1/24. 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.- - - - - - - - - - ranking = 1keywords = epispadias (Clic here for more details about this article) |
2/24. Oculo-ectodermal syndrome: report of two further cases.We describe the clinical findings in two previously unreported, unrelated cases with aplasia cutis congenita and epibulbar dermoids, similar to the cases reported by Toriello et al. [1993]. In addition, one patient had bladder exstrophy with epispadias. These cases provide further evidence for the identification of the oculo-ectodermal syndrome as a distinct entity.- - - - - - - - - - ranking = 0.125keywords = epispadias (Clic here for more details about this article) |
3/24. 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.- - - - - - - - - - ranking = 0.25keywords = epispadias (Clic here for more details about this article) |
4/24. 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.- - - - - - - - - - ranking = 0.375keywords = epispadias (Clic here for more details about this article) |
5/24. 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.- - - - - - - - - - ranking = 0.625keywords = epispadias (Clic here for more details about this article) |
6/24. Cloacal exstrophy in an infant with 9q34.1-qter deletion resulting from a de novo unbalanced translocation between chromosome 9q and Yq.Cloacal exstrophy is a rare malformation, belonging to a spectrum of birth defects, which, in order of severity, includes phallic separation with epispadias, pubic diastasis, bladder exstrophy, and cloacal exstrophy. This malformation overlaps the OEIS complex (O = omphalocele, E = bladder exstrophy, I = imperforate anus, S = spinal defects). The etiology of cloacal exstrophy is unknown to date. It may result from either a single defect of early blastogenesis or a defect of mesodermal migration during the primitive streak period. We report an infant with cloacal exstrophy, exomphalos, right kidney agenesis, ambiguous external genitalia, and axial hypotonia. The karyotype showed a de novo unbalanced translocation between the long arm of chromosome 9 and the long arm of chromosome Y resulting in a 9q34.1-qter deletion. Reviewing the literature, we did not find any observation of cloacal exstrophy associated with a structural chromosomal abnormality. The steroidogenic factor 1 (SF1) gene, included in the deleted region, was a good candidate gene but no pathogenic mutation was found by direct sequencing. We hypothesize that another gene, expressed early in embryogenesis and responsible for cloacal exstrophy, is present in the 9q34.1-qter region.- - - - - - - - - - ranking = 0.125keywords = epispadias (Clic here for more details about this article) |
7/24. Left-sided gastroschisis and pseudoexstrophy: a rare combination of anomalies.Pseudoexstrophy is a rare variant of the exstrophy/epispadias complex, comprised of the musculoskeletal defects of classic exstrophy but with an intact bladder and urethra. We present a case of a neonate with left-sided gastroschisis and pseudoexstrophy, the first reported combination of these two anomalies. The anomalies in this case were unusual but highlight the importance of careful clinical evaluation before and during surgery so as to carefully define the anatomy of the abnormalities, which will assist in planning surgery and further postoperative management. Identifying and reporting these unusual cases is essential to further expand our understanding of these conditions and their potential associations.- - - - - - - - - - ranking = 0.125keywords = epispadias (Clic here for more details about this article) |
8/24. 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.- - - - - - - - - - ranking = 0.875keywords = epispadias (Clic here for more details about this article) |
9/24. 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.- - - - - - - - - - ranking = 0.75keywords = epispadias (Clic here for more details about this article) |
10/24. 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.- - - - - - - - - - ranking = 0.75keywords = epispadias (Clic here for more details about this article) |
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