Variant of Bladder Exstrophy With an Intact Penis: Surgical Options and Approach.

Variants of bladder exstrophy are a rare but diverse spectrum of bladder exstrophy-epispadias complex. This case series describes a group of 4 unique exstrophy variant cases who had an intact phallus, but a completely open bladder plate. These patients underwent exstrophy repair and concomitant umbilicoplasty at the Civil Hospital, Ahmedabad as part of the US-India Multi-institutional Bladder Exstrophy Collaboration and were followed at the same institution. We believe that a detailed assessment of bladder neck prior to reconstructive repair and bladder closure would be beneficial in these cases as the extent of bladder neck involvement would affect reconstructive approach.

Anteroposterior Duplicated Exstrophy: A Case Report.

Duplicated bladder exstrophy is an extremely rare variant of the exstrophy/epispadias complex. Duplicated exstrophy defines an exstrophic mucosal plate in hypogastric area with a normal closed bladder. We present a unique case of an anteroposterior duplicated exstrophy in a female newborn.

Variant Presentations of the Exstrophy-Epispadias Complex: A 40-Year Experience.

OBJECTIVE: To investigate the diagnosis, surgical management, and outcomes in patients with variant EEC. Variant presentations of the exstrophy-epispadias complex (EEC) span a wide range of abnormalities. The rarity and diversity of EEC variants can lead to challenges in the diagnosis and subsequent management of this population. METHODS: The authors reviewed an institutional database of 1336 EEC patients from 1975 to 2018 for variant presentations of EEC. Variant presentations included those with skin covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse. Surgical management and outcomes were assessed. RESULTS: In total, 44 EEC variants were identified. Nineteen (43%) presented with a skin-covered BE variant. Five patients presented with duplicate BE, while 6 presented with superior vesical fistula. Fourteen patients (32%) presented with epispadias with major bladder prolapse. Overall, 36 (82%) EEC variants underwent primary bladder closure, at a median of 135 days after birth (range 1-2010), with 21 (58%) undergoing pelvic osteotomy. Primary closures were successful in 89% of cases. Continence procedures were performed in 17 patients. This includes 5 patients who underwent bladder augmentation. However even without a continence procedure, continence with volitional voiding was found in 8 patients. CONCLUSION: The most common EEC variant is the skin-covered form of BE. In order to expedite appropriate management, accurate diagnosis upon initial presentation is crucial. Still, successful surgical reconstruction often results in continence that is similar to, or better than, nonvariant EEC presentations.

Bladder exstrophy-epispadias complex.

The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal wall, pelvis, urinary tract, genitalia, and occasionally the spine and anus. The vast majority of BEEC cases are classified as non-syndromic and the etiology of this malformation is still unknown. This review presents the current state of knowledge on this multifactorial disorder, including historical retrospect, phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components. These published lines of evidence argue strongly that BEEC occurs as a result of strong genetic predisposition that is yet to be deciphered.

Rare variant of bladder exstrophy associated with urethral, bladder, and colonic duplication.

Urethral duplication, bladder duplication, and bladder exstrophy are rare congenital urinary tract anomalies. We present an extremely rare case of complete urethral and bladder duplication in a boy with bladder exstrophy, omphalocele, and colonic duplication. Bladder augmentation was subsequently performed using the left exstrophic bladder as a patch to augment the right duplicated bladder.

Bladder exstrophy variants.

In the literature it is possible to find many case reports of bladder exstrophy variants, although a thorough classification with all possible associated malformations is not yet available. On the basis of a rare case observed at their Department, the authors studied the embryology of these conditions and their associated malformations. The purpose of this study is to review the literature currently available and suggest a classification for bladder exstrophy variants. Despite the rarity of these variants, surgeons need to know all possible associated malformations in order to have the most complete and correct clinical picture of their patients.

Pseudoexstrophy with epispadias.

The exstrophy variants are uncommon anomalies. The variants of the exstrophy complex have all the stigmata of the classical exstrophy such as divergent recti, widened symphysis pubis, and low-set umbilicus; however, the urinary tract is intact to a varying degree. Pseudoexstrophy, an exstrophy variant, is very rarely associated with epispadias. We report an unusual case of pseudoexstrophy with epispadias, in whom the intact bladder was initially covered by a mucous membrane which later epithelialized. The epispadias was repaired using a penile disassembly technique with posterior and ventral placement of bladder neck and urethra. Although the urethral meatus was hypospadiac, the child had achieved dry intervals with occasional stress incontinence.

Variants of the exstrophy complex: a single institution experience.

