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There are few case reports of bladder agenesis. The outcome of the patients differs and depends on the associated malformations. Different urinary diversions are described to save renal function. Most of the surviving patients with bladder agenesis have undergone incontinent urinary diversions as primary treatment. We describe the case of a female infant with agenesis of the bladder that arrived in our institution after several septic episodes. Treatment of choice was a continent pouch with an appendicovesicostomy. We state that even in young children, a primary continent urinary diversion can be performed with excellent long-term outcome., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objective: To develop a tissue-engineered bladder wall replacement with autologous cells and a biodegradable scaffold, as whenever there is a lack of native urological tissue the bladder is reconstructed with different bowel segments, which has inevitable complications., Material and Methods: Skin biopsies were taken from six minipigs, and primary fibroblast and keratinocyte cell cultures established. A partial resection of the urinary bladder was reconstructed by a cell-seeded scaffold covered with completely differentiated epithelium and supported by a mucosa-free pedicled ileum graft. Each pig was assessed urodynamically and by cystography before operation and every month until explantation; the pigs were killed at 1, 2 and 3 months after augmentation. Control groups (of six pigs each) with bladder augmentation with complete or denuded ileum were used. The bladders were assessed histologically and by distensibility measurements, Results: The differentiated keratinocyte epithelium was still present on the reconstructed bladder wall after 3 months. The overall shrinkage rate was 6.5%. The engineered bladder wall had lower distensibility than the native one. The inflammatory reaction present initially had disappeared after 3 months., Conclusions: The implanted, tissue-engineered substitution of the bladder wall is not only a bridging graft, but also a complete reconstruction. With this model, extended bladder wall substitution seems feasible and should be investigated in further studies.

Partial outlet obstruction of the rabbit urinary bladder causes increased tissue hypertrophy and decreased contractility of that organ; we showed that, in an experimental rabbit model, both correlate closely with alterations in the status and expression of mitochondrial (mt), and mt-related nuclear, genetic parameters in bladder smooth muscle. Here we investigate the rate and overall level of recovery of mt and nuclear genetic function following reversal of outlet obstruction in the same animal model. Release from outlet obstruction at 28 days resulted in improvement in both level of hypertrophy and contractile function in all bladders studied. However, bladders fell into two groups based on whether relative copy mt genome number per cell was above or below that of unobstructed controls. Bladders with high mt DNA content adjusted organellar genome copy number toward normal post-reversal but did not properly adjust mt transcript levels; mt-related nuclear transcripts in these samples showed recovery. Bladders with low mt DNA content showed no adjustment of those levels toward normal post-reversal but did show some adjustment in other mt and nuclear genetic parameters. Thus, a limiting factor for return of normal bladder function following reversal of outlet obstruction may be recovery of normal mt genetic performance.

We evaluated compliance in the developing bladder using a newly developed animal model of posterior urethral valves: partial infravesical obstruction in the fetal rabbit bladder. Partial bladder outlet obstruction was created in fetal rabbits at day 23 of a 31 to 32-day gestation period. An in vitro whole bladder preparation provided data on compliance and an isolated bladder strip preparation provided data on the mechanical properties of the bladder wall. In addition, the influence of calcium on both preparations was evaluated. Partial bladder outlet obstruction in the fetal rabbit resulted in a markedly larger bladder weight (246.4 +/- 22.3 mg, n = 14) than control bladders (90.2 +/- 5.7 mg, n = 13). Isolated smooth muscle strips from obstructed and normal bladders revealed identical stretch-stress patterns. In contrast, obstructed bladders had significantly increased compliance in the whole bladder preparation. Since the increase in compliance was not correlated to mechanical properties of the isolated bladder strips, it must therefore result from the pattern of mass increase of the whole bladder wall. During filling, both the control and obstructed bladders had the same slow, large amplitude spontaneous contractions. In addition, both had rapid contractions: those in the obstructed bladders had significantly lower frequency and higher amplitude than the ones in the control bladders. Removing the calcium from the organ bath eliminated the spontaneous contractions but did not change the baseline pressure or force values, indicating that the compliance of these fetal rabbit bladders is a function of the passive properties of the bladder wall. Three main patterns occur in cystometrograms of patients with posterior urethral valves: myogenic failure, hyperreflexic bladders, and low compliance bladders. Using our model of partial outlet obstruction in the fetal rabbit bladder, we could not imitate the group with low compliance. We therefore hypothesize that the different patterns of bladder dysfunction associated with posterior urethral valves are due to infravesical obstruction occurring with different severities or at different ages of gestation.

