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Pseudoexstrophy associated with congenital pouch colon.

Pseudoexstrophy or covered exstrophy is a rare exstrophy variant. The authors report a case of covered exstrophy that presented as a newborn with widely separated pubic bones and rectus muscles, a low-set umbilicus, and a subcutaneous bladder. The anal opening was absent, and there was a complex malformation of the external genitalia consisting of a small, laterally displaced penis and a right-sided ectopic hemiscrotum. Micturition and urinary continence were normal. The child also had a high anorectal malformation with a coexistent type IV congenital pouch colon (CPC) malformation. Both kidneys were normal. Preliminary surgery consisted of a divided sigmoid colostomy proximal to the colonic pouch. The literature is reviewed and the embryogenesis of pseudoexstrophy and its associated malformations are discussed.

Abnormalities, Multiple↗

Appendicovesicostomy: a useful adjunct to continent reconstruction of the bladder.

Appendicovesicostomy is a useful adjunct to continent reconstruction of the bladder. During the last 3 years we have used this procedure in 14 children with difficult incontinence due to spina bifida (7), exstrophy/epispadias (5), imperforate anus (1) and urethral agenesis (1). In 4 patients in whom previous operations had failed to achieve continence the bladder neck was closed. In the remaining 10 children, all of whom had difficulty or discomfort catheterizing the native urethra, the bladder neck/urethra was preserved or reconstructed to serve as a vent for elevated bladder pressures. Continence was achieved in all 14 children with no complications related to the appendix. All children in whom the bladder neck/urethra was salvaged preferentially empty via the appendix and remain dry, except when the bladder is overly full.

Adolescent↗

Undiverting the ileal conduit.

From 1975 to 1981, 46 patients were evaluated for urinary undiversion. Of these patients 27 completed urinary reconstruction. The patients have been followed for 6 to 80 months, with a mean followup of 32 months and a median followup of 23 months. There has been no death and no rediversion has been necessary, although in 1 patient renal function has deteriorated significantly following urinary reconstruction. Postoperative bladder function was normal in patients diverted for dysfunctional voiding or bladder outlet obstruction but children with meningomyelocele required intermittent catheterization postoperatively. With careful patient selection, meticulous surgical technique and compulsive followup urinary undiversion is a safe procedure.

Adolescent↗

Enterocystoplasty in childhood: a second look at the effect on growth.

OBJECTIVE: To re-evaluate the assumption that enterocystoplasty in children has a detrimental effect on linear growth (which is almost exclusively based upon a chance finding in a retrospective study 10 years ago) in a larger cohort and with a longer follow-up. PATIENTS AND METHODS: The original 12 children who had impaired linear growth in a previous study 10 years earlier were re-measured. A larger cohort was identified from the 242 children and adolescents who had undergone enterocystoplasty between 1982 and 1997. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years) enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. RESULTS: Of the original 12 patients, six had regained or surpassed their preoperative position on their growth charts. In all patients with a known target centile range the final height was within their genetic growth potential. In the cohort of 123 patients, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration, with 83% and 80% of patients growing within two standard deviations of the 50th percentile. After surgery, 85% either remained on the same or reached a higher centile. Nineteen (15.5%) were in a lower position, with a similar tendency in the weight centile. A clinically relevant growth disorder was recognized in four patients with a complete endocrinological evaluation; in none of these was enterocystoplasty thought to be a causal factor. CONCLUSIONS: It is very unlikely that the loss of the preoperative percentile position on the growth curve in 15% of children after enterocystoplasty is a consequence of the surgery. Rather it is a non-specific phenomenon that has to be considered in any clinical population of the same size and age distribution after the same length of time.

Adolescent↗

Future trends in bladder reconstructive surgery.

The incorporation of bowel into the urinary tract is associated with significant long-term complications. Therefore, considerable efforts are being made to avoid the use of enteric epithelium in bladder reconstruction. The simplest of these entail the use of native urothelium that is already available, with techniques such as auto-augmentation, auto-augmentation de-epithelialized enterocystoplasty, and ureterocystoplasty. Unfortunately, in many patients, the bladder is too small, or dilated ureters are not available, and these techniques cannot be applied. Recently, experimental techniques are examining the use of tissue expansion to the ureter and bladder to increase the volume of tissue available. Tissue engineering techniques are being applied to bladder regeneration, and considerable advances have already been made leading to in vivo animal experimentation, the results of which are very encouraging. The details of these most recent advances will be discussed in detail in this report.

Animals↗

The ureter as a pedicle for construction of a ureteral urethra: the double tunnel.

