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Birth Defects Research. Part A,... Jun 2009The bladder exstrophy-epispadias complex (BEEC) represents an anterior midline defect with variable expression comprising a spectrum of anomalies involving the abdominal... (Review)
Review
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.
Topics: Animals; Bladder Exstrophy; Disease Models, Animal; Epispadias; Female; Humans; Male
PubMed: 19161161
DOI: 10.1002/bdra.20557 -
Archivos Espanoles de Urologia 1998To describe the procedures of bladder exstrophy closure, epispadias repair to achieve urinary control. The approaches to the failed exstrophy are also described. (Review)
Review
OBJECTIVE
To describe the procedures of bladder exstrophy closure, epispadias repair to achieve urinary control. The approaches to the failed exstrophy are also described.
METHODS
Bladder closure soon after birth is performed with concomitant pelvic osteotomy. The different techniques are described. The Cantwell-Ransley technique is preferred for epispadias repair and the Young-Dees-Leadbetter procedure for bladder neck reconstruction.
RESULTS
A success rate of 92% and a failure rate of 31% have been reported by different authors.
CONCLUSION
To date there is no definitive solution in the treatment of bladder exstrophy. Continent urinary diversion may be warranted in some cases.
Topics: Bladder Exstrophy; Epispadias; Female; Humans; Infant, Newborn; Male; Osteotomy; Pelvic Bones; Treatment Failure; Urinary Bladder
PubMed: 9773581
DOI: No ID Found -
International Urogynecology Journal and... 1997Bladder exstrophy is one of the most challenging congenital urinary tract abnormalities. Apart from the open bladder the patient also has various other abnormalities,... (Review)
Review
Bladder exstrophy is one of the most challenging congenital urinary tract abnormalities. Apart from the open bladder the patient also has various other abnormalities, including urogenital, musculoskeletal and anorectal defects. The size of the exstrophic bladder varies from patient to patient. In the female the clitoris to bifid and the vagina is anteriorly placed. In this paper various aspects of female bladder exstrophy are reviewed in detail, including incidence, etiology, anatomy and clinical features, together with early, medium-term and long-term surgical management. The aim is to achieve a functional bladder closure, although some patients are better off with a urinary diversion. Considering the complexity of the urogenital problems and the surgical management thereof, all patients require lifelong follow-up.
Topics: Abnormalities, Multiple; Bladder Exstrophy; Female; Humans; Postoperative Complications; Treatment Outcome; Urinary Bladder; Urinary Diversion; Urinary Incontinence; Urination
PubMed: 9297598
DOI: 10.1007/BF02764825 -
Journal of Pediatric Urology Feb 2022Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients' point of view are needed to...
INTRODUCTION
Bladder exstrophy (BE) affects continence and sexual function, impacting on social life and mental health. Long-term data from the patients' point of view are needed to get a real-life perspective on the problem.
STUDY DESIGN
A self-developed questionnaire concerning sexual, psychosexual and psychosocial outcomes was sent to the adult members of the Brazilian Exstrophy Group.
RESULTS
Fifty out of 67 adults from the group (74.5%) responded to the questionnaire. Failure of initial bladder closure attained 62%. Almost ¾ of the patients had augmentation cystoplasty. Bladder lithiasis was common. Esthetic procedures were frequently done. Repetitive UTI (n = 32, 64%) and kidney scars/disease (n = 20, 40%) were frequent. Most (88%) patients either depend on CIC or remain incontinent. Sexual problems predominated in males. Surgery for continence often failed, requiring re-operations, but the prognosis without these procedures was comparatively worse. Continent patients underwent more surgeries (mean 18, 13 and 9 procedures in continent, imperfectly continent and incontinent patients, respectively). Augmented patients more frequently achieved dryness (p = 0.0035). Two-thirds of the women underwent vaginoplasties, but dyspareunia/feeling of "tight" vagina still affected a quarter of them. Four women (15.4%) delivered healthy children. 91.7% of the males reported "normal" erections, but sexual inhibition was common due to feeling of having a small penis (n = 18, 75%). Persistent dorsal curvature and abnormal ejaculation were common (58.3% and 77.1%, respectively). Patients' comments related mainly to mental health issues/need for specialized care, limitations of medicine to cure/treat their disease, unavailability of experts, especially adult specialists, embarrassment over deformities and insufficient information about disease/treatment/prognosis.
