 299 CBE patients from a single institution, the rate of spinal anomalies, excluding normal variants, was found to be 6.7% . Spinal anomalies consisted of uncomplicated scoliosis (2.7%) and spinal dysraphism (4%). In addition to spinal anomalies, 11% of patients presented with normal variants (e.g., spina bifida occulta, lumbarization, sacralization). A single patient with myelomeningocele suffered clinical neurological dysfunction (0.3%).
4.3 Pelvic floor defects
Preoperative magnetic resonance imaging (MRI) among CBE patients reveals several key characteristics of anomalous development. In general, the preoperative CBE pelvic floor consists of a posteriorly positioned, irregularly shaped levator ani and flattened puborectal sling. As a result of these changes, the puborectal slings support twice as much body cavity area than normal . According to most recent literature, the severity of pubic diastasis does not account for the disproportionate curvature of the pelvic floor . The aforementioned abnormalities contribute to incontinence and predispose females to complications including uterine prolapse . Using 3D MRI to compare the pelvic floor of pre- and post-repair CBE patients, Stec et al. discovered closure (1) reshapes pelvis from a boxlike configuration to a more inwardly rotated hammock; (2) redistributes a significant portion of the levator group into the anterior compartment; and (3) facilitates smooth uniform contouring of the pelvic floor .
4.4 Abdominal wall anomalies
Classic bladder exstrophy is associated with a triangular abdominal wall and fascial defect limited superiorly by the umbilicus and inferiorly by the intrasymphyseal band . Occupying the defect is the exstrophied bladder and posterior urethra. Inferiorly the intrasymphyseal band is tethered between the posterior vesicourethral unit and the pubic ramus. Concurrent umbilical hernias are a common, but generally insignificant, finding and may be repaired at time of primary closure. Indirect inguinal hernias are similarly common as a result of a persistent processus vaginalis, large inguinal rings, and linear orientation of the inguinal canal.
4.5 Anorectal defects
Misalignment of the anal canal is a common finding among classic bladder exstrophy patients. The anterior displacement of the anus and anal sphincter, in combination with pelvic floor anomalies, predisposes patients to fecal incontinence. On occasion, CBE patients may present with concurrent omphalocele, imperforate anus, rectal stenosis, and/or rectal prolapse . However, incidence of major gastrointestinal anomalies is more commonly associated with cloacal exstrophy. Anal continence is expectedly imperfect at early ages and improves with time and successful primary closure. Rectal prolapse is frequently found among older untreated CBE children but is often easily reduced. In the case of rectal prolapse following successful primary closure, clinicians should maintain a high suspicion for bladder outlet obstruction and low threshold to evaluate patients by cystoscopy .
4.6 Complex variants
Variants of CBE include skin covered bladder exstrophy, duplicated bladders, superior vesical fistulas, and epispadias with major bladder prolapse . Skin-covered exstrophy presents with the bladder directly beneath an infraumbilical bulge of intact skin with laterally displaced rectus muscles. Duplicated bladders may present as either anterior–posterior or side-to-side. An anterior–posterior duplication may present with a patch of exstrophic mucosa on the infraumbilical aspect of the abdomen. The superior vesical fistula presents as an abdominal wall defect communicating with the urinary bladder.
5 Prenatal diagnosis
Reports on the rate of prenatal diagnosis of classic bladder exstrophy range somewhere between 12.5 and 75% [49,50,51,52,53]. Since the turn of the century, the rate has been approximately 47% and increasing . The hallmark findings for prenatal diagnosis include: (1) absence of bladder filling, (2) a low-set umbilicus, (3) widening pubic ramus, (4) diminutive genitalia, and (5) lower abdominal mass . The diagnosis is often missed or misdiagnosed as omphalocele or gastroschisis. Inability to identify bladder filling in two consecutive ultrasounds 90 min apart and/or a pubic diastasis ≥ 1 cm at ≥ 20 weeks gestation should merit referral to an experienced exstrophy center for evaluation [55, 56]. Existing online health information regarding exstrophy may be incomprehensible for most caregivers, making early prenatal consultation crucial . Prenatal consultation allows confirmatory imaging and the opportunity for family members to receive education on the prognosis of bladder exstrophy, meet members of the multidisciplinary exstrophy team, tour intensive care units, and connect with other bladder exstrophy families [54, 58, 59].
6 Evaluation and management at birth
6.1 Selection of patients for immediate closure
At birth, careful assessment and consideration of the exstrophy patient must be undertaken by an experienced exstrophy surgeon and a pediatric orthopedic surgeon. Examination under anesthesia may yield previously unappreciated bladder and should be considered [41, 60]. If the infant possesses a large, elastic bladder template, free of any polyps, and under the care of an experienced exstrophy team, then, a newborn closure may be considered. Some proponents of early closure claim that a rapid closure allows for early bladder cycling and a resultant larger capacity .
6.2 The inadequate bladder template and elective delayed closure
A small, fibrotic bladder or one with hamartomatous polyps is unsuitable for newborn closure and further assessment under anesthesia by an experienced exstrophy surgeon should be made [60, 62]. Other conditions that may forestall neonatal closure include ectopic bowel within the bladder, penoscrotal duplication, and significant bilateral hydronephrosis. In Baradaran et al., study of delayed primary closures, the authors compared bladder capacity in delayed closure patients due to inadequate templates with those due to late referrals . They concluded that although the total measured capacity of delayed closure was reduced, the annual growth rate was similar [62, 63]. Therefore, surgeons should not hesitate to delay closure in patients with an inadequate bladder template, as it does not risk the growth of the bladder . Attempting to close a small or fibrotic bladder template places the closure at risk for dehiscence and eventual incontinence. If the bladder does not grow sufficiently within 6 to 12 months, consideration should be made for excision of the bladder or incontinent urinary diversion, such as a colon conduit.
7 Surgical reconstruction of bladder exstrophy
The paramount goal of surgical management of classic bladder exstrophy is a successful primary closure as it is associated with decreased overall costs, decreased inflammation and fibrosis of the bladder, improved bladder growth, and decreased need for urinary diversion [65,66,67,68,69,70]. Since the earliest description of staged surgical reconstruction of exstrophy by Sweetser et al. in the 1950’s, debate has persisted regarding the ideal surgical management, timing, and technique of exstrophy closure [71, 72]. Advancements in neonatal anesthesiology and intensive care through the 1970’s brought a shift toward primary closure within the first 72 h of life with a reciprocal decrease in pelvic osteotomies . Pelvic osteotomy is used to deepen the pelvis, reduce pubic diastasis, and release tension on the abdominal wall but may be omitted in select neonatal closures due to pelvic malleability. Further, proponents for neonatal closure contended that early closure facilitated early bladder cycling, improved bladder capacity, and decreased risk of precancerous changes . Delayed closure was primarily used for patients with inadequate bladder templates for neonatal closure (see Sect. 6.2).
Starting in the 1990’s, interest in combined, all-inclusive repairs of exstrophy were rekindled for older children with failed primary closures and eventually in neonatal primary closures [60, 74,75,76,77]. Since the turn of the century, amassing data on the safety and successful outcomes of delayed closure, in combination with pelvic osteotomy, has led to ever-increasing popularity and shifts in clinical practice [72, 78,79,80].
7.1 Modern staged reconstruction of exstrophy (MSRE)
A detailed operative description of MSRE is provided in Pediatric Urology . The MSRE is globally popular and represents the closure technique in up to 58% of exstrophy surgeons [82,83,84]. Dry urinary continence is achieved in up to 70% of MSRE patients with minimal complications [41, 79, 85].
Generally, the modern staged approach separates the repair into three stages: (1) Primary closure of bladder and abdominal wall, (2) epispadias repair, (3) continence surgeries. In recent years, timing of primary closure of the bladder and abdominal wall is customarily scheduled between 6 and 12 weeks of life [26, 72, 79, 83, 86]. Female CBE patients may also receive genitoplasty and urethroplasty with initial bladder closure. Closures may be delayed further if bladder template remains inadequate for closure (see Sect. 6.2). Prior to closure, the infant is managed with frequent saline washes of the exstrophic bladder and cellophane wrappings to protect bladder mucosa. The second stage of closure in males is urethral epispadias repair at approximately 6–10 months of age . The author’s institution utilizes the modified Cantwell–Ransley repair for children with an adequate urethral groove length.
