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Genes Jul 2021The bladder exstrophy-epispadias complex (BEEC) is an abdominal midline malformation comprising a spectrum of congenital genitourinary abnormalities of the abdominal... (Review)
Review
The bladder exstrophy-epispadias complex (BEEC) is an abdominal midline malformation comprising a spectrum of congenital genitourinary abnormalities of the abdominal wall, pelvis, urinary tract, genitalia, anus, and spine. 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 knowledge on this multifactorial disorder, including phenotypic and anatomical characterization, epidemiology, proposed developmental mechanisms, existing animal models, and implicated genetic and environmental components.
Topics: Bladder Exstrophy; Epispadias; Female; Genetic Predisposition to Disease; Genome-Wide Association Study; Humans; Male
PubMed: 34440323
DOI: 10.3390/genes12081149 -
Current Treatment Options in Pediatrics 2022Urinary tract infection (UTI) in children is a major source of office visits and healthcare expenditure. Research into the diagnosis, treatment, and prophylaxis of UTI... (Review)
Review
PURPOSE OF REVIEW
Urinary tract infection (UTI) in children is a major source of office visits and healthcare expenditure. Research into the diagnosis, treatment, and prophylaxis of UTI has evolved over the past 10 years. The development of new imaging techniques and UTI screening tools has improved our diagnostic accuracy tremendously. Identifying who to treat is imperative as the increase in multi-drug-resistant organisms has emphasized the need for antibiotic stewardship. This review covers the contemporary management of children with UTI and the data-driven paradigm shifts that have been implemented into clinical practice.
RECENT FINDINGS
With recent data illustrating the self-limiting nature and low prevalence of clinically significant vesicoureteral reflux (VUR), investigational imaging in children has become increasingly less frequent. Contrast-enhanced voiding urosonogram (CEVUS) has emerged as a useful diagnostic tool, as it can provide accurate detection of VUR without the need of radiation. The urinary and intestinal microbiomes are being investigated as potential therapeutic drug targets, as children with recurrent UTIs have significant alterations in bacterial proliferation. Use of adjunctive corticosteroids in children with pyelonephritis may decrease the risk of renal scarring and progressive renal insufficiency. The development of a vaccine against an antigen present on may change the way we treat children with recurrent UTIs.
SUMMARY
The American Academy of Pediatrics defines a UTI as the presence of at least 50,000 CFU/mL of a single uropathogen obtained by bladder catheterization with a dipstick urinalysis positive for leukocyte esterase (LE) or WBC present on urine microscopy. UTIs are more common in females, with uncircumcised males having the highest risk in the first year of life. is the most frequently cultured organism in UTI diagnoses and multi-drug-resistant strains are becoming more common. Diagnosis should be confirmed with an uncontaminated urine specimen, obtained from mid-stream collection, bladder catheterization, or suprapubic aspiration. Patients meeting criteria for imaging should undergo a renal and bladder ultrasound, with further investigational imaging based on results of ultrasound or clinical history. Continuous antibiotic prophylaxis is controversial; however, evidence shows patients with high-grade VUR and bladder and bowel dysfunction retain the most benefit. Open surgical repair of reflux is the gold standard for patients who fail medical management with endoscopic approaches available for select populations.
PubMed: 37521173
DOI: 10.1007/s40746-022-00242-1 -
African Journal of Paediatric Surgery :... 2022Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem...
INTRODUCTION
Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem in Sub-Saharan Africa. While outlining our challenges, we report our management experience to improve our results.
SUBJECTS AND METHODS
We retrospectively reviewed the records of patients undergoing surgical repair of classic bladder exstrophy at our department between January 2010 and December 2019 (10 years). Epidemiological, clinical, therapeutic and evolution data were analysed.
RESULTS
Twenty-five children with classic bladder exstrophy were treated. Our series included 16 boys and 9 girls with a sex ratio of 1.7. Age ranged from 0 day to 6 years. Twenty-five bladder closures were performed, associated to pelvic osteotomy in 11 cases. Epispadias repair was performed on nine boys. Eight cases of bladder neck reconstruction and three cases of bladder enlargement were performed. We observed six bladder fistulas, four wound dehiscence, of which three partial, two parietal suppurations and six cases of urinary tract infection. Eight children had a continence of 1-2 h.