PURPOSE: Variants of the bladder/cloacal exstrophy complex are rare. Different presentations and subsequent management and outcome are discussed. MATERIALS AND METHODS: We performed a retrospective review of our database of more than 815 patients with the exstrophy complex. Patients with variants of classic epispadias or bladder or cloacal exstrophy were identified. Anatomical presentation, surgical management, type of continence procedures and final outcome were evaluated. RESULTS: Of the 25 patients with variants 13 were treated primarily at our institution and 12 were referred. Time until primary bladder closure ranged from 1 day to 4 years. Followup after continence procedure ranged from 1 month to 39 years. Seven of the 25 patients are awaiting a continence procedure. Six patients are dry without a continence procedure, of whom 4 have superior vesical fistulas. A total of 11 patients underwent bladder neck reconstruction (BNR), of whom 3 are dry, 2 are dry during the day but are wet at night, 1 had a failed procedure and 5 are dry after continent diversion (CD). One additional patient underwent CD initially and is dry. Referred cases of epispadias with bladder prolapse were not recognized at birth and had delayed closure. Impaired bladder growth or failed BNR required CD in 4 patients, and 2 are awaiting a continence procedure. Skin covered and duplicate exstrophy had comparable outcomes to the classic presentations. Duplicated organs were used for reconstructive procedures. Of the 6 patients with cloacal variant 2 are continent of stool and 2 await a Pena procedure. One of these patients has an ileal stoma and 1 has a colostomy. CONCLUSIONS: The initial presentation of exstrophy variants can be confusing, often delaying initial treatment. Superior vesical fistulas permit continence without BNR due to an intact urinary sphincter. Variants such as epispadias with bladder prolapse and duplicate or skin covered exstrophy should be closed at birth with standardized techniques to promote bladder growth for later BNR. These cases are faced with the same long-term problems as the classic presentation. Cloacal variants can present with intact anal innervation, allowing a later Pena procedure.

Exstrophy of the cloaca and exstrophy of the bladder: two different expressions of a primary developmental field defect.

Exstrophy of the bladder (EB) and exstrophy of the cloaca (EC) are generally recognizable as distinct clinical entities. In patients with EB, the posterior bladder wall is exposed through a midline defect of the abdomen. The umbilicus is inferiorly displaced and located close to the superior margin of the exstrophic bladder. Genital abnormalities are common in boys and girls who may present epispadias and a small, split phallus or a split clitoris, a bifid uterus, and a duplicate or exstrophic vagina. In contrast to classic EB, EC is commonly associated with omphalocele, spinal defects, and incompletely formed external genitalia and is always associated with imperforate anus. Some authors state that EC and EB constitute two distinct disorders, but others consider them part of a "continuum," representing different levels of severity within the same spectrum. The use of the acronym OEIS to refer to the combination of omphalocele, exstrophy, imperforate anus, and spinal defects, in our opinion, has not helped to clarify the clinical definition, pathogenesis, or cause of this multiple congenital anomaly (MCA) pattern, mostly because the term makes no distinction between EC or EB. Here we present the epidemiological analysis of a group of characteristics in infants with EC and infants with EB to determine if they constitute two different entities. We also analyze if the different combinations of omphalocele, imperforate anus, and spinal defects are more frequent in infants with EC than in infants with MCA patterns other than EC and EB. The prevalence in our data for EC was 1:200,233 live births and 1:35,597 for EB. The clinical analysis indicated that the study defects (omphalocele, spine defects, spina bifida, and imperforate anus) tend to occur together in the same child with a higher frequency if the child has the EC defect than in infants with MCA patterns that did not include EC or EB. Our findings of low birth weight, twinning, single umbilical artery, and preferentially associated malformations suggest that EC is the result of damage occurring very early in development and that EC and EB are two different expressions of a primary polytopic developmental field defect.

Exstrophy of the bladder.

Exstrophy of the bladder is a rare congenital defect that occurs when the abdominal wall and underlying structures, including the ventral wall of the bladder, fail to fuse in utero. As a result, the lower urinary tract is exposed, and the everted bladder appears through the abdominal opening. Various surgical interventions have been employed with variable success in the hope of achieving complete dryness, full control over delivery of urine, freedom from catheters and external appliances, and a protected upper urinary tract. The most popular surgical approach is the primary bladder closure with secondary bladder neck reconstruction. Comprehensive nursing, medical, and surgical care are necessary to preserve renal and sexual function. The many complex problems experienced by these infants and their families call for a multidisciplinary approach. This article reviews occurrence, clinical presentation, and management of exstrophy of the bladder.

The pelvis of fetuses in the exstrophy complex.

By using a three-dimensional computed tomography (CT) scanner, we compared the anatomic features of the pelvis of three fetuses of same gestational age, one with a normal pelvis representing the reference model, one with classic bladder exstrophy, and one with cloacal exstrophy. The tomography slices were selected at the same levels for each case. Three angles expressing external opening of the pelvis were defined. Comparing normal and abnormal pelvises allowed definition of three criteria for the correction of the malformation: (a) the sum of the differential angles gives the amplitude of the correction needed; (b) a supraacetabular osteotomy appears to allow best closure of the pelvic ring; (c) only three slices of a CT scan are needed, which cannot be harmful, especially for neonates. Therefore, we believe that a CT scan of the pelvis should be performed whenever an osteotomy is planned in the surgical reconstruction of bladder and cloacal exstrophy.

Superior vesical fissure: an exstrophy variant or a distinct clinical entity.

A case of superior vesical fissure is presented to discuss the embryologic origin of the anomaly. In view of the simplicity of the anomaly and the discrepancies between the characteristics of superior vesical fissure and bladder exstrophy, superior vesical fissure is suggested to be a distinct clinical entity with an embryological origin different from bladder exstrophy.