Once a child has received a successful epispadias repair, the patient’s bladder capacity is able to be measured annually via an annual gravity cystogram under anesthesia. The third stage of closure, or continence surgery, is dependent on the child’s bladder growth and desire for continence. The majority of children achieve an adequate capacity for bladder neck reconstruction (BNR) with the potential for spontaneous urethral voiding at a median age of 5.2 years . In children with mild to moderate VUR, concurrent ureteral reimplantation is undertaken at time of continence surgery. Children who are not candidates for BNR, or fail to achieve urinary continence following BNR, may require bladder neck closure, augmentation cystoplasty, and/or continent catheterizable stoma.
7.2 Complete primary repair of exstrophy (CPRE)
A detailed operative description of CPRE is provided in Pediatric Urology . The potential advantage of CPRE is minimalization of overall number of surgeries, hospitalizations, and associated costs of exstrophy care and improve continence rates without the need for formal bladder neck reconstruction. However, recent evidence suggests the majority of patients receiving CPRE require subsequent bladder neck reconstruction [87, 88]. CPRE combines primary abdominal wall and bladder closure with epispadias repair and partial tightening of the bladder neck . Bilateral ureteral reimplantation (BUR) may also be undertaken at the time of CPRE as 50% of children will require BUR within the first year of life following CPRE alone [75, 89]. Long-term outcomes report 48% of CPRE patients achieve eventual urinary continence [88, 90].
While CPRE has many proponents among North American surgeons, there are many recognized complications . The epispadias repair in CPRE is traditionally done by Mitchell Penile Disassembly whereby the urethral plate is fully dissected from the corporal bodies which renders many patients hypospadiac and necessitates later reconstruction (see Sect. 9) . Other complications of CPRE include soft tissue loss [92, 93], urinary retention , and chronic kidney damage . While CPRE is purported to reduce the number of surgeries for CBE, only 16% achieve urinary continence by CPRE alone while the vast majority require subsequent follow-up operations for reflux, incontinence, or closure failure [88, 90].
7.3 Kelly radical soft tissue mobilization repair
Developed in the late 1980’s by Australian surgeon Dr. Justin H. Kelly , radical soft tissue mobilization technique for CBE repair was proposed as a means of closure that obviated the need for pelvic osteotomy [97, 98]. The Kelly Repair is a multistage approach including (1) bladder closure and hernia repair at birth, with (2) reconstruction of proximal urethra and associated sphincteric tissue with penile lengthening and creation of penoscrotal urethrostomy (boys only) between 3 and 6 months of age, and (3) repair of resulting penoscrotal hypospadias at approximately 3 years old . The unique benefit of the Kelly technique is rooted in the radical mobilization of the pelvic floor muscles including dissection of the periosteum of pelvic girdle near attachments sites of sphincteric muscles and the pudendal neurovascular bundle . Sphincteric muscles are wrapped around the reconstructed proximal urethra in an effort to provide continence. Similar to penile disassembly, the urethral plate is dissected from corporeal bodies to create the neourethra. Recent use of the Kelly Repair in single-stage delayed closures of CBE without osteotomy has shown urethral fistula and/or stenosis rates up to 30% .
7.4 Combined bladder closure and epispadias repair
Despite the complications and sustained need for follow-up surgery among CPRE patients, interest in combining stages of CBE repair is a popular pursuit. Combined bladder closure and epispadias repair using a modified Cantwell–Ransley technique in a highly selective patient group have been undertaken successfully as an alternative to CPRE [60, 74, 76, 93, 101]. Combined bladder and epispadias repair are particularly suited for patients with previous failed closure or primary closure beyond 5–6 months of age with an adequate bladder template (> 3 cm diameter), robust urethral plate, and non-diminutive phallic length or size [60, 74, 76, 93]. Rigorous patient selection is key for minimizing complication rates and soft tissue loss. Even with this selection criteria, expected continence rates approach 60% at best .
7.5 Pelvic osteotomies and immobilization
Pelvic osteotomy, or surgical incision of the bony pelvis, has several benefits in application to the closure of the exstrophied bladder including a tension-free approximation of the pubis, deeper placement of the bladder and posterior vesicourethral unit within the pelvis, and improved outcomes of future reconstruction [78, 102]. Osteotomy is recommended for closures with a diastasis over 4 cm, patients over the age of 72 h, or in children with poor malleability of the pelvis as judged by a senior pediatric orthopedic surgeon . Notably, the average pubic diastasis in CBE has been reported as high as 4.8 cm . Several methods of osteotomy have been described within the exstrophy population including posterior iliac osteotomy, bilateral osteotomy of the superior pubic ramus, diagonal iliac wing osteotomy, and combined bilateral anterior transverse innominate and vertical posterior iliac osteotomy. The combined transverse innominate and vertical iliac specifically have been shown to decrease rates of dehiscence and bladder prolapse compared to other forms of osteotomy .
Inherent to the successful impact of osteotomy on CBE closure is the critical role of post-operative pelvic and lower limb immobilization . Many methods of limb immobilization have been described for post-operative management of CBE including modified Buck’s traction, modified Bryant’s traction, spica casting, and “mummy wrapping”. Employment of immobilization methods varies widely by institution and surgical preference. Modified Buck’s and Bryant’s traction exert longitudinal tension along the patient’s lower extremities with the legs extended while supine or with hips supinated in 90 degrees of flexion, respectively. Bryant’s traction is historically used in children closed without osteotomy. Traction is maintained for 4–6 weeks postoperatively. “Mummy wrapping” and spica casting involve wrapping and casting, respectively, the extremities allowing hip flexion without abduction. Proponents of wrapping and casting note facilitation of familial bonding during immobilization and a shorter length of stay compared to traction immobilization . Utilization of wraps and casts have been called into question following reports of increased rates of skin breakdown and inferior outcomes compared to Buck’s or Bryant’s traction [102, 107]. However, in a recent cohort of patients using spica or mummy wrapping, external fixation was found to be a pivotal protective factor in ensuring successful bladder closure . In a challenge on long-held traditions, select institutions have described methods of circumventing the utilization of osteotomy or immobilization with remarkable results [108, 109].
The use of pelvic osteotomy and immobilization is not without risk. Recent reports indicate osteotomy and immobilization may increase operative times, need for blood transfusions, and risk of perioperative complications [78, 79]. Failure of inadequate osteotomy and immobilization can lead to closure failure, wound dehiscence, bladder prolapse, or loss of suprapubic tubes and ureteral stents . Osteotomy complications most often include urinary tract infection, urinary fistula, transient nerve palsy, osteotomy site infection, delayed ileal union, and pin-site infection [78, 110]. Skin inflammation around pin-sites is common and often managed with oral antibiotics.
7.6 Bladder augmentation
In a recent large, survey-based, study of adult exstrophy patients, 50% of adult exstrophy patients required a bladder augmentation . An augmentation cystoplasty is commonly required in CBE patients following failed primary closures and patients with noncompliant and/or insufficient bladder capacity [67, 112]. For example, approximately 40% of patients can be expected to attain adequate bladder capacity for BNR following a single failed closure, with less than half of these patients eventually becoming continent of urine . Chances for continence severely dwindle with two or more failed closures. Techniques for augmentation utilize segments of bowel, stomach, or redundant ureter to expand the bladder wall.
7.7 Continent urinary diversion (CUD)
Patients requiring augmentation cystoplasty typically also require concurrent CUD. Typical CUD options include appendicovesicostomy (Mitrofanoff procedure) or tunneled ileovesicostomy (Monti procedure) to create a catheterizable stomas. Recent literature reports approximately half of adults with bladder exstrophy empty per continent stoma. Approximately 1 in 5 CUD patients report stomal leaks .
7.8 Ureterosigmoidostomy and Mainz Sigma pouch
Ureterosigmoidostomy (USIG), or non-refluxing reimplantation of ureters into the colon, was among the first forms of urinary diversion used in patients with CBE. In North America, use of USIG has dwindled while still being used in other parts of the world. Many patients have reverted to alternative methods of diversion due to ongoing concern for serious complications, including pyelonephritis, hyperkalemic acidosis, rectal incontinence, ureteral obstruction, and delayed development of malignancy [114, 115]. Interestingly, recent published data on long-term continence and renal preservation challenge these assertions [116,117,118]. Specifically, use of the Mainz Sigma pouch has distinct advantages over standard USIG with 95% of patients achieving continence . Less controversy exists regarding higher rates of colorectal malignancy against this population. Colorectal malignancies are identified an average of 38 years after USIG with most common malignant pathology being poorly differentiated adenocarcinoma [118, 120]. Patients who have had mixing of urine and feces at any time during reconstruction remain at high risk for development of cancers [121, 122]. Within our institution, we only see USIG patients as adults who were treated at other locations. We recommend all patients with USIG to have yearly ultrasound and colonoscopy in adult life.
