Cases reported "Epispadias"

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1/35. osteotomy and distraction of the anterior segment of the pelvic ring in epispadias repair: case report.

    We report a new therapeutic approach for bladder exstrophy and epispadias in one case of failed epispadias repair. The width of the pelvis was measured by what we defined as the anteroposterior diameter (APD) on combined transverse computed tomography (CT) scan cuts of the pelvis. The APD was half the normal value in an incontinent patient with failed epispadias repair. He underwent a supraacetabular osteotomy of the pelvis with progressive anterior distraction of the anterior segment of the pelvic ring. Four months later, hardware was removed, and the APD was near normal value. Within 9 months of follow-up, the patient was dry day and night. We believe that in patients with failed exstrophy and epispadias repair, APD seems to be a predictive criterion for continence, and results of the reconstructive surgery with osteotomy should be improved by distraction of the anterior segment of the pelvic ring.
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keywords = epispadias
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2/35. Secondary epispadias repair with rectus abdominis musculocutaneous flap.

    Genital defects in patients with epispadias cause functional, aesthetic, and psychological problems. Successful reconstruction of epispadias should improve the body image and enable the patient to perform sexually. Although various operative techniques have been defined for reconstruction of epispadias--predominantly using local tissue flaps--classic reconstructive methods are sometimes not sufficient in severe cases. Secondary reconstruction is inevitable after failed reconstructive attempts with classic techniques. The authors used the rectus abdominis musculocutaneous flap for severe secondary epispadias repair. The urethra, dorsal chordee, osteo-escutcheon contour defect, and the tissue defect created after dissection of the dorsal surface of the penis were reconstructed successfully using the rectus abdominis musculocutaneous flap. The neourethra was constructed using the skin island of the flap, and the muscle segment was used to reconstruct the dorsal defects. A straightened and lengthened penis and a new urethra without stricture were created during one operative procedure using this method.
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keywords = epispadias
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3/35. 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 = 0.25
keywords = epispadias
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4/35. Seven new cases of familial isolated bladder exstrophy and epispadias complex (BEEC) and review of the literature.

    Clustering of the bladder exstrophy and epispadias complex (BEEC) has been described in 29 families. To explore the genetic contribution we located new families through the German and Austrian bladder exstrophy Support Group and the association for the bladder exstrophy Community (predominantly US and canada). We report six new families with two occurrences of BEEC, one family where the proband is the product of a consanguineous union, and four discordant twin pairs. In conjunction with the published clinical and epidemiological reports this collection suggests that there is a significant genetic predisposition for susceptibility to the BEEC. It also highlights the importance of self-referral groups for recruiting families for multicenter collaborative research efforts to identify susceptibility loci.
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ranking = 0.625
keywords = epispadias
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5/35. An adult female epispadias without exstrophy was presented with urinary incontinence: a case report.

    A 39-year-old woman with three children presented with primary severe urinary incontinence. epispadias without exstrophy was determined in physical examination. The single-stage procedure including vulvoplasty and modified Young-Dees-Leadbetter bladder neck repair was performed to obtain sufficient cosmetic outcome and continence. Excellent functional and cosmetic results were obtained.
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ranking = 0.5
keywords = epispadias
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6/35. female epispadias: are we missing the diagnosis?

    OBJECTIVE: To describe three of four female patients with occult epispadias, as usually epispadias is reported to be a rare condition in females (1/480,000), and obvious and easy to diagnose, but less severe variants may still involve urethral sphincter incompetence in the absence of obvious clinical signs. patients AND methods: The hospital records over a 10-year period (1 January 1991 to 31 December 2000) were reviewed to find all female patients diagnosed with epispadias. Data were collected from the Consultative Council on Obstetric and Paediatric mortality and morbidity for the number of live births per year for the decade studied, and the incidence of female epispadias determined. RESULTS: Over the 10-year period there were 13 patients with a diagnosis of epispadias, from 636,698 total live births; four were female and nine male. Each of the female patients had varying degrees of epispadias; three had subtle signs with apparently normal external genitalia, and only one had a bifid clitoris. However, even the most minor variant case had a palpable notch in the symphysis pubis, and an 'oblong' external urethral meatus. Thus the incidence of female epispadias is at least 1/160,000. CONCLUSION: epispadias in females may be up to three times more common than previously expected, but even experienced clinicians can miss the diagnosis. The importance of a careful examination should be emphasized, especially in female patients with chronic wetting of unknown cause. A bifid clitoris is an important sign but not always present. Key diagnostic features include drug-resistant wetting, a very low leak pressure on cystometrography, a palpable gap in the pubic symphysis and an abnormal oval-shaped external urethral meatus.
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ranking = 1.375
keywords = epispadias
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7/35. Pseudoexstrophy with epispadias.

    The exstrophy variants are uncommon anomalies. The variants of the exstrophy complex have all the stigmata of the classical exstrophy such as divergent recti, widened symphysis pubis, and low-set umbilicus; however, the urinary tract is intact to a varying degree. Pseudoexstrophy, an exstrophy variant, is very rarely associated with epispadias. We report an unusual case of pseudoexstrophy with epispadias, in whom the intact bladder was initially covered by a mucous membrane which later epithelialized. The epispadias was repaired using a penile disassembly technique with posterior and ventral placement of bladder neck and urethra. Although the urethral meatus was hypospadiac, the child had achieved dry intervals with occasional stress incontinence.
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ranking = 0.875
keywords = epispadias
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8/35. Urinary stress incontinence treatment with Remeex in a patient with epispadias: a case report.

    epispadias is a rare condition in women. A postmenopausal 53-year-old woman with a known problem of epispadias came to our outpatient clinic complaining of persistent severe urinary stress incontinence without overactive bladder symptoms. She had previously undergone an inefficacious tension-free vaginal tape (TVT) procedure in another institution. We implanted a Remeex (Mechanic External Regulation) device without applying any tension to the sling. Two tape regulations were performed the day after surgery and 2 months later to gain continence. At 13 months of follow up, this woman was asymptomatic and satisfied. As documented by this case here, the Remeex procedure is particularly suitable in selected high-risk patients where TVT and TVT live slings are less effective.
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ranking = 0.625
keywords = epispadias
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9/35. Treatment of adult female epispadias without exstrophy in the presence of rhabdosphincter function.

    female epispadias without exstrophy is a very rare entity. Symptoms of female epispadias are primary urinary incontinence and anatomical abnormal features. A 24-year-old married women with two children presented with primary urinary incontinence. Due to previous failure of correct diagnosis and ineffective medical treatment, the patient developed psychological problems (anxiety and depression). In the presence of minimal rhabdosphincter function we could achieve socially acceptable urinary continence by bladder neck plication and a Burch colposuspension. The patient did not consent to a major reconstruction.
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ranking = 0.75
keywords = epispadias
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10/35. bladder exstrophy and epispadias complex in sibling: case report and review of literature.

    The bladder exstrophy and classic epispadias represent a spectrum of congenital anomalies with different degrees of anterior midline defect. Although the anomaly usually occurs sporadically there are some cases indicative of a strong genetic component. We present the clinical data of two siblings with bladder exstrophy and epispadias complex (BEEC), who were the product of consanguineous union. All previous reports of familial BEEC in the literature have been reviewed.
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ranking = 0.75
keywords = epispadias
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