DISCUSSION
Most BE patients are well-integrated into society, but feelings of sadness and low self-esteem are common. Most welcome procedures to become dry, despite self-catheterization. The results of bladder neck reconstruction are far from perfect, despite multiple attempts and bladder augmentation was often necessary. Volitional voiding is uncommon. Sexual problems are worse for males, and sexual avoidance is common. Sexual function and self-image are inter-related. It seems reasonable to offer selective esthetic procedures to improve social/sexual interaction. Obstetric complications are common, especially UTI, need for ureteral and/or conduit stenting, abnormal fetal positioning, uterine prolapse, technical problems during surgical deliveries and prematurity.
CONCLUSION
Continence/dryness in BE was mostly eventually achieved, usually depending on multiple interventions, bladder augmentation and self-catheterization. Despite multiple surgeries many adults remain incontinent. Sexual problems and avoidance are the rule in males, due to the feelings of penile inadequacy. Pregnant females deserve expert obstetric care.
Topics: Adult; Bladder Exstrophy; Child; Female; Humans; Male; Pregnancy; Retrospective Studies; Urinary Bladder; Urination; Urologic Surgical Procedures
PubMed: 34876380
DOI: 10.1016/j.jpurol.2021.11.007 -
Current Urology Reports Jul 2019The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary... (Review)
Review
PURPOSE OF REVIEW
The exstrophy-epispadias complex (EEC) represents a group of congenitally acquired malformations involving the musculoskeletal, gastrointestinal, and genitourinary systems. Classic bladder exstrophy (CBE) is the most common and best studied entity within the EEC. In this review, imaging features of CBE anatomy will be presented with surgical correlation.
RECENT FINDINGS
Magnetic resonance imaging (MRI) has emerged as a useful modality for pre- and postnatal assessment of the abdominal wall, pelvic floor, and gastrointestinal and genitourinary systems of children with CBE. The authors' experience supports use of preoperative MRI, in conjunction with navigational software, as a method for identifying complex CBE anatomy. Imaging facilitates surgical approach and improves visualization of complex anatomy, potentially helping to avoid complications. Continued investigation of imaging guidance in CBE repair is needed as surgical techniques improve.
Topics: Bladder Exstrophy; Epispadias; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Urologic Surgical Procedures
PubMed: 31286274
DOI: 10.1007/s11934-019-0916-2 -
Journal of Pediatric Urology Dec 2015Classical bladder exstrophy affects 1 in 30 000 live births. Results of surgical treatment from different institutions employing various surgical techniques are not... (Review)
Review
UNLABELLED
Classical bladder exstrophy affects 1 in 30 000 live births. Results of surgical treatment from different institutions employing various surgical techniques are not uniform, thus there is a need for a consensus on the best technique for bladder exstrophy repair. Surgical correction in bladder exstrophy would be more effective if the exact pathogenetic mechanism was deduced and the procedure was directed to correct the cause, which is responsible for the defect. The anatomy of exstrophy shows that the infraumbilical abdominal wall, the anterior wall of the bladder, and the urethra are split, with splayed out genitalia and musculature along with pubic diastasis. There is no tissue loss and hence embryological defect is unlikely to be the cause of bladder exstrophy. Thus there is a need to examine pathogenesis of bladder exstrophy.
METHODS
A literature search was made of the various hypotheses for cause of bladder exstrophy, and attempts were made to propose a new hypothesis. The present hypothesis is also the basis for a technique of mobilization of pelvic musculature, done in two stages.