8 Management after primary closure
The initial step of the MSRE results in a patient with mid-penile shaft epispadias and incontinence. Immediately following the procedure, patients spend approximately 3 days in the intensive care unit weaning from ventilation. Pain control is optimized with a combination of a tunneled epidural lidocaine administration and intravenous analgesia . Before the suprapubic tube is removed, 4 weeks after surgery the bladder outlet is calibrated with a urethral catheter to ensure drainage. Ultrasound imaging is performed to ensure status of renal pelvises and ureters. Due to the reflux that all patients will have following closure, urinary antibiotics are administered. Imaging is repeated 3 months after discharge and thereafter at intervals of 6 months to 1 year at the surgeon’s discretion, typically for 2–3 years. Prophylactic antibiotics will be given continuously until ureteral reimplantation is carried out, typically performed alongside epispadias repair in the modern staged approach at time of continence procedure. Yearly cystoscopy with cystography under anesthesia is carried out to estimate bladder growth and to evaluate reflux . Dilation of the urethra or intermittent catheterization may prove necessary in patients who develop increased outlet resistance and recurrent infections .
9 Penile and urethral closure in exstrophy
Epispadias repair and penile reconstruction, typically performed between 6 and 10 months of age with MSRE, corrects dorsal chordee, urethral and glandular reconstruction, and penile skin closure . Intramuscular or topical testosterone can improve the quality and quantity of penile skin as well as size of the urethral plate and should be a preoperative consideration with penile closure . The modified Cantwell–Ransley epispadias repair is performed by advancing the urethral meatus to an orthotopic position by utilizing a reverse meatal advancement and glanuloplasty technique . Dorsal chordee is simultaneously released by mobilizing the urethral plate from the corpora from the level of the glans to the prostatic urethra. The corporal bodies are then anastomosed over the dorsal medial aspect of the tubularized urethra. In 1996, Mitchell and Bagli described an additional modification to the Cantwell–Ransley repair, where the urethral plate, corporal body, and hemiglans are dissected free from each other . This repair, dubbed the “Complete Penile Disassembly”, was justified owing to the separate blood supply of each corpora. This repair has been criticized for inducing ischemia in the urethral plate as it shares blood supply with the spongiosum. Lateral dissection, during Mitchell repair, can lead to neurovascular bundle injury and subsequent erectile dysfunction . Mitchell repair, often done in conjunction with CPRE, can often lead to a tubularized urethra being shorter than the corpora, resulting in hypospadias and necessitating a subsequent complicated hypospadias repair .
10 Exstrophy reconstruction failures and complications
10.1 Failed closure
Failure can occur during any step of reconstruction manifesting as bladder dehiscence, bladder prolapse, vesicocutaneous fistula, or neourethral stricture and urinary obstruction . A failed primary closure decreases eventual bladder capacity, chance of spontaneous voided continence, and leaves a lasting negative financial impact [113, 130, 131]. These studies highlight the importance of initial successful closure; therefore, surgeons with minimal experience should consider referral to large centers with experience in treating exstrophy.
Dehiscence or prolapse, possibly due to inadequate pelvic immobilization, abdominal wound tension, or incomplete mobilization of the pelvic diaphragm, requires a 4–6 month recovery period before a secondary closure should be attempted [74, 132]. In certain select patients with failed closure, a combined bladder closure and epispadias repair may be attempted (see Sect. 7.3). After failure of primary CBE closure, the chance of achieving the bladder capacity necessary for BNR, > 100 cc, reduces to 60% . Bladders that do not reach this goal capacity can be augmented, typically with colon or small bowel to expand the bladder wall.
10.2 Failed bladder neck repair
Some patients may remain incontinent after bladder neck reconstruction secondary to a small bladder capacity, decreased compliance, or inadequate outlet resistance. Failure of bladder neck repair is defined as inability to achieve continence, or a 3-h dry period within 2 years after BNR. In those approaching daytime continence, > 2 h dryness, urethral bulking agents may be used to avoid further reconstruction, but the majority of failed BNR patients require augmentation or continent urinary diversion .
10.3 Failed genitourethral reconstruction
Historically there have been few complications following epispadias repair, however, with the advent of the complete penile disassembly more significant complications including loss of the glans, corpora, penile skin, and urethral plate have been reported . Reconstructing these complications may require the use of tissue expansion, buccal mucosa grafting, or full thickness skin grafting. Radial forearm neophalloplasty remains an option for patients with significant penile loss, allowing patients a cosmetic, sensate phallus. For older patients, phallus cosmesis may require further management, with penile scars and short phallus as the most common complaints. Scar revision could require flaps or skin grafting to ensure enough penile skin is available to close in a plastic fashion. Freeing scar tissue and suspensory ligaments may provide additional length to the phallus, but aggressive attempts to lengthen the penis should be cautioned due to the great risk of corporeal denervation and devascularization.
11 Transitional exstrophy patient: adolescent and adult concerns
With innovation and improved CBE surgical management over the last several decades, the childhood survival rate of CBE has increased drastically. As a result of improved management, care for adolescent, adult, and geriatric patients born with bladder exstrophy are an active area of research.
Children with CBE transitioning into adulthood require increasing need to address both long-term functional and psychological aspects of dealing with a multiorgan birth defect. Management of this major congenital defect through multiple reconstructive surgeries predisposes patients to problems of urinary incontinence and sexual dysfunction. As such, it is important to address these topics with the patient and their caregivers from an early age. While research suggests that children with exstrophy do not have clinical psychopathology, many individuals struggle psychologically with adapting their medical care to their desired lifestyle, self-esteem, and social functioning . Notably, urinary incontinence can be particularly stressful for individuals; therefore, reconstructive efforts to obtain dryness once the child is ready carries potential psychological benefit.
11.1 Quality of life
As survival becomes increasingly universal, health-related quality of life (QoL) is becoming an increasingly important topic and driver of reconstructive techniques. Early reports on QoL reported mixed results from decreased QoL in all post-reconstructive patients, to comparable QoL with peers, to increases in QoL among adolescents [135,136,137]. Parents specifically report significantly impaired adolescent general health, family activity, and increased parental emotional distress [137, 138]. As expected, patients with urinary incontinence tend to report lower QoL metrics .
11.2 Male sexual function and fertility
In the BEEC, the penis is 50% shorter and 30% wider compared to normal adult males due to the increased intercorporal and intrasymphyseal distances from the pubic diastasis . Additionally, there is a congenital shortage of anterior corporal tissue and a deep linear scar on the dorsum-lower abdominal wall from which it tethers penis. As BEEC, patients sexually mature penile appearance and function are the focus of reconstruction during late adolescence and early adulthood. The penis is lengthened by incising remnants of the suspensory ligaments before using tissue expanded (TE) penile shaft skin or a full thickness skin graft (FTSG) to provide soft tissue coverage [140,141,142]. Alternatively, a neo-phallus is used for patients where the penis would be too short for penetrative intercourse despite lengthening or those with aphallia, particularly in cloacal exstrophy.
The radial forearm free flap (RFFF) and the pedicled anterolateral thigh flap (PALTF) are the most frequently used techniques for phalloplasty in BEEC [143, 144]. The decision to perform RFFF or PALTF is dependent on vascular anatomy of the non-dominant arm and vascular integrity of the lower abdomen for microsurgical anastomosis of blood vessels. Surgeons should account for patient preference including tactile and erogenous sensation or donor site morbidity and flap bulk. 1 year after phalloplasty, patients undergo insertion of an inflatable penile prosthesis. However, patients do not undergo urethral reconstruction, secondary to the increased risk of complications in BEEC, and patients may have undergone continent urinary diversion [145, 146].
Penile lengthening with TE or FTSG and phalloplasty all resulted in better perception of penile length . Phalloplasty produced the greatest improvement in perception of length though penile lengthening patients reported better sensation. Surgical decision requires careful planning on the most suitable technique while taking patient preference into consideration, and all patients should receive pre- and post-operative psychological evaluation .
