The factors in successful exstrophy closure.
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Exstrophy-epispadias complex has been a difficult disease to treat. As in the minds of the public and most physicians alike, these children are crippled with life-long ailments and multiple operations. Much of the morbidity of this condition relates to failure to preserve urogenital tract function in these children. In recent years, through better understanding of the exstrophic anatomy and improved surgical techniques, complete repair of the exstrophic anomaly has become possible as soon as the child is born. Results of such closure indicate that such repair is not only feasible but mandatory for a successful outcome. Continence rate and cosmetic appearance are superior to the conventional 3-stage technique. Such repair is also socially acceptable as the parents will bring home a normal-looking baby with much fewer operations expected in the future. It is anticipated that complete one-stage exstrophy closure in the newborn period will revolutionize the outcome of all exstrophy patients.
A case we believe to be the longest with successful anatomic closure of exstrophy and epispadias witholt reflux or incontinence is reported. The persistence of the squamous metaplasia in the bladder despite twenty years of "turn-in" is noteworthy. The original method of closure reveals no "secret" to account for the success. The method of repair of traumatic rupture is presented.
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Since 1976, 23 children with bladder or cloacal exstrophy, meningomyelocele, sacral agenesis, the prune belly syndrome and noncompliant bladders associated with urethral valves or prior diversion underwent augmentation cystoplasty. Of these procedures 7 were combined with some type of urinary undiversion. Bowel segments used for augmentation included ileum alone in 10 patients, ileocecal segments in 4, a sigmoid patch in 8 and a hindgut patch in 1. An artificial urinary sphincter was placed at the time of bladder augmentation in 3 patients. There were no urinary fistulas or cases of urinary rediversion. Two patients required oral alkalizing agents as a result of persistent systemic acidosis. One patient required reoperation twice for ureteral obstruction, 1 had removal of the sphincter device secondary to erosion, 1 required reinforcement of the ileocecal valve owing to persistent reflux and 1 required reoperation for small bowel obstruction. Other complications included a superficial wound infection and 5 urinary tract infections, all of which were managed easily. Three patients were voiding and continent, 18 were dry with intermittent self-catheterization, 1 had giggle incontinence and 1 remained incontinent after sphincter removal. Augmentation cystoplasty appears to offer a reliable alternative to urinary diversion in the reconstructive management of children with small capacity bladders.
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Bladder extrophy is a rare congenital malformation that nowadays is surgically corrected in neonatal period. We present a case report of a 71-year-old male with a verrucous squamous cell carcinoma arising in a classical uncorrected form of bladder extrophy.
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PURPOSE: We present a modification of bladder neck reconstruction which resulted in improved continence and voiding compared to other techniques of bladder neck repairs in patients with exstrophy and complete incontinence. MATERIALS AND METHODS: The series consisted of 10 patients with the exstrophy-epispadias complex and complete incontinence who previously had undergone multiple operations for bladder closure, bladder neck reconstruction and epispadias repair. This modification combines bladder neck lengthening and narrowing of the distal half of the urethra, and submucosal embedding of the proximal half of the neourethra in the trigonal area. All patients also underwent bladder augmentation with detubularized sigmoid colon concurrent with bladder neck reconstruction. Additionally the appendical Mitrofanoff principle was applied to 5 cases. RESULTS: Of the 10 patients who underwent bladder neck reconstruction with sigmoid cystoplasty 8 are voiding voluntarily without catheterization and are dry for longer than 4 hours day and night. Only 2 patients are partially dry with stress nocturia incontinence and in both we performed a Mitrofanoff procedure as an adjunct to catheterization and to ensure voiding and continence. CONCLUSIONS: Our modified bladder neck reconstruction provides better overall voiding and continence rates than the other bladder neck/urethral reconstruction procedures in patients with exstrophy and complete incontinence.
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Although epispadias is considered to be the least severe defect of the exstrophy-epispadias complex, the treatment of this anomaly is far from trivial. Epispadias does not involve the body of the bladder or the hindgut but does affect the urethra and can affect the bladder neck. As a consequence, it presents with a spectrum of severity that can affect urinary continence if the epispadias anomaly is proximal enough to affect the urinary sphincter mechanism.