CONCLUSION
The treatment of bladder exstrophy in our context is still limited because of financial difficulties encountered by the population and the insufficient technical platform in our country.
Topics: Bladder Exstrophy; Child; Epispadias; Female; Humans; Infant, Newborn; Male; Retrospective Studies; Urinary Bladder; Urinary Incontinence
PubMed: 35775519
DOI: 10.4103/ajps.AJPS_167_20 -
F1000Research 2019Cystectomy and urinary bladder substitution are rare in children but may be necessary in some cases of bladder exstrophy, in the setting of malignancy, or in other... (Review)
Review
Cystectomy and urinary bladder substitution are rare in children but may be necessary in some cases of bladder exstrophy, in the setting of malignancy, or in other settings in which the bladder is severely dysfunctional. This article details advances in surgical techniques in creating continent urinary diversions in this specialized pediatric population.
Topics: Bladder Exstrophy; Child; Cystectomy; Humans; Urinary Bladder; Urinary Diversion; Urinary Reservoirs, Continent
PubMed: 31824647
DOI: 10.12688/f1000research.16607.1 -
Surgery Research and Practice 2014The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical... (Review)
Review
The exstrophy-epispadias complex is a rare spectrum of malformations affecting the genitourinary system, anterior abdominal wall, and pelvis. Historically, surgical outcomes were poor in patients with classic bladder exstrophy and cloacal exstrophy, the two more severe presentations. However, modern techniques to repair epispadias, classic bladder exstrophy, and cloacal exstrophy have increased the success of achieving urinary continence, satisfactory cosmesis, and quality of life. Unfortunately, these procedures are not without their own complications. This review provides readers with an overview of the management of the exstrophy-epispadias complex and potential surgical complications.
PubMed: 25374956
DOI: 10.1155/2014/587064 -
Cureus Oct 2022Bladder exstrophy (BE) is a rare congenital anomaly caused by an embryological defect in the closure of the abdominal wall. It comprises a spectrum of defects about...
Bladder exstrophy (BE) is a rare congenital anomaly caused by an embryological defect in the closure of the abdominal wall. It comprises a spectrum of defects about severity, including epispadias in the mildest form and cloacal exstrophy in the worst. Surgical correction is required to achieve urinary continence, maintain normal renal function, achieve secured abdominal wall closure, and create cosmetically and functionally satisfactory genitalia. Iliac bone osteotomy is considered essential to achieve the above goals in most patients by reducing the tension of the closed abdominal wall layers, particularly when present late in infancy. Several types of pelvic iliac bone osteotomy have been described to aid bladder and cloacal exstrophy closure. They can be grouped into posterior iliac osteotomy, anterior iliac osteotomy, oblique (also called diagonal) iliac osteotomy, and a combination of posterior and anterior iliac osteotomy. We described here the Y-pelvic osteotomy, which was developed by the Manchester Orthopaedic Group in the United Kingdom. It has the advantage of anterior and posterior osteotomies but also has less risk to the neurovascular structures, less blood loss, and ease of surgical technique. The osteotomy was named the Y-pelvic osteotomy due to the morphological shape it resembles.
PubMed: 36415431
DOI: 10.7759/cureus.30520 -
Frontiers in Pediatrics 2019Although enormous effort has been made to further improve the operative techniques worldwide, the management of bladder exstrophy (BE) remains one of the most... (Review)
Review
Although enormous effort has been made to further improve the operative techniques worldwide, the management of bladder exstrophy (BE) remains one of the most significant challenges in pediatric urology. Today it is universally agreed that successful and gentle initial bladder closure is decisive for favorable long-term outcome with regard to bladder capacity, renal function and continence. Due to a number of reasons, including a lack of comparable multicenter studies, a range of concepts is currently used to achieve successful primary closure. We review the literature of the last 15 years on the current concepts of bladder exstrophy repair with regard to the time of primary closure (initial vs. delayed closure), the concepts of primary closure (single-stage vs. staged approach; without osteotomy vs. osteotomy) and their outcomes. There is a worldwide lack of multicenter outcome studies with adequate patient numbers and precisely defined outcome parameters, based on the use of validated instruments. The modern staged repair (MRSE) in different variations, the complete primary reconstruction of exstrophy (CPRE), and the radical soft-tissue mobilization (RSTM) had been the most extensively studied and reported procedures. These major concepts are obligatory stable now for more than 20 years. Nevertheless, there are still a lot of open-ended questions e.g., on the potential for development of the bladder template, on continence, on long-term orthopedic outcome, on sexuality and fertility and on quality of life. Management of BE remains difficult and controversial. Further, clinical research should focus on multi-institutional collaborative trials to determine the optimal approach.