Adult BEEC males have similar concern with sexual health and relationships as unaffected males. Most have attempted engaging in sexual intercourse with missionary (36.5%) and cowgirl (23.8%) being the most effective positions, attributable to the congenital shortage of anterior corporal tissue . If patients want to father children, assisted reproduction is often necessary (48.0–53.3% of patients) because of infertility from retrograde ejaculation, low or no sperm concentration, or poor sperm motility [149, 150].
11.3 Female sexual function and fertility
Concerns regarding sexual function among females with CBE are predominantly three-fold: appearance of external genitalia, adequacy of vaginal opening, and uterine prolapse. Although initial correction of female external genitalia is undertaken at time of primary closure, surgical revision is frequently performed at puberty. Recurrence of pubic diastasis following closure may lead to flattening of mons pubis, separation of pubic hair, and/or separation of clitoral halves. The vaginal orifice is more vertical and stenotic in appearance, which can be resolved with local tissue rearrangement and post-operative dilation [151, 152]. Uterine prolapse occurs more frequently, and at younger ages, in women with CBE . As many as 60% of pregnant CBE women will develop prolapse [147, 154, 155]. In this population, uterine suspension was only modestly successful leading many experts to recommend uterine fixation [152, 156, 157]. Prophylactic uterine suspension should be considered to prevent prolapse [154, 158].
Sexual desire is reportedly normal in adult women, and most are sexually active [149, 153]. Average age for commencement of sexual activity was 20 years and outside of a few complaints of dyspareunia, most indicated normal orgasms . In some cases, sexual activity was restricted due to perceived cosmetic appearance of external genitalia.
Fertility among women born with CBE is generally preserved with up to 66% of women attempting conception able to achieve successful pregnancies [149, 159]. Cervical and uterine prolapse and temporary urinary incontinence are common complications following pregnancy [149, 160]. Pregnancy in a woman with bladder exstrophy remains high risk for both the mother and the fetus and warrants referral to a tertiary care center for obstetrical care . In most cases, planned cesarean section with a trained urologist present and/or involved in the delivery appears to be the safest mode for delivery.
Classic bladder exstrophy is a debilitating multi-system malformation that proves a formidable challenge to even the most experienced of surgeons. Modern advances in the management of CBE including prenatal diagnosis, delayed operative timing, use of pelvic osteotomy with pelvic and extremity immobilization help to optimal closure outcomes. Long-term urinary continence, cosmesis, sexual function, and fertility among these patients continue to present new challenges for the upcoming generation of pediatric and reconstructive urologists.
Availability of data and materials
Bladder exstrophy-epispadias complex
Omphalocele, exstrophy, imperforate anus, and spinal abnormalities
Classic bladder exstrophy
Magnetic resonance imaging
Modern staged reconstruction of exstrophy
Complete primary repair of exstrophy
Bladder neck reconstruction
Bilateral ureteral reimplantation
Continent urinary diversion
Quality of life
Full thickness skin graft
Radial forearm free flap
Pedicled anterolateral thigh flap
Nelson CP, Dunn RL, Wei JT (2005) Contemporary epidemiology of bladder exstrophy in the United States. J Urol 173(5):1728–1731. https://doi.org/10.1097/01.ju.0000154821.21521.9b
Siffel C, Correa A, Amar E et al (2011) Bladder exstrophy: an epidemiologic study from the international clearinghouse for birth defects surveillance and research, and an overview of the literature. Am J Med Genet C Semin Med Genet 157C(4):321–332. https://doi.org/10.1002/ajmg.c.30316
Cervellione RM, Mantovani A, Gearhart J et al (2015) Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe. J Pediatr Urol 11(6):337.e1-337.e6. https://doi.org/10.1016/j.jpurol.2015.03.023
Ebert AK, Zwink N, Reutter HM, Jenetzky E (2021) A prevalence estimation of exstrophy and epispadias in Germany from public health insurance data. Front Pediatr 9:648414. https://doi.org/10.3389/fped.2021.648414
Shapiro E, Lepor H, Jeffs RD (1984) The inheritance of the exstrophy-epispadias complex. J Urol 132(2):308–310. https://doi.org/10.1016/s0022-5347(17)49605-4
Ives E, Coffey R, Carter CO (1980) A family study of bladder exstrophy. J Med Genet 17(2):139–141. https://doi.org/10.1136/jmg.17.2.139
Systems IC for BDM (1987) Epidemiology of bladder exstrophy and epispadias: a communication from the international clearinghouse for birth defects monitoring systems. Teratology 36(2):221–227. https://doi.org/10.1002/tera.1420360210
Wood HM, Trock BJ, Gearhart JP (2003) In vitro fertilization and the cloacal-bladder exstrophy-epispadias complex: is there an association? J Urol 169(4):1512–1515. https://doi.org/10.1097/01.ju.0000054984.76384.66
Boyadjiev SA, Dodson JL, Radford CL et al (2004) Clinical and molecular characterization of the bladder exstrophy-epispadias complex: analysis of 232 families. BJU Int 94(9):1337–1343. https://doi.org/10.1111/j.1464-410X.2004.05170.x
Gambhir L, Höller T, Müller M et al (2008) Epidemiological survey of 214 families with bladder exstrophy-epispadias complex. J Urol 179(4):1539–1543. https://doi.org/10.1016/j.juro.2007.11.092
Marshall VF, Muecke EC (1968) Congenital abnormalities of the bladder. In: Amar AD, Culp OS, Farman F et al (eds) Malformations. Handbuch der Urologie/Encyclopedia of Urology/Encyclopédie d’Urologie. Springer, Berlin, pp 165–223. https://doi.org/10.1007/978-3-642-87399-7_4
Muecke EC (1964) The role of the cloacal membrane in exstrophy: the first successful experimental study. J Urol 92:659–667. https://doi.org/10.1016/s0022-5347(17)64028-x
Martínez-Frías ML, Bermejo E, Rodríguez-Pinilla E, Frías JL (2001) Exstrophy of the cloaca and exstrophy of the bladder: two different expressions of a primary developmental field defect. Am J Med Genet 99(4):261–269. https://doi.org/10.1002/ajmg.1210
Ambrose SS, O’Brien DP (1974) Surgical embryology of the exstrophy-epispadias complex. Surg Clin N Am 54(6):1379–1390. https://doi.org/10.1016/s0039-6109(16)40493-7
Beaman GM, Woolf AS, Cervellione RM et al (2019) 22q11.2 duplications in a UK cohort with bladder exstrophy-epispadias complex. Am J Med Genet A 179(3):404–409. https://doi.org/10.1002/ajmg.a.61032
Beaman GM, Woolf AS, Lopes FM et al (2022) Narrowing the chromosome 22q11.2 locus duplicated in bladder exstrophy-epispadias complex. J Pediatr Urol 18(3):362.e1-362e.8. https://doi.org/10.1016/j.jpurol.2022.04.006
Ludwig M, Ching B, Reutter H, Boyadjiev SA (2009) Bladder exstrophy-epispadias complex. Birt Defects Res A Clin Mol Teratol 85(6):509–522. https://doi.org/10.1002/bdra.20557
Ince TA, Cviko AP, Quade BJ et al (2002) p63 coordinates anogenital modeling and epithelial cell differentiation in the developing female urogenital tract. Am J Pathol 161(4):1111–1117. https://doi.org/10.1016/S0002-9440(10)64387-8
Ching BJ, Wittler L, Proske J et al (2010) p63 (TP73L) a key player in embryonic urogenital development with significant dysregulation in human bladder exstrophy tissue. Int J Mol Med 26(6):861–867. https://doi.org/10.3892/ijmm_00000535
