| PURPOSE: bladder exstrophy has recently become one of the several congenital malformations detectable prenatally by fetal ultrasonography, which implies the necessity of a correct prognostic evaluation of quality of life on which therapeutic termination of pregnancy may depend within the temporal limits stated by the law. While maintaining a preeminently informative role, prenatal counseling has gained increasing relevance for the quality of life long-term prognosis in adults affected by bladder exstrophy, particularly in regard to urinary continence, and sexual and reproductive problems in male and female individuals. We report a case of bladder exstrophy diagnosed at 22 week of gestation, and comprehensively review the literature on prenatal diagnostic tools and procedures as well as long-term followup in such cases. MATERIALS AND methods: At week 22 of gestation a woman underwent prognostic counseling due to the diagnosis of fetal bladder exstrophy made by prenatal ultrasound. After counseling the parents elected pregnancy termination. In addition, literature data were analyzed in regard to quality of life and bladder exstrophy. RESULTS: We believe that the rarity of the prenatal diagnosis of this abnormality has resulted in the lack of a comprehensive review of necessary counseling criteria. CONCLUSIONS: We suggest what we consider to be a valid counseling approach for parents after a prenatal diagnosis of bladder exstrophy. ( info) |
2/181. OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins. The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a consistent and recognizable pattern of midline abdominal and pelvic defects. It is rare, affecting 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestations. This is an autopsy study of OEIS complex in monozygotic twins after pregnancy termination at 20 weeks of gestation. Unremarkable family history but concordance of monozygotic twins for the defects may support the theory that early malformation complexes, e.g., OEIS, and monozygotic twinning are manifestations of the same disturbance of early blastogenesis. ( info) |
3/181. Duplicate exstrophy of the bladder. Duplicate exstrophy of the bladder is a rare, distinct congenital anomaly without additional major anomalies of the urinary tract. A 15-year-old boy with duplicate exstrophy is reported on, and the possible role of bladder sequestration in the development of classical musculoskeletal deformities in this anomaly is discussed. ( info) |
4/181. 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. ( info) |
5/181. 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) |
6/181. Covered exstrophy with incomplete duplication of the bladder. A rare exstrophy variant in a 1(1/2)-year-old girl is reported. The child had a wide, scar-like lesion over the lower central abdomen with divarication of the recti and a spread symphysis pubis. The underlying bladder was divided into two unequal chambers by a complete sagittal septum with only the right chamber communicating with the single urethra and the left draining by a vesicocutaneous fistula. Both kidneys were normal. The clitoris was bifid and the anus anteriorly placed. Preliminary surgical management consisted of dissection of the bladder from its prevesical covering, excision of the sagittal septum, and cystocystostomy. Postoperatively, the child had normal urinary continence. The literature is reviewed with special reference to the various forms of duplicate exstrophy. ( info) |
| PURPOSE: We report our experience with 5 patients with bladder exstrophy who underwent creation of a Mainz II pouch. MATERIALS AND methods: We retrospectively reviewed the results of the Mainz II pouch as a primary urinary diversion in 2 and a secondary urinary diversion in 3 patients. Each patient underwent multiple surgeries, including osteotomy in 1. All patients were followed postoperatively on a yearly basis. RESULTS: All patients are continent and the upper urinary tract is stable. CONCLUSIONS: The Mainz II pouch is appropriate for children born with a small fibrotic bladder, and as a salvage procedure for those who have endured multiple reconstructive procedures and remain incontinent. Furthermore, this procedure deserves serious consideration in children residing in developing countries. ( info) |
8/181. 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. ( info) |
| 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. ( info) |
| methods: Inflatable penile prosthesis were implanted into sensate radial free-flap phalloplasties in two adult patients with bladder exstrophy. RESULTS: Neither patient reported loss of sensation, and both were able to inflate the prosthesis and engage in sexual intercourse three months later. CONCLUSION: The construction of a functional neophallus is well described however has not previously been reported in exstrophy patients who are suited to this procedure due to the presence of crura, and the absence of a neourethra. Scarring from previous reconstructive procedures however may make implantation difficult, and long-term follow up is required to evaluate this procedure in patients with bladder exstrophy. ( info) |