Purpose: Tadenan is a pharmaceutical agent used in the treatment of BPH. Prior studies demonstrated that pretreatment of rabbits with Tadenan significantly reduced the contractile dysfunction following two weeks of partial outlet obstruction. The specific aim of the present study was to determine the effect of Tadenan pretreatment on the time course of the response to partial outlet obstruction and correlate the effect of Tadenan on the contractile responses to field stimulation, bethanechol, and KCl with both mitochondrial enzyme activity (citrate synthase) and sarcoplasmic reticular function (calcium-ATP'ase activity)., Materials and Methods: Sixty male New Zealands white rabbit (3 to 5 kg.) were separated into 12 groups of 5 rabbits each. Each rabbit in groups 1-6 received Tadenan orally at 100 mg./kg./day for three weeks; each rabbit in groups 7-12 received vehicle (peanut oil). Each rabbit in groups 2-6 and 8-12 received a partial outlet obstruction as described below. One group of Tadenan treated and one group of vehicle-treated rabbits were euthanized at 1, 3, 5, 7, and 14 days following partial outlet obstruction. The non-obstructed groups were studied after 4 weeks of drug or vehicle treatment. Each bladder was rapidly removed and weighed, and 3 longitudinal strips prepared and mounted in individual baths for contractile studies. The remainder of the bladder was frozen for biochemical analysis. The contractile responses to field stimulation, bethanechol, and KCl were determined; and the enzyme activities of citrate synthase (marker for mitochondrial function) and calcium-ATP'ase (marker for sarcoplasmic reticulum) were determined., Results: 1) Tadenan did not reduce the effect of partial outlet obstruction on bladder mass. 2) Although the contractile responses to forms of stimulation were reduced at 1 day following partial outlet obstruction, Tadenan pretreatment resulted in a significant protective effect on the contractile responses to field stimulation, bethanechol, and KCl at 3, 5, 7, and 14 days of obstruction. 3) The activities of both citrate synthase and calcium ATP'ase were reduced significantly at 1 day following obstruction for both Tadenan treated and vehicle treated groups. The activities of both enzymes returned to near normal levels at 7 and 14 days for the Tadenan groups whereas the activities of both enzymes remained significantly reduced in the vehicle treated groups., Conclusions: These results clearly demonstrate that Tadenan pretreatment protected the bladder from both the contractile and metabolic dysfunctions induced by partial outlet obstruction.

Purpose: The loss of a functioning bladder is nowadays mostly managed by replacement with gastrointestinal segments. Management of these urinary diversions is difficult and the long-term effects are unknown. We developed a silicone rubber prosthesis for alloplastic replacement of the urinary bladder and tested it in an animal experiment., Material and Methods: Two artificial bladders were implanted into subcutaneous pockets on each side of the anterior abdominal wall. Cystectomy was performed and each prosthesis was connected to one kidney. Distally, a Y-shaped tube sutured to the urethral stump joined the two bladder outlets. The whole system was successfully implanted in 5 sheep with a mean follow-up of 11.5 months (5-19 months)., Results: Adequate capacity, effective and reliable mechanical function and patent anastomoses to the renal pelvis and the urethra could be achieved. Blood chemistry and renal ultrasound were performed at regular intervals demonstrating neither morphological nor functional deterioration of the renal parenchyma. Histopathological examination in the end of the experiment confirmed the excellent clinical results., Conclusions: The positive outcome of these animal experiments suggests this system would be useful for human bladder substitution. Standardized industrial production of the prostheses will be needed prior to implantation in humans.

Purpose: Tadenan (DEBAT, Paris, France) is a pharmaceutical agent used in the treatment of benign prostatic hyperplasia (BPH). The specific aim of this study was to determine if pretreatment of rabbits with Tadenan reduced either the hypertrophic response of the bladder to partial outlet obstruction or the accompanying contractile dysfunction., Materials and Methods: Twenty-five male New Zealand rabbits (3 to 5 kg.) were separated into 5 groups of 5 rabbits each. Each rabbit in groups 1,2, and 3 received Tadenan orally at 1, 10 and 100 mg./kg./day for 3 weeks. Group 4 received vehicle only (peanut oil); Group 5 were controls. The bladders were evaluated (in vitro studies) after 2 weeks of obstruction., Results: 1) Tadenan did not reduce the effect of partial outlet obstruction on bladder mass. 2) Tadenan pretreatment resulted in a significant protective effect on the contractile responses to field stimulation, bethanechol and KCl., Conclusions: These results clearly demonstrate that Tadenan pretreatment protected the bladder from the contractile dysfunctions induced by partial outlet obstruction.

Excitation-contraction coupling is achieved by translocation of calcium from the extracellular space as well as by the release of calcium from intracellular stores. Thapsigargin has been shown to selectively block the sarcoplasmic Ca-ATPase, thereby preventing the reuptake of calcium into intracellular stores and the participation of these calcium storage sites in the contractile response to stimulation. The current study determined the effect of thapsigargin on the contractile response to field stimulation, bethanechol, and KCl in control rabbit bladders and bladders obtained from rabbits subjected to partial outlet obstruction. Partial bladder outlet obstruction resulted in a marked increase in bladder mass and in significant decreases in the contractile response to field stimulation, bethanechol, and KCl. Thapsigargin (5-40 microM) had no effect on the contractile responses of bladder strips isolated from control rabbits to field stimulation, bethanechol, or KCl. However, bladder strips isolated from obstructed rabbits showed a significant concentration-dependent decrease in the contractile response to field stimulation in the presence of thapsigargin. Thapsigargin had no effect on the contractile responses of bladder strips isolated from obstructed rabbits to either bethanechol or KCl. In general, the data described in this study support our current hypothesis: as smooth muscle cells enlarge (hypertrophy) and the cell volume increases, there is an increased dependence on the release of intracellular calcium from the sarcoplasmic reticulum to mediate the contractile response to field stimulation.