Cheng W, Jacobs WB, Zhang JJR et al (2006) DeltaNp63 plays an anti-apoptotic role in ventral bladder development. Dev Camb Engl 133(23):4783–4792. https://doi.org/10.1242/dev.02621
Mathews R, Wills M, Perlman E, Gearhart JP (1999) Neural innervation of the newborn exstrophic bladder: an immunohistochemical study. J Urol 162(2):506–508
Lee BR, Perlman EJ, Partin AW, Jeffs RD, Gearhart JP (1996) Evaluation of smooth muscle and collagen subtypes in normal newborns and those with bladder exstrophy. J Urol 156(6):2034–2036
Slaughenhoupt BL, Mathews RI, Peppas DS, Gearhart JP (1999) A large animal model of bladder exstrophy: observations of bladder smooth muscle and collagen content. J Urol 162(6):2119–2122. https://doi.org/10.1016/s0022-5347(05)68137-2
Lais A, Paolocci N, Ferro F, Bosman C, Boldrini R, Caione P (1996) Morphometric analysis of smooth muscle in the exstrophy-epispadias complex. J Urol 156(2 Pt 2):819–821. https://doi.org/10.1097/00005392-199608001-00074
Johal NS, Arthurs C, Cuckow P et al (2019) Functional, histological and molecular characteristics of human exstrophy detrusor. J Pediatr Urol 15(2):154.e1-154.e9. https://doi.org/10.1016/j.jpurol.2018.12.004
Wu WJ, Maruf M, Manyevitch R et al (2020) Delaying primary closure of classic bladder exstrophy: when is it too late? J Pediatr Urol 16(6):834.e1-834.e7. https://doi.org/10.1016/j.jpurol.2020.09.003
Khandge P, Harris K, Wu W et al (2022) Mp11–14 achieving goal capacity for continence surgery: a cumulative event analysis of bladder exstrophy patients. J Urol 207(Supplement 5):e162. https://doi.org/10.1097/JU.0000000000002533.14
Diamond DA, Bauer SB, Dinlenc C et al (1999) Normal urodynamics in patients with bladder exstrophy: are they achievable? J Urol. 162(3 Pt 1):841–844. https://doi.org/10.1097/00005392-199909010-00072
Gearhart JP, Peppas DS, Jeffs RD (1995) The application of continent urinary stomas to bladder augmentation or replacement in the failed exstrophy reconstruction. Br J Urol 75(1):87–90. https://doi.org/10.1111/j.1464-410x.1995.tb07241.x
Ko JS, Lue K, Friedlander D et al (2018) Cystectomy in the pediatric exstrophy population: indications and outcomes. Urology 116:168–171. https://doi.org/10.1016/j.urology.2017.09.009
Stec AA, Baradaran N, Gearhart JP (2012) Congenital renal anomalies in patients with classic bladder exstrophy. Urology 79(1):207–209. https://doi.org/10.1016/j.urology.2011.09.022
Ebert AK, Reutter H, Ludwig M, Rösch WH (2009) The exstrophy-epispadias complex. Orphanet J Rare Dis 4:23. https://doi.org/10.1186/1750-1172-4-23
Canning DA, Gearhart JP, Peppas DS, Jeffs RD (1993) The cephalotrigonal reimplant in bladder neck reconstruction for patients with exstrophy or epispadias. J Urol 150(1):156–158. https://doi.org/10.1016/s0022-5347(17)35421-6
Mathews R, Hubbard JS, Gearhart JP (2003) Ureteral reimplantation before bladder neck plasty in the reconstruction of bladder exstrophy: indications and outcomes. Urology 61(4):820–824. https://doi.org/10.1016/S0090-4295(02)02580-3
Silver RI, Yang A, Ben-Chaim J, Jeffs RD, Gearhart JP (1997) Penile length in adulthood after exstrophy reconstruction. J Urol 157(3):999–1003
Ansari MS, Gearhart JP, Cervellione RM, Sponseller PD (2011) The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes. BJU Int 108(6):908–912. https://doi.org/10.1111/j.1464-410X.2010.10018.x
Berkowitz J, Warlick C, North A, Gearhart JP (2007) Duplicate bladder exstrophy with complete duplication of Müllerian structures. Urology 70(4):811.e15–17. https://doi.org/10.1016/j.urology.2007.07.046
Suson KD, Preece J, Di Carlo HN, Baradaran N, Gearhart JP (2016) Complexities of Müllerian anatomy in 46XX cloacal exstrophy patients. J Pediatr Adolesc Gynecol 29(5):424–428. https://doi.org/10.1016/j.jpag.2016.01.124
Sponseller PD, Bisson LJ, Gearhart JP, Jeffs RD, Magid D, Fishman E (1995) The anatomy of the pelvis in the exstrophy complex. J Bone Jt Surg Am 77(2):177–189. https://doi.org/10.2106/00004623-199502000-00003
Sirisreetreerux P, Lue KM, Ingviya T et al (2017) Failed primary bladder exstrophy closure with osteotomy: multivariable analysis of a 25-year experience. J Urol 197(4):1138–1143. https://doi.org/10.1016/j.juro.2016.09.114
Gearhart JP, Di Carlo HN (2020) Exstrophy-epispadias complex. In: Partin AW et al (eds) Campbell-walsh-wein urology, 12th edn. Amsterdam, Elsevier, pp 528–580
Cadeddu JA, Benson JE, Silver RI, Lakshmanan Y, Jeffs RD, Gearhart JP (1997) Spinal abnormalities in classic bladder exstrophy. Br J Urol 79(6):975–978. https://doi.org/10.1046/j.1464-410x.1997.00190.x
Williams AM, Solaiyappan M, Pannu HK, Bluemke D, Shechter G, Gearhart JP (2004) 3-dimensional magnetic resonance imaging modeling of the pelvic floor musculature in classic bladder exstrophy before pelvic osteotomy. J Urol 172(4 Pt 2):1702–1705. https://doi.org/10.1097/01.ju.0000140212.56826.4c
Stec AA (2011) Embryology and bony and pelvic floor anatomy in the bladder exstrophy-epispadias complex. Semin Pediatr Surg 20(2):66–70. https://doi.org/10.1053/j.sempedsurg.2010.12.011
Stec AA, Tekes A, Ertan G et al (2012) Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol 188(4S):1535–1542. https://doi.org/10.1016/j.juro.2012.02.039
Stec AA, Baradaran N, Tran C, Gearhart JP (2011) Colorectal anomalies in patients with classic bladder exstrophy. J Pediatr Surg 46(9):1790–1793. https://doi.org/10.1016/j.jpedsurg.2011.03.019
Baker LA, Gearhart JP (1998) The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol 16(3):205–211. https://doi.org/10.1007/s003450050054
Maruf M, Benz K, Jayman J et al (2019) Variant presentations of the exstrophy-epispadias complex: a 40-year experience. Urology 125:184–190. https://doi.org/10.1016/j.urology.2018.10.049
Xie D, Liang C, Xiang Y et al (2020) Prenatal diagnosis of birth defects and termination of pregnancy in Hunan Province. China Prenat Diagn 40(8):925–930. https://doi.org/10.1002/pd.5648
Goyal A, Fishwick J, Hurrell R, Cervellione RM, Dickson AP (2012) Antenatal diagnosis of bladder/cloacal exstrophy: challenges and possible solutions. J Pediatr Urol 8(2):140–144. https://doi.org/10.1016/j.jpurol.2011.05.003
Jayachandran D, Bythell M, Platt MW, Rankin J (2011) Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol 186(5):2056–2060. https://doi.org/10.1016/j.juro.2011.07.022
Keppler-Noreuil K, Gorton S, Foo F, Yankowitz J, Keegan C (2007) Prenatal ascertainment of OEIS complex/cloacal exstrophy: 15 new cases and literature review. Am J Med Genet A 143A(18):2122–2128. https://doi.org/10.1002/ajmg.a.31897
Wiesel A, Queisser-Luft A, Clementi M, Bianca S, Stoll C, EUROSCAN Study Group (2005) Prenatal detection of congenital renal malformations by fetal ultrasonographic examination: an analysis of 709,030 births in 12 European countries. Eur J Med Genet 48(2):131–144. https://doi.org/10.1016/j.ejmg.2005.02.003
Lee T, Weiss D, Roth E et al (2022) Prenatal diagnosis of bladder exstrophy and OEIS over 20 years. Urology. https://doi.org/10.1016/j.urology.2022.11.020
Gearhart JP, Ben-Chaim J, Jeffs RD, Sanders RC (1995) Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 85(6):961–964. https://doi.org/10.1016/0029-7844(95)00069-4
Antomarchi J, Moeglin D, Laurichesse H et al (2019) The pubic diastasis measurement, a key element for the diagnosis, management, and prognosis of the bladder exstrophy. Fetal Diagn Ther 45(6):435–440. https://doi.org/10.1159/000492817
