Cases reported "Bladder Exstrophy"

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1/19. Exstrophy of the bladder.

    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.
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2/19. Transcolonic retrograde ureteric catheterization assisted by 3-D computed tomography.

    A 42-year-old woman had bladder exstrophy at birth, treated by ureterocolic anastomosis of her single kidney. She suffered recurrent hyperammonaemia, leading to comas, but refused an ileal conduit. During her most recent coma, it was decided to divert her urine to test whether this would reduce hyperammonaemia: this was accomplished by transcolonic retrograde catheterization of the ureter. This was only possible after computed tomography ureterography to show the ureterocolic anastomosis.
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3/19. Catheter knotting in a catheterizable urinary reservoir.

    An 11-year-old boy with bladder extrophy was treated ultimately with an ileocystoplasty and a catheterizable stoma. In attempting to drain his bladder via intermittent catheterization, the catheter became lodged. This case details the surgical treatment of a knotted catheter in an urinary reservoir.
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4/19. Bladder preservation in adult classic exstrophy: early results of four patients.

    OBJECTIVES: To report our experience with the treatment of classic exstrophy of the bladder in a small series of adult patients using ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with flaps. The presentation of exstrophy of the bladder in adulthood is rare. The problems encountered include difficulty in abdominal closure, malignant potential, and upper tract dysfunction. The treatment of choice has been cystectomy with urinary diversion in all reported cases. methods: We treated 4 adult male patients with classic exstrophy of the bladder and complete epispadias. They had not received any previous treatment. Multiple random bladder biopsies revealed nonspecific inflammatory changes with focal areas of keratinization. Three patients were treated in two stages. The first stage included ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with the use of flaps. The epispadias was repaired in the second stage. In 1 patient, the reconstruction was completed in a single stage. RESULTS: All patients were continent at the last follow-up visit, with three using self-catheterization and one voiding spontaneously. The renal parameters and ultrasound scans were normal at a follow-up of 2 to 48 months. cystoscopy performed at 6 months postoperatively revealed normal-looking mucosa in 2 patients and mild inflammation in 1 patient. Three patients were satisfied with the cosmetic results and one complained of the small size of his penis. All patients were evaluated by psychiatrists and revealed anxiety disorders preoperatively. After surgery, all demonstrated improved social interaction. CONCLUSIONS: Vesical preservation with primary reconstruction of bladder exstrophy in adults is safe and feasible in the absence of significant histologic changes in the bladder mucosa. Abdominal closure can be achieved without difficulty with the use of transposition flaps. However, these patients require strict follow-up to detect malignant transformation at an early stage.
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5/19. Augmentation ureterocystoplasty in bladder exstrophy: 5-year follow-up in two cases.

    OBJECTIVE: To report two cases of bladder exstrophy managed successfully by augmentation ureterocystoplasty (UCP) together with bladder neck surgery and continent diversion. patients: Two boys, age 5 and 1 years respectively, had augmentation UCP. The left refluxing megaureter was used in the first case together with bladder neck reconstruction. In the second patient, bilateral obstructed magaureters were used in tandem together with bladder neck division. Both patients had appendicovesicostomy according to Mitrofanoff. RESULTS: In follow-up for over 5 years, both patients are continent with improved upper urinary tracts and normal or stable renal function. They are managed by clean intermittent catheterization (CIC) via the continent stoma with bladder capacities of 220 cc and 150 cc, respectively. Cystogram showed no vesicoureteric reflux (VUR). The procedures were considered to be successful although one patient was on oxybutinin because urodynamics showed high intravesical pressures. CONCLUSIONS: Augmentation UCP should be considered in patients with bladder exstrophy when a suitable megaureter is available. This may be combined with simultaneous bladder neck reconstruction together with a continent diversion.
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6/19. Patent urachus with bladder eversion.

    An infant was born at term after having received a diagnosis of omphalacele on prenatal ultrasound scan. She did not have an omphalacele but rather had a patent urachus with bladder eversion onto the abdominal wall. The remainder of the genitourinary tract was normal, and it was possible to pass a soft catheter through the urethra and out of the evaginated bladder. The prolapse was repaired without complications, and the infant recovered uneventfully. A voiding cystourethrogram and renal ultrasound scan were normal at 2 month follow-up. The embryology and malformations of the urachus are discussed, and the rare case reports of similar anomalies are reviewed.
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7/19. Can epithelized neobladder-cutaneous fistula provide urinary continence?

    The paper presents a case of a boy born with a bladder exstrophy, who underwent several surgical procedures, including bladder augmentation. During the last procedure, the patient received a suprapubic catheter that accidentally slipped out. The epithelized neobladder-cutaneous fistula resulted in a continent and easily catheterizable abdominal stoma.
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8/19. Spontaneous bladder rupture after colocystoplasty. Case report.

    An 18-year-old girl had bladder extrophy managed by sigmoid cystoplasty with clean intermittent catheterization. Spontaneous bladder rupture occurred 12 months after reconstructive surgery. The diagnosis was made by ultrasound with abdominal tapping and cystography under fluoroscopy. Management included intravenous antibiotics, laparotomy and closure of the perforation. The diagnosis was delayed and postoperative intraperitoneal abscess formation occurred.
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9/19. Urethral duplication with single bladder and multiple genitourinary abnormalities: an example of continent urinary diversion.

    A 13-year-old girl with multiple genitourinary malformations, incomplete bladder exstrophy, urethral duplication with single bladder, septate vagina, and total urinary incontinence is presented. Prior to admission she had undergone surgery for teratoma and calculi of the bladder and partial fecal incontinence. A continent urinary diversion was done by bladder augmentation using a cecal-colonic segment and by surgical closure of the bladder neck. The distal end of the appendix was brought to the skin as an inconspicuous, easily catheterizable, watertight stoma, our modification of the Mitrofanoff procedure. Excision of the vaginal septum, creation of an introitus, and unification of the split clitoris improved the cosmetic appearance of the external genitalia and improved the outlook for a normal sexual life and pregnancy.
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10/19. Perforation of the augmented bladder in patients undergoing clean intermittent catheterization.

    During urological reconstructive surgery small or large bowel is used to increase bladder capacity or to create a urinary reservoir. In most patients clean intermittent catheterization is necessary for urinary drainage. We report on 4 patients with a sigmoid cystoplasty who perform clean intermittent catheterization and who have experienced a serious long-term complication, urinary reservoir perforation, which was fatal in 1. Two were adolescent girls with myelodysplasia and 2 were prepubertal boys with bladder exstrophy. One patient experienced 2 separate reservoir perforations. rupture occurred 15 to 48 months (mean 30.2 months) after reconstruction. diagnosis was made by a static cystogram, which demonstrated extravasation in 2 of 4 patients, and ultrasound. In 2 patients the cystogram was normal. Management included intravenous antibiotics and open abscess drainage in all patients. In patients who have undergone augmentation cystoplasty or continent diversion and in whom abdominal pain and distension develop reservoir perforation should be considered in the differential diagnosis.
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