Cases reported "Bladder Exstrophy"

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1/27. 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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ranking = 1
keywords = closure
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2/27. Inferior vesical fistula.

    Exstrophy variants are uncommon developmental anomalies of the bladder; the variants involving only the bladder neck are extremely rare. There are only two case reports of inferior vesical fissure to date, and inferior vesical fistula (IVF) as an exstrophy variant has not been described previously. We report a case of IVF in a 20-month-old female. The urethra was normal in calibre and position and the bladder capacity was adequate. Simple closure of the fistula was followed by a normal micturition pattern.
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keywords = closure
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3/27. bladder exstrophy in a neonate at risk of transient myasthenia gravis: a role for remifentanil and epidural analgesia.

    Infants born to mothers with myasthenia gravis may exhibit a transient form of the disease, with similar sensitivity to non-depolarizing neuromuscular blocking drugs. We report the case of an infant at risk who required major surgery when 48 h old for closure of bladder exstrophy. A combined epidural-general anaesthetic technique, with remifentanil supplementation, enabled us to avoid unnecessary neuromuscular blocking drugs and prolonged intensive care, which had been anticipated. The potential benefits of remifentanil and epidural analgesia in neonates are discussed.
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keywords = closure
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4/27. 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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ranking = 4
keywords = closure
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5/27. bladder exstrophy associated with complete urethral duplication: a rare malformation with excellent prognosis.

    PURPOSE: We report the association of complete urethral duplication and bladder exstrophy in 5 males, and describe the main characteristics to achieve diagnosis. MATERIALS AND methods: From 1983 to 2000, 5 males with single bladder exstrophy and complete urethral duplication were seen at 3 institutions. Although bladder exstrophy was obvious at birth, only 1 patient had the preoperative diagnosis of associated urethral duplication. All patients were evaluated with abdominal ultrasound, voiding cystourethrogram after bladder closure and cystoscopy. RESULTS: All patients were totally incontinent, including 4 after primary bladder exstrophy closure. Although it was initially unnoticed by the urologist, most patients had leakage from the tip of the penis during the valsalva maneuver. In all cases bladder exstrophy was single and deeply situated in the pelvis with excellent elasticity. All patients had a normal sized penis with less severe dorsal chordee and conical shaped glans. The duplicate epispadiac urethra did not have a verumontanum. Only 1 of the 5 patients had the correct diagnosis made before primary closure of bladder exstrophy, while in the other 4 diagnosis of a duplicate ventral urethra was made at the time of epispadias or fistula repair. There were no other associated malformations. In all cases the dorsal epispadiac urethral plate was excised and bladder emptying occurred through the ventral urethra, which at cystoscopy had a normal verumontanum. Postoperatively, all patients became continent and voided with good flow through the ventral urethra and, as opposed to many cases of exstrophy and epispadias, had normal size and excellent cosmesis of the penis. CONCLUSIONS: Complete urethral duplication is a rare variant of the exstrophy-epispadias complex. patients with this anomaly present with a larger and more deeply situated bladder plate than classical exstrophy and a larger penis. A high index of suspicion is necessary to make a preoperative diagnosis. Excision of the dorsal urethral plate and maintenance of the ventral urethra are the treatment of choice, resulting in a continence and normal voiding.
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ranking = 3
keywords = closure
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6/27. Development of a large bladder calculus on sutures used for pubic bone closure following extrophy repair.

    bladder exstrophy is a rare congenital condition that occurs in 1 to 30,000 live births. Primary bladder closure is usually performed in the first days of life in conjunction with an iliac osteotomy in order to achieve a more secure bladder closure. We report a case of a large bladder stone with secondary right-sided hydronephrosis in a 3-year-old child who underwent exstrophy repair at the age of 7 months. During the exstrophy repair a no. 1 braided, polyester, non-absorbable suture was used to close the pubic bones and served as a nidus for intravesical stone formation. This case substantiates the lithogenic nature of non-absorbable sutures in contact with urine as well as the need for close post-operative follow-up in these patients.
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ranking = 6
keywords = closure
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7/27. Use of cadaveric fascia lata for closure of bladder exstrophy with diplomyelia.

    We describe a technique for the repair of bladder exstrophy using cadaveric fascia lata. The graft is folded in a four-layer configuration, passed through the obturator foramina, and then wrapped and anchored in front of the pubis. The resultant ring-shaped graft is useful when complex deformities preclude proper apposition of the pelvis anteriorly.
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ranking = 4
keywords = closure
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8/27. A combined vertical and horizontal pelvic osteotomy approach for repair of bladder exstrophy: the Dana experience.

    BACKGROUND: bladder exstrophy is a severe congenital defect that requires a multidisciplinary treatment approach. Soft tissue repair may be successful during the first few days after birth, but a combination of pelvic osteotomies and bladder reconstruction is necessary later in life. The combination of externally fixed anterior and posterior osteotomies has biomechanical advantages over previous techniques for achieving primary bladder closure. OBJECTIVES: To describe our experience with a combined vertical and horizontal pelvic osteotomy approach for the repair of bladder exstrophy. methods: Four children underwent bladder exstrophy closure; the mean age at surgery was 19 months (range 9-33 months)... We stabilized the osteotomies with a small Synthes AO external fixator, 4.0 mm rod diameter. RESULTS: All four patients had successful bladder repair with no dehiscence; two of them achieved partial continence, and bladder neck reconstruction is planned for the other two. Three of the four patients sustained neurologic injury; two completely recovered, and the third continues to suffer from right drop foot. The average follow-up was 39 months (range 10-60 months). CONCLUSIONS: Vertical and horizontal pelvic osteotomies stabilized by external fixator and bladder repair is an effective treatment for bladder exstrophy.
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ranking = 2
keywords = closure
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9/27. Mucocolpos associated with bladder exstrophy: a case report.

    INTRODUCTION: We present the first reported case demonstrating an association of mucocolpos and bladder exstrophy. MATERIALS: A term baby girl presented with bladder exstrophy and underwent a successful primary bladder closure and ureteral reimplantation. Subsequently, she presented with a fluid-filled pelvic mass which was found to be a mucocolpos. RESULTS: Surgical drainage of the collection was required. CONCLUSION: Mucocolpos should be considered in the differential diagnosis of a pelvic mass in a patient with bladder exstrophy.
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ranking = 1
keywords = closure
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10/27. Case report: azotemia secondary to bilateral ureteral kinking from ureteral stents placed at the time of bladder exstrophy closure.

    We report the case of a neonate who underwent bladder exstrophy repair on the second day of life. Bilateral ureteral stents were placed intra-operatively. Postoperatively azotemia developed secondary to kinking of the proximal ureter bilaterally. Issues in diagnosis and management are discussed.
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ranking = 4
keywords = closure
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