Haffar A, Morrill C, Garcia A, Werner Z, Crigger C, Gearhart JP (2022) Complicating the already complex? Readability scores in bladder exstrophy and its treatment. Front Urol. https://doi.org/10.3389/fruro.2022.1044639
Cacciari A, Pilu GL, Mordenti M, Ceccarelli PL, Ruggeri G (1999) Prenatal diagnosis of bladder exstrophy: what counseling? J Urol 161(1):259–261
Kelly-Hedrick M, Geller G, Jelin AC, Gross MS (2022) Perceived value of prenatal ultrasound screening: a survey of pregnant women. Matern Child Health J. https://doi.org/10.1007/s10995-022-03515-1
Gearhart JP, Mathews R, Taylor S, Jeffs RD (1998) Combined bladder closure and epispadias repair in the reconstruction of bladder exstrophy. J Urol. 160(3 Part 2):1182–1185. https://doi.org/10.1016/S0022-5347(01)62734-4
Inouye BM, Tourchi A, Di Carlo HN, Young EE, Gearhart JP (2014) Modern management of the exstrophy-epispadias complex. Surg Res Pract. 2014:587064. https://doi.org/10.1155/2014/587064
Di Carlo HN, Maruf M, Jayman J, Benz K, Kasprenski M, Gearhart JP (2018) The inadequate bladder template: its effect on outcomes in classic bladder exstrophy. J Pediatr Urol 14(5):427.e1-427.e7. https://doi.org/10.1016/j.jpurol.2018.03.023
Baradaran N, Cervellione RM, Stec AA, Gearhart JP (2012) Delayed primary repair of bladder exstrophy: ultimate effect on growth. J Urol 188(6):2336–2341. https://doi.org/10.1016/j.juro.2012.08.037
Ferrara F, Dickson AP, Fishwick J, Vashisht R, Khan T, Cervellione RM (2014) Delayed exstrophy repair (DER) does not compromise initial bladder development. J Pediatr Urol 10(3):506–510. https://doi.org/10.1016/j.jpurol.2013.10.026
Dodson JL, Surer I, Baker LA, Jeffs RD, Gearhart JP (2001) The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome. J Urol 165(5):1656–1659
Nelson CP, North AC, Ward MK, Gearhart JP (2008) Economic impact of failed or delayed primary repair of bladder exstrophy: differences in cost of hospitalization. J Urol 179(2):680–683. https://doi.org/10.1016/j.juro.2007.09.093
Gearhart JP, Ben-Chaim J, Sciortino C, Sponseller PD, Jeffs RD (1996) The multiple reoperative bladder exstrophy closure: what affects the potential of the bladder? Urology 47(2):240–243. https://doi.org/10.1016/s0090-4295(99)80424-5
Baradaran N, Cervellione RM, Orosco R, Trock BJ, Mathews RI, Gearhart JP (2011) Effect of failed initial closure on bladder growth in children with bladder exstrophy. J Urol 186(4):1450–1454. https://doi.org/10.1016/j.juro.2011.05.067
Oesterling JE, Jeffs RD (1987) The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985. J Urol 137(2):258–262. https://doi.org/10.1016/s0022-5347(17)43972-3
McMahon DR, Cain MP, Husmann DA, Kramer SA (1996) Vesical neck reconstruction in patients with the exstrophy-epispadias complex. J Urol 155(4):1411–1413
Sweetser TH, Chisholm TC, Thompson W (1952) Exstrophy of the urinary bladder; discussion of anatomical and surgical principles applicable to its repair, with preliminary report of a case. Minn Med 35(7):654–657
Haffar A, Manyevitch R, Morrill C et al (2023) A single center’s changing trends in the management and outcomes of primary closure of classic bladder exstrophy: an evolving landscape. Urology. https://doi.org/10.1016/j.urology.2022.12.064
Rubenwolf PC, Eder F, Ebert AK, Hofstaedter F, Woodhouse CRJ, Roesch WH (2013) Persistent histological changes in the exstrophic bladder after primary closure-a cause for concern? J Urol 189(2):671–677. https://doi.org/10.1016/j.juro.2012.08.210
Gearhart JP, Jeffs RD (1991) Management of the failed exstrophy closure. J Urol 146(2 (Pt 2)):610–612. https://doi.org/10.1016/s0022-5347(17)37869-2
Grady RW, Mitchell ME (1999) Complete primary repair of exstrophy. J Urol 162(4):1415–1420
Baird AD, Gearhart JP, Mathews RI (2005) Applications of the modified Cantwell–Ransley epispadias repair in the exstrophy-epispadias complex. J Pediatr Urol 1(5):331–336. https://doi.org/10.1016/j.jpurol.2005.02.003
Baird AD, Mathews RI, Gearhart JP (2005) The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences. J Urol. 174(4 Part 1):1421–1424. https://doi.org/10.1097/01.ju.0000173127.81878.2b
Sholklapper TN, Crigger C, Haney N et al (2022) Orthopedic complications after osteotomy in patients with classic bladder exstrophy and cloacal exstrophy: a comparative study. J Pediatr Urol 18(5):586.e1-586.e8. https://doi.org/10.1016/j.jpurol.2022.09.005
Morrill CC, Manyevitch R, Haffar A et al (2023) Complications of delayed and newborn primary closures of classic bladder exstrophy: is there a difference? J Pediatr Urol. https://doi.org/10.1016/j.jpurol.2023.01.001
Haney NM, Crigger CB, Sholklapper T et al (2022) Pelvic osteotomy in cloacal exstrophy: a changing perspective. J Pediatr Surg. https://doi.org/10.1016/j.jpedsurg.2022.06.020
Purves JT, Gearhart JP (2010) The bladder exstrophy-epispadias-cloacal exstrophy complex. In: Gearhart J et al (eds) Pediatric urology, 2nd edn. Amsterdam, Elsevier, pp 386–415
Ebert AK, Zwink N, Reutter HM et al (2020) Treatment strategies and outcome of the exstrophy-epispadias complex in Germany: data from the German CURE-net. Front Pediatr 8:174. https://doi.org/10.3389/fped.2020.00174
Zaman MH, Young EE, Maruf M et al (2020) Practice patterns in classic bladder exstrophy: a global perspective. J Pediatr Urol 16(4):425–432. https://doi.org/10.1016/j.jpurol.2020.02.017
Inouye BM, Purves JT, Routh JC et al (2018) How to close classic bladder exstrophy: are subspecialty training and technique important? J Pediatr Urol 14(5):426.e1-426.e6. https://doi.org/10.1016/j.jpurol.2018.02.025
Baird AD, Nelson CP, Gearhart JP (2007) Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 3(4):311–315. https://doi.org/10.1016/j.jpurol.2006.09.009
Wu WJ, Maruf M, Harris KT et al (2020) Delaying reclosure of bladder exstrophy leads to gradual decline in bladder capacity. J Pediatr Urol 16(3):355.e1-355.e5. https://doi.org/10.1016/j.jpurol.2020.03.019
Di Carlo HN, Manyevitch R, Wu WJ et al (2020) Continence after BNR in the complete repair of bladder exstrophy (CPRE): a single institution expanded experience. J Pediatr Urol 16(4):433.e1-433.e6. https://doi.org/10.1016/j.jpurol.2020.05.011
Ellison JS, Shnorhavorian M, Willihnganz-Lawson K, Grady R, Merguerian PA (2016) A critical appraisal of continence in bladder exstrophy: long-term outcomes of the complete primary repair. J Pediatr Urol 12(4):205.e1–7. https://doi.org/10.1016/j.jpurol.2016.04.005
Jarosz SL, Weaver JK, Weiss DA et al (2022) Bilateral ureteral reimplantation at complete primary repair of exstrophy: post-operative outcomes. J Pediatr Urol 18(1):37.e1-37.e5. https://doi.org/10.1016/j.jpurol.2021.10.012
Weiss DA, Shukla AR, Borer JG et al (2020) Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model. J Pediatr Urol 16(4):435.e1-435.e6. https://doi.org/10.1016/j.jpurol.2020.05.153
Mitchell ME, Bägli DJ (1996) Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol 155(1):300–304
Kasprenski M, Maruf M, Davis R et al (2020) Penile disassembly in complete primary repair of bladder exstrophy: time for re-evaluation? Urology 137:146–151. https://doi.org/10.1016/j.urology.2019.12.020
Cervellione RM, Husmann DA, Bivalacqua TJ, Sponseller PD, Gearhart JP (2010) Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms. J Pediatr Urol 6(5):450–456. https://doi.org/10.1016/j.jpurol.2010.04.007
