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Revista Da Associacao Medica Brasileira... 2016Bladder exstrophy is a rare congenital anomaly resulting from failure of fusion of the middle of the pelvis line tissues during embryogenesis. It is characterized by...
Bladder exstrophy is a rare congenital anomaly resulting from failure of fusion of the middle of the pelvis line tissues during embryogenesis. It is characterized by malformation of the lower abdominal wall involving the genitourinary tract and the musculoskeletal system. Its incidence is estimated at 1:30,000 to 1:50,000 live births, and it is 2 or 3 times more frequent in males. The child's age is important and the best results are obtained when treatment is performed shortly after birth.
PubMed: 27310539
DOI: 10.1590/1806-9282.62.03.197 -
European Journal of Pediatric Surgery :... Dec 2021Bladder exstrophy-epispadias complex (BEEC) represents the severe end of the uro-rectal malformation spectrum and has profound impact on continence, sexual, and renal... (Review)
Review
Bladder exstrophy-epispadias complex (BEEC) represents the severe end of the uro-rectal malformation spectrum and has profound impact on continence, sexual, and renal function. Treatment of BEEC is primarily surgical, and the main goals are safe closure of the abdominal wall, urinary continence while preserving renal function, and adequate cosmetic and functional genital reconstruction. Psychosocial and psychosexual outcomes and adequate health-related quality of life depend on long-term multidisciplinary care. The overall outcome is now considered very positive and affected individuals usually lead self-determined and independent lives with the desire to start their own families later in life. Certainty about the risk of recurrence and the provision of information about the current state of knowledge about the identified genetic causes with high penetrance will have an impact on family planning for healthy parents with an affected child and for affected individuals themselves. This review addresses this information and presents the current state of knowledge.
Topics: Bladder Exstrophy; Child; Epispadias; Genetic Counseling; Health Status; Humans; Quality of Life
PubMed: 34911128
DOI: 10.1055/s-0041-1740336 -
Journal of Indian Association of... 2021Incidence and recurrence of bladder stone in augmented exstrophy bladder rate is high. So, recurrent open cystolithotomy is not a preferred procedure; particularly...
AIMS
Incidence and recurrence of bladder stone in augmented exstrophy bladder rate is high. So, recurrent open cystolithotomy is not a preferred procedure; particularly through scarred tissues, consequence of previous surgeries. Percutaneous cystolithotomy (PCCL) is an old but standard procedure for retrieval of bladder stones in adults. We extrapolated PCCL for bladder stone in augmented bladders in children.
PATIENTS AND METHODS
In three patients, we made suprapubic (SP) needle track with initial puncture (IP) needle under cystoscopic guidance. Following that laparoscopic cannula was placed through dilated SP track that was crafted with Alken's dilators and bladder stones were removed with grasper.
RESULTS
On cystoscopy, we also observed the patches of skin tissues in native bladders. Continence and bladder capacity were not affected following PCCL.
CONCLUSION
PCCL in augmented bladder showed good outcome. High recurrence of bladder stone is possibly due to presence of keratin in dermal tissue; invaded mucosa in open bladder plate. It seems shaving or fulguration of those dermal elements during bladder reconstruction might decrease incidence of stone formation. However, we haven't attempted fulguration during PCCL.
PubMed: 34385769
DOI: 10.4103/jiaps.JIAPS_128_20 -
Transfusion Medicine and Hemotherapy :... Sep 2016Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need... (Review)
Review
Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need urinary diversion or augmentation cystoplasty as when untreated they may cause severe bladder dysfunction and kidney failure. Currently, the gold standard therapy of end-stage bladder disease refractory to conservative management is enterocystoplasty, a surgical enlargement of the bladder with intestinal tissue. Despite providing functional improvement, enterocystoplasty is associated with significant long-term complications, such as recurrent urinary tract infections, metabolic abnormalities, stone formation, and malignancies. Therefore, there is a strong clinical need for alternative therapies for these reconstructive procedures, of which stem cell-based tissue engineering (TE) is considered to be the most promising future strategy. This review is focused on the recent progress in bladder stem cell research and therapy and the challenges that remain for the development of a functional bladder wall.
PubMed: 27781020
DOI: 10.1159/000447977 -
Journal of Indian Association of... 2022Crucial requirement of exstrophy bladder repair is to make patients continent as well as to preserve kidney functions. We analyzed our patients' data retrospectively to...
PURPOSE
Crucial requirement of exstrophy bladder repair is to make patients continent as well as to preserve kidney functions. We analyzed our patients' data retrospectively to study their continence and to find out the justification behind continence and preservation of renal functions.
PATIENTS AND METHODS
We selected files of 18 fully continent patients from 52 patients operated. Eleven out of 18 patients were presented from beginning and 7 were referred after around 8 to 14 years, as incontinent bladder following good repair of bladder neck and posterior urethra. Eleven were operated with complete primary repair of exstrophy along with pubic osteotomy minimal and were kept on cystostomy track (CT) till augmentation to vent out vesical pressure. In seven patients, we did reduction of caliber of posterior urethra and bladder neck along with CT followed by augmentation after 6 months.
RESULTS
All 18 patients are maintaining dry period for 24 h. Two patients had enuresis but are manageable with partial fluid restriction from evening. Seventeen out of 18 patients are maintaining their renal functions.
CONCLUSION
No tension abdominal wall closure with rectus muscle apposition is essential to preserve repaired bladder exstrophy. Osteotomy prevents lateral drag to overcome failure of whole reconstruction. Increased "systolic" vesical pressure from contraction of small bladder might destroy the mechanism of continence and renal functions. Hence, venting of vesical pressure through CT is obligatory till augmentation which is of necessity to be done as early as possible to create a low-pressure continent system.
PubMed: 36530804
DOI: 10.4103/jiaps.jiaps_250_21 -
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