1/46. 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. ( info) |
| 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. ( info) |
3/46. 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. ( info) |
4/46. Congenital prepubic sinus: an epispadiac variant of urethral duplication: case report and review of literature. Urethral duplication is rare; the duplicated urethra is almost always dorsal to the normal urethra, which contains the sphincteric mechanism. It is very rare to present as a prepubic sinus. Urethral duplication does not represent a uniform entity making it difficult to find an unequivocul and comprehensive classification. A case of epispadiac variant of urethral duplication is reported in which the duplicated urethra presents as a prepubic sinus, with complete cure and normal urinary and sexual functions after maturity. ( info) |
| 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. ( info) |
| 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. ( info) |
| 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. ( info) |
| Exstrophic bladder and intramedullary teratomas are rare congenital anomalies. To the authors' knowledge, the coexistence of these 2 anomalies has not been reported previously. The authors report on a newborn with thoracal intramedullary teratoma and exstrophic bladder. The possible embryogenetic background also is discussed. ( info) |
9/46. Complete duplication of bladder and urethra in coronal plane with no other anomalies: case report with review of the literature. Complete duplication of the bladder and urethra is a very rare congenital anomaly that is usually associated with other congenital anomalies. We report the case of a 7-year-old boy with complete duplication of the bladder and urethra in the anteroposterior plane, without any other associated congenital anomalies. ( info) |
10/46. 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. ( info) |