Sack BS, Borer JG (2019) A single-institution experience of complete primary repair of bladder exstrophy in girls: risk factors for urinary retention. J Pediatr Urol 15(3):262.e1-262.e6. https://doi.org/10.1016/j.jpurol.2019.02.019
Joshi RS, Eftekharzadeh S, Shukla AR et al (2022) Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: results from the international bladder exstrophy consortium. J Pediatr Urol. https://doi.org/10.1016/j.jpurol.2022.04.018
Kelly JH, Taghavi K, Mushtaq I, Justin H (2022) Kelly and his procedure for bladder exstrophy and epispadias. J Pediatr Surg 57(2):314–321. https://doi.org/10.1016/j.jpedsurg.2021.09.005
Ansell JS (2002) Surgical treatment of exstrophy of the bladder with emphasis on neonatal primary closure: personal experience with 28 consecutive cases treated at the University of Washington Hospitals from 1962 to 1977: techniques and results, 1979. J Urol 168(1):214–217
Leclair MD, Villemagne T, Faraj S, Suply E (2015) The radical soft-tissue mobilization (Kelly repair) for bladder exstrophy. J Pediatr Urol 11(6):364–365. https://doi.org/10.1016/j.jpurol.2015.08.007
Kelly JH (1998) Exstrophy and epispadias: Kelly’s method of repair. In: O’Neill JA et al (eds) Pediatric surgery, 5th edn. Maryland Heights, Mosby Publishing, pp 1732–1759
Leclair MD, Faraj S, Sultan S et al (2018) One-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results. J Pediatr Urol 14(6):558–564. https://doi.org/10.1016/j.jpurol.2018.07.013
Borer JG, Vasquez E, Canning DA et al (2017) Short-term outcomes of the multi-institutional bladder exstrophy consortium: successes and complications in the first two years of collaboration. J Pediatr Urol 13(3):275.e1-275.e6. https://doi.org/10.1016/j.jpurol.2017.01.006
Meldrum KK, Baird AD, Gearhart JP (2003) Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success. Urology 62(6):1109–1113. https://doi.org/10.1016/s0090-4295(03)00791-x
Inouye BM, Lue K, Abdelwahab M et al (2016) Newborn exstrophy closure without osteotomy: is there a role? J Pediatr Urol 12(1):51.e1–4. https://doi.org/10.1016/j.jpurol.2015.07.010
Gearhart JP, Forschner DC, Jeffs RD, Ben-Chaim J, Sponseller PD (1996) A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy. J Urol 155(2):689–693
Haffar A, Morrill C, Crigger C, Sponseller PD, Gearhart JP (2022) Fixation with lower limb immobilization in primary and secondary exstrophy closure: a saving grace. J Pediatr Urol. https://doi.org/10.1016/j.jpurol.2022.12.009
Shnorhavorian M, Song K, Zamilpa I, Wiater B, Mitchell MM, Grady RW (2010) Spica casting compared to Bryant’s traction after complete primary repair of exstrophy: safe and effective in a longitudinal cohort study. J Urol 184(2):669–673. https://doi.org/10.1016/j.juro.2010.03.057
Zaman M, Kasprenski M, Maruf M et al (2019) Impact of pelvic immobilization techniques on the outcomes of primary and secondary closures of classic bladder exstrophy. J Pediatr Urol 15(4):382.e1-382.e8. https://doi.org/10.1016/j.jpurol.2019.04.009
Mushtaq I, Garriboli M, Smeulders N et al (2014) Primary bladder exstrophy closure in neonates: challenging the traditions. J Urol 191(1):193–197. https://doi.org/10.1016/j.juro.2013.07.020
Hofmann A, Haider M, Promm M, Neissner C, Badelt G, Rösch WH (2022) Delayed primary closure of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to neonatal surgery. J Pediatr Surg 57(10):303–308. https://doi.org/10.1016/j.jpedsurg.2021.12.017
Sponseller PD, Jani MM, Jeffs RD, Gearhart JP (2001) Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Jt Surg Am 83(2):184–193. https://doi.org/10.2106/00004623-200102000-00005
Harris KT, Villela NA, Alam R et al (2022) The exstrophy experience: a national survey assessing urinary continence, bladder management, and oncologic outcomes in adults. J Pediatr Urol. https://doi.org/10.1016/j.jpurol.2022.11.014
Chalmers D, Ferrer F (2011) Continent urinary diversion in the epispadias-exstrophy complex. Semin Pediatr Surg 20(2):102–108. https://doi.org/10.1053/j.sempedsurg.2010.12.005
Novak TE, Costello JP, Orosco R, Sponseller PD, Mack E, Gearhart JP (2010) Failed exstrophy closure: management and outcome. J Pediatr Urol 6(4):381–384. https://doi.org/10.1016/j.jpurol.2009.10.009
Duckett JW, Gazak JM (1983) Complications of ureterosigmoidostomy. Urol Clin N Am 10(3):473–481. https://doi.org/10.1016/S0094-0143(21)01709-2
Spence HM, Hoffman WW, Fosmire GP (1979) Tumour of the colon as a late complication of ureterosigmoidostomy for exstrophy of the bladder. Br J Urol 51(6):466–470. https://doi.org/10.1111/j.1464-410x.1979.tb03580.x
Koo HP, Avolio L, Duckett JW (1996) Long-term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol 156(6):2037–2040
Gobet R, Weber D, Renzulli P, Kellenberger C (2009) Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol 5(3):190–196. https://doi.org/10.1016/j.jpurol.2008.11.007
Pettersson L, Tranberg J, Abrahamsson K, Pettersson S, Sillen U, Jonsson O (2013) Half century of followup after ureterosigmoidostomy performed in early childhood. J Urol 189(5):1870–1875. https://doi.org/10.1016/j.juro.2012.11.179
D’elia G, Pahernik S, Fisch M, Hohenfellner R, Thüroff JW (2004) Mainz Pouch II technique: 10 years’ experience. BJU Int 93(7):1037–1042. https://doi.org/10.1111/j.1464-410X.2003.04777.x
Honeck P, Kienle P, Huck N, Neisius A, Thüroff J, Stein R (2017) Adenocarcinoma in continent anal urinary diversion: is a sigma rectum pouch a surgical option after failed ureterosigmoidostomy? Urology 103:209–213. https://doi.org/10.1016/j.urology.2017.01.013
Smeulders N, Woodhouse CR (2001) Neoplasia in adult exstrophy patients. BJU Int 87(7):623–628. https://doi.org/10.1046/j.1464-410x.2001.02136.x
Novak TE, Lakshmanan Y, Frimberger D, Epstein JI, Gearhart JP (2005) Polyps in the exstrophic bladder. A cause for concern? J Urol 174(4 Pt 2):1522–1526. https://doi.org/10.1097/01.ju.0000179240.25781.1b
Stec AA, Baradaran N, Schaeffer A, Gearhart JP, Matthews RI (2012) The modern staged repair of classic bladder exstrophy: a detailed postoperative management strategy for primary bladder closure. J Pediatr Urol 8(5):549–555. https://doi.org/10.1016/j.jpurol.2011.09.007
Baker LA, Jeffs RD, Gearhart JP (1999) Urethral obstruction after primary exstrophy closure: what is the fate of the genitourinary tract? J Urol 161(2):618–621. https://doi.org/10.1016/s0022-5347(01)61983-9
Gearhart JP, Jeffs RD (1987) The use of parenteral testosterone therapy in genital reconstructive surgery. J Urol 138(4 Part 2):1077–1078. https://doi.org/10.1016/S0022-5347(17)43507-5
Gearhart JP, Mathews R (2000) Penile reconstruction combined with bladder closure in the management of classic bladder exstrophy: illustration of technique. Urology 55(5):764–770. https://doi.org/10.1016/s0090-4295(00)00458-1
Shnorhavorian M, Grady RW, Andersen A, Joyner BD, Mitchell ME (2008) Long-term followup of complete primary repair of exstrophy: the Seattle experience. J Urol 180(4 Suppl):1615–1619. https://doi.org/10.1016/j.juro.2008.04.085
Frimberger D (2011) Diagnosis and management of epispadias. Semin Pediatr Surg 20(2):85–90. https://doi.org/10.1053/j.sempedsurg.2011.01.003
Massanyi EZ, Shah B, Schaeffer AJ, DiCarlo HN, Sponseller PD, Gearhart JP (2013) Persistent vesicocutaneous fistula after repair of classic bladder exstrophy: a sign of failure? J Pediatr Urol 9(6 Pt A):867–871. https://doi.org/10.1016/j.jpurol.2012.11.016