PURPOSE: Clean intermittent self-catheterization has been shown to be an effective method for the prevention of upper tract urinary disease in patients who are unable to void efficiently. When the urethra is not available for catheterization surgeons can construct a catheterizable channel from the appendix or retubularized bowel. However, for patients without an appendix and limited bowel segments we have reimplanted the ureter to prevent reflux and tunneled the distal portion of the same ureter to construct a catheterizable stoma, the "double tunnel." MATERIALS AND METHODS: We retrospectively reviewed our 10-year experience with the double tunnel ureteral pedicle. A total of 10 patients (8 girls and 2 boys) were included in this study. The left ureter was used in 7 cases and the right ureter was used in 3. Mean followup for these patients was 4.1 years, with a range of 0.5 to 8.5 years. Outcome measures included intraoperative complications, reoperation rates, postoperative complications and upper urinary tract deterioration. RESULTS: There were no intraoperative complications. Furthermore, there were no cases of stomal stenosis or strictures that required reoperation or excision of the ureteral pedicle in the followup period. In our cohort the renal function associated with the ipsilateral double tunnel ureteral pedicle remained stable or improved. All patients reported that their catheterizable stoma is still functional. CONCLUSIONS: The double tunnel ureteral pedicle provides another option in the urological armamentarium when there are minimal resources with which to construct a catheterizable stoma.

Bladder Exstrophy↗

Isolated vesicorectal internal urinary diversion: a 37-year review of the Boyce-Vest procedure.

The challenges that management of exstrophy of the bladder pose the pediatric urological surgeon have resulted in a multitude of ingenious operations and a voluminous literature on exstrophy. Despite this intense interest in an uncommon anomaly, no consistently satisfactory approach to achieve urinary continence in children with exstrophy has evolved. We summarize a 37-year clinical experience with a form of urinary diversion that provides sphincteric urinary continence, and unexcelled long-term preservation of renal function and upper urinary anatomy. From our review it appears that the infant with exstrophy is served best by neonatal closure of the exstrophic bladder with attempts to establish urinary continence later in childhood. When other methods to establish urinary continence are unsuccessful and satisfactory anal continence has been demonstrated, a diverting colostomy combined with anastomosis of the isolated bladder to the isolated rectal stump offers an acceptable long-term alternate form of urinary diversion to produce sphincteric urinary continence with relative freedom from upper urinary deterioration.

Adolescent↗

Modified Young-Dees-Leadbetter bladder neck reconstruction: new concepts about old ideas.

INTRODUCTION: The modern Young-Dees-Leadbetter bladder neck reconstruction is a procedure that has evolved during the past 82 years. During this time, the modifications and contributions of several urologic surgeons have improved the procedure and ultimate patient outcomes. We review the evolution and describe in detail our version of the Young-Dees-Leadbetter bladder neck reconstruction procedure. TECHNICAL CONSIDERATIONS: Optimally, patients should have a minimal bladder capacity of 85 mL and be mature enough to participate in a postoperative voiding program. Adequate radical exposure of the lateral aspects of the bladder and bladder neck are important. Cephalotrigonal or cross-trigonal ureteral reimplantation is typically performed to mobilize the ureters in relation to the proposed bladder neck and correct reflux. A mucosal template 15 mm wide by 30 mm long is created that will serve as the reconstituted posterior urethra and bladder neck. Triangular lateral bladder mucosal wedges are marked and demucosalized. The neourethra is closed over an 8F Firlit tube. The demucosalized flaps are brought over the urethra sequentially in a "vest-over-pants" fashion with the final layer consisting of suspension sutures. Finally, ureteral stents and a suprapubic tube are used, but no urethral catheter is left in place. CONCLUSIONS: The modern Young-Dees-Leadbetter bladder neck reconstruction is the result of the contributions of various urologic surgeons during 82 years. When patients are appropriately selected, it is an effective method of lower urinary tract reconstruction in cases of congenital urinary incontinence.

Bladder Exstrophy↗

Further experience of free muscle transplantation in children with urinary incontinence.

Sixteen patients, 10 boys and 6 girls with severe urinary incontinence due to congenital malformations, were treated by free autogenous muscle transplantation. The mean follow-up period was 2.3 years. In 10 patients the results were good, implying increase in functional bladder capacity and abandoning of napkins during the day. Two patients were improved and 4 were failures. The earliest signs of improvement appeared 2 to 6 months after surgery. Most patients showed further improvement for at least 2 years post-operatively. Cine studies of micturition clearly showed activity of the transplant, most patients with good results being able to interrupt completely the urinary stream at will. Seven patients exhibited normal urinary flow post-operatively. In 5 patients the maximal urinary flow was around the lower normal limit, in two because of detrusor-sphincter dyssynergia. In boys, erection was unimpaired post-operatively. The prognosis seems to be better in girls than in boys, especially when comparing cases of epispadias. Patients with no sign of improvement during the first 6 months post-operatively had a poor prognosis. A relatively large bladder capacity before operation is advantageous, but not a prerequisite for continence, as bladder capacity can develop after transplantation. The ideal age for transplantation is 5 to 6 years.

Adolescent↗