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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 -
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 -
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 -
Orphanet Journal of Rare Diseases Oct 2009Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and... (Review)
Review
Exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations ranging in severity from epispadias (E) to classical bladder exstrophy (CEB) and exstrophy of the cloaca (EC). Depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. Prevalence at birth for the whole spectrum is reported at 1/10,000, ranging from 1/30,000 for CEB to 1/200,000 for EC, with an overall greater proportion of affected males. EEC is characterized by a visible defect of the lower abdominal wall, either with an evaginated bladder plate (CEB), or with an open urethral plate in males or a cleft in females (E). In CE, two exstrophied hemibladders, as well as omphalocele, an imperforate anus and spinal defects, can be seen after birth. EEC results from mechanical disruption or enlargement of the cloacal membrane; the timing of the rupture determines the severity of the malformation. The underlying cause remains unknown: both genetic and environmental factors are likely to play a role in the etiology of EEC. Diagnosis at birth is made on the basis of the clinical presentation but EEC may be detected prenatally by ultrasound from repeated non-visualization of a normally filled fetal bladder. Counseling should be provided to parents but, due to a favorable outcome, termination of the pregnancy is no longer recommended. Management is primarily surgical, with the main aims of obtaining secure abdominal wall closure, achieving urinary continence with preservation of renal function, and, finally, adequate cosmetic and functional genital reconstruction. Several methods for bladder reconstruction with creation of an outlet resistance during the newborn period are favored worldwide. Removal of the bladder template with complete urinary diversion to a rectal reservoir can be an alternative. After reconstructive surgery of the bladder, continence rates of about 80% are expected during childhood. Additional surgery might be needed to optimize bladder storage and emptying function. In cases of final reconstruction failure, urinary diversion should be undertaken. In puberty, genital and reproductive function are important issues. Psychosocial and psychosexual outcome depend on long-term multidisciplinary care to facilitate an adequate quality of life.
Topics: Abnormalities, Multiple; Bladder Exstrophy; Cloaca; Counseling; Diagnosis, Differential; Epispadias; Female; Humans; Male; Osteotomy; Prenatal Diagnosis; Prognosis
PubMed: 19878548
DOI: 10.1186/1750-1172-4-23 -
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 -
International Journal of Molecular... Jun 2022In the case of pediatric urology there are several congenital conditions, such as hypospadias and neurogenic bladder, which affect, respectively, the urethra and the... (Review)
Review
In the case of pediatric urology there are several congenital conditions, such as hypospadias and neurogenic bladder, which affect, respectively, the urethra and the urinary bladder. In fact, the gold standard consists of a urethroplasty procedure in the case of urethral malformations and enterocystoplasty in the case of urinary bladder disorders. However, both surgical procedures are associated with severe complications, such as fistulas, urethral strictures, and dehiscence of the repair or recurrence of chordee in the case of urethroplasty, and metabolic disturbances, stone formation, urine leakage, and chronic infections in the case of enterocystoplasty. With the aim of overcoming the issue related to the lack of sufficient and appropriate autologous tissue, increasing attention has been focused on tissue engineering. In this review, both the urethral and the urinary bladder reconstruction strategies were summarized, focusing on pediatric applications and evaluating all the biomaterials tested in both animal models and patients. Particular attention was paid to the capability for tissue regeneration in dependence on the eventual presence of seeded cell and growth factor combinations in several types of scaffolds. Moreover, the main critical features needed for urinary tissue engineering have been highlighted and specifically focused on for pediatric application.
Topics: Animals; Child; Humans; Male; Regenerative Medicine; Tissue Engineering; Urethra; Urethral Stricture; Urinary Bladder; Urology
PubMed: 35742803
DOI: 10.3390/ijms23126360