Inouye BM, Di Carlo HN, Young EE, Tourchi A, Gearhart JP (2015) Secondary reclosure in classic bladder exstrophy: challenges and outcomes. Urology 85(5):1179–1182. https://doi.org/10.1016/j.urology.2015.01.029
Hesh CA, Young E, Intihar P, Gearhart JP (2016) The cost of failure: the economic impact of failed primary closure in classic bladder exstrophy. J Pediatr Surg 51(8):1312–1316. https://doi.org/10.1016/j.jpedsurg.2015.11.011
Gearhart JP, Peppas DS, Jeffs RD (1993) The failed exstrophy closure: strategy for management. Br J Urol 71(2):217–220. https://doi.org/10.1111/j.1464-410x.1993.tb15922.x
Burki T, Hamid R, Duffy P, Ransley P, Wilcox D, Mushtaq I (2006) Long-term followup of patients after redo bladder neck reconstruction for bladder exstrophy complex. J Urol 176(3):1138–1141. https://doi.org/10.1016/j.juro.2006.04.055
Montagnino B, Czyzewski DI, Runyan RD, Berkman S, Roth DR, Gonzales ET (1998) Long-term adjustment issues in patients with exstrophy. J Urol 160(4):1471–1474
Jochault-Ritz S, Mercier M, Aubert D (2010) Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study. J Pediatr Surg 45(8):1693–1700. https://doi.org/10.1016/j.jpedsurg.2010.03.032
Wittmeyer V, Aubry E, Liard-Zmuda A et al (2010) Quality of life in adults with bladder exstrophy-epispadias complex. J Urol 184(6):2389–2394. https://doi.org/10.1016/j.juro.2010.08.022
Dodson JL, Furth SL, Yenokyan G et al (2010) Parent perspectives of health related quality of life for adolescents with bladder exstrophy-epispadias as measured by the child health questionnaire-parent form 50. J Urol 184(4 Suppl):1656–1661. https://doi.org/10.1016/j.juro.2010.03.122
Spencer KA, Ramji J, Unadkat P et al (2022) Caregiver distress: a mixed methods evaluation of the mental health burden of caring for children with bladder exstrophy. Front Pediatr 10:948490. https://doi.org/10.3389/fped.2022.948490
Schaeffer AJ, Yenokyan G, Alcorn K et al (2012) Health related quality of life in adolescents with bladder exstrophy-epispadias as measured by the Child Health Questionnaire-Child Form 87. J Urol 188(5):1924–1929. https://doi.org/10.1016/j.juro.2012.07.014
Johnston JH (1975) The genital aspects of exstrophy. J Urol 113(5):701–705. https://doi.org/10.1016/s0022-5347(17)59557-9
Harris TGW, Maruf M, Leto Barone AA, Redett RJ, Gearhart JP (2020) Utility of skin grafting and tissue expansion in penile reconstruction for the exstrophy-epispadias complex. Urology 136:231–237. https://doi.org/10.1016/j.urology.2019.10.017
Fainberg J, Gamal A, Hanna MK (2021) Outcome of penile lengthening of adolescents and young adults born with bladder exstrophy and epispadias. J Pediatr Urol 17(3):394.e1-394.e6. https://doi.org/10.1016/j.jpurol.2021.01.036
Massanyi EZ, Gupta A, Goel S et al (2013) Radial forearm free flap phalloplasty for penile inadequacy in patients with exstrophy. J Urol 190(4 Suppl):1577–1582. https://doi.org/10.1016/j.juro.2012.12.050
Harris TGW, Manyevitch R, Wu WJ et al (2021) Pedicled anterolateral thigh and radial forearm free flap phalloplasty for penile reconstruction in patients with bladder exstrophy. J Urol 205(3):880–887. https://doi.org/10.1097/JU.0000000000001404
Segal RL, Massanyi EZ, Gupta AD et al (2015) Inflatable penile prosthesis technique and outcomes after radial forearm free flap neophalloplasty. Int J Impot Res 27(2):49–53. https://doi.org/10.1038/ijir.2014.30
Lumen N, Monstrey S, Ceulemans P, van Laecke E, Hoebeke P (2008) Reconstructive surgery for severe penile inadequacy: phalloplasty with a free radial forearm flap or a pedicled anterolateral thigh flap. Adv Urol 2008:704343. https://doi.org/10.1155/2008/704343
Harris TGW, Khandge P, Wu WJ et al (2022) Sexual health outcomes after penile reconstruction in the exstrophy-epispadias complex. J Pediatr Urol 18(6):747–755. https://doi.org/10.1016/j.jpurol.2022.02.016
Hankinson JC, Eldridge MA, Ostrander R et al (2014) Emotional and behavioral functioning in children with bladder exstrophy-epispadias complex: a developmental perspective. J Pediatr Urol 10(1):136–141. https://doi.org/10.1016/j.jpurol.2013.07.013
Harris K, Villela NA, Alam R et al (2021) Pd15-08 the exstrophy experience: a national survey assessing sexual function and fertility outcomes in adults with bladder exstrophy-epispadias complex. J Urol 206(Supplement 3):e275–e275. https://doi.org/10.1097/JU.0000000000001997.08
Baumgartner TS, Lue KM, Sirisreetreerux P et al (2017) Long-term sexual health outcomes in men with classic bladder exstrophy. BJU Int 120(3):422–427. https://doi.org/10.1111/bju.13866
Cervellione RM, Phillips T, Baradaran N, Asanuma H, Mathews RI, Gearhart JP (2010) Vaginoplasty in the female exstrophy population: outcomes and complications. J Pediatr Urol 6(6):595–599. https://doi.org/10.1016/j.jpurol.2010.01.011
Stein R, Fisch M, Bauer H, Friedberg V, Hohenfellner R (1995) Operative reconstruction of the external and internal genitalia in female patients with bladder exstrophy or incontinent epispadias. J Urol 154(3):1002–1007
Mathews RI, Gan M, Gearhart JP (2003) Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int 91(9):845–849. https://doi.org/10.1046/j.1464-410x.2003.04244.x
Woodhouse CR (1999) The gynaecology of exstrophy. BJU Int 83(Suppl 3):34–38. https://doi.org/10.1046/j.1464-410x.1999.0830s3034.x
Quiroz Y, Llorens E, Novoa R et al (2021) Pregnancy in pateints with exstrophy-epispadias complex: are higher rates of complications and spontaneous abortion inevitable? Urology 154:326–332. https://doi.org/10.1016/j.urology.2021.01.061
Anusionwu I, Baradaran N, Trock BJ, Stec AA, Gearhart JP, Wright EJ (2012) Is pelvic osteotomy associated with lower risk of pelvic organ prolapse in postpubertal females with classic bladder exstrophy? J Urol 188(6):2343–2346. https://doi.org/10.1016/j.juro.2012.08.034
Miles-Thomas J, Gearhart JP, Gearhart SL (2006) An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy. J Gastrointest Surg Off J Soc Surg Aliment Tract 10(4):473–477. https://doi.org/10.1016/j.gassur.2006.01.001
Stein R, Fisch M, Black P, Hohenfellner R (1999) Strategies for reconstruction after unsuccessful or unsatisfactory primary treatment of patients with bladder exstrophy or incontinent epispadias. J Urol 161(6):1934–1941
Deans R, Banks F, Liao LM, Wood D, Woodhouse C, Creighton SM (2012) Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study. Am J Obstet Gynecol 206(6):496.e1-496.e6. https://doi.org/10.1016/j.ajog.2012.03.016
Giron AM, Passerotti CC, Nguyen H, da Cruz JAS, Srougi M (2011) Bladder exstrophy: reconstructed emale patients achieving normal pregnancy and delivering normal babies. Int Braz J Urol Off J Braz oc Urol 37(5):605–610. https://doi.org/10.1590/s1677-55382011000500006
The Kwok Family Foundation of Hong Kong supports the exstrophy database and laboratory research.
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Ethics approval and consent to participate
This research adheres to the ethical research criteria established and approved by our institutional review board.
Consent for publication
No new patient data have been provided in this review paper.
The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.
About this article
Cite this article
Morrill, C.C., Haffar, A., Harris, T.G.W. et al. Current management of classic bladder exstrophy in the modern era. Afr J Urol 29, 27 (2023). https://doi.org/10.1186/s12301-023-00356-4
- Bladder exstrophy
- Transitional urology