PubMed: 30984727
DOI: 10.3389/fped.2019.00110 -
Urology Case Reports Nov 2021The Exstrophy - Epispadias Complex (EEC) is a spectrum of rare congenital malformations involving the urinary, genital and musculoskeletal systems. We present an...
The Exstrophy - Epispadias Complex (EEC) is a spectrum of rare congenital malformations involving the urinary, genital and musculoskeletal systems. We present an atypical or variant case of EEC in which a bladder plate is found involving a small omphalocele, separated pubic bones and bladder and urethral duplication. The treatment had a favorable outcome, with bladder control and the child voiding normally. Perhaps the best accepted embryonic theory to explain exstrophy and its variants should be reviewed as it fails to satisfactorily explain the alterations we found.
PubMed: 34285880
DOI: 10.1016/j.eucr.2021.101769 -
Wiley Interdisciplinary Reviews.... 2013Congenital anomalies of the lower urinary tract (CALUT) are a family of birth defects of the ureter, the bladder, and the urethra. CALUT includes ureteral anomaliesc... (Review)
Review
Congenital anomalies of the lower urinary tract (CALUT) are a family of birth defects of the ureter, the bladder, and the urethra. CALUT includes ureteral anomaliesc such as congenital abnormalities of the ureteropelvic junction (UPJ) and ureterovesical junction (UVJ), and birth defects of the bladder and the urethra such as bladder-exstrophy-epispadias complex (BEEC), prune belly syndrome (PBS), and posterior urethral valves (PUVs). CALUT is one of the most common birth defects and is often associated with antenatal hydronephrosis, vesicoureteral reflux (VUR), urinary tract obstruction, urinary tract infections (UTI), chronic kidney disease, and renal failure in children. Here, we discuss the current genetic and molecular knowledge about lower urinary tract development and genetic basis of CALUT in both human and mouse models. We provide an overview of the developmental processes leading to the formation of the ureter, the bladder, and the urethra, and different genes and signaling pathways controlling these developmental processes. Human genetic disorders that affect the ureter, the bladder and the urethra and associated gene mutations are also presented. As we are entering the postgenomic era of personalized medicine, information in this article may provide useful interpretation for the genetic and genomic test results collected from patients with lower urinary tract birth defects. With evidence-based interpretations, clinicians may provide more effective personalized therapies to patients and genetic counseling for their families.
Topics: Chromosome Mapping; Humans; Signal Transduction; Ureter; Urethra; Urinary Bladder; Urinary Tract; Urogenital Abnormalities; Vesico-Ureteral Reflux
PubMed: 23408557
DOI: 10.1002/wsbm.1212 -
Wiener Medizinische Wochenschrift (1946) Mar 2019Bladder augmentation is a demanding surgical procedure and exclusively offered for selected children and has only a small spectrum of indications. Paediatric bladder... (Review)
Review
Bladder augmentation is a demanding surgical procedure and exclusively offered for selected children and has only a small spectrum of indications. Paediatric bladder voiding dysfunction occurs either on a basis of neurological dysfunction caused by congenital neural tube defects or on a basis of rare congenital anatomic malformations. Neurogenic bladder dysfunction often responds well to a combination of specific drugs and/or intermittent self-catheterization. However, selected patients with spinal dysraphism and children with congenital malformations like bladder exstrophy and resulting small bladder capacity might require bladder augmentation. Ileocystoplasty is the preferred method of bladder augmentation to date. Because of the substantial long-and short-term morbidity of augmentation cystoplasty, recent studies have tried to incorporate new techniques and technologies, such as the use of biomaterials to overcome or reduce the adverse effects. In this regard, homografts and allografts have been implemented in bladder augmentation with varying results, but recent studies have shown promising data in terms of proliferation of urothelium and muscle cells by using biological silk grafts.
Topics: Anastomosis, Surgical; Child; Humans; Plastic Surgery Procedures; Urinary Bladder; Urodynamics; Urologic Surgical Procedures
PubMed: 30084093
DOI: 10.1007/s10354-018-0645-z