RESULTS
The functional outcomes of 38 children with bladder exstrophy managed over a period of 10 years were reviewed. At a mean follow-up of 4.5 years (range 2.5-8 years), 82% of patients were functionally continent.
CONCLUSIONS
The exact embryopathogenesis of bladder exstrophy is unknown. In this study a new hypothesis is proposed, with the aim of tailoring the surgical procedure to correct this defect. Bladder exstrophy epispadias complex (BEEC) is a deformative disruption occurring after embryogenic phase and pubic diastasis, and is central to exstrophy development. A working hypothesis can be formulated in line with our observation so that future experiments based this new hypothesis can aim to elucidate the exact pathogenesis.
Topics: Bladder Exstrophy; Humans; Infant, Newborn
PubMed: 26316280
DOI: 10.1016/j.jpurol.2015.05.030 -
Current Opinion in Urology Nov 1999The modern staged approach to bladder exstrophy reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron in the 1970s. Although... (Review)
Review
The modern staged approach to bladder exstrophy reconstruction has undergone significant changes since it was first advocated by Jeffs and Cendron in the 1970s. Although varied surgical approaches have been tried in the bladder exstrophy condition, the staged approach to bladder exstrophy repair has withstood the test of time. Progress continues to be made in evaluating the outcome of older types of staged reconstruction, with continuing modification and improvements in the modern approach to staged reconstruction. This treatise will update the reader on recent advances in the treatment of bladder and cloacal exstrophy.
Topics: Bladder Exstrophy; Epispadias; Female; Humans; Male; Penis; Pregnancy; Prenatal Diagnosis; Plastic Surgery Procedures; Urinary Bladder
PubMed: 10668568
DOI: 10.1097/00042307-199911000-00002 -
Fetal and Pediatric Pathology Aug 2012Bladder exstrophy is a very rare congenital malformation in which the anterior wall of the bladder is absent, and the posterior wall is exposed externally. The...
Bladder exstrophy is a very rare congenital malformation in which the anterior wall of the bladder is absent, and the posterior wall is exposed externally. The differential diagnosis includes omphalocele, gastroschisis, and cloacal exstrophy. Ultrasound and Doppler examinations are the main diagnostic tools. Although mortality is low, termination of pregnancy should be discussed due to serious morbidities.
Topics: Abortion, Eugenic; Bladder Exstrophy; Cloaca; Diagnosis, Differential; Female; Gastroschisis; Hernia, Umbilical; Humans; Pregnancy; Ultrasonography, Doppler; Ultrasonography, Prenatal; Young Adult
PubMed: 22432588
DOI: 10.3109/15513815.2011.650286 -
Neonatal Network : NN Mar 1999Exstrophy 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... (Review)
Review
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.
Topics: Bladder Exstrophy; Counseling; Humans; Infant, Newborn; Male; Neonatal Nursing; Parents; Patient Care Planning; Perioperative Care; Perioperative Nursing
PubMed: 10362996
DOI: 10.1891/0730-0832.18.2.17 -
Journal of the Royal Society of Medicine Jan 1996Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging... (Review)
Review
Exstrophy of the bladder is rare and the incidence of bladder exstrophy is calculated to be from 1 per 30,000 to 50,000 live births with male to female ratio ranging from 1.5-5 to 1(1-4). It was found that persistence or overgrowth of the cloacal membrane on the lower anterior abdominal area, prevents normal mesenchymal ingrowth. This causes divergence of the lower abdominal muscular structures and forces the genital ridges to fuse caudal to the cloacal membrane. The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders.
Topics: Adolescent; Adult; Age Factors; Bladder Exstrophy; Child; Child, Preschool; Epispadias; Female; Fetal Diseases; Humans; Infant, Newborn; Male; Mental Disorders; Pregnancy; Prenatal Diagnosis
PubMed: 8709084
DOI: 10.1177/014107689608900112